THE
AMERICAN JOURNAL OF THE MEDICAL SCIENCES.
NOVEMBER, 1892.
THE SURGICAL TREATMENT OF EPILEPSY.' By A. G. GersTErR, M.D.,
PROFESSOR OF SURGERY IN THE NEW YORK POLYCLINIC, AND B. Sacus, M.D., PROFESSOR OF MENTAL AND NERVOUS DISEASES IN THE NEW YORK POLYCLINIC.
In the following report we record our joint experiences in the surgical treatment of epilepsy. We have attempted, in an unbiased fashion, to study the effects of various surgical procedures upon the course of epilepsy, and in recording all cases that we have operated upon, no matter what the result, we are more apt to give a correct view of how much or how little surgery can accomplish in this special field than those can give who describe their short-lived successes and do not report the many dismal failures.
The cases operated upon were selected with considerable care. They were either cases of distinctly traumatic origin or in which a strictly localized convulsion pointed to a limited focus of disease. Cases of general epilepsy of non-traumatic origin were not subjected to operation.
The following table’ gives a succinct account of the chief points of interest in ten cases; seven of these may be designated as cases of traumatic epilepsy, one was due-to ear disease, one was a case of infantile cerebral hemiplegia with epilepsy, and one was a case of non-traumatic localized epilepsy due to early acute brain disease—a case of infantile cerebral palsy without palsy, but with epilepsy.
1 Read at the American Neurological Association, June, 1892.
2 We are indebted to Dr. E. Sternberger, house surgeon at Mt. Sinai Hospital, for his assistance in keeping and furnishing the records of most of these cases. VoL. 104, No. 5.—NOVEMBER, 1892.
504
GERSTER, SACHS,
SURGERY IN EPILEPSY.
Ei &¢ } II. | W.C.H. | | III. | M. K | IV. | E.L.M | v.| J.D. vi. | T.C.
Sex
age.
M. 6
M.
Se
om
M 26
No. | Name, |and |
| ing spells became worse. |
Date of | Occurrences History of case. opera- Coe | after tion. - * | operation. |Said to have had brain Dec. 29, Exposure by | Recovery ex- | fever at age of 10 mos.;| 1890 chiselling . of cellent; no at age of 5% years first motor area of | convulsions | right-side convulsion, right arm, de-| up to Feb. 2,
| repeated at interval of
one week ; right hemi-
paresis since first attack;
athetoid and associated | movements.
|At age of 12 years was | Feb. 13,
| pushed back over pole of & wagon; supposed to have struck back of | head (?); unconscious | for a few minutes, but | worked as usual; one | week later general epi- | leptic convulsions ; has petit mal, and above all | Jacksonian epilepsy in- | volving muscles around | right half of mouth. | Occasionally eyes are involved. No loss of consciousness with ma- jority of attacks. |
| 1891
fell out of window; 1891 since that time epileptic attacks at varying inter- |
vals; has had tremen-
dous doses of bromides ; ill-tempered and stupid; stopped bromides; no | attacks for three weeks, |
then left-sided convul- |
sions becoming general. |
| Traumatic injury to right | July 24,
| side of head; general, 1892 | epileptic attacks.
Traumatism at7 months; 1st oper. 6 months previous to | Aug.14, operation began to de- 1891 velop auditory and ol-
factory aure and then
general epileptic spells; | 2d oper, if ear ceased discharg- —
At age of 11% years was Nov.20,
severely kicked by a| 1891 man over the right side of occiput; 6 months
later epileptic attacks
| which have continued | nocturnally about every
6 weeks since. No hemi- anopsia. Marked de-
| pression in skull; was | | for 8 weeks without any |
attack ; attacks then re- | turned.
}
|At age of 18 months | Feb. 23,
termined
by faradization.
| Dura tense and | charged from | | adherent; punc-|
ture; no cyst found.
Exposed centre for representa- tion of angle of mouth accord- ing to Horsley; adhesions un- der the button of bone ; small
on puncture a little bloody fluid. Faradi-
caused contrac- tion only of r. angle of mouth. Large opening ;
placed,
Large trephine opening over motor area for arm and leg, right side of skull,
Trephining over occipital de- pression ; ad- hesions over the depression.
Opening of mas- toid and remov- ing two seques- tra of bone.
|Mastoid opened again and silver canula to secure permanent drainage,
Trephining and chiselling over scar ; dous exostosis almost doubling underlying part of brain.
cysts on dura; |
zation over dura
button not re-
tremen- |
| 1891, when | boy was dis-
hospital.
| Attacks re- turned after operation and have not been diminished, and eyes are
| more fre-
quently ‘n- volved.
|
Good recovery, but no cessa- tion ofattacks
Did very well except that he developed de- lusions of per- secution.
Facial palsy of left side. Sept.10th first attack; re- peated attacks
Nov. 18th, con- vulsion of
| rightside, but
| none since ;
| last report Au-
gust 15, 1892.
Nov 22, short spells ; none while in hos- pital; dis- charged Dec, 15; has gone out West ; has attacks every six weeks, but milder; re- ports that his memory is better.
General result.
Immediate diminution | of attacks ; after leaving hospital had one mild at- tack ; not heard from since.
No improve- ment.
No improve- ment,
Attacks re- turned with- | in 2 weeks ; alcoholic excesses,
Great im- provement after second operation ; no spells up to date.
Some im- provement in severity of attacks eight months after opera- tion.
GERSTER, SACHS, SURGERY IN EPILEPSY. 505
| | Sex Date of Occurrences | No. | Name. |and History of case. opera- | Gamat of | after | Cae | lage. tion, | - * | operation. | :
VIL. H. L. | M. Six years ago felldown; Jan. 29, |Motorarm centre | 24 |
thinks he struck on 1892 | on left side ex-
right side of occiput ; | posed; part of
one year later first at- dura but nocor-! | tack, right hand and | tical tissue re- | | leg convulsed. At first | moved,
had attacks six times
daily, lately three or 2d oper.|;Removed arm | Repeated at- No improve-
| four times in two weeks. | Mar. 8, | centre as deter-| tacks after | ment worth
| } 1892 | mined by elec-| both opera- | mentioning.
| | | | trical tests,with tions. | slight resulting! paresis. H
VIII.| C. D. | F. | Fell out of bed at six | Feb. 15,) Large trephine | Attacks not | Slight im- | months, striking head 1892 | opening over quite so fre-| provement. | | against bare floor; at | left side of head quent as be- | ten months a tedious (motor area), fore. | | illness, slow in develop- | | | ment; at age of 5 years | | | | began to have innumer- | | able convulsions (at | | | least 50 per day). } | Idiocy. Parents insist- | | ing on operation. | .| K.A. | M. | At age of 5 years had a April 12,| Excision of hand Did very well | Some imme- | 9 | ‘congestive chill’’ and 1892 | centre in right in hospitalfor| diate im- | spasms; no paralysis at | hemisphere ; six weeks, but} provement,
©
| the time; two or three | removed con- | as soon as he | but no last-
| years later developed | siderable tissue; left hospital | ing benefit
| Jacksonian epilepsy be- | hand was pare- and ran about) from opera-
| ginning in left hand | tic for a few! got severe at-| tion ; in without loss of con- | days. tack with loss! later attacks
| sciousness ; no evidence { of conscious- | right arm
| of palsy ; boy feels left ness and in-| wasa
| hand drawn up in voluntary pas-| involved, cramp. sage of urine.
X.| E.S. | M. |Two years ago was run May 27, Trephining and | Report not 12 | over; fell backward, 1892 | chiselling over yet made. | striking curbstone ; un- | occipital region. conscious for ten days, | and had _ convulsions during this time; one year later convulsions became regular and | frequent. Scar in left occipital region near | median line.
Remarks, BY Dr. Sacus.
I wish, first, to have a word to say regarding the determination of the brain areas to be operated upon. Working upon the rules laid down ‘ by Reed, Thane, and others, we have been accustomed to map out upon the skull with the greatest care, in advance of the operation, the exact site of the various divisions of the motor areas. Excellent as this prac- tice is, I am now firmly of the opinion that in most cases it is quite un- necessary, and that the application of the faradic current to the dura will help us to localize centres much more carefully than any of the customary rules will.
At the last meeting of this Association doubt was expressed whether
| a
} | j | j | |
506 GERSTER, SACHS, SURGERY IN EPILEPSY.
faradization of the dura was apt to be successful.' My experiences during the past year have helped to strengthen the opinion expressed at that time, and I feel so certain of the absolute reliability of this pro- cedure, that I would be willing to have the skull opened at any reason- able point over the motor areas and by means of the faradic tests determine the exact location of the centres. I have demonstrated this again and again to the satisfaction of Dr. Gerster and a large number of medical men present at these operations. The method is furthermore so accurate that I believe we shall be able to determine the .exact subdivisions of the motor area in the brain of man as they were determined by the physiologists upon the brains of the monkey and other animals. The success of this method was particularly marked in Case II., a case of Jacksonian epilepsy with convulsive movements of the muscles about the right angle of the mouth. In Horsley’s article I found an area which he claims to contain the representation of the upper face and angle of the mouth. I located this area as closely as possible upon the skull, and after trephining, on applying the faradic current to the dura, caused contraction of those muscles and of those muscles only which were, as a rule,involved in the epileptic convulsion. I need not add that the same tests applied to the cortex will give equally satisfactory results; but the chief advantage of determining these areas before opening the dura is that we may be sure that we are right before exposing any large part of the brain cortex.
While I am very certain that this method will be found entirely sat- isfactory in all cases of ordinary brain lesions, I have a suspicion that in cases of tumor,’ or in cases in which the brain tissue has been seriously altered by disease, the morbid brain tissue may not respond as promptly to the current. In such cases the older method of determin- ing the areas will have to be resorted to.
The first and most notable result of the operations we have recorded is that we cannot claim a single decided cure. In several of the cases there has been a marked diminution of the attacks immediately after the operation ; in some the improvement lasted for a few months; but in every single case the attacks recurred after a lapse of several months or even less. The case which promises best is Case V., in which the epilepsy—characterized, by the way, by interesting auditory and olfac- tory auree—was due to ear disease, and in this case the improvement that has set in has now continued nine months; but a second operation was necessary to bring about this result. The quiet of hospital life after
1 At the meeting of this year the same doubts were still entertained by some Phila- delphia colleagues. We can do no more than ask them to try and see for themselves.
2 Since writing the above, the “electrical method” proved to be thoroughly satisfac- tory in a case of cystic tumor of the motor area.
GERSTER, SACHS, SURGERY IN EPILEPSY. 507
an operation, and the unusual care which patients receive during this time, undoubtedly contribute to the cessation of attacks, and may in some way account for the successes so frequently reported, as was illus- trated in Case IX. Not a single attack occurred for six weeks after operation, but as soon as the boy was dismissed from the hospital and allowed to roam about at his own free will, the attacks returned and possibly even with greater force than before.
In the first six cases reported, the operation consisted simply in open- ing the skull, possibly the dura also, without excision of cortical tissue. In Cases VII. and IX. cortical tissue was removed; but as far as our ex- perience goes, excision of cortical tissue or excision of a diseased area is not superior to the older method of simple trephining. In these very cases of traumatic epilepsy it is not always an easy matter to excise the area which is supposed to be the starting-point of the epileptic disease. If the motor area happens to be the part injured, this can of course be attempted ; but if, as in two of our cases, the traumatic injury caused a depression over the occipital areas, excision of the cortex, with its prob- ably resulting disturbances of vision, would not be advisable or even fair to the patient.
If we seek for some special reason why operative procedure has ac- complished so little in these cases, it is to be found in the fact that they came under our notice after the epilepsy had been established for many years. - It is now generally conceded, as I pointed out in a former paper,’ that, though a focus of disease is the actual cause of the epilepsy, this epilepsy does not manifest itself, as a rule, until widespread changes have appeared throughout the entire brain.
The time that elapses between the infliction of the initial lesion and the development of these secondary changes corresponds quite accurately with the period of time between the traumatic injury or the initial disease and the development of epilepsy.
If we operate upon cases which have run a course of many years, removal of the initial focus of disease will have little effect upon the general sclerosis that has been established. It is this general sclerosis that keeps up, as it were, the epileptic habit. The inference to be drawn from this is, that we should remove the focus of disease before secondary changes have been set up. This is equivalent to asking prompt surgical interference in all cases of traumatic injury to the skull in which there is any reason to suppose that serious harm has been done to the brain, and also in those cases in which the occurrence of a local- ized hemorrhage of non-traumatic origin is more than likely to give rise to epilepsy later on. The operations themselves, if skilfully done, are
1 Sachs: “ What Can We Expect from the Surgical Treatment of Epilepsy?” N. Y. Med. Journ., February, 1892.
508 GERSTER, SACHS, SURGERY IN EPILEPSY.
borne so well by persons above the age of five years that a more ener- getic surgical treatment in the earlier stages of these troubles will be productive of great good in the way of preventing the development of epilepsy. I have not yet got beyond the point of believing that the only way to cure epilepsy is to prevent its development.
The old method of trephining for traumatic epilepsy has been so frequently recorded as productive of good that we must endeavor to find some explanation for the effects of this procedure. It will not do to classify it simply, as Dr. White does, as one of those cases in which the operation per se helps; I am inclined rather to believe that the fre- quent occurrence of cysts in traumatic cases, and also in cases of old cortical hemorrhages, accounts for the improvement following upon the release of pressure over a cystic area. The excision of cortical tissue which has been considered the only rational method in the treatment of epilepsy since Horsley first recommended it, seems to me, after all, to be of questionable merit. If the disease involved the motor area, exci- sion of the part is apt to be followed by paralysis. This most patients are willing to bear, provided they can be promised a freedom from future attacks. In all but the most acute cases such promise can scarcely be given. Moreover, if the injury happened to involve other than motor areas, particularly if it involved the occipital or even the frontal portions of the brain, excision of any considerable cortical tissue would be in the nature of a rather dangerous experiment, inasmuch as the resulting loss of function could not well be foretold. Taking this in connection with the fact that the excision of tissue does not seem rational after the development of a general sclerosis, or gliosis as Chaslin would have it, it is evident that this “ rational method ”’ is applicable to only very few cases.
It is our intention to continue this series of operations in the hope of being able to determine those cases in which the operations promise good results. For the present I am bound to acknowledge that the prospects are rather gloomy, and that the successful cases will probably be those in which there is some very tangible organic lesion which has been removed at a very early period, or those cases in which, after trau- matic injury to the skull, trephining has been done before the effect of depression of the skull upon the brain has resulted in the development of epilepsy. Considering the seriousness of epileptic disease and the slight danger attending the opening of the skull, I believe it to be the surgeon’s duty, in every case in which there is the shadow of a doubt about the effect of a traumatic injury to the skull or brain, to trephine the skull and thus remove the cause of an epilepsy that would be apt to be developed.
DILLER, TUMOR OF THE PONS.
A CASE OF TUMOR OF THE PONS IN WHICH TAPPING OF THE LATERAL VENTRICLES WAS DONE FOR THE RELIEF OF INTRA-CRANIAL PRESSURE.
By THEODORE DILLER, M.D., FELLOW OF THE PITTSBURG ACADEMY OF MEDICINE; PHYSICIAN TO THE NEUROLOGICAL DEPARTMENT OF THE PITTSBURG FREE DISPENSARY.?
THE following case was referred to me by my friend, Dr. R. W. Stewart, for my opinion as to the propriety of a surgical operation. The patient was in the Mercy Hospital :
A. T., male, thirty-three years old; born in Germany ; two years in the United States; married seven years. He was a butcher by occupa- tion. Enjoyed excellent health and worked regularly. No history of syphilis or intemperance. In October, 1890, while working temporarily in a rolling mill, he fell from a gondola car upon the “ back of his head.” The fall must have been one of considerable distance. The man, how- ever, was not rendered unconscious by it, but, after a short rest, con- tinued his work the same day; nor did he lay off work for some time after this accident, but often complained of “giddiness” and muscular lassitude. He worked up to February, 1891, when, on account of his feeling of insecurity and of these subjective sensations, he gave up his occupation. At this time he was frequently troubled with occipital and occasionally by frontal headaches. His wife first noticed an unsteadiness in his gait, which was vascillating and uncertain. About the same time he began to lose some muscular power in his left arm and leg. He often lapsed into a state of semi-stupor from which he was aroused with some difficulty and into which he soon relapsed when let alone. His vision became slightly impaired. For several weeks the headaches were severe and persistent. June 14, 1891, he was admitted to the Mercy Hospital. While in the hospital he had some difficulty in micturition, and was several times catheterized. He remained in the hospital about three weeks.
In May he experienced a sudden and peculiar loss of sensation on the affected side, for which he consulted a physician in this city.
In July, 1891, he experienced for the first time some difficulty in talking and swallowing; had occasional attacks of choking spells. All his other symptoms, especially headache and difficulty in walking, became more pronounced. He would not venture out alone, but was always accompanied by his wife. His eyesight had failed so much that he was no longer able to read. Shortly after this his headache began to be less severe and gradually disappeared altogether.
I first saw the man in March, 1892, at the Mercy Hospital, at which time the following notes were taken :
Status presens. A well-developed, muscular man, about six feet tall.
1 Paper read for the writer by Dr. Charles K. Mills, of Philadelphia, before the Sec- tion of Neurology of the American Medical Association, at Detroit, June 8, 1892.
2 The case was briefly referred to at a meeting of the Pittsburg Academy of Medicine, when the brain was exhibited.
510 DILLER, TUMOR OF THE PONS.
A careful search (the scalp was shaved) fails to reveal the presence of any scars or cicatrices; but just to the left of the median line, over the leg-centre, a small bony elevation about the size of a pea is noted. This exostosis does not fade away gradually into the surrounding bone, but is set sharply upon the bone, as though placed there from the outside. The face is distinctly drawn to the right side, there being considerable paresis of the left side. This paresis is still better seen when the patient attempts to laugh or show his teeth. There is marked paresis of both the left arm and leg, but not absolute paralysis, as the man is able to raise the leg readily and to move the arm in all directions. The mus- cular power in the right arm and leg and face apparently unimpaired. The power in grasp of left hand is about one-fifth that of right hand. The — measurements were taken at corresponding points on the two sides :
Right side. Left side. Arm r - 28cm. Arm . ° - 28 cm. Forearm . > - = Forearm ; - 25.5 “ Calf of leg . a Calf of leg . _— = Thigh . ‘ . Thigh . . - 4 «
A careful test of muscular sense shows no impairment. Patient is able to discriminate sharply between heat and cold, and also quickly feels the pain of a pin-prick. There is some impairment of tactile sense over the entire left side; on left arm can distinguish two points no nearer than 30 cm.; on the left forearm, no nearer than 15 cm.; on left thigh can distinguish at 14 cm.; over instep, 3.5cem. Right arm distinguishes points 9 cm. distant; forearm, 9 cm.; back of hand, 2 cm.; palm, 2 cm.
Slight increase of patellar tendon reflex and ankle clonus on left side as compared with right.
A slight deafness of the left ear is noted. Can hear tick of watch no further away than five inches. Right ear normal.
Slight paresis of extra-ocular muscles. Cannot move eyes to left or upward or downward as far as in normal condition. Pupils dilated (both equally). Very little contraction to light. Patient is able to read only very large letters. Can recognize persons he knows.
His speech is very thick and muffled, but he comprehends questions, and attempts to make intelligent answers, but he cannot always be un- derstood. Swallows with great difficulty. Food is very apt to get into larynx and provokes a coughing spell. He frequently points to his head and asks for an operation. Takes food slowly; even then often has severe coughing spells. He is unable to control his bladder, but can control his bowels.
It is difficult to estimate precisely his mental condition. While there is no very gross defect—no pronounced aberration, there is doubtless a slowness of comprehension and some loss of memory. Patient lies in bed constantly. He is unable to walk even a very short distance alone. This is probably not so much owing to his defective vision and hemi- paresis as to his difficulty in codrdination.
Dr. W. F. Robeson made an ophthalmoscopic examination of the eyes and found rather advanced optic atrophy, rather more marked in the left than in the right eye.
The history was gotten from the man himself and from his wife. On all the main points their statements were in accord. Both were par-
DILLER, TUMOR OF THE PONS. 511
ticularly questioned as to whether the patient had ever experienced any convulsions or spasmodic twitchings. Each was sure there had been no such symptoms.
The main points in the case may be briefly summarized as follows:
A man who was previously strong and robust receives a severe injury to the head, and dates therefrom a train of grave symptoms, viz.: head- ache, of diffuse character generally, but at times localized, which for a period was very severe; drowsiness or tendency to lethargy, even in spite of headache ; left hemiparesis; cerebellar titubation or ataxia of a certain sort; some failure of vision; difficulty in walking, which is most likely more owing to inability to coérdinate than to dimness of vision or left-sided muscular weakness; marked dysphagia and aphonia ; advanced optic neuritis.
These symptoms were enough to make the diagnosis of an intra-cranial lesion certain. The absence of convulsive seizures of any sort at any time; the early appearance of incodrdination ; the comparatively slow progress of the hemiparesis ; the tendency to sleep; but more especially the marked loss of control of power over the muscles concerned in swal- lowing and in phonation (the bulbar symptoms)—made it seem most likely that the lesion was somewhere in the base of the brain. It seemed to me that most likely it was the pons or medulla or else at such a locality in the cerebellum that the vermiform process of the lesser brain was involved or pressed upon, as well as the pons and medulla. The symptoms of the case seemed to make this conclusion inevitable. Yet there were two features in the case which threw some element of doubt upon this diagnosis; these were the presence of the small bony exos- tosis referred to and the stout declaration of the patient that it was in that locality the injury occurred to his head. True, there was no scar at this place, nor could local tenderness on percussion or elevation of temperature be elicited. If the view were adopted that the lesion was at that point indicated by the exostosis it would have been impossible to have accounted for the bulbar symptoms save upon the theory of general intra-cranial pressure ; and this seemed scarcely tenable because of the paresis being so distinctly one sided.
All the symptoms had progressively increased, and it seemed certain that the man would die before many weeks had passed unless some relief were afforded by surgical interference. The patient himself realized his critical condition, and was anxious to have an operation performed.
It seemed to me, in view of all the circumstances of the case, that it would be justifiable to tap the lateral ventricles to relieve the intra- cranial pressure. The plan of procedure determined upon was to tre- phine just to the right of the median line so as to escape the longitudinal sinus and bite enough bone away to the left as to include that part of the skull upon which was situated the bony excrescence. A search
512 DILLER, TUMOR OF THE PONS.
was then to be carefully made for depressed bone, tumor, abscess, soften- ing, blood-clot, or anything else which would be sufficient to account for the symptoms. If a morbid product were found in this situation it would be dealt with as the surgical exigencies of the case might require, and the project of tapping the ventricles would be abandoned. If the structures in this locality were found to be healthy, or if but slight disease were found, then the operation of aspirating the ventricles and inserting a drainage-tube in them would be performed.
Operation.‘ The operation was performed by Dr. R. W. Stewart. The patient’s head having been previously shaved and prepared anti- septically, he was brought into the operating-room. Ether was admin- istered by Dr. McCloud. Dr. Stewart was assisted by Drs. J. J. Buchanan, Pool, and the writer. Drs. J. B. Murdoch, M. R. Ward, Stoner, and others were present.
By previous measurements it was determined that the bony exostosis was situated over the superior parietal convolution. A horse-shoe flap was made. A large-sized trephine was used. The operation of trephining, as it neared completion, was proceeded with with great caution, so that the dura or the longitudinal sinus, if it happened to run a little further to the right than normal, might not be injured. The trephining was done in the place previously determined upon. When the button of bone was removed there was distinct bulging of the dura mater, which was very tense to the touch. It seemed to some of the gentlemen present that an incision through the dura would surely reveal the presence of a morbid product. The dura was then incised, after which procedure the — was seized with a coughing spell, when a rather formidable cere-
ral hernia ensued ; but a subsidence of the coughing was followed by a considerable subsidence of the protruding convolutions. The cortex under the pia presented a bluish, congested appearance. A careful exploration of the exposed convolutions by Dr. Stewart with his finger failed to reveal the presence of any mass of greater or less consistency than the normal brain-tissue. The opening was now enlarged to the left, by the use of the rongeur forceps, enough to include the small bony exostosis. Nothing of unusual character was found either in the con- formation of that part of the internal table of the skull or of the under- lying dura. The brain substance was now carefully probed in several directions by the operator, but no feeling of greater or lesser resistance was communicated to his hand through the probe. The opening was next enlarged slightly downward to the right, but nothing unusual was found. Dr. Stewart now inserted a small trocar and canula into the lateral ventricle. No difficulty was experienced by him in finding this cavity. Upon the withdrawal of the trocar a clear straw-colored stream of considerable force issued from the canula. It was estimated by Dr. Murdoch that at least two ounces escaped. The canula was then with- drawn, and a drainage tube was inserted into the ventricle through the canal made in the brain by the canula. The tube was conducted to the outside of the scalp through a hole made in the centre of the horse-shoe
1 In the absence of my colleague, Dr. Stewart, who is in Europe, it devolves upon me to describe the operation. I regret that this is necessary, as this part of the subject must lose something of interest by this substitution.
DILLER, TUMOR OF THE PONS. 513
flap. The wound was now closed, the button of bone not being replaced. But little bleeding occurred during the operation, which was conducted on the strictest antiseptic principles.
The patient came out from the influence of the ether slowly. He stood the operation badly. His temperature went up to 102° on the evening of the day of the operation. The next morning it was noted that the paresis of left side had increased. He was seized with three or four convulsions which, the nurse states, were general in character. - By noon of the second day the temperature had risen to 103.5°. Up to this time, about twenty-four hours after the operation, there had been a con- stant discharge of clear fluid through the drainage-tube. The dressing was saturated, and the nurse was compelled to change the pillow several times. It would be impossible to estimate the amount which escaped, but it must have been several ounces. As it seemed to Dr. Stewart and myself that the drainage-tube had accomplished all the good it ever would, and that it was a likely source of irritation and cause of the rise of temperature, it was decided to withdraw it at once. This was done without any difficulty. But the patient was on the downward turn. Dyspnoea became marked ; cedema of the lungs ensued, and death oc- curred about thirty-six hours after the operation.
Autopsy was performed about twenty-four hours after death, the head only being examined. No pus was noted about the wound. A slight union of the edges of the wound was found to have taken place. The flap was somewhat swollen and congested. There was nothing unusual of note either about the bones of the cranium or the brain membranes. The calvarium was carefully explored for an old fracture, but no trace of any could be found. The convolutions were full, regular, typical, and healthy in character. A considerable amount of fluid was found between the pia and dura. But some wrinkling of the dura was noted, evidently the result of the escape of the large amount of fluid from the drainage-tube; for while some of this fluid came from the ventricles, doubtless much of it—especially of that which leaked out while the patient was in bed—came from what was doubtless a considerable accu- mulation between the dura and pia. Upon removal of the brain the primary seat of the trouble at once became apparent. It was a tumor, about the size of a walnut, situated on the left side of the pons. The growth did not extend over the median line to the right, but did press upon the upper portion of the medulla. The pyramidal tract and oli- vary body were found pressed downward and somewhat attenuated in character by the encroachment of the tumor. To the touch the growth was hard. It presented a rather finely granulated appearance. It was very firmly incorporated with the pons and appeared to have grown from its centre. An incision into the substance of the tumor revealed the presence of a thick, creamy fluid. Nothing else was found of a morbid character.
A microscopical examination of the neoplasm, made by Dr. Pool, showed it to be a sarcoma.
The question might be asked: If the location and nature of the tumor could have been exactly determined during life, would the operation of trephining have been justifiable? If we may be guided by the high authority of Victor Horsley it was justifiable, for this brilliant operator
514 DILLER, TUMOR OF THE PONS.
has trephined several times simply for the relief of intra-cranial pressure. Philip Coombs Knapp’ has recently reported an instructive case where, in a case of tumor of the cerebellum, trephining was done for the relief of intra-cranial pressure. In Knapp’s case there were, as in my own, no Alistinctive focal symptoms. He concluded that the growth was either in the right temporal lobe or in one of the lateral lobes of the cerebellum. On account of the uncertainty of his diagnosis, Knapp, acting upon the suggestion of Weir and the experience of Horsley, trephined over the right temporal lobe, with a view of relieving intra-cranial pressure. The autopsy showed that the tumor was in the left lobe of the cerebellum. But it seemed to me that in my case trephining alone, with puncture, would not fully accomplish the purpose of relieving intta-cranial pressure, and, being guided by the experiences of Horsley, Duncan, and Knapp, I advised, conditionally, the more radical measure of tapping the ven- tricles. This operation has already been done by Duncan and is advised by Knapp.
The experience is perhaps yet too limited to warrant us in drawing up conclusions. But both Knapp’s case and my own were very desperate ones. In my case the operation doubtless shortened the man’s life; yet he could not have lived very long at best, and his remaining days would have been full of misery and distress to himself and his family.
Whether, if trephining alone, without tapping the ventricles, had been done, the patient would have stood the operation better is, of course, a question difficult to answer. I cannot persuade myself that this pro- cedure added much to the gravity of the operation, for the careful intro- duction of a small trocar into the ventricle could not have caused very great destruction of brain-matter; byt the introduction of the drainage- tube added somewhat to the gravity of the operation. Possibly a more favorable result would have occurred had the drainage-tube been omitted.
In this case I should not have strongly urged the operation upon the patient ; but in view of the fact that he himself, as well as his wife, was anxious for an operation, the course adopted seems to me to have been justifiable.
So far as I am able to learn, Duncan’s’ case and this one are the only cases on record in which tapping of the lateral ventricles was done in a case of intra-cranial growth. But Ayers, of this city, tapped the ventri- cles of a boy aged five, for acquired hydrocephalus, with favorable results.* Keen has, in five cases of hydrocephalus, tapped the lateral ventricles. In one of his cases a tumor of the cerebellum was suspected. All of Keen’s cases were children—the oldest being fourteen years—and
1 Journal of Nervous and Mental Diseases, Feb., 1892. 2 Philadelphia Hospital Reports, vol. i. % Pittsburg Medical Review, March, 1889.
OLIVER, LEUKAEMIA. 515
all died, the one which lived longest after the operation surviving but forty-five days. This operation has been done a number of times.
In estimating the gravity of the operation of tapping the ventricles for the relief of intracranial pressure it would not be fair to group this one case with those of Keen, for in this case the intra-cranial pressures was the result of a local disease; in Keen’s cases it was the result of a general brain or vascular disease. I cannot see that tapping the ven- tricles in cases of congenital hydrocephalus is a justifiable operation. Only with more experience will we be able to determine whether an operation such as I have here described is justifiable under like circum- stances.
A CASE OF LEUKZMIA WHICH TERMINATED FATALLY BY RETRO-PERITONEAL HEMORRHAGE.
By Tuomas OLIver, M.A., M.D., F.R.C.P., PHYSICIAN TO THE ROYAL INFIRMARY, NEWCASTLE-UPON-TYNE; PROFESSOR OF PHYSIOLOGY, UNIVERSITY OF DURHAM.
Deatu from hemorrhage is not an infrequent termination of leukemia. All through the course of the disease a tendency to bleeding is often met with. In the case about to be reported the end came, not less gradually than unexpectedly, from a large retro-peritoneal hemorrhage, and, from this peculiar fact alone, it deserves to be recorded.
E. W., aged nineteen years, single, was admitted into the Newcastle Infirmary, under my care, on January 21st, complaining of great de- bility and of swelling of the abdomen of six months’ duration. Her family history was good. Eight months previous to this she felt tired and wearied on the least exertion, and had complained of great thirst. She noticed, too, that she was losing flesh. Three months after this she took to bed, and has since then scarcely ever left it. About this time she was occasionally sick, and she noticed that a lump could be felt in the left side of the abdomen. It was hard, but not painful; it has not increased much, patient thinks, since then. When asked if she could explain the presence of this lump, she said she attributed it to an injury to her side. There has never been any vomiting of blood, but every three or four weeks she has had recurrences of epistaxis, which have lasted for two or three days at a time. She began to menstruate at the age of twelve, and has continued regular until twelve months ago, when the intervals lengthened, so that no menses have now been seen for at least seven months. She thinks little of this, however, as she regards the epistaxis as having replaced the menstrual flow.
Within the last few days a short troublesome cough has developed. She is emaciated. Tongue red, papille are prominent. There is no longer a feeling of thirst. Bowels are regular. Many of the glands in the right side of the neck are enlarged, but are not painful. Here and there upon her limbs can be felt subcutaneous nodules, the size of a
516 OLIVER, LEUKAMIA.
small bean; they are painless, are not discolored, and come and go in crops, each crop remaining out for two or three days. Pupils are slightly dilated. Since the commencement of the illness patient has become rather deaf. Chest: Intercostal spaces are wide and deeply grooved, mamme are ill-developed. Heart: Apex is felt beating about ‘one inch below and just internal to left nipple. The sounds of the heart are healthy, the second aortic being particularly well marked. Lungs: The breath sounds are coarse over each apex, but the expiratory murmur is not prolonged.
The abdomen is somewhat tense and resistant, and the superficial veins are much distended. There is a very apparent fulness over the hepatic area, the upper margin of which begins at the level of the fifth rib, and is carried down to the level of the umbilicus. Over the epigastric and splenic areas the resistance is great, and there is felt a hard dense mass, with a well-defined border projecting toward the umbilicus.
Fie. 1.
Case of E. W., showing increased areas of hepatic and splenic dulness.
The lower border is particularly well defined and sharp. The fingers can be pushed well under this, and it is noticed that on deep inspiration the dulness on the left side extends from the sixth rib to one and one- half inches above the crest of the ilium. The mass is not painful on pressure ; it is perfectly smooth on the surface. There is no albumin in the urine and no sugar. Its specific gravity is 1020, and it is acid. Temperature is normal. Pulse, which is very compressible, is always rapid—116 per minute. Blood: A prick of the finger by a needle is not quickly followed by the presence of blood, but when it does come it is seen to be pale and watery. Under the microscope the field seems to be occupied almost entirely by colorless cells, which vary in size, many being larger whilst others are smaller than ordinary leucocytes, ex- tremely granular and showing a large nucleus. Between these cells are noticed here and there dirty reddish-yellow masses, evidently composed of fused red blood-cells. No rouleaux are observed. Gowers’ hemo-
OLIVER, LEUKAEMIA. 517
cytometer showed that in 1 c.mm. of blood 1,610,000 cells were present ; of these the red cells formed 980,000, and the colorless corpuscles 630,000. Patient was put upon arsenic. Under this line of treatment she improved. All seemed to be going on well with her when she un- expectedly died.
he day before her death she complained of severe pain running:
down her right thigh, chiefly along the course of the sciatic nerve. The pain, of which at this stage no explanation could be found, was evidently very severe. It had been somewhat relieved by an appli- cation of aconite and menthol before I saw her, and the relief continued more or less during the whole of the day. Toward evening pain re- turned in the leg with increasing severity, and remained constant. Vomiting now occurred, and it was noticed that the swelling in the right half of the abdomen had gradually increased in size since the morning, chiefly in a downward direction, and that it was tense and very oe Death came quietly and without convulsions.
Autopsy. Abdomen: on reflecting the skin there was brought into view a very Jarge spleen; it extended well toward the middle line, and was seven or eight times the size of the normal organ. After removing it there was more completely brought into view a large cyst, with dark bluish-black walls, which lay in the right half of the abdomen, extend- ing from beneath the liver to nearly the brim of the pelvis. The intes- tines were displaced by it, portions of the bowel being driven deeply into the pelvis. The ileo-ceecum was pushed upward, and there was matting between it and the omentum. On clearing away the intestines this cyst was found to be the size of a man’s head, and its walls were formed by the retro-peritoneum. The cyst was completely filled by thin reddish semi-coagulated blood of a peculiarly flaky color. It was not connected with the liver, although it could be traced up under this organ to near the diaphragm. From this point it extended all the way down to the brim of the pelvis, but on approaching it the cyst became narrowed and not nearly so distinct. The right kidney was pushed upward, forward, and to the left, and was apparently in no way connected with it. The anterior wall was composed entirely of retro-peritoneum. At the right side the peritoneum all at once glanced off from its parietal attachment to encircle the tumor in front, and then passed down more or less behind it, on the left. It was as impossible to remove this blood-cyst as it was impossible to make out with which vessel or vessels it was connected. It is sufficient to say that it was not connected with liver, kidneys, or adrenals, nor with anything in the pelvis. The uterus, ovaries, and bladder were healthy and free from any adhesions. The kidneys were normal. Liver seemed healthy. Gall-bladder contained healthy-looking bile. The a. as already stated; was enlarged. On its anterior surface was a thick white patch, the size of the palm of the hand, and about one-eighth inch in depth. It was firm, and on section its tissue was coarse-looking, and showed pale trabecule. The heart was extremely firm and hard to the touch; it was a little larger than the normal. The left ventricle was hypertrophied, its wall being nearly one inch thick, whilst its cavity was not dilated. This cavity contained a large quantity of flaky blood. The other chambers of the heart contained large quantities of the same kind of blood, and were healthy. Lungs presented nothing remarkabie. Pericardium and pleurz were healthy.
VOL. 104, NO. 5.—NOVEMBER, 1892. 34
518 OLIVER, LEUKAEMIA.
This patient, whilst possessing a large spleen and suffering from leukemia, died from the effects of an internal hemorrhage; the blood forcing its way behind the retro-peritoneum on the right side, throw- ing it forward, and giving rise to a blood-cyst.
Going back upon this case of leukemia, it is difficult to account for it. There is mention of an injury to the patient’s side from a fall against a doorway, a circumstance which should not be forgotten, since De Chapelle has shown what an important part injury plays in the causation of this disease. Epistaxis, too, was an early and an important symptom in her case, and its monthly recurrence may have had some- thing to do with the abolition of the menstrual function, which up till that time had been normal.
It is difficult to explain the deafness. Is there any reason to suppose that an internal or labyrinthine hemorrhage had occurred—an accident to which has been ascribed the loss of hearing in leukemia? It is well known that deafness is frequently met with in leukeemia—so frequently, in fact, that it cannot be a coincidence. Vidal and Izambert found it present in 10 per cent.—a percentage which Gradenigo, of Padua, who has given considerable attention to this subject, regards as too high. In Gradenigo’s case—one of mixed leukeemia—hearing was affected in both ears, and there was tinnitus aurium, which was relieved by catheteriza- tion of the right Eustachian tube. Epistaxis, too, was not less an obstinate than a prominent symptom. Death occurred in six weeks— preceded by a recurrence of hemorrhagic diarrhea. At the post-mortem there was found a gelatinous yellowish-red substance in the drum- cavities, extending backward toward the mastoid cells. The fluid pressed from this mass showed under the microscope red and white blood-cells. There was found newly-formed connective tissue, with hemorrhagic infiltration. Gradenigo, therefore, believes that in the course of a leuk- semic process there sometimes occurs in the organ of hearing compli- cations which depend upon exudative processes in the middle and in- ternal ear, and this quite independently of anything like a hemorrhagic diathesis. This extract from the Internat. Journ. Med. Sciences, 1887, conveys the opinion of most writers upon this subject. In some cases the deafness has been found to have been due to labyrinthine disease. The tendency has been, however, in nearly all of them, for hemorrhage to occur into some of the deeper parts of the auditory apparatus. At the post-mortem of my case we were not allowed to examine the head, so I cannot express more than a theoretical opinion upon this point.
The condition of the blood is interesting. The total number of cor- puscles in 1 c.mm. was only 1,610,000—and of these 98,000 were red cells, and the remaining 63,000 were colorless. Pepper (Syst. Med,, vol. iii. p. 912) considers it rather the exception for the number of blood-cells to fall below 2,000,000 per c.mm. I have seen the number even lower than this. In one case, that of a woman near the middle term of life,
OLIVER, LEUKAMIA. 519
only 540,000 cells, red and white, were present in 1 c.mm. of blood. But to return; given the normal relationship of 1 white blood-cell to 400 red in 1 c.mm. of blood, we have in the present case the two kinds of cells almost equal—the relationship being one white to one and one-half red. It is, therefore, a veritable leucocytosis that we have, and about it I would incidentally make this remark, viz.: that if we, in these cases,
Fie. 2.
Fre. 3.
Spleen : subcapsular tissue. Showing dilated bloodvessels with thin walls, and filled mostly with colorless cells.
Spleen with thickened capsule, show- ing development of new vessels. -
were to form an opinion as to the condition ot the blood from an ex- amination of the patient’s face and lips alone, we would completely fail to realize to what an extent the blood had become morbidly altered. In other words, there may be not only a marked diminution in the number of cells as a whole in the blood, but the most pronounced leuco-
520 OLIVER, LEUKAMIA.
cytosis may be present, and yet almost no indication of this will be revealed in the face or lips of the patient. Occasionally the lips of the invalid may be well colored—not in any way even suggesting anzmia ; and when the blood is examined under the microscope there is found - not only a diminution of red cells, but almost an equal number of colored and colorless corpuscles.
Without going into the morbid anatomy of leukemia, I would simply allude to the great thickening of the capsule that is frequently met with in leukemia—drawings of which are subjoined.
Sims Woodhead, in his Practical Pathology, 1892 ed., p. 431, alludes to this irregular thickening of the capsule of the spleen, “under which there are at times purple patches, the result of hemorrhage.” Examina- tion of the drawings shows not only a thickened capsule, made up of an excessive development of fibrous tissue, but a rich network of blood- vessels, the walls of which are extremely thin, and therefore liable to rupture. Large blood-sinuses, too, are noticed in the splenic pulp im- mediately under the capsule. The one morbid feature which is charac- teristic of leukemia is the irregular accumulation of leucocytes in patches in and around the small bloodvessels in spleen, liver, and sub- serous spaces ; it is thus easy for embolic hemorrhages to occur. Numer- ous small hemorrhages may thus at first have taken place underneath the retro-peritoneum of E. W., and been the precursor of that large hemorrhage which caused death.
Disseminated capillary hemorrhages in leukemia were observed by Byrom Bramwell (Brit. Med. Journ., June 12, 1886) in brain-tissue, associated with hemorrhages of varying size—some as large as a hen’s egg, others only visible to the naked eye. In his case the bloodvessels and capillaries throughout the brain were enormously dilated and dis- tended with white corpuscles, multitudes of which had escaped into the lymphatic sheaths surrounding the larger vessels. The hemorrhages were composed for the most part of white corpuscles, and it is to the presence of these cells in excess that I attribute the peculiar flaky con- dition of the blood in the retro-peritoneal hemorrhage in my own case.
Of late the infective nature of leukemia has attracted the attention and received the support of Pawlowsky (Deutsche med. Wochenschr., July 7, 1892, and alluded to in Supplem. Brit. Med. Journ., Aug. 13, 1892), in whose case—one of considerable leucocytosis (1:4) and enlargement of the spleen—there were found in the blood short bacilli with spores. In the blood and organs of five other patients similar bacilli had been found, as well as in the blood of leeches which had abstracted blood from leukemic patients. The presence of these microdrganisms in the blood of six patients led Pawlowsky to regard them as peculiar to leuk- zemia, and standing in direct relation to it. Whether subsequent ex- aminations by other bacteriologists will confirm his observations remains to be seen—for, relying upon these facts, Pawlowsky has come to regard
DERCUM, ADIPOSIS DOLOROSA. 521
leukemia as a disease of the blood. The bacilli are considered to exercise a certain influence upon the leucocytes in the blood-forming organs. These cells multiply, many of them entering into the blood in an imperfectly formed condition. Carried all through the system by the blood, the microérganisms are retained in the spleen, lymphatic glands, and medulla of bone. In the spleen is supposed to occur the fight between leucocytes and microbes (phagocytosis). How far this is purely hypothetical remains to be seen before there can be general acceptance of Pawlowsky’s opinion that the hyperplasia of the spleen and other blood-forming organs is the result of the reaction of the indi- vidual against the poison circulating in the blood.
THREE CASES OF A HITHERTO UNCLASSIFIED AFFECTION RESEMBLING IN ITS GROSSER ASPECTS OBESITY, BUT ASSOCIATED WITH SPECIAL NERVOUS SYMPTOMS— ADIPOSIS DOLOROSA.'
By F. X. DEeRcum, M.D., CLINICAL PROFESSOR OF DISEASES OF THE NERVOUS SYSTEM IN THE JEFFERSON MEDICAL COLLEGE ; NEUROLOGIST TO THE PHILADELPHIA HOSPITAL.
Ar the meeting of the American Neurological Association held in Washington in September, 1888, the writer reported an anomalous case found in the nervous wards of the Philadelphia Hospital, and as it was not possible to classify the condition found, the description was prefaced by the title, “ A Subcutaneous Connective-tissue Dystrophy of the Arms and Back associated with Symptoms resembling Myxcedema.” Sub- sequently the case was published in the University Medical Magazine for December, 1888.
Some two years later, another and apparently similar case was dis- covered in the medical wards of the Philadelphia Hospital, and was reported at a meeting of the Philadelphia Neurological Society in December, 1890, by Dr. Frederick P. Henry. It was described as a case of myxcedematoid dystrophy and afterward published in the Journal of Nervous and Mentai Disease for March, 1891. Dr. Henry stated that he adopted the term dystrophy in order to bring the case “into the same category with the very similar one” reported by the writer.
In October, 1891, a third case, resembling the others, made its appear- ance in the nervous wards of Blockley. This will presently be detailed, but before doing so ‘let us review, as briefly as possible, the previous cases, in order that all three may be considered together.
1 Read before the meeting of the American Neurological Association, New York, June, 1892.
522 DERCUM, ADIPOSIS DOLOROSA.
Case I.—Before described by the writer, as stated, in 1888. History in abstract as follows :
M. G., aged fifty-one, female, widow, native of Ireland, domestic.
Family history. Father died at forty-five, of erysipelas. Mother had eighteen children; died at forty from some complication incident to the menopause. Of brothers and sisters, seven died in early childhood, one
Fie 1. (Case I.)
Fie. 2. (Case I.) -
in adult life, of pleurisy ; a sister, in childbirth; a brother and two sisters, of phthisis ; while the remaining five are living and apparently in aver- age health. None of the patient’s relatives had ever suffered, as far as she knew, from symptoms similar to her own. No history of insanity, epilepsy, or other neurosis.
Personal history. As a child, had measles, whooping cough, and scar- let fever. Menstruated normally at fifteen. Married at eighteen. Some years after, had an attack of pneumonia but made a good recovery.
DERCUM, ADIPOSIS DOLOROSA. 523
Had in all seven children and one miscarriage. Five children died in infancy or childhood, one from cholera infantum, two from measles, one from “congestion of the brain,” and the fifth from “spasms.” Meno- pause set in abruptly at thirty-five. From this time up to within two or three years her health had continued good. She had undergone some increase in weight, but beyond this nothing worthy of mention could be recalled. Syphilis and alcoholism denied.
When forty-eight or forty-nine years of age noticed that her arms were becoming very large. The upper arms and shoulders appeared swollen. On some days the swelling seemed more decided than on others. It continued steadily to increase, and for about a year was unattended by any other symptom.
In November, 1886, she was admitted to the surgical wards of the Philadelphia Hospital for the rupture of a varicose vein of the leg. In the following February she was transferred to the medical wards for a severe attack of bronchitis. Later she had an attack of severe pain and swelling in the right knee, attended by chill and fever. She was treated for rheumatism and promptly relieved. Two weeks after this she com- plained of a sharp darting pain in the right arm. It began on the outer aspect above the elbow, and gradually increased in severity and extent, spreading upward to the shoulder and neck and downward to the fore- arm and hand. It was different, she states, from the pain previously experienced in the knee. It was shooting and burning. She felt at times as though hot water were being poured upon the arm, and again as though the hand and fingers were being torn apart. No rise in temperature was noted. The pain was often paroxysmal, being very much worse for hours and days at a time, but even during the intervals it was never altogether absent. On June 4, 1887, she was removed to the nervous wards, when she came under the writer’s care.
Her appearance at this time was striking. She was a tall, large-framed woman who looked as though she had at one time presented a fine phys- ical development, but she seemed unnaturally broad across the back and shoulders. On removing the clothing an enormous enlargement of these parts was disclosed. The enlargement affected both helen, the arms, the back, and the sides of the chest. It was most marked in the upper arms and back, forming there huge and somewhat pendulous masses. It was elastic, and yet comparatively firm to the touch, and it was impossible to produce pitting. In some situations it felt as though finely lobulated, and in others, especially on the insides of the arms, as though the flesh were filled with bundles of worms. The sensation to the fingers was very much like that experienced in examining a varicocele, except that the structures appeared more resistant. The skin itself was evidently not thickened. It did not take any part in the swelling and it was not adherent to the subjacent tissues. It was slightly roughened over the forearms, less so on the arms, shoulders, and hands, while over the fin- gers it was quite smooth and even glistening. Further, over the fore- arms and hands it was slightly darkened, small brownish patches and minute epithelial scales being observed ; lastly, it was quite 5.
The right arm was extremely painful on motion. The head, at this time, was also held in a fixed position for fear that movement of the neck would give rise to pain in the shoulder. In addition, the arm was also very sensitive to pressure. Pronounced pressure appeared to be absolutely unbearable. The nerve trunks also were exquisitely sensi-
524 DERCUM, ADIPOSIS DOLOROSA.
tive, but this painful condition was not by any means limited to them, but permeated the swollen tissues as a whole.
In marked contrast to the right, the left arm could be handled with im- punity. Transient pain was, however, at one time noted in the left wrist.
The muscles were evidently not involved in the swelling. On grasp- ing, for instance, the enlargement over the left biceps and directing the patient to flex and extend the arm repeatedly, the mass was felt to be unaffected by the movements of the underlying muscle. The affected parts were, however, quite weak. The grip of the right hand was almost nil, while that of the left was greatly diminished. Examined electrically, the muscles of the shoulders and arms yielded a negative result, partly because of the great resistance caused by the intervening tissue. Slight quantitative and qualitative changes were noted in the muscles of the forearms, while in the hands distinct reaction of degeneration was noted in the thenar and hypothenar groups, more evident on the right side.
Cutaneous sensibility was much diminished. On the right arm various areas existed in which no response whatever was given to the esthesi- ometer. - They were large and irregular in shape and very sharply defined, and were present on both the inner and outer aspects. In the finger-tips of the same side the points could not be at all separated. In the left arm, on the other hand, the response was prompt and accurate, except on the outer aspect of the forearm, where some delay and uncer- tainty existed. In the finger-tips, also, sensation was decidedly below normal, the points being separated at not less than one-half or three- quarters of an inch. Sensibility to heat and cold was also diminished.
Examining the legs, it was found that cutaneous sensibility was dis- tinctly lessened on the right, while showing little or no impairment on the left. No enlargement was, however, noted at this time in any por- tion of the body, save in the regions already mentioned. No swelling or anesthesia was found about the face. The latter was pale, as were also the mucous membranes. There was, however, a little color in the cheeks, more noticeable at times. Her features were well formed and intelligent. Her hair was dark and fine, and somewhat thin over the vertex. Her mind was unimpaired, except that at times she was much abstracted. Sometimes she gave conflicting answers to questions, so that the latter had often to be repeated. Her speech was not slowed or otherwise altered. At times she was irritable and quarrelsome, and frequently gave much trouble to her nurses.
The above abstract fairly represents her condition at the time of her admission to the nervous wards.
June 13th, ten days later, she had a chill, followed by fever and a painful herpetic eruption over the upper portion of the left arm and the upper and anterior portion of the left side of the chest. June 19th, another crop of vesicles made its appearance on the back and on the front of the chest.
For some three months following, among other studies, a careful record of the axillary temperature was made. It proved to be very nearly normal. At one time, however, a temperature of only 97° was recorded.
Nothing worthy of note occurred until October 13th, when the patient had another severe attack of bronchitis, which was accompanied by much dyspnea.
In the latter part of December it was ‘noticed that during one of her poe of pain the swelling of the right arm became more decidedly obulated. The arm became more sensitive than ever, and on examina-
DERCUM, ADIPOSIS DOLOROSA. 525
tion hard, cake-like masses were felt, resembling, as the resident physi- cian expressed it, the caking of milk in a breast. This caking, or increased lobulated feel, was subsequently repeatedly noticed during paroxysms of pain. At this time, also, she suffered from an attack of pain in the right knee, and in the popliteal space a diffused swelling was felt which exhibited the same curious nodulated or leech-like feel as did the swelling elsewhere. It was also very painful, but subsided in a few days, and no permanent alteration of the tissues could be detected.
At various times subsequently paroxysms recurred, during one of which swelling was noticed in the posterior triangles of the neck, which seemed later to be permanently fuller than normal. Bronchitis also recurred, accompanied by dyspnoea, and at one time with free expectora- tion of bloody mucous.
In April following she experienced an attack of unusual severity. The pain, which involved the right arm and shoulder, right side of trunk and back of neck, now for thé first time spread to the face and head. The right side of the face and neck became distinctly swollen, and presented to the touch the same nodulated feel so characteristic of the swelling in other portions. At the same time, the tongue and prob- ably the pharyngeal tissues became swollen. Her tongue, she said, felt much too large for her mouth, and this certainly appeared to be the case. Her speech was much interfered with. Her voice was very hoarse, and she spoke with great difficulty. This condition persisted for upward of a week, when the swelling slowly subsided. For some time subsequently she spat blood, the source of which was not deter- mined, though it appeared to come from the throat. The reddish color in the cheeks also became more pronounced, until it covered the entire forehead like an intense blush. This blush was afterward observed to recur with other paroxysms of pain.
During the summer of 1888 the patient’s condition underwent some change. The paroxysms of pain became less frequent and less severe. Hand-in-hand with this improvement, sweating became very abundant. However, paroxysms accompanied with marked dryness of skin occurred from time to time, and upon one occasion a thick, welt-like swelling, exquisitely painful, was observed extending from the upper and inner angle of the scapula perpendicularly down the back to very nearly the lumbar region. Upon another time, swelling again made its appearance in the right popliteal space, as well as on the inner aspect of the knee. In the latter locality the swelling became permanent and the tissue pre- sented the same peculiarities as noted elsewhere. ;
Pain now occasionally appeared in the left arm. Prolonged attacks of cardiac dyspnoea recurred every week or two, and apparently inde- pendently of bronchitis.
Examination of the eyes by Dr. de Schweinitz revealed contraction of the fields of vision for form and colors, most marked in left eye. The other special senses, hearing, taste, and smell appeared to be somewhat obtunded.
An analysis of the urine yielded a negative result. A blood-count failed to reveal an increase of white corpuscles.
Since the notes from which the above account is condensed were taken, the patient has at various times during attacks of pain vomited blood. Upon several occasions this was observed by the writer himself. The quantity could not be accurately estimated, but while it was never in large bulk at a single emesis it was constantly brought up in repeated
526 DERCUM, ADIPOSIS DOLOROSA.
vomiting during an entire night or day. The last attack occurred in August, 1891.
Siessemene were made of this patient at various times, and there has been a steady increase in the bulk of the arms up to the present time.
As a whole, however, the patient has not suffered as intense pain as formerly. Cardiac dyspnea, though, is a frequent and very distressing symptom. Pulse soft and rather rapid, ranging from 95 to 110. Face still flushed. Of late has had shooting pains in the abdomen, and ex- amination discloses an extensive deposit of tissue in this region, and to which the pain is referred. A large longitudinal wheal, especially sensi- tive, is found in the left lumbar region.
A deposit of tissue (or swelling) has also made its appearance over the left hip and to some extent over the right. Thighs and buttocks do not seem to be especially enlarged, but soft masses are now found on the inner sides of both knees, the right larger than the left, the former more painful to pressure.
A small nodule to the right of the scorbiculus is especially painful.
At various times, by means of a Duchenne trocar, fragments for microscopic examination were removed from either arm. They revealed connective tissue and fat cells present in varying degree. It was observed that the former was decidedly embryonal in type, the cells being large and fusiform, and their nuclei correspondingly large and prominent. The fat-cells for the most part were associated with these connective- tissue cells, and occasionally individual fat-cells were seen in which the fatty metamorphosis had not been complete. (In one of the fragments removed the writer was fortunate enough to find nerve elements which had probably been included in the grasp of the trocar by the latter graz- ing a bloodvessel, as the fibres were non-medullated. Their connective tissue was denser than normal and they presented an unusual number of nuclei.) For a detailed description, with drawings, the reader is referred to the original article (doe. cit.).
Case II.—This, as already stated, was reported Dr. F. P. Henry (doc. cit.). Subsequently in November, 1891, she came under the care of the writer, having been transferred to the nervous wards, where she finally died about a month later. The following account is abstracted partly from the report of Dr. Henry and partly from my own notes.
E. W.., aged sixty-four, married, native of England.
Family history. Father dead of alcoholism at middle life. Mother dead at twenty-eight of oedema of brain, verified post-mortem. Has living an elder brother and sister and one younger brother. The younger brother when a child was “ peculiar ”—he would ‘run to people in sudden fright and say that he was drowning or the like. He is now in average health, but drinks heavily. Has a contracture of the ring finger. Has nine children, all of whom appear to be well. The older brother suffers periodically from violent headache ; also, since a young man has suffered from constantly cold feet—this so severe as to disturb sleep and cause great distress. He had five sons and two daughters. One son died of tetanus (traumatic) ; the others are well. One daughter has a contracted middle finger of the right hand; has never suffered pain in the finger. Patient’s sister is living, sixty-five years old, and healthy; no children.
Previous history. Does not remember having the ordinary diseases of childhood. At early infancy began to have fits, which at times occurred daily, at other times weekly. Consciousness was lost during the fits, and they were followed by great pain in the forehead. After the seizures she
DERCUM, ADIPOSIS DOLOROSA. 527
slept. During this time was relieved of lumbricoid worms—vomiting them; and some time later recovered from the fits.
Was married at seventeen. Had two sons, the older of whom is now forty and has seven healthy children; the younger died at two years of
Fig. 3. (Case II.)
Fie. 4. (Case II.)
hemorrhagic diarrhea. Patient had no miscarriages and no stillbirths. Left her husband because of a venereal disease contracted by the latter. Was told by a doctor that she had escaped infection. A year later,
528 DERCUM, ADIPOSIS DOLOROSA.
however, she had sore-throat with white patches. Had been an immoderate drinker for many years. For weeks at a time was intox- icated every night.
Menstruation began at eleven and ceased abruptly at thirty-five. Lost habitually an unusual quantity of blood, but never suffered any discomfort.
History of present disease. Her malady began about fifteen years ago, when she was forty-nine years old. At that time she was living in California. The first thing noticed was a constant feeling of coldness about the knees, followed by swelling, which gradually increased. At first she thought the swelling was due to her growing fat, but later was astonished to see that there was a localized mass on the inner aspect of each knee. At the same time there was dull aching pain in the affected parts. Later the right arm became involved, a mass making its appear- ance on the outer aspect. Her body she now noticed also became larger, as her stays were too small for her. During this time, while still in California, her inability to perspire, except at the Turkish bath, was marked and was part of her reason for coming East. Since she has been in Philadelphia the lack of perspiration has not been as marked as before. Various plans of treatment were tried, but did not influence the progress of the disease (that is the growth of the swelling). Five or six years ago injections of chloroform were made into the swellings on the inner side of the knees, but no good was done. Painful ulcera- tions were the result, and scars of considerable size mark their location,
About five years since a slight swelling appeared in the epigastrium. This gradually increased in size until it resembled the breasts in shape. and afterward spread so as to involve nearly the whole abdomen.
From the knees the process extended to the thighs, and gave rise to the large masses on their outer side and about the hips.
To Dr. Henry she stated that pain had never been a well-marked.
feature of the disease, which differed, however, from her statements made to the writer. To both, however, she stated that at various times she had suffered with pains apparently situated in the enlarged tissues or running down the limbs. Sometimes these attacks were fairly well localized, in one limb, in one side, or about a joint.
Five years ago her attention was called to a peculiar condition of the right hand. The last phalanx of the second finger began to be fixed in a flexed position, while the end of the finger appeared to be growing somewhat smaller. Later the remaining fingers of this hand became involved and all the phalanges deformed. The deformity as seen now is flexion of first phalanx, marked over-extension of second, and half- flexion of the third. The thumb is also stiff, but all of its joints are flexed. For some time she has noticed the thumb of the left hand becoming like that of the right.
A year ago the patient had a quasi-rheumatic attack affecting the deformed hand and the arm. The pains seemed to run up and down in the arm rather than about the joints.
Some months ago had pneumonia of the right lung, and made a good recovery.
For several months past had slight uterine hemorrhage at times, associated with which were dull, aching pains, resembling those formerly felt before menstruation.
On November 27, 1891, she was transferred, as already stated, to the nervous wards. Here, on questioning her in detail, I was able to con-
-™
DERCUM, ADIPOSIS DOLOROSA. 529
firm the points of the history, as described by Dr. Henry. In addition, she said that the enlargement had spread from the knees to the thighs and buttocks unequally, that the left thigh and buttock had been earlier and more conspicuously enlarged than the corresponding parts on the right side. Gradually, however, the latter became enlarged to an almost equal degree. Later, swelling appeared over the left arm, and later still on the back and sides of the trunk, and wherever appearing it gradually became diffused and finally reached very great proportions. The patient further volunteered the statement that she had formerly been very slight in build.
To ordinary obervation she merely presented the appearance of an excessively » person. However, examination soon revealed that the enlarged tissue was very unevenly distributed. In the region of the knees, where it had first made its appearance, it was excessively irregu- lar and lumpy. To the fingers it resembled, in a remarkable degree, the swollen tissues of Case I. It gave the same nodular and elastic feel, and could not be made to pit on pressure. At the time of the examina- tion no tenderness existed in any of the lumps, but shooting pains were referred to them in various situations. This was particularly the case in the mass over the right hypochondrium. In addition, she complained of scalding sensations on the inside of the right cheek and the right side of tongue. Nothing abnormal could be discovered in the mucous membrane of these parts. No tenderness existed in any of the nerve- trunks at the time of the examination. The patient was excessively weak, and could move about her bed or sit up only with great difficulty. Her grip was almost ni/. No tendon jerks could be cliclted—gaokelily due to purely mechanical difficulties. For the same reason, an electrical examination could not be made.
Examination of the cutaneous sensibility confirmed, in general, the findings of Dr. Henry, except that some areas had become entirely anesthetic. Dr. Henry found that there was slight analgesia, and diminished temperature and tactile sense, and further that the “changes of sensory acuteness were not more marked over the distribu- tion of any of the cutaneous nerves, but seemed dependent entirely upon the amount of the subcutaneous tissue.” Dr. Henry, it appears, found no area of absolute anzesthesia anywhere. However a year later, such an area undoubtedly existed on the back of the left arm, and extended thence over the posterior aspect of the left shoulder. On the opposite side, anesthesia was not present, although no marked difference, if any, existed in the amount of the subcutaneous tissue. A marked increase in this subcutaneous tissue had, however, everywhere taken place during the past year. Comparing, for instance, the measurements of the arms made by Dr. Henry and myself, it was found that the left forearm had increased one and seven-eighths inches, and the right forearm one and three-eighths inches ; the left arm one and a half, and the right arm two inches. This increase seemed to be maintained throughout.
Subjectively, the patient complained much of headache. Her face was very much flushed, and she suffered greatly from cardiac dyspnea. It was a persistent and distressing symptom.
Examination of the eyes proved negative, as did also that of the urine. Perspiration, according to the patient’s statement, was scant. Face not involved in the enlargement. No subnormal temperature. Hair thin, but not excessively so. No difficulty in speech. No mental impairment. ‘
530 DERCUM, ADIPOSIS DOLOROSA.
The patient remained very much in the same condition for some two weeks following her admission to the nervous wards, when her dyspnea greatly increased. Her pulse, already soft and compressible, became irregular and intermittent. This condition, although relieved from time to time, persisted until hands and feet became puffy, the face cyanotic, and the lungs cedematous and congested. Death occurred on December 22, 1891.
Autopsy, December 23d. Body of a very large woman. Weight esti- mated at about three hundred pounds. Face dark from venous conges- tion. Some discoloration on under surface of body and thighs. A number of large white scars on either side over the knees. Legs and feet cedematous. Body distorted and flattened, as though by its own weight.
Scalp and calvarium revealed nothing abnormal. Veins of dura and longitudinal sinus full. Venous congestion of the pia. Cortex a little darker than normal. Puncta vasculosa prominent. Brain other- wise normal. Spinal cord appeared normal. Skin of thorax appears normal. The subcutaneous tissue is fatty and moist.
Thyroid gland small, indurated and infiltrated by calcareous matter in both lobes.
Right lung cedematous and tightly adherent to chest walls. Left lung cedematous, with hypostatic congestion posteriorly. Both pleural cavities contain a large excess of fluid.
Pericardium contained some six to eight ounces of fluid, in which was suspended some flocculent lymph. Weight of heart twenty-seven ounces ; the right side dilated, the moderator band much thickened. Walls of
left side also much thickened ; marked hypertrophy of the columnz ‘ carnee and papillary muscles. Some fatty change, especially in walls of right ventricle.
Over the abdomen the subcutaneous fatty tissue was three inches thick. About a pint of ascitic fluid in abdomen. Stomach much di- lated. Intestines normal, Liver showed some fatty infiltration, other- wise normal. Spleen apparently normal, though somewhat dark. Kidneys both reveal, except slight adhesion of the capsules, nothing specially abnormal. :
In the pelvis, an ovarian cyst containing some six ounces and a hydro- salpinx were found on the left side. Uterus seemed a trifle larger than normal, Bladder normal.
Brain, cord, some of the nerve trunks, pieces of skin and subcutaneous tissue, pieces of the liver, kidneys, and spleen, a fragment of muscle, and the whole of the thyroid gland were removed for microscopic examina- tion. The specimens were left in the care of Dr. H. W. Cattell, assistant to the pathologist of the hospital. Unfortunately, Dr. Cattell fell ill with scarlet fever, and during his absence the specimens, together with those of the next case (Case III.), were thrown away by an attendant.
Case III.—M. M., aged sixty years, widow, a tailoress by occupation, and a native of Germany, but a resident of America for twenty-six years. Admitted to the nervous wards of the Philadelphia Hospital October 7, 1891. Memory very poor. History obtained in part from relatives.
Family history. Father and mother were healthy. Mother died of heart disease. Had seven brothers and sisters, all apparently well. Had no children, no pregnancies.
DERCUM, ADIPOSIS DOLOROSA. 531
Previous history. Many years ago a lump appeared at the back of the neck, for which she consulted Dr. Gross at the Jefferson Medical Col- lege, but for some reason no operation was performed. At various times thereafter swellings made their appearance in various situations.
Fig. 5. (Case III.)
Fie.'6. (Case III.)
Lost more blood at menstrual periods than normal. Occasionally suf- fered from hzematemesis and epistaxis. Climacteric at forty-six. No history of any intercurrent affections. Mental impairment had been noticed for about two years.
Present condition. Patient is excessively feeble. For some two weeks past has been unable to walk. Lies, for the most part, in a quiet apathetic state, though when aroused answers questions intelligently, but slowly. Is, in addition, somewhat deaf.
Examination reveals so‘t, fat-like masses or swellings in various situations. Thus a large soft mass is found over either biceps, and others, somewhat smaller, over the outer and posterior aspect of either upper arm. ‘Two large masses are found over the belly, separated above the umbilicus by a deep transverse crease. Another gives exces- sive prominence to the mons Veneris. From the back of the neck, at its lower part, springs a big mass like a hump, while a diffuse swelling gives a cushion-like coating to either half of the back, and extensive
532 DERCUM, ADIPOSIS DOLOROSA.
deposits give unnatural prominence to either hip. In marked contrast, the deposit is absent from the forearms and hands, from the face, from the thighs and legs and from the buttocks. The gluteal regions, in fact, seem flattened and sloping.
The deposit at the back of the neck and over the abdomen seemed tolerably firm and resistant ; over other portions it was quite soft, though elastic, and exhibited the same nodular feel noted in the previous cases. Further, it was discovered at once that these masses were painful to the touch, the patient complaining very much when only moderate pressure was exercised. This was especially true of the deposits over the arms and back of the neck. In addition, the patient complained of stabbing
ains in the deposits, more marked in the regions just mentioned. here was no tenderness over the nerve-trunks. She complained also of headache.
In making the examination, it was also further noted that the left radius was rough and nodular for about two and a half inches in its middle third ; also, that there was a large discolored area on the outer aspect of the left forearm resembling a syphilitic sear. Both tibize were somewhat nodular, though no scars were discovered on the legs. A few white scars were seen on the forehead. Quite a number of purpuric spots were also observed on the forearms, thighs, legs, and back.
The skin of the forearms and hands, and that of the legs and feet to a less extent, was dark, dry, and much roughened.
Cutaneous sensibility was found generally diminished, while a few patches of anzsthesia were noted. One of these was an area diffused over the right side of the trunk and the right shoulder. They appeared to be constant, and were confirmed at various examinations.
Owing to the extreme weakness of the patient, the study of the eyes could not be made satisfactorily, but, as far as it went, was negative.
The urine contained albumin. No casts were found.
In answer to questions, the patient said that she had not been sweat- ing freely for years, but owing to her mental condition no importance was given to this statement. She at no time presented a subnormal temperature. Her hair was well preserved.
Patient seemed to fail gradually, although diet and stimulants were freely used. Her dementia gradually deepened, and for some’ days before death she voided urine and feces involuntarily. She finally died in a comatose state on November 5th.
Autopsy, November 6, 1891. Body of a large woman with irregularly distributed fat-like masses. Some discoloration of the back. Small bed- sores beginning on the buttocks.
Scalp and calvarium normal. Dura normal. Pia very cedematous. Brain very soft and cedematous. Cord revealed nothing abnormal.
On incising the skin of the chest and abdomen it was found to be normal in appearance, but the subcutaneous tissue, which looked like a very white fat, was excessively thick, reaching below the umbilicus a depth of seven inches.
he thyroid gland was larger than normal, harder to the feel, and much calcified, especially the right lobe.
The heart weighed eight and a half ounces. Both aortic and mitral valves were slightly thickened. Heart substance evidently fatty. The lungs were emphysematous. The mucous membranes of the stomach revealed a chronic gastritis. The liver weighed forty-four ounces, and
DERCUM, ADIPOSIS DOLOROSA. 533
beyond some fatty infiltration, was practically normal. Spleen normal. The kidneys, however, showed decided shrinking and loss of cortical substance, with somewhat adherent capsules. Nothing noteworthy in pelvic organs.
As in Case II., brain, cord, nerve-trunks, skin and subcutaneous tissue, thyroid gland, and portions of other viscera were removed for microscopic examination, with the subsequent unfortunate loss of the specimens already mentioned.
It is not without some hesitation that I bring these cases before you. I am well aware that without a microscopic examination to supplement the autopsies their study is incomplete, and yet the cases are in them- selves so interesting, and appear to be so unusual, that their publication in a group with such data as are at hand is more than warranted. Cer- tainly these cases differ radically from ordinary cases of lipomatosis, and certainly the nervous symptoms present are not without a special sig- nificance. To begin, the enlarged tissue makes its appearance in a very irregular way. Nodules of soft tissue are, at first, deposited in some one situation, or perhaps in corresponding places of the upper or lower extremities. For a time the deposit is limited to these original areas, but subsequently it makes its appearance elsewhere, and may become very extensive. Regions, however, may exist which remain permanently uninvaded. In Case I. the enlargement was first noticed in both upper arms, and later in the back. Subsequently swelling made its appear- ance on the inner aspect of the right knee, to be followed months after by a similar swelling in a corresponding position over the left knee. Later still, it made its appearance in various other situations. However, the legs, with the exception of the knees, have remained free from involvement, while the thighs and buttocks have only receutly shown a doubtful change. In Case II. the enlargement began onthe inner aspect of either knee, and then gradually spread unequally over the thighs and buttocks. Later, the left arm became involved; next the sides and back, and finally the entire trunk. In Case III. the enlarge- ment began in the back of the neck, and thence at various times in other situations. It remained absent from the face, the forearms, the legs, thighs, and buttocks. It is a peculiarity of this case, further, that the enlargement tended to produce distinct segregated masses.
Not only is the development of the enlargement irregular and even capricious in these cases, but there is, in addition, another important fact to be remembered, and that is: that at some time or other the enlargement is accompanied by pain or other nervous symptom. Thus, in Case II., pain and a sensation of cold preceded the appearance of the nodules on the inside of the knees. In Case I. pain was noticed a year after the swelling of the upper areas had begun to show itself, and in
Case III. pain was evidently present at the time of the examinations. VOL. 104, No. 5,—NOVEMBER, 1892. 35
534 DERCUM, ADIPOSIS DOLOROSA.
In Case I., again, which I had the opportunity of studying in great detail, pain was observed at numerous times. Occasionally it was observed in old areas of enlargement, and again in regions free from the latter, but in which it subsequently appeared. This was especially the case in the swelling on the inner aspect of the right knee and certain welt-like formations in the back. Finally, pains, shooting or stabbing in character, were present in all cases, both at yarious times in the history and at the examinations. Very suggestive, indeed, were some of the paroxysms of pain observed in Case I. In some of them decided and sudden increase took place in the swelling of a part attacked, and it became, for the time being, firmer and more resistant, and occasionally more nodulated than before. Further, as already pointed out, a per- manent increase or a new focus of swelling made its appearance. It should be remembered, too, that some of the nerve-trunks, especially those of the right arm, were very sensitive to pressure; that some of the muscles—e. g., the thenar and hypothenar groups—revealed reaction of degeneration, and, more significant than all, that the patient suffered on two occasions from herpes zoster.
In Cases IT. and III. tenderness over the nerve-trunks could not be elicited. However, in Case I. this symptom has at present disappeared. Indeed its absence has been noted for some time past. This circum- stance leads to the suspicion that Cases II. and III. were further advanced than Case I., and that the latter was really observed during a developmental period and whilst more active changes were going on.
Among the nervous symptoms must also be placed the anesthesia ux diminished cutaneous sensibility already described, as well as the exces- sive motor weakness. It is probable that the absence or diminution of sweating also belongs to this category. It will be remembered that this symptom was undoubtedly present in Cases I. and II., and doubtfully in Case III. We are here reminded forcibly of myxedema, in which diminution or absence of perspiration is so prominent a symptom, and, at the same time, these cases are still further removed from ordinary obesity, in which excessive sweating is the rule. Headache was also noted in all the cases.
Among other symptoms present in these cases should be noted hzematemesis in Case I., hematemesis and epistaxis in Case III., and a recurrence of uterine flow many years after the cessation of menstrua- tion in Case III. In Cases I. and II. the menopause occurred at thirty- five, and in the latter the flow was said to have been unusually free. In Case III. the menopause occurred at forty-six, and menstruation was likewise said to have been excessive Finally, Case III. also presented a well-marked purpura. What the significance of these symptoms may be it is impossible to say. It may, however, not be out of place to recall
PR ew eee ow geo
DERCUM, ADIPOSIS DOLOROSA. 535
the not infrequent occurrence of uterine hemorrhages in women who subsequently suffer from myxcedema.
Bronchitis is a most frequent and persistent symptom in Case I., while both Case I. and Case II. suffered markedly from cardiac dyspnea. Both of these symptoms were absent in Case III. By their presence we are again reminded of myxcedema, in which they are frequently present.
As already stated, fragments of the enlarged tissue were removed from Case I. by the Duchenne trocar, as also from Case II. It both instances fat-cells and connective tissue were found in various proportions, though at times the latter was decidedly embryonal in type; this was especially so in Case I., in which in certain areas embryonal connective tissue pre- dominated. It would seem that this is the case in the more recent for- mations, while in the older areas a fully formed adult fatty tissue appeared to be present. It is especially to be regretted that the loss of the specimens from the autopsies of Cases IT. and III. prevented a con- firmation of these results. The autopsies, however, are not without interest when it is called to mind that in both cases the thyroid gland was found indurated and much infiltrated by calcareous deposit. It is impossible, however, to correctly interpret this condition in the absence of microscopic studies.’
Now, with the above data before us, what view are we to hold in regard to these cases? Evidently the disease is not simple obesity. If so, how are we to dispose of the nervous elements present? Equally plain is it that we have not myxcedema to deal with. All of these cases lack the peculiar physiognomy, the spade-like hands, the infiltrated skin, the peculiar slowing of speech, and the host of other symptoms found in true myxedema. It would seem, then, that we have here to deal with a connective-tissue dystrophy, a fatty metamorphosis of various stages of completeness, occurring in separate regions, or at best unevenly distributed and associated with symptoms suggestive of an irregular and fugitive irritation of nerve-trunks—possibly a neuritis. That this, how- ever, does not embrace the whole truth is evidenced by such symptoms as the diminished sweating, the headache, and the contraction of the visual fields noted in Case I. However, the above inference is all that we are justified in making.
Inasmuch as fatty swelling and pain are the two most prominent features of the disease, I propose for it the name Adiposis Dolorosa.
1 In the light of these interesting findings, it seems desirable that in all cases of obesity, whether typical or otherwise, the thyroid gland be studied.
RICHEY, ETIOLOGY OF GLAUCOMA.
THE PRIME ETIOLOGICAL FACTOR OF GLAUCOMA IS CONSTITUTIONAL.’
By 8. O. Ricuey, M.D.,
OF WASHINGTON, D. C,
Tuis paper will be occupied with the presentation of one idea, for a résumé of the literature of glaucoma would unprofitably occupy much space, as so much has been written offering diverse views of its different features, each with a show of reason.
Mr. Jonathan Hutchinson, in the Bowman Lecture, 1884, discusses the relation between certain diseases of the eye and gout. The tissues of an individual long subject to the causes of gout may become modified in such a way that they are liable to suffer in a peculiar manner when exposed to the ordinary causes of disease; the nervous and vascular sys- tems are specially so disposed. Rheumatic gout may have such a rela- tion to true gout, and he names “ hot eyes,” calcareous bands of the cornea, arthritic iritis, relapsing cyclitis, glaucoma, and retinitis hemor- rhagica as having such connection ; and asks if it can attack any of the structures of which the nervous system is composed.
In the London Lancet, January, 1873, he describes an iritis occurring at an early age, differing from other forms of arthritic iritis, in being persistent and insidious, rather than paroxysmal. Without any attack of acute inflammation, adhesions quietly form between the iris and the capsule of the lens. . . . This affection usually begins in but one eye, and advances to almost entire loss of vision in it, before attacking the other. It is insidious, and for the most part painless, but is liable to exacerbations and periods of improvement. It is remarkably intract- able, prone to attack both eyes, and to end in blindness.
Such is the position of an -acute observer as to the influence of gout upon the eye.
The question as to the cause of increased tension is still open, whether due to too rapid infiltration, or to impeded excretion, with a leaning to the latter.
Opposed to Mr. Priestley Smith’s theory, that “glaucoma of every form is essentially a disease of retarded excretion,” are the conclusions of Schnabel,® supported by clinical and pathological studies, that “ glau- coma may be present without obliteration of the sinus of the anterior chamber; that the latter can exist without glaucoma; that glaucoma
1 Read at the meeting of the American Ophthalmological Society, July 20, 1892. 2 Trans. Seventh International Medical Congress, vol. iii. p. 84. 3 Archiv Ophthalmol., vol. vii. p. 14.
RICHEY, ETIOLOGY OF GLAUCOMA. 537
can be cured without obliteration of the sinus of the chamber being removed.”
“Tt has been proved by Mr. Windsor,’ of Manchester, that acute glaucoma may occur where there is congenital absence of the iris.”
A doubt, which reaches almost a denial, is general as to whether excavation of the disc is due to pressure, or not. In Rydell’s’ case, blind from acute glaucoma of three weeks’ standing, without excavation, pain was relieved and tension reduced, but vision was not improved. Mauthner’ claims that “‘ We find in the beginning of an excavation that pressure frequently is not increased. I have recently examined the left eye of a patient, in which there is the beginning of a pressure excavation, of which there was not the slightest sign a year ago, when I saw him for paresis of one of the muscles. The functional disturbance is extra- ordinary, and shows itself in transitory ohscurations ; central S. is less than in R. E., which has S = 6/v1, while with L. E. a few letters of 6 are not seen at 6 m. distance. Without glasses the patient, who is forty- five years old, reads with R. E., J. 2, with the left eye J. 3: F. undis- turbed. The well-known appearance of the vessels is very marked at the upper lateral edges of the papilla. T. is precisely the same in both eyes, and falls even below the physiological maximum. Would such a press- ure produce such a picture?”
“Some morbid process has attacked the intra-ocular end of the optic nerve, causing a diminished resistance (softening) of the lamina cribrosa, so that it yields to even normal pressure in the eye, but at the same time there is going on in the optic nerve an alteration, which has the greatest resemblance to that in the lamina cribrosa, and leads to a softening, to a giving way of the supporting connective tissue.” *
Reading these comments on glaucoma with a free mind, our previous ideas are subverted, because we must conclude that increased tension is not necessary to excavation ; that excavation is not always present, even when increased tension has existed sufficiently long to produce it ; that excavation may result from® “some morbid process” in the nerve, lessening its resistance; that increased tension is not dependent upon obstruction of the channels of excretion.
1 A Practical Treatise on Diseases of the Eye, by Haynes Walton, London edition, p. 1170. See Ophthalmic Review.
2 Von Graefe’s Archiv, 1872, vol. xviii. pp. 1-51.
3 Archiv Ophthalmol., vol. viii. p. 38. 4 Vide supra, p. 39.
5 Garrod, on “ Rheumatoid Arthritis,’ Reynolds’ System of Medicine, p. 553: “In the early stage, when swelling is prominent, a considerable increase of synovial fluid is found, and the joint exhibits the same appearance as in case of ordinary inflamma- tion. The lining membrane is often red from over-injection of the bloodvessels. If the bone be sawn through, it is often found spongy, and contains a large amount of oily matter, from the occurrence of a species of fatty degeneration.” N.B. All italics are my own.
538 RICHEY, ETIOLOGY OF GLAUCOMA.
If the last proposition be true, that increased tension is not dependent upon obstruction of the channels of excretion—and Schnabel supports his conclusion by dissections of the organ which he had observed while affected with the malady—then increased tension must be caused by too rapid infiltration, or secretion. Schnabel argues further,’ that glaucoma is a disease of the bloodvessels of the eye, which develops either gradu- ally, or at once, in the region supplied by the long anterior and posterior ciliary arteries, the central bloodvessels, and those of the sclerotic circle; that the disturbances of nutrition and function are the direct result of these disturbances of circulation, etc.
Mr. Priestley Smith’s theory was obviously derived from the study of glaucoma of local origin; and yet, as Mr. Brailey, of London,’ says, “it fails to account for temporary glaucoma, for glaucoma without the characteristic application of the iris, for glaucoma in young persons, for one-sided glaucoma, for glaucoma in aphakic eyes, and especially for cases where a traumatic dislocation of the lens backward has been quickly followed by increased tension. It does not, also, explain the invariable inflammation and atrophy of the ciliary body and optic nerve.”
To the theory of increased secretion, or more properly too rapid infil- tration, a vis a tergo, some derangement of the general system, is a sine qua non. The uric acid diathesis, of which gout is a characteristic feature in many instances, offers the most satisfactory explanation: true gout, of acute inflammatory glaucoma; rheumatic gout, of chronic simple glaucoma.
In nearly all particulars acute gout of the toe and acute inflammatory glaucoma are alike. Observe the points of resemblance :
Acute INFLAMMATORY GLAUCOMA.
1. An inherited tendency.
2. Most frequent after the period of presby opia.
3. First attack is usually in cold weather.
4. Premonitory symptoms: Impaired A.; premature presbyopia, increased H. ; halo, rising clouds or smoke, heaviness of brow, shooting pains in the eye, increased tension. These may be so slight as to cause no anxiety.
5. Sudden seizure, usually at night.
, Acute Gout or THE Great Tor.
1. An inherited tendency.
2. Most frequent after the beginning of senile changes.
3. First attack, usually in winter, or spring.
4. Premonitory symptoms may be so slight as to pass unnoticed, or may be very distressing.
5. Attack is sudden, usually between two and five o’clock in the morning. (Garrod.)
1 Archiv f. Augenheilkunde, vol. xv. p. 311.
2 Trans. Seventh International Medical Congress, vol. iii.
i ‘4
RICHEY, ETIOLOGY OF GLAUCOMA.
Acute InFLAMMATORY GLAUCOMA.
6. Constitutional disturbances; febrile excitement, with some nausea and vomit- ing.
7. Circumorbital pain, peri-corneal and sub-conjunctival injection, slight protru- sion of globe, sluggish, dilated iris; cornea dull and anesthetic, humors greenish, ischemia.
8. As the attack passes off there is great chemosis, lachrymation, and photophobia. The cornea becomes roughened.
9. The inflammatory attack passes off in a few days or weeks.
10. The disease is not arrested: there may be a recurrence of acute inflamma- tory attacks, chronic inflammatory exa- cerbations, or the disease may progress insidiously.
11. No pus.
12. Urine. ?
13. No analysis of aqueous humor, so far as I know.
14. The disease may attack first one eye and then the other.
15. Occurs most frequently in women.
539
Acutr Gout or THE Great Tor.
6. Chilliness, heat of skin and per- spiration, thirst, loss of appetite, a white tongue, constipation, and restlessness.
7. Toe is swollen, red, hot, and ex- quisitely tender. Veins proceeding from the toe are turgid with blood, and the joint is stiff. Great tension of the skin.
8. As the attack passes off there is pit- ting of the skin (edema), then desquama- tion.
9. Duration, from four days to three weeks. ¥
10. Gout recurs, and the frequency of the paroxysms increases.
11. No pus.
12. Urine scanty, high-colored, and de- posits a colored sediment on cooling.
13. Synovia contains urate of soda.
14. Gout not uncommonly seizes first one great toe, then the other. 15. Is rare in women.
Thus, each may be inherited and have the premonitory symptoms; the attack is sudden and at night; in each it is characterized by great pain, engorgement, and tension, followed by cedema and exfoliation ; duration, from a few days to a few weeks; recurrence of the affection,
possibly to attack the other side, or to become chronic.
Such is the clinical picture.
No pus.
That acute inflammatory glaucoma is more frequent in females, and gout of the great toe more frequent in males, may be due to the greater emotional tendencies of women ; for, according to Schweigger “ mental emotion and loss of sleep favor acute glaucoma.”
While women derive a certain immunity from podagra by reason of menstruation (Hippocrates), yet at the approach of the climacteric, a period of greater or less tendency to vascular cerebral disturbance, aris- ing from the intermittence of the derivative action of this function, acute inflammatory glaucoma is most frequent, and chronic simple glaucoma develops. ;
“The great toe’ contains a considerable amount of tissues peculiarly liable to become the seat of the deposition of urate of soda; as, for ex- ample, the cartilages and ligaments, tissues having either little vascu- larity, or nourished independently of bloodvessels; the great toe being
1 Garrod, “ Pathology of Gout,” Reynolds’ System of Medicine, vol. i p. 535.
540 RICHEY, ETIOLOGY OF GLAUCOMA.
very remote from the heart, the circulation is weaker there.
The reasons for the great toe on one side of the body being affected apply equally to the other; and hence, the disease not uncommonly attacks first one toe and then the other, within the short space of a few hours or days.”
Anatomically, the eye is an extremity of the body, not quite so far from the heart as the toe, and is exposed to variations of temperature and to injury; the sclerotic, the cornea, and the tendons of the extrinsic muscles are of dense fibrous tissue, with little vascularity; the stroma of the choroid and iris is of reticular connective tissue, supporting pig- ment cells, bloodvessels, etc., the zonule of Zinn is a fibrous perforated membrane, the lens capsule is a structureless membrane, the corpus vitrei depends upon bloodvessels not its own for nutrition, and con- tains mucin, and (Picard) 0.55 per cent. of urea, and about 0.75 per cent. of sodic chloride. The posterior surface of the iris and ciliary body secrete the aqueous humor (synovia?) which contains a small amount of albumin, sugar, and sodic chloride, equal to z'; of its volume.
With increase of blood-pressure and intra-ocular pressure, there is in- crease of albumin and the production of fibrin in the anterior chamber. (Jessner and Griinhagen.)
Taken with the fact that a local derangement, as a dislocated lens, does not seem sufficient to cause the whole train of symptoms, general as well as local, called glaucoma (though it may precipitate an attack which would probably have taken place at a later date), the clinical history of a seizure and the anatomical peculiarities of the regions under consideration present a picture of such mimicry as we find nowhere else repeated. The crucial test, the presence of urate of soda, I have had no opportunity to apply since recognizing the resemblance.
To again read Garrod, “The impure state of the blood, due to the presence of urate of soda, is probably the cause of the disturbance which often precedes the gouty paroxysm ; that is, of the so-called pre- monitory symptoms. Urate of soda in abnormal quantity in the blood is essential to an attack of gout, . . . . but does not constitute gout; . . . . that the amount of deposited urate of soda is not in proportion to the intensity of the inflammation, and that in some the infiltration may ensue and give rise to scarcely any inflammatory action.
The inflammation of the gouty paroxysm tends to the destruction of the urate of soda in the blood of the inflamed part, and probably of the salt also which is thrown out.” Soelberg Wells’ ob- serves that “males who are attacked by glaucoma frequently suffer from gout, or disorders of the digestive organs :” of primary glaucoma, “ when
! Reynolds’ System of Medicine, vol. i. p. 533. 2 A Treatise on Diseases of the Eye, 3d Amer. ed., 1880, p. 589.
RICHEY, ETIOLOGY OF GLAUCOMA. 541
once the one eye has become affected by glaucoma there is great ten- dency in the disease to invade the other also.”
Mr. Hutchinson’ asserts that “all forms of rheumatism, and all forms of gout, are included in the common term, arthritic. But we cannot limit the term to the joints, as its etymology might seem to require, but must allow it also to apply to certain affections of the muscles, fasciz, tendons, and other fibrous structures which have been proved to be dependent upon the same peculiar state of health, . . . . Under the term rheumatism we include all arthritic maladies which are not proved to be gouty. . . . . I must protest, at once, against any attempt to limit the term gout to cases in which attacks of acute in- flammation of the great toe occur. . . . . Rheumatism differs from gout in being of nerve origin, and due to reflex disturbance of nutrition; . . . . it is, according to my hypothesis, the basic diathesis to which a small minority of cases of gout is superadded.”
The younger Garrod says that rheumatic gout lacks the distinguish- ing feature of gout, urate of soda.
Many of the manifestations of rheumatic gout are associated with chronic glaucoma, viz.: enlarged or distorted joints, a peculiar senile pallor, or muddiness of the skin ; periods of mental depression, and other symptoms, attributable only to changes in the nervous system. I have found nowhere any reference to pathological alteration of nerve tissue in gout, although the existence, character, and specific cause of such changes, which are presumed to exist because of the nervous symptoms present in lithiasis, would have important bearing upon the subject in hand, in explaining the structural changes in the lamina cribrosa and the intra-ocular end of the optic nerve, the condition of diminished re- sistance associated with excavation without increase of tension, in cases of chronic glaucoma.
Dr. W. W. Johnston,? Washington, D. C., published some thoughts “On the Nature and Treatment of Forms of Disease characterized by Indigestion, the Presence of Bile, Urates, and Uric Acid in the Urine, and by Nervous Symptoms,” which suggest a possible cause and expla- nation of the nerve changes in chronic glaucoma. In his own words, “The question of the continuous production of toxic substances in the intestinal canal in health, and the protection of the organism by physio- logical elimination, as well as the auto-intoxication of the organism by the absorption of poisons in alterations of the gastro-intestinal tract, was developed in detail by Professors Albertoni and Silvia at the meet- ing of the Fourth Italian Congress of Internal Medicine, held in Rome. Professor Silvia enumerates the following substances as probable
1 Trans. Seventh International Medical Congress, vol. ii. p. 92. 2 The Medical News, March 12, 1892.
542 RICHEY, ETIOLOGY OF GLAUCOMA.
poisons: peptoxine, organic bases (ptomaines and leucomaines), indol, phenol, lactic acid, ammonia, sulphuretted hydrogen, acetone, etc. The direct proof of the fact that the nervous phenomena in such cases are due to the absorption of toxic matters from the intestines is not yet found, but the argument is a forcible one. The existence of indigestion is known by the symptoms: the presence of toxic matters in the in- testine in health is proved. . . . . The relationship of acute indigestion and nervous disturbances, and the association of fermentative dyspepsia with nervous symptoms, and an excess of these products in the urine and feces, give sufficient grounds for adopting this theory as reasonable.” '
Dr. Johnston has given much attention to the subject of digestion, and if a reference to his able paper will induce those who have the care of cases of chronic glaucoma to read it, it will probably divert attention from glaucoma, except as a local manifestation of a general malady (although he does not refer to glaucoma), broaden the view of the subject, and enable us to comprehend the changes in nerve tissue going on elsewhere in the system in rheumatic gout, by that which takes place in the intra-ocular end of the optic nerve, exposed to obser- vation, in chronic glaucoma.
Returning to the subject of intra-ocular tension, Mr. Priestley Smith’ claims that “ high tension depends more upon an excess of blood in the eye than upon an excess of intra-ocular fluid,” while Dr. Spender’ has observed, as early symptoms of arthritis, increase of pulse rate with high arterial tension.
Mr. Hutchinson® concludes that “it is probable that there are many different forms of inflammation of the eye, or of parts of it, which are in connection with gout. They may be divided into two groups: a, those which go with acquired, humoral, or renal gout ; 6, those which depend upon inheritance of structures damaged, or, at any rate special- ized, by gout in predecessors. The difference between the two classes of affections is very marked. In the one, attacks of a transitory nature are the rule, and the attacks are often acute and attended by much pain. In the second group, although a tendency to temporary recovery and recurrence is often observed, yet, there is a great proneness to chronicity, and persistence. The invasion is often insidious, but the disease is usually in the end destructive.”
If the difference between the forms of acute inflammatory and chronic simple glaucoma had been in the mind of Mr. Hutchinson the description could not have been more effective than in the specification of the two groups named above.
1 Ophth. Rev., vol. vi. p. 196. 2 Garrod: A Treatise on Rheumatism. Am. ed., 1890, p. 245. % Ophth. Rev., vol. iii. p. 385.
RICHEY, ETIOLOGY OF GLAUCOMA. 5438
His address will bear reading with this thought.
Ordinarily, when both eyes are attacked by the same disease process, we rationally conclude that the cause is constitutional, and do not treat an expression of the dyscrasia, but rather its cause.
In chronic glaucoma, a local manifestation is treated (for, sooner or later, both eyes are attacked), and then we wait to see what “turns up,” with about the results presented by Dr. Bull,’ of New York, to the American Ophthalmological Society, in 1889; the detailed history of ninety cases of chronic simple glaucoma, subjected to the operation of iridectomy, during a period of seventeen years. The paper is most inter- esting and instructive, especially the summing up: “One hundred and fifty-four operations were done on the one hundred and eighty eyes under consideration. Vision was temporarily improved by iridectomy in both eyes in two cases, and in one eye in six cases; but in all eight cases, after a few months, a steadv loss of vision and narrowing of the field set in, and continued progressively as long as the patients were under observation.
“ Vision remained unchanged, neither better nor worse, after the oper- ation, for a period of one year or longer, in both eyes in eight cases, and in one eye in twenty cases.
“Vision grew slowly and steadily worse after the operation, in both eyes in forty cases, and in one eye in twenty-nine cases.
“Vision grew rapidly worse after the operation, in both eyes in two cases, and in one eye in eight cases.”
He concludes that “the health and age of the patient exert a decided influence upon the operation, and any marked evidence of senility is distinctly unfavorable to the operation.”
Dr. Gruening,’ of New York : “ In chronic glaucoma with degenerative changes, neither iridectomy nor anterior sclerotomy will give the patient the desired relief; posterior sclerotomy may do it at times.”
Mr. Power,’ of London: “ In cases of chronic glaucoma no operation is of much service.” This terse statement, it seems to me, covers the whole ground.
The good results of operation in chronic glaucoma are in compara- tively small ratio, and are therefore accidental, and not scientific; for it often precipitates disaster by additional irritation. So long as the two chief clinical characteristics of glaucoma, increase of tension and excavation of the disc, are not satisfactorily explained, the management of such cases must beempirical. The author of iridectomy for glaucoma acknowledged it to be empirical, and only experience has taught us in
1 Trans. Amer. Ophth. Soc., vol. vi., part 2, pp. 246, 291. 2 Ibid., 1889. 5 Trans. Seventh Internat. Med. Congress, vol. iii. p. 106.
544 RICHEY, ETIOLOGY OF GLAUCOMA.
what cases it is of most value, those of acute inflammatory glaucoma ; for here it saves the eye until another time ; it does not cure the disease. Dr. Bull’s statistics do not teach us to do iridectomy in chronic glaucoma, cases of which form of the disease are in excess of any other, unless upon the plea of dernier ressort—because we know of nothing better. They indicate that the majority of eyes are worse after an operation ; in a few the status quo ante is maintained ; in a still smaller percentage there is some improvement. With this diversity of result, who, save in the occasional case of immediate gain, or loss, to the eye, can say what influence is attributable to operation? Might the case not have done just as well without interference? Is the surgeon justified in a feeling of certainty that he has done a service? If all such cases followed approximately a given course he would have a guide; but they vary so much. If it progresses slowly after an operation, it might have done so without it. If it remains stationary for a time, can that be attributed to operation? If the patient goes rapidly blind, has hea right to re- proach the surgeon? In operation is possibility, not probability. In simple glaucoma it has a questionable rationale, and experience teaches that, if done at all, it must be done with caution. It is double-edged, and may cut either way.
It is a prime necessity that a quiet, healthy, out-door life should be led, apart from occupations of much nervous excitement, causing loss of reserve force ; that a condition of self-possession should be maintained ; that the dietary should be regulated as to time, quantity, and quality ; for over-feeding and bad feeding is a conspicuous vice of the age. In adult life the effort should be to preserve the balance between waste and repair, and to see that both processes are normal. This is a duty which the family physician may share.
As such cases pursue so chronic a course, it would seem wise to die cover the constitutional cause, and to begin with that, instead of with the last expression of the disease, leaving the cause in action.
Rational management of the disease involves a study of the general condition and a correction of all the habits of the individual. This is difficult, but our function is advisory, and each sufferer must “ work out his own salvation ” with our guidance.
By controlling the quantity of food productive of uric acid, and by reducing the whole quantity to the possibility of easy digestion and assimilation, thus lessening the amount of toxic substances in the in- testinal tract; by the regular entire excretion of what is excessive by way of the kidneys and bowels, harm in this way is obviated. Tonic aperients (not irritants and excitants), which encourage natural action of the intestines, serve a good purpose when used with judgment. Hunyadi water, taken at bedtime, lies in the tract all night, does not purge, but by its solvent power prevents accretions. Nothing should be
POTN SRN Ame:
RICHEY, ETIOLOGY OF GLAUCOMA. 545
done to lessen the digestive power, and a quantity of food should be taken, small enough to inswre its digestion and proper disposal. Any- thing (as coffee) which retards digestion must be rejected for obvious reasons.
Salicylate of phenol, it is claimed, has been found in the joints of gouty persons taking it; therefore its purpose is apparent.
Lithia waters secure the excretion of some uric acid; piperazine, a new synthetical compound, is commended as having twelve times the solvent power of lithia upon uric acid. Strychnine acts by stimulating the functional activity of all the organs of the physical economy.
Galvanism, if properly and steadily used, is profitable. After ten minutes’ use of two milliampéres direct current to the sympathetic, in an ordinary case, ocular tension is lessened, the pupils seem more active, and the patient becomes calm, often almost falls to sleep. By the experi- ments of Onimus and Legros’ it has been shown that if the direct current (positive pole at the nerve centre) be employed, the circulation is aug- mented ; within a few moments the arteries have increased in bulk, and the whole network of capillaries is seen in great commotion. Faradiza- tion contracted the bloodvessels, but after a time contraction ceased, and the arteries became larger than before the application. The continuous current, on the other hand, renders circulation more active, and reéstab- lishes it when it has been arrested. The induced current causes spas- modic contraction of the unstriped muscle, while the continuous current produces a vermicular contraction (Bartholow). The latter thus favors the natural movement of the vessel, and while directly increasing the amount of blood passing, by reaction the amount of blood in the part supplied by the vessel is reduced to the normal. The object to be gained, stimulation of the cervical ganglia, the trophic centres of the region of the trigeminus, is accomplished as well with the cathode held in the hand as in contact with the affected region ; yet, when placed on the temple, or above the eye, it has some additional mental effect, which is not unde- sirable.
The writer has endeavored to cover the ground as concisely as possible : to offer the salient points of a view of the subject he has entertained for several years, especially in regard to too much food. He thinks that in the hypothesis discussed we find the true etiological factor of the most intractable of diseases, chronic glaucoma; that acute inflammatory glaucoma is a paroxysmal expression of the same affection; that local irritation, or trauma, excites an attack of glaucoma only in the presence of the dyscrasia ; that operation saves the eye during a paroxysm ; that operation serves little purpose in chronic glaucoma, even when it does not, by irritation, hasten the disease process or precipitate a paroxysm ;
1 Traité d’Electricité Médicale, Paris, 1872.
546 VAN DE WARKER, HYSTERICAL MANIA.
that chronic glaucoma is a neurosis—a progressive atrophy with the feature of inflammation with deficient power, varied by periods of seem- ing rest; that correcting and controlling individual habits, especially in the amount and character of food taken, will do more to preserve vision than operation ; and that there may be a possibility of aborting chronic glaucoma, if the tendency to it be recognized at an early stage.
HYSTERICAL MANIA.
By ELty VAN DE WARKER, M.D., OF SYRACUSE, N. Y.
GivinG the broadest possible range to the definition of insanity, there are yet certain mental states fully as incapacitating and equally diffi- cult to treat that, by common consent, are not included under this term. Without attempting to define what hysteria really is, I am safe in asserting that the phenomena are manifested within the intellectual field. Even if it is manifested as a bodily condition, with no apparent trace of a mental associated error, yet these bodily conditions are largely subjective ones, and have their real status in a perversion of a conscious state—a condition as evidently objective as a phantom tumor that can be made to depend upon a mental impression. In this relation I use the term mania, not in the sense that the diseased mind has originated a delusion, but because the mind has lost the power of diverting itself from certain fixed ideas, and about which it circles in an endless itera- tion. So far as an alienist is able to define, the disease which it is his so-called specialty to treat does not exist without a delusion. But we as gynecologists must recognize the fact that the mental function may be entirely unhinged without the trace of a delusion. The mind is as much in a pathological state, so far as normal logical and associated ideas are concerned, as though dementia or active mania existed.
Mental hysteria expresses itself along certain well-marked lines that sharply define it from insanity. This form of hysteria may be classified in groups just as insanity is classified, and like the latter has a compara- tively narrow range. Take as an illustration the melancholic form. Every waking moment is devoted to tears, or is absorbed in the deepest gloom. But no man who is familiar with the work of the gynecologist would say that it was melancholia in the sense of the alienist, and what is still more remarkable, it never will be, so far as there exists any tendency in the disease itself to degenerate into insanity. Many of these cases drift into asylums, to their great misfortune, and I am sorry to be obliged to add the deplorable fact that the actual mental status of
VAN DE WARKER, HYSTERICAL MANIA. 547
the patient is never recognized by the professed insanity doctor. This is the type of case that belongs to the gynecologist, and has for a quarter of a century afforded ground for the demand that a competent expert be assigned to each hospital. We have never been able to bring this simple fact before asylum managers in a practical form. They have so completely misunderstood the position we have taken that they have consented to the appointment of female physicians without in any way exacting a practical knowledge of gynecology. Basing my conclusion upon my own personal experience, I believe that ten per cent. of female inmates of asylums—under thirty-five years of age—can be restored to society by proper treatment and removal from the society of the insane. I regard removal from the environment of the asylum as essential to recovery, on account of the marked initiative tendency in the mental action of the hysteric. Mental operations in this class partake largely of automatic action, and the force of example is sufficient to constitute a morbid impulse. ;
Many of these cases unhappily drift into asylums, and are rarely ever discharged. It is one of the attending evils of massing large num- bers of the insane together, that proper classification and discrimination cannot be made. It would require an insanity doctor to understand physical diseases, as very few of them at this age of the world do, to realize that many of the inmates of his asylum are not insane at all, but are simply imitating what they see around them. This brings to mind a matter that many years ago I contended for, supported by the powerful alliance of the late Dr. Wilbur, a distinguished alienist, that asylum physicians ought to be appointed from the ranks of the general prac- titioners of not less than five years’ service. The majority of asylum physicians, aside from the fact that they have passed through a medical college and have received a diploma, are not medical men at all. It is ample proof of this, that the usual practice is to call in the local general practitioner to treat common diseases among the inmates of even large institutions.
The most common type of mental hysteria is the melancholic form. Its diagnostic features are that the cause and its mental reflex are equally apparent, and exist on parallel lines. You observe in the objective conditions sufficient reasons for the mental wreck. Every mental operation is attended with logical coherency ; there are no delu- sions or morbid suspicions. Why, then, should we say that she approaches the insane type? Simply because her will is absolutely powerless to divert her mind from a fixed and dominating line of thought. Its direction is always turned inward upon herself. Her wasted life, her neglected duties, and the poverty of hope that she will ever get well. There is an iteration about it that Shakespeare has well called dam- nable. In hospital work I have known the strongest nurses completely
1
548 VAN DE WARKER, HYSTERICAL MANIA.
broken down from constant association with such a case. She is not a monomaniac, for that implies a fixed delusion coexisting with a more or less normal remnant of mental action. The train of thought that pro- duces this state of morbid mental fixation is based upon actual facts, the importance of which she may not in any way exaggerate. She simply comes to a fixed and morbid conclusion that renders her mind as useless for the purposes of life as though she was beset by the wildest delusions.
Another phase is the hysterical type of the dement. I measure it by this well-known form of insanity because outwardly there is not a trace of a thought or emotion. She will not answer a question or express a wish. She is not resistant; everything about the outbreak is passive. She is receptive and tractable, but cannot originate an idea. Several days will pass in this condition, when the will power suddenly regains its force, and normal mental action be recovered. This form frequently marks the menstrual crisis, or follows a prolonged outbreak of the melancholic form, oftentimes attended with unappeasable tears. A more rare form of the hysterical dementia follows surgical operations, in which food is refused, and the patient positively but stubbornly refuses the personal attentions of her nurse. This form is not without danger, as I have recently experienced in a very delicate emaciated blonde approaching middle life, and on whom there was recently per- formed a simple operation for fistula-in-ano. In the hospital she could be fed by the care and skill of her nurse, but when removed to her own home died from exhaustion after a week or so. Her removal was the result of the very common notion that such patients will quickly recover if they can only be removed to their own home. While this form of hysterical dementia always embarrasses the conduct of a surgical case, it rarely has the unfortunate result just noted. Indeed, what forms a strong diagnostic line between the hysterical form and insanity as a mental disease is the self-limited character of the former. Knowing his patient, the surgeon can rest assured that a comparatively brief time will see his patient restored to a normal mind, while in the latter it is among the most hopeless of mental diseases.
The last form of hysterical alienation is that form known as mania traumatica. To classify this comparatively common condition among the chronic mental diseases is entirely to misunderstand its nature. It was well understood about forty years ago, with a fairly abundant liter- ature, which was entirely lost sight of, so that a few years ago it found its place in current literature as a new discovery of a surgical compli- cation, until the profession was better informed by a review of its history from the pen of Dr. Mary Putnam Jacobi. It is an interesting fact that surgical mania began to attract attention about the beginning of the era of anesthesia in surgery. A case of my own proved in a most
VAN DE WARKER, HYSTERICAL MANIA. 549
pointed way this connection. A middle-aged German was admitted to the Central New York Hospital for a genito-plastic operation. She passed out of the ether stage only to pass into most violent mania, with morbid suspicions and active motor disturbances. In about a week the mental disturbance had passed, and she made a quick recovery. I afterward learned that a similar attack had followed anesthesia for a dental operation. My repair operation was a failure and had to be repeated—this time without an anesthetic and without any mental dis- turbance. The literature of the subject shows that occasionally a man develops surgical mania, but the vast majority are women. Mania of this character, of a few hours’ duration, is very frequently met with after an anesthetic, and always gives the surgeon a few bad hours, especially after a severe operation.
The brief recital of a few cases to more fully illustrate the different forms of the mental type of hysteria will not be out of place:
Mrs. 8., aged thirty-five years, sterile, retroversion, utero-pelvic adhe- sions, defective nutrition, obstinate cervical catarrh, constipation, occa- sional dysuria ; a delicate blonde, and a sufferer for years from sacralgia and pelvic pain. She would have attacks of spasmodic action of the abdominal muscles, simulating tremors; always excited by manipula- tion. Her sole subject of conversation was her peculiar symptoms. She slept fairly well, and without cause would suddenly refuse to speak or answer questions. She would do as she was told, but always passively. Would not dress or undress herself, but offered no resistance to the atten- tion of her nurse. These attacks would last three or four days, when she would talk freely. During these attacks there were no delusions. When asked why she refused to speak, she replied that she was tired of talking, as it did no good, and she would never get well.
Mrs. B., aged fifty-four years ; four adult children ; a German by birth; sub-involution and great hypertrophy of vaginal portion ; double lacera- tion; masses of scar tissue; large retention cysts, giving the cervix a nodular appearance ; climacteric at fifty years. Was admitted to the hospital as a case of cancer of the cervix. Operation under ether. The second day became very restless, and during the night got out of bed several times and insisted upon going home. Before the third day she became violent in response to the gentle force necessary to keep her in bed. Her one delusion was that she was deserted by her husband, and she must leave in search of him. Bringing her husband and chil- dren into the room in no way changed her delusion or caused her to desist from her violent efforts to leave the hospital. The active mania subsided into a melancholic form, which cleared away in about ten days, and the mind became normal. The repair operation was a failure, and was repeated in a few months without an anesthetic and without mental disturbance, and with good results. A feature of the mental outbreak greatly resembled the efforts to resist that a patient will make in initial anesthesia with the form of delusions that follows the narcosis.
Mrs. V., aged thirty-eight years ; three children—eldest twelve years, youngest four years. Uterus retroverted, enlarged ; double laceration of neck ; perineum torn to the sphincter. Admitted to the hospital for a
vo. 104, No 5 —NOVEMBER, 1892. 36
550 VAN DE WARKER, HYSTERICAL MANIA.
repair operation. She attended to her domestic duties until about a year previous to admission, when she became unfitted for social life by her profound melancholy. She was free from delusions ; her naturally affec- tionate and demonstrative nature remained unchanged. Nearly every waking hour was passed in tears. She could not meet a mere acquaint- ance without bursting into tears. A caress from one of her children or a pleasant word from her husband was always a signal for an outbreak. Naturally of a studious habit, she lost all interest in reading, and would talk or think of nothing but her illness and its hopeless nature. She was regarded as a melancholic and narrowly escaped an asylum. After her operation the restoration to a normal mental tone was so gradual and slow that we were fearful that she never would be restored. In about a year, however, the mind was acting in nearly a natural manner, and she sees to take her old place in society and the home.
The practical question is, What shall we do with these cases? If we class them among the insane they cannot be admitted legally into any institution, public or private, other than an asylum, unless a man is en- gaged who has had a certain number of years’ experience in the so- called medical treatment of the insane. This is the law in the State of New York. No institution can admit them that is not provided with this lay figure. Only a few months past I tested the matter, and found that the law made it illegal to admit a patient known to have any mental defect. Many of these cases are the outcome of chronic salpin- gitis and ovaritis, and a laparotomy would safely and quickly cure them; but even for the purposes of operation you are not entitled to the legal custody of these patients. These cases are received into gyne- cological sanitariums and hospitals, but it is done in violation of the law. It is evident to all right-thinking people that a law ought not to be upon the statute-books the violation of which is compelled by the higher law of common humanity. When cases of this class take on the periodical form of mental disturbance they are, among the laboring classes and people of indigence, sent to asylums, and, as I have already stated, rarely restored to sanity. The law ought to be so modified that cases of this character that require the attention of gynecologists should first be given all needful attention. Insanity is already becoming such an important matter in our present social development that any method that would serve but ever so little to lessen the vast aggregate of the insane ought to receive prompt attention. Reform, as the past fully proves, will never come from the ranks of the asylum managers, but must originate in the indignant protests of the medical profession.
ha e
DUHRING, MORPH@A WITH MACUL ATROPHICA. 551
MORPHA WITH MACULZ ATROPHICZ:.'
By Lovis A. DuHRING, M.D., PROFESSOR OF SKIN DISEASES IN THE UNIVERSITY OF PENNSYLVANIA.
THE patient is an Englishwoman, fifty-five years of age, a brunette, spare, but in good general health. There is no apparent cause for the disease. It first manifested itself a year and a half ago, and has been gradually spreading. The regions invaded are the nape of the neck, and the adjoining scalp to a slight extent ; the chest just below the left clavicle; the wrists and forearms. Several stages of the process exist, and the lesions are so different as to require separate description. Three distinct kinds are noted :
1st. Whitish patches of skin with manifest structural change in the true skin, of the nature of a peculiar fatty degeneration of this structure, constituting the commonest form of the disease. 2d. Distinctly circum- scribed, depressed, and cicatriform whitish spots, varying in size from a small to a large pea, which plainly exhibit wasting and thinning of the true skin, the lesions resembling scars from syphilis, being thin, soft, and pliable. 3d. Patches of mottled, brownish-red, pigmented, struc- turally altered, atrophic skin, with a broken border or margin of firm, variously sized, irregularly shaped, papular elevations. Over these patches here and there are distinctly marked bluish-purple veins running in various directions. The skin in the central portion is thinned, and the border, as stated, is thickened, but nowhere is the skin bound down to the subcutaneous tissue, the whole patch being freely movable over the tendons and fascie. 4th. Enlarged, bluish-purple veins, identical with those on the patches of the wrists, which run up the forearm and are not associated with the other forms of disease de- scribed, although they are in the neighborhood of some atrophic macules. Having thus outlined the chief features which characterize the affection as a whole, the individual lesions and their distribution upon the several regions involved may be referred to more definitely.
On the back of the neck at the line of the hair, partly on the scalp and partly on the non-hairy portion of the neck, there exists an irregu- larly shaped, sharply defined, whitish patch of skin, the normal struc- ture being changed into a whitish, lardaceous, non-indurated, soft, pliable, freely movable patch. The sense of touch with the fingers does not determine any increased thickness or structural change, so that in
icking up the skin with closed eyes one would hardly detect disease.
his patch is about two inches in diameter and is not surrounded by any border or hyperemic zone or by injected veins. The hair of the scalp growing from the patch is of natural color, blackish, and not whitish as might be expected. This lesion represents one of the varieties of morpheea, and according to my experience the earliest and mildest phase of the disease. ;
Near by this patch, on the back of the neck, are several pea-sized, rounded, sharply defined, slightly depressed, shallow, whitish or pearl colored atrophic macules with thin skin which at first glance resemble scars from the large pustular syphiloderm or from burns resulting from
1 Read before the College of Physicians, February 3, 1892.
552 DUHRING, MORPHGA WITH MACULZ ATROPHICE.
the application of a hot iron. They are disseminated upon the neck on either side of the median line, and show no special distribution or arrangement. They are in no way different from the typical macule atrophice which are met with occasionally upon various regions, and usually without other forms of cutaneous disease.
Upon the flexor surfaces of the wrists are two symmetrical, rounded, atrophic patches, the size of a silver half-dollar, defined in outline, with a raised, indurated, irregular, uneven, papular border, and pigmented, of a mottled, brownish-red hue. The central portions of these patches are somewhat wasted and depressed, the skin being thinned, but soft and supple. Some enlarged and purplish veins run irregularly over the surface. On the flexor surface of one forearm, running up toward the elbow, there exist several pea-sized, whitish, atrophic macules, identical in character with those on the back of the neck. They incline some- what to take on a linear arrangement rather than to be widely dis- seminated.
The lesions which have been described constitute the whole disease. The several varieties have no association with one another, but they are plainly due to the same cause. They are not stages of one process, but are distinct forms of cutaneous change, beginning and running their course as such. The patient complains of no pain or serious incunven- ience from the disease, and seeks medical advice because of the disfigure- ment and of the tendency of the process to waste and atrophy.
The case represents an unusual phase of the somewhat rare disease morphea. Many years ago I pointed out that this affection was charac- terized not only by the so-called “ patch,” but moreover in some cases by a variety of lesions, which might occur either singly or in combination. In the third edition of my Treatise on Skin Diseases (published some years ago) attention was directed to the observation that atrophic macules sometimes were present with the characteristic lardaceous patch. The occurrence of these two forms of lesion together, however, as in the case before us, I regard as rare, one or the other variety of atrophy alone usually existing.
Morphea must be classified with the atrophies, and not with the hypertrophies, as it has been by some prominent authors. The process is distinctly atrophic in all its essential features, especially in its course and termination, which are characterized by degeneration of the skin and subcutaneous tissue with usually more or less thinning, shrinking, and wasting or degenerative atrophy. The present case illustrates the close relationship pathologically of macule et strize atrophice with the common plaque of morpheea, as this latter was originally described by Addison and E. Wilson. At that date the affection seemed to have been scarcely known in Germany, and moreover even now it appears to be rarer there than in England or in this country. Many years ago, during a long sojourn in Vienna with daily attendance upon the clinics for skin diseases, I do not recall having observed @ case.
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ELLIOT, DERMATITIS HERPETIFORMIS. 553
Concerning the diagnosis, no difficulty can exist, it seems to me, if we are in the habit of studying cutaneous disease from the standpoint of anatomy and pathology, and more particularly the latter. This classi- fication of skin diseases, upon the basis of general pathology, is not only the most scientific, but what is of more importance, is also the most practical and useful for our daily dealings with these diseases. The affection before us belongs manifestly to the atrophies, the process at work being essentially degenerative and atrophic in its phases. This point established in our minds, there remains merely to find a place for it in this class, and it plainly must be grouped with atrophies of the true skin. Such forms of atrophy are comparatively rare, the true skin not being prone to take on atrophy as a primary process. The several affections of this kind which may be classed together are atrophy of the skin proper (atrophia cutis propria); macule et striz atrophice ; morphea; and some forms of scleroderma, the two latter affections sometimes coexisting.
The treatment of these cases is generally unsatisfactory, the prognosis, however, depending a good deal on the variety of the disease present and on the stage of the process. In some cases arsenic internally is useful, but in the patient before us local inunctions with stimulating ointments and oils, with massage, electricity, and frictions will probably prove more beneficial.
THREE CASES OF DERMATITIS HERPETIFORMIS ORIGI- NATING FROM CAUSES CONNECTED WITH THE UTERINE ORGANS.
By GEorGE T. Ex.iot, M.D., DERMATOLOGIST TO THE NEW YORK INFANT ASYLUM, THE DEMILT DISPENSARY, AND ASSISTANT VISITING PHYSICIAN TO THE NEW YORK SKIN AND CANCER HOSPITAL, ETC.
THE admirable work done by Dr. Duhring and supplemented by Brocq has so familiarized the symptomatology of dermatitis herpeti- formis that the diagnosis of the cases of the disease presenting them- selves has become a matter of comparative ease. The same degree of progress has not, however, been made in our knowledge of the etiology and pathology of the dermatosis, nor does there exist unanimity of opinion in regard to its nature; so that the careful recording of all cases furnishing details bearing upon these points is of the greatest importance, in order that, in time, a critical and comparative analysis, and perhaps the formulation of conclusions satisfactory to all, may be made in regard to the pathogeny and nature of the process. For this reason I would report the following cases, which present many features of interest and importance.
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554 ELLIOT, DERMATITIS HERPETIFORMIS.
Case I. (Demilt Dispensary.)—Female, aged thirty-five years, intelli- gent and bright; consulted me July 19, 1890, giving the following details in regard to herself and the cutaneous disease from which she was suffering. Her general health had always been good, but at the age of twenty-five she had had an attack of gastric dyspepsia, and off and on since then there had been occasional returns of the same trouble. Married at twenty-four, she has had three children—an interval of five years occurring between the first and second, and of four years between the second and third. The skin disease had begun in the third month of this last pregnancy, appearing first upon the feet, and then rapidly becoming universal, under the form of intensely pruritic “hives,” as large as a fifty-cent piece, which appeared in successive crops. When about five months pregnant, her husband died and she experienced intense worry and anxiety, as she was thus obliged to provide for both herself and her children. The eruption immediately became aggravated, fresh crops of lesions of various types appearing, the pruri- tus and subjective discomfort becoming almost unbearable, and all the distressing circumstances surrounding her culminated in a miscarriage at the seventh month. Temporary amelioration of the cutaneous pro- cess ensued, but only for a short time, for in a few weeks a severe relapse occurred, and the course of the disease ever since has been one of alternate outbreaks and periods of comparative ease and quiescence. These latter, however, would always be shortened by mental or moral irritation of any kind, by anger, or by increased worry and anxiety —a fresh outbreak occurring or an aggravation of existing symptons ensuing. After her miscarriage she became a wet-nurse, and pursued that occupation until about May 1, 1890. For several months pre- viously she had been much worried and very despondent, because her earnings had not been sufficient to support her children, and in April, 1890, the severest attack of the disease began to appear. Prior to this outbreak, erythematous patches and groups of papules and vesicles alone had appeared, but in this relapse, and for the first time, bull also occurred more or less generally over the body. The disease had now existed nearly two years, but her functional health had been very good, except that there were occasional attacks of dyspepsia.
When the patient was seen by me, this last outbreak had subsided to a considerable extent. There were still, however, numerous lesions over the body, especially on the extremities, and the pruritus was still intense in character. On the surface, papules and small vesicles arranged in
roups of variable extent were seen, while here and there were irregu- arly-shayed, tense and flaccid bullz. Numerous crusts and scratch marks, and diffuse and small circumscribed pigmented areas, the site of former lesions, were also present.
While the patient was under observation, a marked feature observed both before and during an outbreak was excessive general hyperidrosis, but especially of the hands, feet, and axille. A number of relapses also occurred, and they varied in intensity and extent as well as in the type of the lesions—papules alone, or papules and vesicles, or both and bullz. The pruritus and burning sensation was at times only slight, but then again would attain such a pitch as to bring on a severe hyster- ical attack, and after this an outbreak would regularly follow, consist- ing of groups of vesicles and papules or erythematous patches. While she was under my care, the patient’s material circumstances did not
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ELLIOT, DERMATITIS HERPETIFORMIS. 555
improve; her worries and anxieties persisted; she was despondent and melancholic, given to brooding, and she obtained benefit from no form of treatment used. Undoubtedly these mental and moral disturbances nullified all attempts at improving her condition, for, as they had previously done, they continued to call into existence new outbreaks -* the process. The patient was seen by me continuously until in Sep- tember, 1890, she ceased her visits. She was practically then in the same condition as at first, and the disease followed the same course as during the previous two years. Recently, however (March, 1892), the woman returned most unexpectedly to tell me how she was. She stated that from the time I had last seen her she had not followed any treat- ment, but in December, 1890, considerable amelioration of her material circumstances had come about; her two children were taken care of by her husband’s relatives, and she was thereby relieved from her constant worrying and anxiety on their account. She also obtained a better situation and earned enough money to support herself well, to obtain proper food, and to enjoy life. As a result, there was a diminution in the intensity of the process, relapses became infrequent, and about April, 1891, entire cessation of all the objective and subjective manifes- tations of the disease occurred. Since that time she has remained abso- lutely well, and, in fact, when I saw her she was in perfect health; the cutaneous surface was without a trace of the former trouble beyond slightly pigmented areas here and there.
From the clinical history of this case it can be seen that the primary lesions were erythematous patches of various sizes—in fact, the patient pointed out similar ones at the time I first sew her—which remained in association, or were quickly replaced by papules and vesicles appear- ing in successive crops, all being accompanied from the beginning by the most intense pruritus and burning pain. When these features, the clinical course of the process, and the condition—pregnancy—under which it arose are taken into consideration, the dermatosis agrees so closely with the herpes gestationis of authors that there can be no question but that it primarily represented an example of that cutaneous affection, though in its subsequent history it differed in some particulars from other recorded instances of the same disease. It is thus the first and only case which from its very inception ran a chronic course, per- sisting with undiminished severity for an indefinite period of time after pregnancy had terminated, while all the other cases in literature under- went a temporary aggravation after delivery, to gradually disappear at the end of a few weeks, and a chronic course became instituted only after a series of suecessive attacks developing during successive preg- nancies.
It does not, however, appear to me difficult to explain this deviation in type. The reason for it, I would find in the neuropathic condition of the patient, induced by the severe mental and moral emotions she experienced in the fifth month of pregnancy, by the death of her hus- band, by the worries and anxieties entailed upon her by the inadequate
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556 ELLIOT, DERMATITIS HERPETIFORMIS.
support furnished her children by her own work, and by the miscarriage in the seventh month, a condition which was not terminated by the emp- tying of the uterus, but one which persisted not only unchanged, but even in an increasing degree for very many months after. It is to the existence of this neuropathic state and the constant repetition of the mental and moral emotions, the anxieties, etc., that I would, therefore, ascribe the persistence of the process and the unceasing occurrence of relapses after the termination of the pregnancy. In other words, the primary cause—the pregnant uterus—being removed by the miscarriage, the cutaneous process in all probability would, as in the other recorded cases, have disappeared, but the mental troubles then coming into play as secondary exciting causes, the disturbed conditions of which the skin affection was the expression were kept up, and the persistence and chronic course of the dermatosis was the result. The importance of these circumstances in connection with the process was not judged from the patient’s assertion and observation alone, but I myself noted so repeatedly and regularly the occurrence of relapses and an aggravation of the cutaneous symptoms after any increased worry, anxiety, or mental emotion, that no other conclusion could be reached but that a most intimate connection existed between the dermatosis and disturb- ances of the nervous system, brought about through the channels of the mental and moral faculties. In consequence, it appears to me per- fectly logical to conclude that if these factors operated at one time in the manner mentioned, they must have had the same influence at other times also, and, therefore, it would be perfectly justifiable to ascribe to their occurrence the persistence of the process after the termination of the original inducing cause.
The herpes gestationis of authors has been claimed by Dr. Duhring not to be an independent disease by itself, but to be merely a phase of his dermatitis herpetiformis—a claim, moreover, recognized and allowed by Brocgq in his analysis of the whole subject ; and, judging by this case, I would certainly accept the opinions of both of these dermatologists. I would do so for the reasons that, when the patient came under my care and while she remained under my observation, the case in its entire symptomatology and behavior was absolutely undistinguishable from any case of dermatitis herpetiformis the outcome of any other cause than pregnancy, and furthermore, its entire clinical history from the very first presented those marked features attached to that form of cutaneous disease, as shown by its chronicity and frequent relapsing, its multi- formity of lesions—erythematous patches, vesicles, papules, bulle ; its intense subjective symptoms—pruritus and burning pain, and its marked rebelliousness to treatment. Under these circumstances, it would be difficult to include the case in any other category than the one mentioned, even though its primary inducing cause was pregnancy
ELLIOT, DERMATITIS HERPETIFORMIS. 557
and not some other factor. My own cases of dermatitis herpetiformis —eighteen in number—have, however, shown me that there is not needed for the production of the process any one single or specific cause, but that in a person possessing a certain degree of predisposition almost any exciting factor may so disturb the equilibrium in the nervous sys- tem that the dermatosis develops. In fact, such diverse exciting causes as mental shock and emotion, malarial fever, the menopause, etc., have been in my cases the determining influence in the production of the process, and if these could act in that manner, there can be no objection whatever to regarding pregnancy in the same light. A further corrob- oration of the view that influences originating in the generative tract— of which pregnancy would be one—can be productive of the dermatitis herpetiformis of Duhring is furnished, moreover, by the following two cases of the disease, which have been under my care, and in both of which the menopause was the exciting factor.
Case II. (Private practice).—Female, aged fifty-three years; seen by me in August, 1891. She stated that she had always enjoyed good health, although of nervous temperament, anemic, and usually a little below par. Her menstruation had always been regular, but she had had ulceration of the os uteri for several years after the birth of her second and last child, twenty-nine years ago, and also ever since a most profuse leucorrheea and a lacerated cervix. She was accustomed to take
daily cold baths during menstruation, but not at other times. The climac- teric began in April, 1889, the appearance of the menses becoming irregular, the flow scanty and finally ceasing, not to return, in July, 1890.
The cutaneous process first appeared in May, 1890, and since that time she has not been free from its manifestations, with the exception of two months, when she had an attack of erysipelas of the face and head. The disease was not so severe while the periods still came, but it became very much aggravated as soon as they had entirely stopped. The first lesions developed at the bend of each elbow as erythematous patches, which became covered with vesicles; then similar phenomena over the knees, then upon the back, and finally cropping out generally over the entire surface. Both halves of the body have always been affected, but the right half invariably more severely than the left. All these manifestations have been accompanied by the most intense pruritus and burning sensation, increasing at night and when in any way she became heated or excited. Since the primary outbreak the patient has expe- rienced relapse after relapse, separated by longer or shorter intervals of comparative ease—papules, vesicles, bulle, and more lately erythe- matous patches appearing in successive crops or more or less mixed to- gether. Her general health has remained about the same, that is, she was not robust but could not complain of any particular illness. When she came to me, constipation was present, and she stated that for some time she had passed urine very frequently and abundantly, and had occasionally noticed a “ brick-dust” sediment. The urine on examina- tion showed the presence of urates, uric acid, and oxalate of lime crystals, but no albumin, sugar, or renal epithelium or casts.
The cutaneous efflorescences were distributed generally over the entire
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558 ELLIOT, DERMATITIS HERPETIFORMIS.
surface of the body, the mucous membranes of the mouth and vagina being entirely free, and having always been so. They consisted of groups of papules or papules and vesicles, while here and there were single pea-sized bulle, or a group of several of these, and widely dis- tributed were discrete and grouped small and large areas of pigmenta- tion, the sites of former lesions and groups of lesions. The vesicles were for the most part flattened, angular, and irregular in shape, quite resistant and not rupturing easily, and the bulle presented very much the same characteristics, though on a larger scale. The pruritus and burning pain were complained of intensely, as they deprived the patient of sleep and rest. Since the case has been under observation there have been periods of improvement and others of aggravation ; crops of lesions having the characteristics mentioned above have succeeded each other at irregular intervals, the patient at times covered with the eruption, at others presenting only a few groups of vesicles or papules and bull; but ae the agonizing subjective sensations have persisted without change or abatement, and only temporary relief has been obtained from treat- ment.
In January, 1892, she experienced a most intense relapse, which de- veloped — after taking “laughing gas” for the extraction of some teeth. More lately (March, 1892), another severe outbreak mani- fested itself, after grief and the natural emotion caused by the death of her mother. On the whole, it may be said that there has not been any material change in the patient’s cutaneous disease since its inception.
Case III. (New York Skin and Cancer Hospital; Dr. Bulkley’s service.)—Female, forty-eight years of age; first seen by me in Janu- ary, 1889. She had been married twenty years, but had never become pregnant. She had always been of neurotic temperament, easily frightened, subject to attacks of melancholia, and a sufferer from severe neuralgias—facial and occipital—for twenty years. The inception of the menopause was dated by her in April, 1886, and her last period had occurred in May, 1887. The earliest cutaneous symptom noticed by the patient was a most severe burning and itching of the entire skin, which came on in the first part of June, 1887, and which at the end of from two to three weeks was followed by the appearance of bullous elevations as large as a 25-cent piece, first on the legs and then on the arms. While these were still present, outbreaks of papules and vesicles began coming out and have kept appearing in rapidly-succeeding crops, but no bulle have reappeared. hen the case was seen by me the process had been in existence about eighteen or nineteen months, and it had become universal. There were groups of papules and of vesicles of variable size, erythematous patches and pigmented areas distributed without arrangement over the entire cutaneous surface, which, in addi- tion, was thickened and infiltrated and scratched in every direction. The burning pain and pruritus were intense and paroxysmal in charac- ter, and during the paroxysms the patient suffered from excessive thirst and consumed large quantities of water.
While the patient remained under observation the dermatosis pursued the same course as it had hitherto, that is, successive crops of vesicles, papules, or areas of erythema would appear, preceded or accompanied, by the same severe subjective symptoms. No abatement or relief’ seemed to be obtained from treatment, and when she was last seen the process was in every particular in statu quo ante.
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We thus find in these two cases the same major clinical characteristics as were observed in the one which primarily developed under the influ- ence of pregnancy, and, in fact, in their entirety they agreed accurately with the latter, though differing from it in their initial moment of causation. I do not think that there can be any doubt but that Cases II. and ITI. were examples of dermatitis herpetiformis of Duhring, inas- much as clinically their mode of behavior, their objective and subjective symptomatology were precisely such as pertain to that of dermatosis ; and this being the case, it cannot but be evident that the process can and does originate under the influence of conditions, other than pregnancy, existing or arising in the generative organs.
It would appear to me justifiable to ascribe to the climacteric the role of being the exciting cause of the dermatosis in these two cases, for the reason that, notwithstanding the existence of a neuropathic condition in the patients, of a lowered nervous and general systemic tone for a long period of time, yet they were able to-withstand all the rioxious influences surrounding them and in operation, and they succumbed only after they had been subjected to the additional severe strain produced by the manifold changes occurring in connection with the menopause. The clinical history given by these cases would lead me, therefore, to regard all these factors existing for years prior to the cli- macteric as instituting a predisposed condition of the general system, while the menopause itself acted similarly to a nervous shock or mental or moral emotion—that is, as the determining cause which produced the final changes necessary for the development of the disease. In what manner the pregnant uterus or the climacteric act in calling the dermatosis into existence, it is difficult to state, except speculatively. In view, however, of the manifold neurotic disturbances which arise under the influence of these conditions, there are strong presumptive reasons for believing that the action of these exciting causes is through the nerv- ous system, their effects being manifested in a reflex manner, and the cutaneous symptoms being simply an objective expression of a neuropathic condition induced by the disturbances having the uterine and generative organs for their seat. It would certainly appear as though the nature of the influences under which the process in Case I. continued after the miscarriage, demonstrated that the dermatosis was the outcome ‘of disturbed conditions induced in the nervous system ; besides this, the clinical history—to judge from the constant aggravation and relapsing of the dermatosis upon the repetition and recurrence of the same influences—pointed out that a most intimate connection existed between the objective and subjective symptoms and any and all dis- turbances taking place in the nervous system; or, in other words, the cutaneous manifestations were purely the objective expression of the neuropathic condition. No better proof of this view can be desired than
560 ELLIOT, DERMATITIS HERPETIFORMIS.
was furnished by this patient’s case, since the disease persisted without change as long as those influences operating upon the nervous system lasted, but disappeared after their removal and cessation, the woman regaining her health and remaining free from the dermatosis. A stronger proof, I repeat, of the neurotic nature of the process than this could not, in my opinion, be obtained ; and it is one which I have observed in a number of my cases, in which similar conditions were in action, and in which removal of these latter was likewise followed by entire relief from the process; when they recurred, so did a more or less extensive relapse become manifested. In Cases II. and III. similar observations were made as in Case I., though in a minor degree; that is, independent of the climacteric, influences in the former similar in nature to those noted in the latter excited likewise the same effects, and were followed by relapses, etc. In these two cases, the material conditions of the patients were better than in Case I., so that the repetition of the neurotic disturbances was not so frequent nor so intense in character, but, nevertheless, their occurrence would be invariably signalized by outbreaks and aggravation of the cutaneous symptoms.
When these facts are repeatedly observed, and not alone in these three cases, but also in many others, and they are taken in conjunction with the neuropathic etiology furnished by the majority of the patients, with the intensely marked evidences of disturbed sensory innervation— pruritus, burning pain, neuralgias—invariably present, it certainly seems to me that there is every reason and ground enough to conclude that the process is a dermato-neurosis, the outcome of disturbances produced in the nervous system by one cause or another. In another article upon this subject I have already committed myself to that belief, and cer- tainly the more cases of the disease that I see and the more I study the dermatosis, the more convincingly do the array of facts presented by them strengthen and corroborate the opinion expressed.
In regard to the treatment of dermatitis herpetiformis, my experience has certainly shown me that there are no drugs which exert any influ- ence upon its manifestations. In view of the conditions under which the cases originate and their behavior after development, this failure is what would be expected ; so that, beyond remedying whatever functional or other disturbances of health which may be present or arise in the course of the disease, I can see no result to be obtained from the adminis- tration of medicines. Of course, if the etiological elements of causation can be reached by drugs, then certainly those indicated should be exhibited; but, unfortunately, such cases have not come under my observation. On the contrary, the only good results which have been obtained by me have been in such cases as permitted the removal and avoidance of all influences, surroundings, or circumstances which acted in any way detrimentally upon the nervous system, or caused a disturb-
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561
ELLIOT, DERMATITIS HERPETIFORMIS.
ance of whatever nature in it. In consequence, the treatment should, in my opinion, rest upon the basis of the case’s etiology, and be directed with a view to guarding the patient from every emotion, shock, or occurrence which might act in the manner mentioned. If this cannot be done, I would not expect the dermatosis to be helped, but to con- tinue indefinitely, relapsing and recurring as often as the patient was exposed to one or another of such influences, though it is perfectly possible that in some instances the process may terminate of its own accord, the individual ceasing to react to the causes mentioned as pri- marily being so actively determinative of the eruption. As evidence I would advance, besides the two cases previously reported by me,’ Case I. in this paper and Cases I. and IV. in another article already published.’ In these, the clinical histories show that the patients got well when the influences surrounding them were removed or guarded against, but not before, notwithstanding that medication of all kinds was made use of. On the other hand, Cases II. and III. in this paper, and two others in the one just referred to, have persisted and continued irrespective of treatment, and in them it was not possible to remove or prevent the repetition of the various factors and other cir- cumstances productive of the relapses. It seems to me, therefore, that the disease has to be dealt with upon the broad therapeutical basis mentioned, and its treatment not limited to the exhibition of one drug after another; rather let entire attention be given to the removal of all the etiological exciting and determining factors appearing to participate in the production and persistence of the process.
Locally, a certain amount of benefit can be obtained from treatment, in so far that relief from the distressing and oftentimes agonizing sub- jective symptoms can be given. ‘The eruption is also by this means to a certain extent diminished in severity, by lessening the scratching and wounding of the skin by the patient—thereby increasing the inflamma- tory changes. Ichthyol has given me by far the best results of all sub- stances used or tried, and patients have invariably obtained so much relief from its application that they have strongly objected to any change being made. I have not, however, observed that it had any influence in preventing new outbreaks or had any other action but that of giving relief to the intense suffering caused by the pruritus and burning.
1 Journal of Cutaneous and Genito-Urinary Diseases, September, 1891. 2 Some Cases of the Dermatitis Herpetiformis of Duhring, etc.,” New York Medical Journal, May 28, 1892.
562 POWERS, AMPUTATION IN SENILE GANGRENE.
THE QUESTION OF EARLY HIGH AMPUTATION IN SENILE GANGRENE.
By CHARLEs A. Powers, M.D., SURGEON TO THE CANCER HOSPITAL, NEW YORK.
I am able to adduce but a single case in illustration of the topic which I present for the consideration of my readers, and that case a fatal one. It serves, however, to emphasize the one point to which I invite your attention, namely, that in cases of senile gangrene involving the lower extremity, early recourse should be had to ampu- tation through the thigh when the process has extended from the toes to the foot.
Mr. D., a heavily built man, aged sixty-seven years, was referred to me on Sept. 15,1891. He had suffered with syphilis in early manhood, but had had no later manifestations. He had used alcoholics to a moderate degree. Four months before seen he had been troubled with slight itching on the dorsum of the right foot. Some months previously this had given place to dull, undefined pains in the foot and leg, slight at first, but later becoming so severe as to prevent locomotion. Areas of diminished and lost sensation, had in turn appeared, and when comin under observation the entire foot was without surface sensibility, livid cold, its dorsum covered with occasional blebs. The leg, as far up as its middle, showed scattered areas of insensibility and patches of a lightish-purple mottling, there being, however, no distinct line of demarcation. No evidence of recent or old injury to the toes or foot could be found.
There was no pulsation to be felt in either of the tibials, in the pop- liteal, or in the femoral at Hunter’s canal. The latter vessel pulsated beneath Poupart’s ligament. The heart-sounds were weak, but other- wise normal. The urine was negative upon examination.
The continuous pain had tended to weaken the patient’s general strength ; he had developed a slight daily fever.
Dr. William T. Bull kindly advised, commending immediate opera- tion. Accordingly, thirty-six hours after I first saw him, amputation was made under chloroform through the middle of the thigh by antero- posterior flaps, care being taken that these should not contain an undue amount of tissue. The femoral artery was thickened, calcareous, occluded by a firm clot. It was secured with heavy catgut and did not pulsate ave removal of the bandage which had been lightly placed about the thigh at the upper third. Occlusion did not, however, extend to the other vessels in the stump, all of which seemed competent.
Bleeding was checked in the usual manner and the stump closed, drained, and dressed in the customary way.
The operation was followed by no shock. Twenty-four hours after- ward, however, respiration became labored. Physical examination re- vealed hypostatic pneumonia over both lungs below and behind, and the patient succumbed to this at the end of the third day.
POWERS, AMPUTATION IN SENILE GANGRENE. 563
An autopsy was not permitted, yet I was allowed to examine the stump and found it the seat of complete primary union.
The flaps were approximated throughout, there were no areas of mal- nutrition, and there was no pus.
Proposition to resort to early amputation through the thigh in cases of senile gangrene due to arterio-sclerosis was first seriously made by Jonathan Hutchinson,’ who presented, in 1883, an exceedingly clear and forcible argument before the Medico-Chirurgical Society of London,’ calling attention to the fact that amputation in obstructive gangrene due to arterio-sclerosis has been largely discountenanced because followed by sloughing of the stump, and urging that this only takes place when the part is removed too near to the disease.
When amputation is done at a low point the condition of the vessels will rarely be found to be such as to admit of repair; gangrene of the stump usually occurs immediately and places the patient’s life in much more danger than before operation.
By the “ high amputation” which he urges in these cases, he means that in the case of gangrene of the foot the amputation should be made above the knee, and in that of the hand, at or near the shoulder-joint.
In gangrene due to arterial calcification the interference with the blood-supply is usually greatest in the distal part of the arterial system, and is of such nature as to be steadily on the increase. Hence the hope- lessness of improvement and the great danger of advance. Hutchinson adduces a number of cases in which he successfully amputated through the lower third of the thigh for gangrene of the foot, and avers that the procedure is not attended with much danger, even in advanced years and with most extensive calcareous degeneration of the arteries. -He has never seen secondary hemorrhage in such cases, nor has he encoun- tered difficulty in securing the vessels at the time of operation.
In this connection I beg to draw your attention to a recent and most instructive paper by Heidenhain,* who, in September, 1891, published the cases of senile gangrene of the lower extremity which he had seen in the clinic of Kiister at the Augusta Hospital in Berlin.
He makes prefatory reference to a paper by Israel,‘ in which the latter arranges the conditions under which spontaneous gangrene occurs under three heads: 1. Imperfect access of normal blood to the affected parts ; 2. Perfect access of abnormal blood; and 3. Imperfect access of ab- normal blood. In the first category belong the cases of senile gangrene caused through arterio-sclerosis or obliterating endarteritis; in the second, those of gangrene of the tip of the nose, lips, toes, etc., after
1 Mr. Hutchinson accredits earlier commendation of the measure to James. 2 Medico-Chirurgical Transactions, 1884, vol. Ixvii. p. 91.
3 Deutsche medicinische Wochenschrift, 1891, p. 1087.
* Berliner klinische Wochenschrift, 1882, p. 705.
564 POWERS, AMPUTATION IN SENILE GANGRENE.
acute infectious diseases; while in the third class one must number a great part of the cases of diabetic gangrene, inasmuch as Israel found arterio-sclerosis in no less than 13 out of 20 diabetic patients who con- sulted him.
Heidenhain includes, then, cases of gangrene in diabetics as well as those in which the urine was free from sugar, citing 25 in all—11 with diabetes and 14 with simple arterio-sclerosis.
Thirty primary amputations were carried out on these 25 patients, three of whom demanded double amputation, and one triple. Besides these 30 primary amputations there were 10 secondary operations, the latter made necessary by gangrene of the stump following a primary amputation. Heidenhain says: “ Kijister had at first contented himself with the simpler form of interference, or low amputation. The con- stant occurrence of gangrene in the amputation wound, however, in these cases, regularly compelled further high amputation. So that he (Kiister) is led, through his practical experience, to amputate at or above the knee in every case in which the gangrene has extended from the toes to the dorsum or sole of the foot.”
Analyzing these cases we find that four times, in circumscribed gan- grene of a toe, Kiister disarticulated, but that in every instance gangrene of the flaps occurred and extended to the foot. Lisfranc’s amputation was secondarily carried out on one of these patients; he developed further gangrene and died of sepsis. A second was further amputated at the knee and again higher, the latter operation accomplishing cure. Both of the others were healed only after amputation through the femur. In three cases of primary amputation through Chopart’s joint, the. gan- grene progressed in two and required femoral amputation. Primary amputation through the leg was employed six times: one case died from gangrene of the flaps and sepsis; three were saved by amputation through the thigh’; the other two were healed only after gangrene of the edges of the flaps and necrosis of the sawn surface of the bones. Of these 13 low primary amputations, only 2 went on to healing, these in leg amputations; 2 patients died of gangrene of the flaps and sepsis; the remainder, 9, were saved by secondary amputation at or above the knee.
Of the 17 primary amputations through or above the knee-joint, 9 were cured, while 8 died of diabetic coma or heart weakness. Of the 10 secondary amputations, all recovered. Separating, now, the diabetic from the non-diabetic cases, we fiud that of the 11 diabetic patients 6 were cured, while 5 died ;' and of the 14 patients with simple
1 It is worthy of note that three of these five fatal cases were in patients whose urine was both saccharine and albuminous. Of four patients whose urine contained albumin in addition to sugar, only one recovered, and he was a man in whom the amount of sugar, as well as of albumin, was small.
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COPLIN, BEVAN, UNUSUAL CASE OF TUBERCULOSIS. 565
senile gangrene, 9 were cured, 5 dying. The fatal result was due to gangrene of the flaps and sepsis in two cases (one Lisfranc’s amputation, one through the leg, both in the early days of antisepsis) ; in one case a man, aged eighty years, died at the end of nine days, from heart failure ; one man, aged seventy-eight years, succumbed to hypostatic pneumonia ; and another, aged fifty-two years, died at the end of nine days, with myocarditis, nephritis, and ascites. The list shows that through the high amputation all patients were saved who were not severely afflieted with some general disease.
Careful study of the cases of Hutchinson and Kiister, together with the observation of others in which disaster has followed low amputation, serves to convince me of the wisdom of the course indicated, and in so far as we may be guided by present knowledge, I think that we may accept as authentic this statement of Heidenhain’s :
“So long as the gangrene be confined to one or two toes, one may wait and abstain from other than general antiseptic treatment, with high position of the limb, allowing the part to be spontaneously thrown off. If the process extends, however, to the dorsum or sole of the foot, one should amputate above the condyles of the femur.
“ Amputation below the knee is almost always followed by gangrene of the flaps, and brings the patient in danger. High amputation is indicated, then, when the gangrene progresses, even though the patient be without fever.”
AN UNUSUAL CASE OF TUBERCULOSIS. By W. M. L. Copuin, M.D.,
ADJUNCT PROFESSOR OF HYGIENE AND DEMONSTRATOR OF PATHOLOGY, JEFFERSON MEDICAL COLLEGE, ADJUNCT PROFESSOR IN THE PHILADELPHIA POLYCLINIC, A. A. SURGEON MARINE HOSPITAL SERVICE,
AND D. BEvAN, M.D.,
INSTRUCTOR IN HYGIENE AND CLINICAL MICROSCOPY IN JEFFERSON MEDICAL COLLEGE.
E. H., Concord Street, Philadelphia, male, aged twenty-nine years. Of tuberculous family; general health had been good prior to July, 1888, at which time he had a small ischio-rectal abscess, which was opened by Dr. Hearn, and completed into a fistula, the second operation for which was made early in August of the same year. The fistula was slow in healing; otherwise his general health was good. He returned to his occupation, that of a clerk, and did not complain of any symptoms until February, 1890. About the middle of February he was taken with an acute attack of pleurisy, which presented some unusual charac- teristics. It affected more particularly the left side, the pain and fric- tion being well marked at the apex, while the lower and anterior surface VOL, 104, NO. 5.—NOVEMBER, 1892. 37
566 COPLIN, BEVAN, UNUSUAL CASE OF TUBERCULOSIS.
of the pleura was the seat of well-marked and extensive dulness—so marked, indeed, that the effusion at one time almost demanded tapping ; however, under suitable internal remedies and counter-irritation it disappeared.
There had been marked hyper-resonance over the right lung and apparently over the left apex ; this now gave way to slight dulness over the left apex with crepitant riles. The evidences of pleurisy had disap- peared, and the patient’s general condition was so good that he failed to consult his professional attendant until the middle of June, when the following symptoms and physical signs were noticed.
The symptoms were slight pain over the left apex, not increased by inspiratory efforts nor by coughing; slight hacking cough, which did not annoy him very much and was attended by a very slight expectoration, at no time bloody, nor was it thick and muco-purulent in character. He complained of some shortness of breath on exertion, with decided hectic fever in the afternoons; had lost considerable flesh, but his appe- tite and digestion remained good.
The physical signs which presented themselves were as follows: Marked dulness over the left apex, extending downward as far as the fourth intercostal space, there were no respiratory murmurs to be heard over the dull area; the lower portion of the lung seemed to be hyper- resonant, although emitting some crepitant riles. In the third inter- costal space, about one inch to the left of the sternum, was a soft nodule which gave distinct fluctuation and became tense and hard during any attack of coughing. The right apex might have been slightly dull, although no note was made of it at the time.
This examination was made on July 3, 1890; he was prescribed
7 of hydriodic acid in teaspoonful doses, three times a day; he bore this well, and returned July 27, very much improved, as he thought. At this time the physical signs did not seem to have changed; a heart note was made—no lesions could be noted. Treatment continued
He was not seen again until August 13, when Dr. Coplin was sum- moned, and found the abscess had opened and discharged about two ounces of thick creamy pus containing small flocculi, slightly blood- stained. The opening through which it discharged was about as large as a pea, its edge thin and undermined, exposing to view the third costal cartilage, which was eroded and almost divided through.
This small opening communicated with a cavity about as large as a teacup, and could readily be made out, although its walls could not be discerned with any clearness. During the first twenty-four hours after the opening of the abscess no air passed in or out during the respiratory movements. However, on the second day after its opening the patient had a slight hemorrhage from the wound, amounting to one or two drachms and associated with slight hemoptysis; at the same time air was noticed to have entered through the opening, and from that time until his death whenever the opening was exposed respiration could be carried on entirely through it with the nose and mouth closed. His general condition seemed good, he was cheerful, had slight night sweats, appetite and digestion good, bowels regular, and his sleep very good, although at times annoyed by slight coughing. He had almost no expectoration.
About the middle of August Dr. O. P. Rex was asked to see the case. At that time there was a slight dulness over the right apex, with
COPLIN, BEVAN, UNUSUAL CASE OF TUBERCULOSIS. 567
no other evidences of further tubercular infection. The method by which he would empty this cavity was extremely interesting. He would seat himself on the side of the bed and take a large basin in his lap; he would then take a deep inspiration, leaning well back, followed by suddenly throwing himself forward over the basin, and, closing his mouth and nose, force the expired air out through the hole in his chest, bringing with it at least three or four ounces of thick tuberculous sputum; he would then feel relieved and lie down and énjoy an hour or so of quiet rest. The opening had now extended in size until it was as large as a silver half-dollar ; the third costal cartilage, which had bridged the opening, was entirely destroyed and the two eroded ends could be seen at the inner and outer margins. With a bull’s-eye lantern the large cavity could be readily seen, and its walls, posterior, external, and internal, could be easily made out. Extending across the cavity at various points, were remnants of bloodvessels or bronchial tubes, or possibly both, with branches jutting off in different directions, many of them uniting and some of them hanging as shreds from larger trunks ; some of these showed saccular enlargements, and many of the saccular enlargements were open, showing small cavities inside, evidently com- municating with other bronchial trunks through which air could be seen passing in and out. The cavity looked sufficiently large for the admission of two good sized fists.
Upon the floor of the cavity—that is, the inferior boundary—could be distinctly noticed the filling and emptying of the cardiac auricle; the filling could be distinctly noticed as synchronous with the pulse. This seemed to indicate that the auricle filled while the ventricle was emptying itself. Pulsation could not be observed in any of the branches which passed through the abscess cavity.
After the initial hemorrhage, which occurred shortly after the abscess opened, no further hemorrhages were observed. Many of the branches which traversed the cavity disappeared, and its opening gradually enlarged to nearly twice the size of a silver dollar. His general con- dition very gradually became worse, and death occurred on the 27th of October from exhaustion.
TREATMENT.—The treatment was entirely symptomatic. There were few symptoms which called for treatment ; his cough rarely demanded it, and when it did, seemed to be relieved more by inhalation of one or two drops of chloroform upon a handkerchief held over the opening in the chest. The opening was kept surrounded and covered by corrosive sublimate gauze, and as at one time the discharge became fetid he was ordered a gauze moistened with a solution of eucalyptol, chloroform, and iodide of ethyl, each a drachm in four ounces of fifty per cent. alcohol, this controlled the cough and entirely removed the fetid odor. It was used only when occasion demanded it, and then for brief inter- vals, 15 or 20 minutes; it rarely produced any symptoms of anzsthesia and but slightly accelerated the heart’s action.
Laxsoratory Nores.—The pus from the abscess was stained both by Gram’s method and by the Koch-Ehrlich method ; by Gram’s method streptococci and staphylococci, also diplococci and bacteria were well
568 COPLIN, BEVAN, UNUSUAL CASE OF TUBERCULOSIS.
stained. These were afterward isolated and proven to consist of the following microérganisms :
1. Streptococcus pyogenes.
2. Staphylococcus pyogenes, areus and albus.
3. Bacteria termo and lineola.
4, Various forms of mould, not isolated.
A diplococcus was noted as present in the pus, but the culture experi- ments failed to isolate it.
By both Gram’s and the Koch-Ehrlich methods the bacillus of tubercle was well stained and found to be present in large numbers. The sputum expectorated did not show the bacilli of tubercle until after the opening externally of the abscess, when they were present in large numbers, as well as in discharges from the abscess cavity. Numerous examinations were made of the sputum and discharge, the observations extending over several months, and at no time was there absence of the micro- organisms above stated.
The accompanying cut shows the organisms present ; also the fact that moulds were growing in the abscess cavity; these were proven to be members of the aspergillus group.
Beck’s , objective, homogeneous immersion, Oc. c, composite field ; camera lucida drawing.
a,a.a. Tubercle bacillus. 4, 4, 4, Tubercle bacillus with spores (?). 5. Micrococci in zooglea. c. Streptococci. d. Diplococci. e. Tetracocci. f. Leptothrix. g. Mould fungi. A. Yeast fungi.
RemarkKs.—While this case presents many features of unusual in- terest, its history, associated with minute study of the accompanying
COPLIN, BEVAN, UNUSUAL CASE OF TUBERCULOSIS. 569
process, shows that it is but an extraordinary combination of complex infective processes. It is to be assumed that the pleurisy which he had was plastic in character and firmly united the visceral and parietal layers of the apiceal pleura together, thus occluding the lower half of the left cavity from that portion of the pleura which covered the apex. That this pleurisy was tubercular in origin and assdciated with active tubercular processes in the apex is hardly to be doubted; these even- tually becoming infected with microdrganisms of suppuration (secondary infection), thus leading as an inevitable result to the development of pus, which extended and burrowed through the anterior chest-wall. One of the most interesting and at the same time most benevolent features of this unique pathological process is the fact that its walls so thoroughly limited and prevented its opening into some of the larger bronchi until an external opening: had been provided; it is also a very remarkable occurrence in the fact that this extensive process was unassociated with any hemorrhagic development. Whether the ischio-rectal abscess was of tubercular origin or not cannot be definitely stated, as no examinations were made for the bacilli of tuberculosis. It is, however, to be presumed that the pulmonary process was probably caused directly from infection through a primary cheesy nodule or caseous mass somewhere else than in the lung itself; this, of course, cannot be proven.
It is extremely unfortunate that more minute observations could not have been made upon the cardiac condition, and if possible graphic re- presentations of the inter-thoracic viscera have been made. It is also to be regretted that no post-mortem could be obtained.
REVIEWS.
A TEXT-BOOK OF THE PRINCIPLES AND PRACTICE OF MEDICINE. By Henry M. Lyman, A.M., M.D., Professor of the Principles and Practice of Medicine in Rush Medical College, Chicago. Philadelphia: Lea Brothers & Co., 1892.
WHILE a review of a text-book of the Practice of Medicine may be of value to one contemplating the perusal of such a work, it is to be remem- bered that there are no points of striking original merit to which atten- tion can be called, in view of the fact that the only good Practice for the use of student and practitioner is one that presents a complete picture of the subject, without misleading or false statements, and containing all that is essential, including recent researches that have been or are being made at the time of publication. It will be seen, therefore, that not much can be said in reviewing a book of this character when the statement has once been made that the book is complete, accurate, and up to date.
The work before us consists of 926 pages, with many illustrations. Its general make-up is excellent, and the mechanical part of the execu- tion leaves nothing to be desired.
Part I. is devoted to Preliminary Considerations, including chapters upon the organization of the body, growth and development, disturbances of nutrition, tumors, disorders induced by disturbances of the circula- tion, contagion and infective diseases, inflammation, and fever.
Part II. is devoted to the Parasitic and Infective Diseases. While it at first sight seems strange to find grouped together diseases so far removed —for example, as intestinal worms and pulmonary consumption—it is manifest that, if we agree with the general view as to the etiology of the eruptive fevers, dysentery, tuberculosis, etc., it is not so extremely out of the way to arrange many of the diseases caused by living animal or vegetable parasites together. It would, however, seem to the reviewer that the placing of diseases caused by intestinal parasites among intes- tinal disorders would be far more appropriate than inserting them in the same portion of the book with infectious diseases. There is certainly no such connection between verminous intestinal diseases and malaria as there is between dysentery and malaria. The animal parasites of the intestinal canal are, so to speak, outside of the body proper, and are so closely connected with local disorders of the parts concerned that the proper treatment of the parasitic disease does not, as a rule, complete the cure, the correction of the accompanying catarrhal condition of the mucous membrane being as important as the removal of the parasite itself. With dysentery the question is to a certain extent different, in that the truly infective character of the disease allows of its more prop- erly being considered with such diseases as malaria. In the present volume the author connects the diseases due to the invasion of living
LYMAN, PRINCIPLES AND PRACTICE OF MEDICINE. 571
plants and animals by considering the intestinal animal parasites first ; then the protozobn of malaria ; by this leading up to dysentery, and this in turn being followed by actinomycosis, anthrax, typhoid fever, and so on through the list of infective diseases.
While he does not so state in positive terms, it is evident that the author believes in the now well-recognized plasmodium of Laveran as the cause of the malarial manifestations. In regard to the réle played by the ameeba coli in the causation of dysentery, the author is very cautious in giving an opinion, his reference to it being in the following terms: “Tn the tropics and elsewhere various forms of amceba have been iden- tified in the colon and in the hepatic or pulmonary abscesses that some- times complicate the disease, and they have been considered as its cause.” With the diseases now generally supposed to be caused by bacteria, the author is much more positive in his statements as to their etiological importance. Full consideration is given to the facts at present known regarding acquired and artificial immunity.
Among the infective diseases the author includes the description of syphilis and tuberculosis in their various forms, so that, instead of describing cerebral and spinal syphilis and pulmonary and laryngeal tuberculosis among the diseases of the nervous and respiratory systems respectively, they are treated under the head of the general disease as localized in the different parts. This arrangement has many very de- cided advantages that are apparent after one has become used to this change from the former classification, whereby the general disease was treated of in one portion of the work and the specially localized forms of the same disorder under the various anatomical divisions. This classi- fication is also much more in accordance with etiological facts, and per- mits the author to avoid much repetition and to give a connected account of the infective process as it involves various structures.
The thirteen remaining parts, into which the rest of the book is divided, deal with the diseases of the various systems of the body, each division receiving careful consideration.
Under the Diseases of the Stomach the author gives a full description of the various tests for determining the presence or absence of free hydro- chloric acid, the peptogenic power of the gastric juice, the rate of absorp- tion from the stomach, and the amount of the muscular power of the viscus. The chapters upon Diseases of the Intestines and organs of the chylopoietic system require no notice, being fully up to the requirements demanded by the importance of the diseases to which they belong.
Under Diseases of the Organs of Respiration the author considers fibrin- ous pneumonia, although he describes it as an infective disease. It is well, however, that the author has considered it in relation to the diseases that are closely connected with it anatomically, as the importance of the local lesion in the lungs is at present far greater than that of the general systemic disturbance, of which the former may be simply the outward and visible sign. In regard to the treatment of pneumonia, the author condemns the use of quinine, tartar emetic, veratrum viride, digitalis, and venesection as measures to be avoided. As the author makes no mention of the extreme danger arising from over-distention of the right side of the heart in this disease, it would seem that he has never wit- nessed the intense relief affurded by the loss of a small quantity of blood from the congested venous system when this unfortunate condition threatened life.
ae
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572 REVIEWS.
The portion of the work that deals with Diseases of the Circulation is divided into chapters upon endocardial diseases, diseases of the muscular substance of the heart, neuroses of the heart, diseases of the pericar- dium, and diseases of the aorta. Following a section upon Diseases of the Blood, Part VIII. is devoted to the Diseases of Nutrition, including rickets, osteo-malacia, obesity, diabetes mellitus, diabetes insipidus, gout, nodular rheumatism, acute rheumatism, alcoholism, morphinism, cocainism, and chronic nicotinism. The classification of these diseases together avoids the usual absurdity of considering diabetes mellitus and insipidus among renal diseases, with which they have no possible con- nection, save only that the most characteristic changes in both groups are found in the urine. The portion devoted to renal diseases and to those of the urinary organs is full and carefully written. The last four ww are taken up with a consideration of Diseases of the Nervous
ystem, being divided into sections upon diseases of the peripheral nerves, of the brain and its membranes, of the spinal cord, and functional diseases of the brain.
Looking upon the book as a whole, it may be said to fully carry out the purpose for which it was written: the giving of a reliable text-book of practice of medicine to the student and practitioner. ¥. A.
Diz ENTSTEHUNG DER ENTZUNDUNG UND DIE WIRKUNG DER ENT- ZUNDUNGERREGENDEN SCHADLICHKEITEN, NACH VORZUGSWEISE AM AUGE UNGESTELLTEN UNTERSUCHUNGEN. Von Dr. THEODOR LEBER, Professor der Augenheilkunde an der Universitit Heidelberg. Mit 8 lithographischen Tafeln und 2 Holzschnitten.
THE ORIGIN OF INFLAMMATION AND THE ACTION OF Noxious InN- FLAMMATION-EXCITING SUBSTANCES, AS SHOWN IN INVESTIGATIONS INSTITUTED MOSTLY ON THE EYE.
Most German professors seem to think that their thoughts are better embalmed when presented to the public in weighty quartos, and, conse- quently, nearly all essays on election to university chairs, and anniver- sary mementoes of the retirement of noted professors from their posts as teachers, are spread out in huge volumes with wide margins. Prof. Leber is no exception to the rule, and soon after his transfer from the chair of ophthalmology in Géttingen to that in Heidelberg, has published the results of his eleven years of careful work in the study of inflammation in a quarto of five hundred and thirty-five pages, printed on heavy paper, and illustrated by eight lithographic plates and two woodcuts. All his es writings, from that on the circulation of the blood in the eye-
all to this treatise on inflammation, have shown careful and pains- taking observation and thoughtful reasoning, and we were therefore led to expect a great deal from the present treatise; and we have not been disappointed. :
In conducting his experiments on inflammation, the author has usually chosen the eyeball as the organ of the body in which to per- form ~ A because in most instances the reaction produced does not
LTR
LEBER, ON INFLAMMATION. 573
extend beyond the sclerotic coat, and because the progress of the in- flammation in the cornea and iris can usually be advantageously studied during the life of the animal used for experiment. The substances selected to cause inflammation have been most varied in character, e. 7., the mould-producing fungi, the bacteria of decomposing fluids, the staphylococcus aureus, particles of iron, copper, lead, silver, gold, mer- cury, and arsenic, introduced into the tissues, and a long list of organic substances such as gamboge, indigo, quinine, croton oil, uric acid, etc. In experimenting with fungi and bacteria, pure cultures only were em- ployed, and in the case of other material it was in every instance care- fully sterilized and introduced into the eye with sterilized instruments, under the strictest aseptic precautions. Horner had already described a keratitis mycotica as occurring in man, and these experiments with mould fungi and the bacteria of putrefaction were therefore of direct practical interest. It was found that some fungi, such as penicillium glaucum, produced only slight and transient irritation, owing to their inability to exist and grow at the —— of the rabbit’s body. The alkaline reaction of the serum of the tissues is probably also unfavorable to the growth of many varieties of fungus. On the other hand, aspergillus fumigatus grew luxuriantly, and produced intense inflammation. If a portion of a pure culture of this fungus was inoculated in the centre of the cornea, it proliferated to some extent in the corneal tissue, causing a local necrosis and ulcer, and ee this was a clear area, where the intensity of the poison secreted by the fungus caused a more gradual death of the corneal tissue. This area was absolutely free from either fungus or from pus corpuscles, while in it also the corneal corpuscles were also shrinking and dying. Beyond this necrotic area and surrounding it was a ring densely packed with leucocytes. At this stage there was also marked inflammation of the iris and ciliary body, the pupil becoming plugged with lymph, and the iris being covered with fibrinous effusion both on its anterior and posterior surfaces, while the pectinate ligament and ciliary processes were crowded with leucocytes. When the fungus is introduced into the anterior chamber, a violent irido-cyclitis is pro- duced, but no mycelium is found free in the anterior chamber. On its non-vascular walls it develops, however, being found in the membrane of Descemet and in the anterior capsule of the lens. The layers of the cornea anterior to the membrane of Descemet were full of fibrinous exudation, but free from all traces of aspergillus. When a small por- tion of the aspergillus fumigatus was introduced into the vitreous, there followed a proliferation of mycelium throughout its tissue, with the pro- duction of pus in the retina and choroid, and eventually, also, in the anterior chamber, although no mycelium was found in the latter situa- tion. An injection of a neutral watery extract of aspergillus fumigatus into the cornea also produced inflammation, but in a lower grade than that caused by the living fungus. One of the most interesting of the conclusions arrived at by Leber is that the intense suppurative inflam- mation caused by the introduction of the staphylococcus aureus into the eye is due to a poisonous principle produced by these microbes, and that this chemical agent can be extracted from them, and will then, in turn, give rise to intense suppurative inflammation. Pasteur has already found that boiled cultures of the staphylococcus w6uld produce active inflam- mation. Leber confirmed this result, and found, further, that such de- coctions when treated with ether yielded a crystalline principle, called
574 REVIEWS.
by him phlogosin, which, when introduced into the tissues, produced local necrosis, and the most violent surrounding inflammation. The pus produced either by the use of the decoctions of the staphylococcus or by the phlogosin was in either case absolutely sterile and non- microbic, as proved both by repeated careful attempts at culture in different materials, and also by the most thorough microscopic investi- gation, after staining with fuchsin. Either sterilized mercury or steril- ized croton oil injected into the anterior chamber caused a limited puru- lent irido-cyclitis, with destruction of a part of the deeper corneal layers and the production of an internal corneal ulcer.
Clinical experience has long since taught us that very minute pieces of metal, if entirely aseptic, may remain in the anterior chamber for a long time without exciting any notable reaction. Leber proved by experiment that small aseptic pieces of glass and metal would often become coated with an exudation, consisting mainly of proliferated endothelium and of giant cells, and thus coated remain harmless. In one instance, a piece of steel needle introduced into the anterior chamber lay there for over a year with very little irritation, and finally, in five hundred and twenty-seven days after its introduction, was spontaneously evacuated through the wound of entrance. On the other hand, even substances like glass and gold, when in a state of minute sub- division, appear to undergo slow solution and to exercise an irritative and deleterious action on tissues. Thus finely powdered aseptic gold injected into the vitreous produced a slow contraction of the retinal arteries, the veins being in like manner affected, but to a less degree, while there was swelling of that part of the retinal fibres which retained their marrow sheath (rabbit’s eye), and final atrophy of the fibres. These changes were accompanied by alterations in the distribution of the pigment in the retinal epithelium, causing the eye-ground when examined by the ophthalmoscope to appear marbled. Similar but more marked changes followed more rapidly from the introduction of copper, lead, iron, etc., into the vitreous. Arsenic seems to have a marked necrotic influence on the adjacent tissues. All these changes would appear not to be due to any mechanical action of the foreign substances thus introduced, but to a gradual solution of them by the fluids of the economy and the irritant action of the solution on the tissue. In every case they were more marked when the metal was introduced in a state of minute subdivision. The action of jequirity was also noteworthy. A large piece of a bean of the Abrus precatorius introduced into the anterior chamber of a rab- bit’s eye caused the death of the animal, with marked congestion of the lungs and kidneys and with extravasations of blood into the mucous membrane of the stomach and bowels. A smaller quantity of the drug produced a diffuse opacity of the cornea, with swelling of the lids and the well-known characteristic exudation on the surface of the conjunc- tiva. Leber considers that this action is due to jequiritin and not to the action of microbes. The slight effect of uric acid introduced into the anterior chamber of rabbits’ eyes is noteworthy. It was rapidly absorbed, causing but little inflammation. This was contrary to what might have been expected, because, in the experiments of Ebstein on birds, where he caused retention of uric acid by tying the ureters, the tissues infiltrated with it subsequently underwent necrosis. As already mentioned in speaking of the action of staphylococcin, so in numerous experiments with other substances we have abundant and repeated evi-
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dence of the possible occurrence of severe non-microbic inflammation and suppuration, the most careful culture experiments and searching microscopic examination failing to prove their presence, either in the tissues or in the pus corpuscles. The main clinical difference between microbic and non-microbic inflammation and suppuration seems to be that the former, owing to the continued secretion of irritant substance by the microbes, is of longer duration, more intense, and extends further into adjacent tissue. Purulent softening and disintegration in inflamed parts, or, as