Chapter C: O——, aged twenty-two, is a reporter attached to one of the New York

afternoon papers, who received a severe injury of the head when but three years old. He fell from the second story of an unfinished building to the cellar, striking the upper and back part of his head upon a beam. He was rendered unconscious, and remained so for a day or more. He recovered from the immediate bad effect, but has suffered from severe general headaches, which recur every week or so, with an increase in the amount of urine excreted. About six months ago he began to have epileptic convulsions of a violent character almost every day, and sometimes more often. These were precipitated by excitement, and he had a great many when worried about his wife at the time of her delivery. Upon one occasion he fell down stairs and injured himself quite severely. The attacks were, as a rule, preceded by an epigastric aura of long duration, and occasionally by a visual aura, and, according to the testimony of his associates, he became strange and queer. When in such a dazed condition he would restlessly wander about his office, and suddenly, without any cry, become convulsed. After the attack he slept soundly. The bromides of sodium and ammonium and digitalis did little or no good, but the bromide of nickel appeared to have some influence. During the past month he has had only two or three attacks, but these have been of a quite irregular character. He told me that there were times when he felt like doing himself an injury, and that he had impulses to kill some one else. His companions said he was irritable, pugnacious, and easily thwarted, and his brother-in-law stated that upon several occasions he had queer turns, when he would raise his hand to strike some member of the family—that he subsequently knew nothing of his conduct, and when it was detailed to him he appeared greatly astonished.

Mr. O—— came to my office in company with a friend at ten o'clock in the morning of December 27, 1883. He had had one of his attacks at the newspaper office, of rather more severe character than usual, at eight o'clock, with a psychical aura, during the existence of which he was very morose and sullen. Upon recovery he was speechless, though he could communicate by signs. Upon his arrival at my office his manner was composed and he appeared somewhat dazed. His pupils were dilated, but contracted readily to light. I asked him one or more questions regarding his inability to speak, which he perfectly understood, and when I gave him a pencil and a piece of paper he replied without difficulty in writing. When told to make a great effort to speak he did so, and I thought I detected the word ‘To-day,’ but he could not repeat it, though he tried and expressed great annoyance. He was unable to utter any sound except a sort of groan, which could not in any way be taken as an element of speech. I examined his larynx, but found nothing which could explain his impaired phonation, and I sent him to Dr. Asch, who found absolutely no abnormal appearances to account for the speech {485} difficulty. The patient could not phonate, and though he made attempts to enunciate the vowel-sounds, and the vocal cords were approximated, he made no orderly sound. Asch found a slight laryngitis of no importance.

The patient went home, and remained speechless all day, and was seen by my associate, G. de Forrest Smith, in the evening. What occurred during and after that gentleman's visit is contained in his notes: “I was called to see patient about 8.15 P.M. He was lying upon the bed, but had not slept; recognized me and motioned that he could not speak, and I found that he could only say one or two words, and this with the greatest effort, and so all my questions were put so that he could answer them by nodding or shaking his head. He knew that he had had an attack in the morning, that he had seen Hamilton and Asch, and recalled various incidents of the day, answering intelligently my questions in regard to them. He indicated by motions that his inability to speak was due to a lump in his throat. When asked if he had any trouble to think of the word he wanted, he shook his head, but shortly afterward hesitated in an answer, and when asked if this was due to his inability to think of the word, said ‘Yes.’ Was asked if he had any loss of power in either side, and he motioned to his right arm and leg, and said that he felt a numbness and pricking on that side. On his grasping my hands with his, the right was perceptibly weaker.

“At one time he seemed confused as to which was his right or left side, and put up both hands, and after looking at first one and then the other in a puzzled manner, at last decided correctly, then smiled apparently at his confusion.

“All this time he had been half lying on the bed. He now intimated that he was tired, put his head down on the pillow and began to belch up wind, and as he appeared about to vomit I called for a basin; but this was only the beginning of an attack; the muscles of the neck and right side assumed a state of tonic spasm, the extensors predominating, so that the head was turned a little to the left and forcibly thrust back into the pillow, and the right arm and leg were firmly extended. He remained in this position about one minute; then, taking two or three full inspirations, put his hand to his throat and said plainly, ‘Something has fallen from there.’ On being asked ‘What?’ he replied, ‘A bone has fallen from my throat.’ I told him it was well that the bone had fallen, as now he could speak. ‘Why,’ said he, ‘I have had no difficulty in talking.’ On being asked why he had seen Asch, he said ‘Who is Dr. Asch? I never saw any such person.’ Further questioning showed that all the occurrences of the day (except those which had taken place immediately before the first attack) were an absolute blank, and he thought it still morning. He asked the time, and I told him half-past eight o'clock in the evening. At this he seemed much surprised and said, ‘Why, I went to work this morning; how did I come here?’ I then explained to him that he had been ill. After further conversation he said he felt sleepy, and, after resting a few minutes, he arose, put on his slippers, and came out into the room. He walked with difficulty, because of the loss of power in the right side, which he said felt numb and sore, as if it had been pounded, also a sensation of pins and needles. After the attack his mind was perfectly clear, and he could talk as well as ever, and all that had happened before the attack in the morning he could remember {486} perfectly well, but the interval between the two was a complete blank. His inability to speak seemed due, not to lack of knowledge of what he wanted to say, but rather to want of power to form the words, although there was no paralysis of the vocal muscles. When he did manage to say a word, it was invariably the correct one, but it was always done with the greatest effort. The day after the attacks he remained at home; the next day he went to work, but his head felt heavy and confused. Two days after he complained of a pressure on the left side and back part of the head; otherwise he was all right. At this visit he said that after I had left him on the night of the attacks he intently thought, striving to recall the incidents of the day, and after a time concluded he could remember being at Thirty-third street, but did not know how he got there. He thought he could recall going to see Asch, but would not know him if he should see him. I then asked him how questions were answered by him on that day; he answered he did not know, as he had not thought of that; then, after a few moments' reflection, said he must have written the answers. He was then shown some of the answers he had written, which he recognized, and by an effort of memory could recall some of the incidents of writing them. He was still unable to remember anything that occurred after his arrival home previous to the last convulsion.”

January 27, 1885: This patient subsequently suffered from several attacks in which the psychical element predominated. His head presented a remarkable deformity, there being a prominence posteriorly which might be compared to a caput succedaneum, only it was entirely osseous. The upper margin was separated from the anterior parts by a deep sulcus.

Under such circumstances we find very often that acts of great violence are committed by such epileptics for which they are entirely irresponsible. Two or three cases of the kind occur to me now. One of them was a boy who always bit every one and everything—his family, the domestic animals, and inanimate objects; another, a most dignified and lady-like woman, who violently struck different members of her family; and within the past week a woman was brought to me who hurled a kerosene lamp at a perfect stranger with whom she was quietly talking before the seizure was precipitated. Numerous instances are related where individuals while in the masked epileptic state have wandered for long distances and committed a variety of purposeless acts, and undoubtedly many of the mysterious disappearances are of this order.

SENSORY EPILEPSY.—Some years ago Hammond referred to certain peculiar epileptic attacks in which sensory manifestations were very pronounced. To this condition he gave the name thalamic epilepsy, believing the condition to be one of the optic thalamus. Among the large number of unclassified and irregular cases reported by various authors there are many so much resembling each other that I think they should be relegated to a special place.[32] The notable examples of Sommers, Bergmann, Tagges, Guislain, and others belong to this category.

[Footnote 32: I shortly afterward, believing the term a misnomer, invented that in use: “On Cortical Sensory Discharging Lesions or Sensory Epilepsy,” _New York Med. Journal and Obstetrical Review_, June, 1882; also see “A Contribution to the Study of Several Unusual Forms of Sensory Epilepsy which are probably Dependent upon Lesions of the Occipital Cortex,” _New York Med. Record_, April 4, 1885.]

{487} The features of this form of epilepsy are (1) the expression of some hallucination (prodromal stage), or hemiopia; (2) supraorbital neuralgia; (3) aphasia, formication; (4) slight loss of consciousness, and little if any motor disturbance.

I may present two illustrative cases:

A few months ago I was consulted by a medical gentleman in regard to a patient who had for years presented a curious train of nervous symptoms, which afterward assumed a form leading me to think she might have sensory epilepsy. She would, in the presence of the gentleman who consulted me, who was a personal friend of the patient and a medical man, stop short in the midst of an animated conversation, look fixedly ahead, appearing momentarily lost, remaining abstracted for a short period, possibly a minute, and on recovering herself go on, finishing the sentence she had commenced before the seizure. At this time she constantly had hallucinations of a visual character, when she saw animals, birds, figures of men and women, who approached her, as well as a variety of other objects. A common hallucination, which had been repeated quite frequently, consisted in visions in which green leaves and white rabbits and other objects familiar to her in childhood figured extensively. Upon one occasion, while sitting in the drawing-room, opposite a door which communicated with the hall, she suddenly called her companion's attention to the hand of a man which she saw clasping the baluster rail. The hand was seemingly disconnected from the arm. She was somewhat agitated, and it was nearly half a minute before the vision was dismissed. Sometimes she would call attention to the hallucinations before the attack, but more often she became transfixed, apparently lost, and then recovering she described her visions minutely. She has apparently been able to foresee the attacks and ward them off by a strong voluntary effort. So far as can be learned, there is no hysterical element in the case, but her seizures are more frequent at the time of menstruation. In a private note it is stated that “the family history of the patient is very good, and she has always seemed remarkably healthy and robust, and has shown more than usual intellectual ability. She has appeared to persons generally to be of a contented, happy disposition.... At night, when she closed her eyes, she suffered from these hallucinations, especially after a day of fatigue. Her pupils are usually dilated, but her color undergoes no change during the seizure.”

The second case is one of a more complex type:

J. B——, a bright boy aged sixteen, was sent to me by F. H. Bosworth in April, 1883. He comes of nervous stock, his mother being subject to epilepsy, and his father is an eccentric man who manifests his mental peculiarities chiefly in a morbid restlessness and irascibility. There is a brother who is healthy. The attention of the parents was first called to the boy's condition by his recital of a sudden attack which occurred during the summer of 1882. While rowing upon a river he suddenly and completely lost the visual use of the right eye, so that in looking at a number of ducks swimming near his boat he failed to perceive those upon one side of the flock. This condition lasted for twenty minutes, and after a brief and severe pain over the right eye he became unconscious, the unconsciousness being preceded by a tingling and numbness of the hand, forearm, arm, and left side of the tongue. He has {488} subsequently had eight or ten of these attacks, of which the following is an example: Usually without any bad feelings, physical or mental, he, while engaged in any duty or at any time, suffers a sudden unilateral blindness. This is never gradual, and not like the form of amblyopia in which the visual field is gradually reduced. There is some hemichromatopsia. It would seem as if the retinal anæsthesia was unequal, for while usually the loss is complete and universal, it sometimes happens that there is only a limited loss. Upon one occasion, while reading, he suddenly lost the printed matter of the right lower half of the page below a diagonal line extending from the right upper corner to the left lower corner. There is never diplopia. This deprivation lasts anywhere from ten to twenty minutes; meanwhile, a distal anæsthesia, coming very gradually, involves at first the fingers of the opposite hand, and successively extends to the forearm, arm, and other parts, as I have already mentioned. There seems to be analgesia as well as anæsthesia, for a pin may be run into the muscles without producing pain, and upon one occasion the gum was freely pricked without any discomfort to the patient. It invariably happened that the cutaneous sensory trouble occurred upon the side opposite to the hemianopsia and neuralgia, and in the greater number of instances the left side was that affected. The third stage of the attack consists in migrainous headache of a very severe kind, and which sometimes lasts for an hour or more. There is a subjective feeling as if the eye was pushed forward. This disappears with nausea and relaxation. More often he loses consciousness when the anæsthesia reaches its limit, which seems to be the extension of the anæsthesia to the gums. Occasionally there are slight convulsive movements upon the anæsthetic side. While the attacks involve the left side of the body as a rule, it happens that when there is primary left hemianopsia and right-sided anæsthesia the boy becomes very much confused in speech, and sometimes is paraphasic, the trouble being but transitory. He is sometimes unable to speak at all, though perfectly conscious and in possession of his faculties. No pupillary disturbance has been noticed at any time. Upon two occasions there was a swelling of gums and tongue, which was not only subjective, but perceived by the mother. Occasionally he sees prismatic colors and rays before the blindness, but this has been only once or twice. During his early life he had attacks of slight numbness of the hands and feet which were not thought much of, and he had headache as well. He has been a somnambulist.

MORBID ANATOMY AND PATHOLOGY.—The literature of the experimental physiology of epilepsy is enriched by the observations of a variety of careful students, among them Sir Astley Cooper, Kussmaul and Tenner, Brown-Séquard, Nothnagel, Schroeder Van der Kolk, Pitres, Hughlings-Jackson, and the followers of the localization school, as well as many others more or less distinguished.

The experiments of many of the early writers were directed for the purpose of ascertaining the relations of circulatory variations to convulsive seizures, and the most notable were those of Burrows and Kussmaul and Tenner. These latter produced compression of the carotid arteries, and instituted cerebral anæmia by free and exhausting hemorrhages. As a consequence, the emptying of the cerebral vessels was followed by a loss of consciousness and by epileptiform convulsions, and it was {489} necessary to produce the same result to compress all the great afferent vessels of the brain. The experience of surgeons generally is, that ligation of the common carotid upon one side of the neck is sometimes very apt to produce an alarming anæmia, with occasional convulsions, and sometimes fatal consequences.

The experiments of Hall, Landois, Hermann, and others, as well as those of the writers just mentioned, show that carotid compression results in capilliary anæmia and venous hyperæmia, and that with cessation of this pressure there is a sudden congestion of all vessels. The susceptibility of the brain is greatest at its posterior part and between the optic thalami and the cord. When the bulb was subjected to sudden changes in its nutrition—such, for instance, as followed the experiments of Hermann, who ligated simultaneously the superior and inferior venæ cavæ of a rabbit—there were not only convulsions, but various cardiac and other disturbances which were undoubtedly due to central impairment of function. Kussmaul and Tenner conducted their experiments with watch-glasses luted into the cranium—a procedure which, however, at best, is unreliable.

Brown-Séquard some years ago in part established an important pathological truth, the theory of epileptic zones, and demonstrated in certain animals that bruising and injury of the great nerve-trunks, especially the great sciatic, would give rise to epilepsy, and that irritation of certain tracts would precipitate the paroxysms. He further announced that the progeny of animals in whom epilepsy had been thus induced very frequently inherited the epilepsy of the parent. By some it was held that such epilepsies were purely peripheral, and Brown-Séquard even believed in spinal epilepsy. His spinal epilepsic theory has, for the most part, been explained by the anatomical researches of Hitzig and the doctrine of interrupted spinal inhibition. In fact, many of the spinal epilepsies are examples of exaggerated reflexes.

The epileptiginous zone theory, which, while it induced many to believe that the disease might have its origin outside of the brain, gave rise to the false assumption that attacks with distal auræ were primarily non-cerebral, has been discarded, and most observers have arrived at the conclusion that even in these cases the first explosion is due to some cerebral cell-discharge.

Hughlings-Jackson's grand work has revolutionized the views held prior to his first published writings, about twelve years ago. He believes that any part of the gray matter may, through over-excitability, give rise to convulsive attacks.

The production of convulsions by cortical irritation is now an old story.

The experiment of Pitres and Frank[33] bears upon the sensorial function of the cortex in showing that, when the cortex is irritated, epileptiform convulsions follow, but if the exposed surface be subjected to the ether spray the same irritation will only produce definite movements, but no convulsions.

[Footnote 33: _Gazette des Hôpitaux_, No. 38, 1883.]

The investigations of Van der Kolk especially, and his followers, certainly give the medulla an important place as the locus morbi of the malady; and it must be assumed, bearing in mind the existence of the vaso-motor centres of Dieters and the presentation of symptoms indicative {490} of disturbance at the floor of the fourth ventricle, that the most important pathological changes must be looked for in this part of the brain.

Jackson's cortical explanation is, however, fully in consonance with the medullary theory. If we study the different stages of the attack, we shall find that there is probably a suspension of cortical inhibition—that a derangement of the cortical cells or discharge may cause a resulting disturbance in the bulb. On the other hand, a reflex irritation through the pneumogastric or from some distal part brings about the same disturbance of equilibrium. There is anæmia due to irritation of the vaso-motor centre, an inhibition of the great ganglion-cells, and a disturbance of function of the important cranial nerves. The primary anæmia and unconsciousness are accounted for by this primary irritation of sympathetic filaments and vascular constriction; the secondary hyperæmia is explained by the experiments of Kussmaul, which demonstrated the succeeding congestion; or by irritation of the spinal accessory and contraction of the muscles of the neck and compression of the large veins. The pupillary, ocular, respiratory, and other symptoms indicate the disturbance of the nerve-nuclei in the bulb. The respiratory difficulty and the interrupted decarbonization of the blood undoubtedly account for the secondary unconsciousness.

Van der Kolk[34] in localizing the lesion in the medulla found capillary dilatations in the neighborhood of the hypoglossal nuclei in tongue-biters. In epileptic patients who were in the habit of biting their tongues during the fit the vessels were wider than in those who did not bite the tongue, on an average in the course of the hypoglossus by 0.096; in the corpus olivare, which certainly here plays an important part, by 0.098 mm.; and in the raphé by 0.055. In those who did not bite the tongue, on the contrary, the vessels in the path of the vagus were 0.111 wider than in those in the first, Table A.[35]

[Footnote 34: “On the Minute Structure and Functions of the Spinal Cord,” by J. L. C. Schroeder Van der Kolk, _New Syd. Soc. Trans._]

[Footnote 35: TABLE.
--------------------+-----------+-----------+-----------+----------
Different | Hypo- | Corpus | |
Epileptics. | glossus. | olivare. | Raphé. | Vagus.
--------------------+-----------+-----------+-----------+----------
Table A—tongue | 0.306 | 0.315 | 0.315 | 0.237
biters | | | |
Table B—non biters | 0.210 | 0.217 | 0.217 | 0.348
+-----------+-----------+-----------+----------
Difference | +0.096 A. | +0.098 A. | +0.055 A. | +0.111 B.
--------------------+-----------+-----------+-----------+----------]

Nothnagel[36] is of the opinion that the anæmia of the brain is not the cause of the convulsions, but that the “excitation of the vaso-motor centre and that of the centre for the muscles are co-ordinate—that both go on side by side, and are independent of each other.”

[Footnote 36: _Ziemssen's Encyclopædia_, vol. xiv. p. 268.]

He by this theory explains the occurrence of those forms of petit mal in which there is loss of consciousness without convulsions, and, on the other hand, twitchings before the coma.

The best argument in favor of this hypothesis is in Jacksonian epilepsy, when monospasms exist oftentimes with a succeeding extension.

In those cases which are the outgrowth of migraine the pathological condition is probably an exaggerated tendency to angio-spasm, the original impaired vascular tonus in the beginning giving rise simply to pain and lesser troubles, while after repeated changes of calibre not only {491} nutritive alterations ensue, but hyperexcitability of the bulbar convulsion centres as well.

The labors of those who have endeavored to connect epilepsy with cerebral-tissue alterations have been attended by nothing very definite or positive, so far as pathological explanation is concerned. The post-mortem appearances have varied widely, and the only conclusion to be reached is that which shows that almost any morbid gross alteration of the cerebral mass may be symptomatized by convulsions, but such a production of paroxysmal trouble is much more likely to be the case, and in a more definite manner, when the cortical motor-centres are subject to destructive disease or irritative pressure. This is even not always the case, for numerous cases of injury of the paracentral lobe have been recorded with no showing of resulting convulsions. The long list of autopsies which I will not here consider show that an epilepsy may owe its origin to the pressure of a spicula of bone, or to the pressure exercised by depressed fragments of the same—to tumors or adventitious products, meningitis, cortical encephalitis, vascular degeneration, ventricular œdema, contusio-cerebri, and many other morbid processes which result in rapid or tardy degeneration. Of course, in such cases the genesis of the disease depends not so much upon the nature of the lesion as the location. The fruitful collections of cases of Ogle and Jackson are full of examples of limited growth or disease involving the cerebral cortex, while numerous cases collated by other writers show disease of the bulb or various peripheral parts which have been closely connected with the growth and behavior of the affection.

Several able pathologists have independently and repeatedly found that sclerotic degeneration of the hippocampal folds often existed. Delasiauve and Lébert first observed this lesion, but many modern authorities—among them Meynert, Nothnagel, and Charcot—who have also found this appearance, regard the change as of purely secondary, and consequently unimportant, character.

Tamburini[37] reports a case of hemiplegic epilepsy with induration of the left optic thalamus and the left cornu ammonis, in which aphasia existed. Pfleger[38] and Henkes have also found the sole lesion to be induration of the cornu ammonis. Of Pfleger's[39] 43 autopsies, atrophy and sclerosis of the cornu ammonis were found twenty-five times, and it was noted that the extent of the morbid change bore relation to the violence and frequency of the seizures.

[Footnote 37: _Sallanzani_, Modena, 1879, viii. 550-557.]

[Footnote 38: _Allg. Zeitschrift f. Psychiatrie, etc._, Berlin, 1879, xxxvi. 359-365.]

[Footnote 39: _Ibid._, lxxvi., and _Arch. de Neurologie_, No. 2, 1880, p. 299.]

In many examples, especially where the disease has been found to be unilateral and associated with more or less hemiatrophy, the autopsy disclosed a corresponding hemiatrophy of the brain. Many such cases are reported. I have frequently found epilepsy in association with cerebral hypertrophy, and as a symptom of cerebral tuberculosis it has long been recognized, and numerous cases are reported in which for a long time the paroxysms were the only manifestations of the condition. In one of these cases, reported by Luys,[40] the bulb was found involved by tuberculous matter.

[Footnote 40: _Archives gén. de Méd._, 1869, ii. 641 _et seq._]

{492} Convulsions have very frequently been noted in association with imperfect cerebral development, and Echeverria laid great stress upon the hyperplastic increase in volume of certain parts of the brain.

Marie Bra[41] has thus summed up her conclusions relating to the morbid anatomy of epilepsy:

“1. The mean weight of the brains of epileptics is less than the physiological mean.

“2. The cerebellum is greater than the physiological mean.

“3. There frequently exists an asymmetry between the lobes (not peculiar to epilepsy). The increase of weight is sometimes found on the right and sometimes on the left side. Equality is the exception.

“4. In no form of mental disease (excepting perhaps general paresis, which is accompanied also by epileptiform crises) have we met with so marked and constant a variation between the weights of the hemispheres as exists in epilepsy.”

[Footnote 41: Referred to by Axenfeld.]

Drasche, Green, Greenhow, Löbel, and others have detailed cases in which tuberculous deposits were undoubtedly the causes of the disease.

Kussmaul and Tenner, Hoffman, and others have held that a stenosis of the superior part of the vertebral canal may explain, through pressure upon the cord, the genesis of the attack, and Kroon found asymmetry of the medulla oblongata.

The microscopical changes that have been found in brains where no gross lesion was apparent are by no means distinctive. I have myself examined the brains of many epileptics with discouraging results. The varying granular cell-degeneration, capillary dilatation, and exudative changes are common enough. In several cases of cortical epilepsy I found more or less advanced degeneration of the great cells in limited regions.

By far the most important and exact changes are those observed in the cases of sensory epilepsy. I have elsewhere collected some continental cases. In brief, areas of occipital softening or degeneration have been discovered in those cases with hallucination, sensory expressions, and hemiopia. In one case attended by hallucination of smell the autopsy disclosed the following: