In the hereditary form the disease in the great proportion of cases begins before the twentieth year; and, so far as my experience goes, this kind of the disease makes its appearance at a very early age. The part played by particular predisposing influences appears to be well defined. The children of syphilitic parents develop the disease at a more advanced age than when alcoholism is found to exist. In adult males, when the disease develops late in life, it is almost always possible to find syphilitic or coarse brain diseases, while among women the history of antecedent migraine or menstrual derangement is nearly always present, and the convulsions in a very large number of instances have a hysteroid character.
The excitingCAUSESof the disease are quite numerous. Traumatism is a frequent and important etiological factor, and the head-injuries may be recent or remote. It is quite common to find old fractures, with depressions which have existed for years without any seeming bad effects, suddenly lighting up convulsions under the influence of some new excitement. Under such circumstances the depressed bone is quite apt to give rise to symptoms suggestive of meningeal irritation and inflammation, so that the diagnosis is comparatively easy. Several observers have called attention to epilepsy which has been undoubtedly due to cicatrices not only of the scalp, but elsewhere, and these may or not be found in association with osseous lesions. The literature of the subject is replete with curious cases which go to show that epilepsy may occur from a few days to many years—even twenty—after the initial head-injury. Unsuspected cortical pressure, the inner table being alone depressed, is common; in fact, the cases in which the most serious mischief is done seem to be those where the only external evidence of violence is the contused scalp. As a consequence of such injury we may have exostoses developed.
The influence of syphilis in the production of epilepsy is one of very great importance. Not only has specific epilepsy characteristics of its own, but its origin may be distinctly traced to syphilitic infection. Cases dependent upon gross cerebral disease, such as meningitis or gumma, are excluded from consideration, but it is conceded by all syphilographers that an epilepsy may mark the second stage of the disorder, and its pathological dependence is probably a simple vascular disturbance which cannot be determined after death. So-called specific epilepsy may be congenital.
Orwin1mentions as a cause the influence of prolonged lactation. In several cases I have seen a metrorrhagia, or a loss of blood from hemorrhoids has been followed by a readily curable epilepsy.
1Prov. Med. and Surg. Journal, London, 1862, v. 48.
As eccentric irritating causes may be mentioned intestinal worms, but I am convinced that it is too often the fashion to ascribe convulsions in children to intestinal parasites: in very young children, however, there are frequent examples of the disease in which the attacks are precipitated by worms. The fits are usually very severe, and are not regular in their appearance, occurring at night-time more often than during the day, and, though they usually disappear when the bowels are cleared of their unpleasant occupants, may recur when once initiated, even though anthelmintics of the most powerful kind are employed. Gall-stones are mentioned by Ross as an eccentric cause of the disease, but I have never witnessed a case of this nature.
Sudden terror, fright of all kinds, morbid example, and other psychic causes are detailed, and undoubtedly all have more or less influence. Hysteroid attacks are notably precipitated by these mental causes, and all forms of the disease are greatly modified by abnormal exercise of the mind.
A number of writers, among them Baly2and Booth,3have called attention to cases of the disease dependent upon carious teeth. I have seen but one such case, where a wisdom tooth produced so much violent inflammatory action that middle-ear disease followed, and with it subsequent extension to the brain took place.
2London Med. Gazette, 1851, xlviii. 534-540.
3Am. Journ. of the Med. Sciences, 1870, N. S. lix. 278.
In rare cases the administration of anæsthetics is followed by epilepsy, and Gowers alludes to a case in which convulsions were due to the inhalation of nitrous oxide gas.
Concussion of the brain as the result of railroad injury or falls may give rise to a progressive epilepsy which is usually of serious character.
Reflex causes play a prominent part in many instances, though I am inclined to think that their importance has been greatly exaggerated. This is especially true of so-called uterine epilepsy. It cannot be doubted that difficult menstruation, ovarian neuralgia, etc. are found in connection with epilepsy, but whether as a cause or effect it is not always possible to say. The fact that in some women we find accès at periods identical with menstruation would point to a very close relationship. Carstens4reports a case due undoubtedly to stenosis of the cervix; Cohen,5an example in which there was a uterine fibroid; and others have spoken of erosion of the cervix, etc. as possible explanation of the seizure.
4Detroit Lancet(8), 1880, N. S. iii. 153.
5Wochenschrift f. d. ges. Heilkunde, Berlin, 1839, vii. 648, 673.
The toxic forms of epilepsy hardly need discussion in this article. Metallic poisoning, which gives rise to a veritable plumbic encephalopathy, is rather the cause of a symptomatic than generic epilepsy. Curious cases of epilepsies which have followed the use of oil of tansy (Mitchell6), ergot, absinthe (Magnan7), and various drugs show that occasionally their mode of origin may explain the convulsive seizure. Alcoholic epilepsy I do not regard as being the rare affection some authors consider it. In cases of prolonged saturation, where perhaps there are no other symptoms of chronic alcoholism, I have found it perhaps associated with the trance state (cataleptoid) or appearing in the psychic form.
6Cincinnati Lancet and Clinic, 1881, N. S. vi. 479.
7Recherches sur les Centres nerveux, Paris, 1876.
In old persons I have found gout to have an undoubted influence in producing the disease, there being a spasm of the cerebral vessels which usually betokens a condition of uric-acid saturation. In these cases the painful symptoms were not decided. Such epilepsies have quite often preceded serious evidence of arterial degeneration.
Malarial epilepsy is rare: Jacobi8reports a case and Payne9another. A young man was brought to me last year who had lived for many years in a part of Pennsylvania which was exceedingly malarious. His attacks, which were more or less periodic, were violent, and his convulsions weregeneral and attended by very great preliminary rise of temperature and intense congestion of the face and head. The patient was unusually somnolent, and between the paroxysms frequently suffered from facial neuralgia. The influences of change of habitation and quinine determined the correctness of my diagnosis.
8Hospital Gazette, New York, 1879, v. 41-43.
9Indian Ann. Med. Sci., Calcutta, 1860-61, vii. 597et seq.
Day10and Kerr11have both ascribed epilepsies seen by them to hepatic congestion.
10Clin. Histories, etc., London, 1866, 143-145.
11Med. Times and Gazette, London, 1871, i. 568.
The important etiological relation of the exanthemata to epilepsy has been the subject of much attention. The books are full of cases which owe their origin not only to scarlet fever and measles, but to whooping cough, diphtheria, and the various zymotic fevers. In 35 cases tabulated by Gowers12(p. 28) no less than 19 were due to scarlet fever, while the first fit followed measles in 9 cases. So far as my individual experience goes, I have in 23 cases found epilepsy to be the result of scarlet fever, and this form of the disease was often associated with other manifestations of coarse brain trouble. Cerebro-spinal meningitis was the undoubted cause of epilepsy in 6 cases I examined. It is probable that just as smallpox acts upon the nervous centres, so does scarlet fever, and I agree with Gowers that the convulsions that may begin during or just after the fever are not always due to uræmia. Bright's disease may give rise to an epilepsy, but this can hardly be regarded as a distinct affection.
12Op. cit.
There are many cases, especially in adults, which grow out of a prolonged depletion of the brain—a continued cerebral anæmia. Among these cases are some which depend upon pressure upon the great vessels of the neck, and some due to debilitating disease of a general character. Schulz13has seen a case which followed compression of the jugular vein, and enlarged cervical glands have in other cases acted as mechanical agents. Hammond alludes to the influence of prolonged general anæmia in the production of epilepsy.
13Cor. Bl. Deutsches Gesellsch. f. Psych., Neuwied, 1855, ii. 35.
While I do not place much reliance on the claims that have been made regarding the frequent dependence of the disease upon preputial irritation, or that it may arise from phymosis or urethral stricture, it cannot be denied that some cases have originated in difficulties of the kind, and especially the former. Simmons14reports a case of attachment of the prepuce to the glans in which a cure followed separation. Disease of the testicles in certain cases plays a part in its genesis, and Liégey15details an epilepsy clearly due to testicular pressure. Some years ago I saw a case in which the pressure of an improperly applied truss, I am now convinced, had much to do with the development of the convulsions. The alleged sexual causes are many, and some of them are very doubtful. A fanciful continental writer, Montmeja,16believes that sodomy explained the appearance of the disease in one of his patients.
14Am. Journ. Med. Sci., 1880, N. S. lxxix. 444.
15Gaz. méd. du Strasbourg, 1856, xvi. 105-107.
16Rev. Photo. des Hôpitaux de Paris, 1873, v. 229-232.
The existence of vesical calculi as an exciting cause cannot be disregarded, and, while rare, the observations of Duncan,17Muscroft,18and oneor two others have shown that the removal of a stone was followed by a cure of the epilepsy.
17Ed. Med. Journal, 1868-69, xiv. 140.
18Arch. Sci. and Pract. Med. and Surg., 1873, 1360.
Masturbation is a popular etiological factor. I really do not believe, even in face of the numerous alleged cases that have been recorded, that the habit of self-abuse often results in genuine epilepsy. So far as my experience goes, onanism is practised by epileptics as well as by healthy boys, and when indulged in to excess is due to the congenital moral deficiency which is so common, especially in hereditary cases. In rare examples the frequently-repeated act may give rise to a form of the disease of the nature of petit mal.
Gastric disorders have attracted much attention, especially from Paget, who speaks of a gastric epilepsy and reports cases. While I do not believe in the sole etiological influence of digestive derangement, I have too often witnessed examples in which disorders of this kind markedly influenced the precipitation of attacks and the duration of the disorder.
Occasional cases of peripheral origin have been from time to time presented, and go to support in some measure the pathological views of Brown-Séquard. Among observers who have brought forward cases besides those referred to on a previous page are Lande,19who reported a case of epilepsy dependent upon injury of the right median nerve, and Short,20in whose case a neuroma explained the cause of the convulsions. Billroth,21Garnier,22Brown-Séquard,23and Raymond24have brought forward cases where injury of the sciatic nerve was the origin of the trouble, and in more than one instance a cure was effected by excision. It seems strange that a bone dislocation should have anything to do with the genesis of epilepsy, yet in one case reported a severe dislocation at the shoulder-joint explained the appearance of the attacks, and reduction was speedily followed by cure.
19Mém. et Bull. Soc. de Méd. et Chir. de Bordeaux, 1878, i. 56-65.
20Med. Essays and Observation Soc., Edin., 1737, iv. 416.
21Archiv f. klin. Chir., Berlin, 1872, xiii. 379-395.
22Union médicale de Paris, 1872, 3d S., xiii. 656-658.
23New York Medical Record, 1872, vii. 472.
24Rev. méd. de Limoges, 1869-72, iii. 102-105.
I have seen several cases where disease of the internal ear or injury of the temporal bone gave rise to the most obstinate and violent epilepsies. Westmoreland25and others report such cases, but more often the epilepsy is only symptomatic of pachymeningitis or abscess. Some years ago I presented26a case of genuine epilepsy in which the seizure was produced at will by irritating the meatus auditorius. By simply blowing into the ear the same effect would be produced. Since then Blake and others have related examples. Quite lately a writer inBrainhas collected other cases of this species of auditory epilepsy. A year or so since I examined a patient in whom not only hemi-epilepsy, but other unilateral symptoms, followed erosion of a large part of the mastoid and petrous portion of the temporal bone as the result of a bullet wound.
25Atlanta Med. and Surg. Journal, 1876-77, xiv. 717-719.
26New York Medical Record, 1878, xiii. 107-109.
The influence of climate and varying barometric pressure has been considered by Delasiauve. His conclusion was that the attacks were much more common during the season of the year when the prevailing winds were from the north-west, north, or south-west.
PRODROMATA.—There are various minor disturbances of sensation and motion which may not amount to the dignity of an epileptic attack. These may be so fugacious as to escape the attention of the persons in whose company the patient may happen to be, or he himself may be unaware of any disordered state of feeling. They may precede a severe paroxysm, when they are known as warnings or auræ. The term aura was originally applied to the familiar premonitory sensation which is so often likened by the subject to the blowing of wind over the skin, from whence it receives its name, but it has come to be applied to all primary indications of a fit. Such auræ may be sensory or motor—in the preponderance of cases the former, for motor precursors are quite rare, and when they occur are most likely to be but one stage, though a slight one, of the convulsion itself. There is no general rule about the occurrence of an aura, but, so far as my experience goes, there is great constancy in the character of the warning in each particular case. The sensory disturbance may vary from a vague feeling of confusion to a well-marked sensation. In many instances the patient speaks of an indescribable mental disturbance, which may precede the attack and last anywhere from a few minutes to several hours. This confusional state or psychical aura is most protean in its expression. It may simply be a heavy feeling, a feeling of tension, a sleepy feeling; a restlessness which is manifested by the patient changing his position frequently or wandering forth into the streets; an irritability of temper which often lasts twenty-four hours or more, and during the display of which he rebukes those who may be solicitous about him, or wantonly destroys articles of furniture, or vents his spleen upon inoffensive persons. I have had epileptic children under my charge who were wont to bite their little brothers and sisters or their nurses. A feeling of terror sometimes precedes the attack, and very often there is a sense of impending danger which has no basis whatever, and with it is associated a depth of depression which is very painful. In other cases the patient manifests a strange exhilaration, which may precede the occurrence of the attack for a period of from one or two hours to two or three days; and this is made manifest by great loquacity and a lively play of spirits. It is not rare to find errors in the speech as indications of an approaching attack. A minor degree of aphasia, slowness of speech, or anarthria betrays occasionally the preparatory state which is the precursor of a severe convulsion. By far the most common warnings, however, consist of disorders of the special senses, and generally these are visual. From an inspection of my notes I find that the patients saw colored lights, rings of fire, bright objects, dark spots, luminous clouds, a flood of light, sparks, stars, bright balls, lights which approached them, lights which receded, fireworks, and all became dark. While many were unable to define the color perceived, I found among those who were positive that red was the color most frequently seen, while blue came next; and this is a conclusion which I believe is accepted by Jackson and others who have analyzed their cases.
Hemiopia and diplopia in rare cases precede the major attacks, and are sometimes associated with distal pain and anæsthesia and with supraorbital pain as well.27Among these ocular warnings we find constriction of the visual field to be often present, especially in cases where there is ahistory of migraine. Vague disorders of hearing, which may even amount to the dignity of hallucination, are complained of by some persons. There may be simply roaring in the ears or a sound of bells, and in one instance my patient declared that he heard whisperings at the time of the seizure. Some patients smell smoke or other foul odors, and in exceptional instances the odor of some particular flower or of some aromatic substance, such as camphor, turpentine, or tar, is perceived by the epileptic; and these are probably psychical.28Sometimes there is a feeling of great suffocation, constriction of the chest or of the throat, palpitation, or vertigo.
27See Sensory Epilepsy.
28For curious examples of this kind consult Sir Charles Bell'sNervous System of the Human Body.
There are disorders of cutaneous sensation of great diversity of character, but those auræ which are of the most constant occurrence are the epigastric, which consist of a vague sensation starting below the sternum and ascending, its arrival at the throat being coincident with the commencement of the fit, and the patient very often likens its culmination to the violent grasp of a strong hand. So, too, we find crawling sensations starting in the extremities and running up to the trunk. These have been compared to the contact of insects in motion or to the blowing of wind over the surface. There may be tingling in one or two fingers or the whole hand, and such sensations may be unilateral or bilateral. It is quite common for the sensory warning to begin in the hand and foot of one side and to run up to the knee and elbow. Sometimes the tongue becomes hyperæsthetic, and I have frequently found that the gums became exquisitely tender just before the attack. According to Gowers, 17 per cent. of his cases began with unilateral peripheral auræ, but I think this is too small a proportion, for, so far as I have observed, at least 30 per cent. of all my cases in which any auræ at all could be ascertained presented the history of a one-sided warning, beginning most often in the right hand. Gowers says that in three-fourths of his cases in which the attack began in the hand consciousness was lost before the seizure extended beyond the arm, while in the others it extended much farther before the actual fit was precipitated. In Gowers's cases he rarely found that unilateral auræ were associated with other warnings; and his experience, which is like my own, goes to prove that unilateral sensory auræ and one-sided initial motor expressions go together, and very often indicate gross organic disease. In some cases there may be for several days a decided unilateral or general muscular weakness or recurring chronic spasms which may be frequently repeated. In aborted or irregular attacks there are also peculiar motor symptoms, to which reference will be made later on.
In two or three patients I have found that the attack was immediately preceded by a profuse discharge of saliva, and in one case the patient had frequently bleeding from the nose. Several authors have called attention to certain local vaso-motor disturbances which are expressed by limited patches of cutaneous hyperæmia or anæmia, so that the fingers—or, in fact, the whole hand—may either be swollen and of a dusky-red color, or, contrariwise, there may be blanching and an apparent diminution in size.
Sometimes the patient immediately before the attack makes more or less automatic movements, pressing his hands to his head, clasping his breast,or clutching at some imaginary object, and after this he loses consciousness and falls to the ground. In some irregular cases the patient runs aimlessly for some distance or describes a circle, and after a variable time, which rarely exceeds a minute or two, the actual fit begins. Here are two examples:
Case I.—Boy aged fifteen, has been subject to somnambulism; attacks began two years ago. He frequently when sitting at table rises suddenly, and runs either around the room or out into the street if possible. In thirty seconds or so he falls to the ground in a convulsion. Always falls backward in opisthotonos.
Case II.—Man aged thirty-four. Attacks always begin by stage of violent temper. While unconscious he runs about office, striking all who try to restrain him; finally falls to the floor. Convulsions, when they occur, are always severe. Sometimes running attack is the sole feature.
This disposition to run is no less remarkable than another queer prodroma I have seen in several patients, who began to take off their clothing when first seized, no matter where they were or under what circumstances. This is not, as has been suggested, the remains of a half-formed idea that they must seek their beds because of their impending trouble, but it is a much less complex mental act, and the several patients I have seen were fully unconscious when they did this, and were in places where there was no bed within reach. Equally curious mental precursors of the attack have come to my notice, and these I will detail subsequently.
SYMPTOMATOLOGY.—The Light Attack.—The epileptic attack may, as I have said, be scarcely perceptible to those about the patient, or may consist simply of a momentary loss of consciousness and very feeble convulsive movements. Reynolds has described two forms: (1) That without evident spasm; (2) that with evident spasm. Such seizures are always fugacious, and consist merely in some transient loss of consciousness and very little or no convulsive movement. The tonic spasms prevail, if any, and the disorder of motility may often consist simply in the arrest of some act in the performance of which the patient is engaged. While playing the piano the patient's hands may for a moment remain suspended over the keys he is about to strike; if eating, the hand which holds the fork may be arrested between the plate and his mouth. The attack consists sometimes in the rolling upward of the eyeballs, or when crossing the room the patient may stop, remaining quiet for an instant. Temporary unconsciousness, shown by cessation of conversation, by change of color, and absence of intelligent expression, accompanies the other trouble. In a well-marked attack of petit mal the patient may move his lips convulsively, and remain otherwise quiet, but bereft of consciousness, for one or two minutes.
The patient sometimes loses himself and loses the thread of the conversation, repeating what he has just said or showing his want of appreciation of what his companion has said. To this light grade belongs the case reported by Jackson of the individual who blew his nose upon a piece of paper and gave the conductor £2 10s.instead of twopence halfpenny.
The Major Attack.—An attack of epilepsy of the familiar severe form may or not follow an aura. The first intimation to the bystander may be a noise made by the patient, which is either a loud, startling, wild cry, or a gurgling groan due to compression of the thorax and theforcible escape of wind through the vocal cords. There are three stages of the attack: (1) The stage of tonic convulsion; (2) the stage of clonic convulsion; (3) the reactionary stage.
The first stage of the attack is symptomatized by tonic spasms, which may be local or general, usually the latter. It is very often unrecognized, for its duration may be so short that it is lost in the stage of clonic spasms, which is much more protracted. There is usually unilateral seizure, the muscles of the face being primarily involved, then those of the hand and upper extremity, and then those of the lower extremity; and finally there is a general involvement, so that the patient may be in a position of opisthotonos. In some cases there is strong tonic contraction to one side, or pleurosthotonos. The notes of a case which illustrates the beginning and development of convulsion with reference to the parts involved, which I observed carefully, are the following:
Bindewald: Epileptic attack observed at hospital for paralyzed and epileptic, Sunday, Mar. 12, 1882:
1. Long, shrill cry which attracted my attention. It probably lasted five seconds. At same time patient threw up arms and became unconscious, and fell to floor. Nurses ran to him and placed him upon bed.
2. Tonic convulsions began by fine twitchings at right corner of mouth. These became gross, and were separated by succeeding long intervals. Eyes directed to left side, face pale.
3. In twenty seconds twitching began in right hand and arm, which were rigidly flexed (five seconds); then leg and foot of right side became agitated, the face meanwhile changing in color successively from red-gray to purple; lips purple, ears livid and purple, edges white; eyes still turned to the left, pupils dilated, eyes widely open; breathing stertorous and irregular.
4. General convulsions of right side.
5. Head suddenly twisted to left side; position of eyes the same. Chin drawn down, movements moderated; still livid. A fit of coughing and expectoration of much frothy mucus. Left side, with exception of head, not implicated. Whole attack lasted about one minute and thirty-five seconds. Deep sleep afterward, lasting forty-five minutes.
This attack was one of many in a confirmed epileptic, and is a fair example of those commonly met with, though not as general as we sometimes find. In most cases the attack appears to be very much longer than it usually is, and the phenomena noted above, which seemed to occupy a considerable space of time, really lasted but little more than a minute and a half. Axenfeld and Beau fix the average period of the attack as follows: “Duration of the complete attack, which Beau divides into four phases: first stage, tetanic stage, five to thirty seconds; second stage, clonic convulsions, from one to two minutes; third stage, stage of stupor, three to eight minutes; fourth stage, return of sensibility and intelligence. It is not complete until the end of from ten to thirty minutes.”
To be more explicit, the manifestation of an ordinary epileptic attack of the more severe kind is very much like this: Usually without any warning to those about the patient he utters a shrill, peculiar cry of a character never to be forgotten if once heard, and then, perhaps throwing up his hands, he falls to the ground rigid and contorted. His body maybe arched laterally or antero-posteriorly, his legs are thrown out, his forearms flexed, and his fists doubled, the thumbs being beneath the other fingers. His face may be for a moment flushed, but the color recedes, and it assumes a dusky-bluish tint, the lips being ashy-gray. The eyes are usually open, and the balls are rolled up and the pupils widely dilated. The breathing may for a moment be suspended, but it soon quickens, and becomes labored and noisy, and the pulse grows hard and full. The tonic contractions are succeeded in a very short time by more or less violent clonic contractions, which increase in violence and rapidity; the jaws work and the lips are covered with foam, which is blown in and out by the rapid inspirations and expirations. It may be tinged with blood in the severe attack if the tongue is bitten, which is by no means an uncommon accident. The teeth are sometimes firmly set and the jaws locked. The head is usually drawn to one side in the first stage, but afterward may be rolled from side to side. The movements are now more or less general, and occasionally the agitation is so great that the patient throws himself from the bed on which he may be lying. The face grows more pale, or rather more livid, and toward the end of this stage there may be a puffiness and congestion such as are seen in partially asphyxiated individuals, for this alteration in color is due to dyspnœa and consequent imperfect oxidation. The patient may defecate or pass his urine unconsciously, and sometimes we find seminal emission. The movements, after a period varying from ten seconds to a minute or two, become less violent, and he may talk in a silly manner, as a person does who is recovering from profound ether unconsciousness; or deeply sigh, and he is restless. The pulse is now much weaker and more rapid, and may be irregular. The color returns to the face, the patient closes his eyes, and the body is covered with profuse perspiration. The fingers are unlocked and every evidence of spasmodic movement disappears. He falls asleep, and remains so for several hours, awaking with a confused feeling, headache, and no remembrance of the attack, and is only reminded that something has happened by his wounded tongue or lips, the bruises he has received, or by the information of friends. He looks jaded and tired, and is indisposed to exert himself for several days if the attack has been at all severe. The transition from the attack to the normal state is not always the same. Some patients do not sleep at all, but after being dazed go about their occupation. This is even true occasionally of the severe form of disease.
The usual termination of the attack is, however, by sleep preceded by a period of confusion. The patient, after coming out of the clonic stage, mutters incoherently. He is apt to pass large quantities of wind from his bowels, or vomits. This is attended by a subsidence of the spasmodic movements, and perhaps by oscillation of the eyeballs. The pulse loses its rapid, hard character, and the reaction brings with it diminished frequency of respiration and the evidence of exhaustion.
Special Symptoms.—The eyes are, as a rule, open, and, there being spasmodic movement of the ocular muscles, we find that the balls are either rolled up or directed away from the side in which the spasms begin. This is especially true in those epilepsies due to cortical disease, and the same law of conjugate deviation laid down by Bourneville may be remembered.
The pupils are dilated pretty much throughout the fit, though they may vary, and a transient contraction may occur at the commencement of the first stage. During the clonic stage, especially toward the end, they not infrequently undergo a species of oscillation. The interparoxysmal state is revealed by a very great mobility of the pupil, which has been observed by Gray and others. Gray is disposed to consider it a diagnostic indication of value, but so far I have found it only in two-thirds of my cases. Dilatation of the pupil I believe to be a very constant feature of epilepsy.
The ophthalmoscope reveals in certain cases an abnormal increase in the circulation at the fundus, in others a very decided emptiness of the retinal vessels. Jackson is disposed to consider that certain visual auræ depend upon spasm of the arterioles in this location. Loring, whose opportunities for research have been very great, is not disposed to attach much importance to the ophthalmoscopic appearances, at least during the periods between the fits.
The pulse of the epileptic between the paroxysms is small and irritable. Voisin has found the following changes: “Two or three seconds before the attack it becomes rapid, sharp, and the sphygmographic curves are higher, rounded, and nearer together. When the attack begins we see five or six little undulations in the course of the ascending line, and the curves are higher and more accentuated. Several minutes after the attack there is dicrotism, and the line of descent is very sharp, the angle with the ascending being quite acute. This form of pulse lasts an hour or half hour after the attack. There is in some cases great irregularity, with paroxysms of cardiac pain resembling angina pectoris. There is occasionally epistaxis or more marked hemorrhages.” Parrot speaks of hemorrhages from the eyes and ears, and occasionally the cerebral congestion is so great as to result in cerebral hemorrhage in old subjects.
The temperature is usually lowered before the attack, but the surface temperature is increased during or after the second stage.
Cutaneous sensibility is often very much disturbed. Spots of hyperæsthesia and anæsthesia are sometimes left after the attack. The scalp is not infrequently exceedingly tender. The sensory troubles have been alluded to as prodromata of the ordinary motor attack. Some attacks of the sensory variety in which psychical excitement plays a part are characterized by unilateral and persistent formications.
A consequence of some epileptic paroxysms is the appearance of petechiæ, chiefly upon the face, neck, and upper extremities. The skin of old epileptics is harsh, cold, and rough, and the face is apt to be studded with spots of acne even when the patient is not taking the bromides. The hair is stiff and dry, and the ears and tip of the nose are apt to be the seats of a passive and old hyperæmia.
The urine of epileptics is apt to contain evidence of muscular waste, and an increase in the amount of earthy phosphates as well. Zapolsky found, however, that immediately after the attack there was diminution in the quantity of the phosphates. The occurrence of glycosuria has been noted by numerous clinicians. De Renzi29has published an interesting article. I have seen no less than six well-marked cases within two years in which constant glycosuria was a feature of the disease, but in two ofthe patients well-marked symptoms of disease of the pons were present. After the paroxysm it is by no means uncommon to find the urine loaded with albumen. Otto, Mabille, Saundby, Bazin, and other writers speak of a transitory albuminuria. Kleudgen,30however, does not attach much importance to this feature, believing that the albumen is often due to semen.
29Gior. internaz. d. Sc. Med., Napoli, 1880, ii. 357-359.
30Archiv für Psychiatrie, etc., 1880, xi. 478-506.
The tendinous reflexes are frequently abolished during the attack, but not always so, and in several cases in which I was enabled to make a test during the convulsion I found that the patellar reflex was very active, and in one case elsewhere reported it was transferred. The skin reflexes are ordinarily exaggerated.
Tongue-biting is, I think, a more common feature of the nocturnal than the other attacks. It is rare in infantile epilepsy, and is always a bad feature. The wound is sometimes very serious, and cases are mentioned where the tongue has been severed. An occasional sequel of the attack is a urinary difficulty and vesical tenderness. It is sometimes connected with great urethral irritability and spasmodic stricture, which prevents the introduction of a sound or catheter. Romberg speaks of the supervention of asthma and dysphagia.
THEIMMEDIATE ANDREMOTEEFFECTS OF THEEPILEPTICPAROXYSM.—As a result of violence we often find wounds and bruises, quite rarely fractures, but more often dislocations. Cases have been communicated to me where as a result simply of the great muscular force the humerus has been dislocated at its superior articulation. Muscular pain of great severity, and sometimes of great persistency, follows unusually severe fits, and rupture of muscular substance is not uncommon. An epiolecranon bruise in one of my cases produced a severe neuritis which was very intractable. In old cases, according to Axenfeld, there may be great muscular hypertrophy, the sterno-mastoidii attaining the size of the biceps, and in other cases there is fatty degeneration. He also calls attention to defects that may be due to frequent exercise of violence upon bones through repeated exaggerated muscular contraction. Paralysis of nerves which supply convulsed members is mentioned.
The psychical effects are various. For several days following the attack there may be simply confusion of ideas, irresolution, or drowsiness, which subsides in a short time. In not a few cases I have regarded the attack as beneficial in the sense of an explosion of relief when perverted mental states had preceded it. In such persons the discharging lesion was followed by a very conspicuous restoration of the mental equilibrium.
Occasionally the attacks are terminated by great violence or screaming or the commission of purposeless acts.
Post-epileptic aphasia has been described by Winslow, Moreau, and others, and among my own cases of the sensory variety of this disorder I have met with speech disturbance. The aphasia is of short duration, and consists either in a total incapacity for verbal expression or a transposition. It is not rare for it to be associated with the commission of a number of quasi-automatic actions.
Unilateral epilepsies are quite apt to leave behind them a species of paresis which may last even for several days. The loss of power is confined to the convulsed members, and may be accompanied by tingling.In the greater number of instances, however, there is some central organic change, and the epilepsy is purely symptomatic.
Deafness, amaurosis, and other pareses of the organs of special sense are rare sequelæ of the epileptic state.
The remote effects of the grave disease are not so decided as when the patient has been the subject of petit mal. Slight repeated losses of consciousness are apt to be followed by mental decay. The ultimate result is mental enfeeblement, a progressive and very great loss of memory, which advances to such an extent that a veritable dementia ensues. With this there is usually a very decided perversion of the emotions and affections, so that a good-natured, amiable child may in a few years become everything that is bad and trying, and the acts of mischief are almost inconceivable. Theft, incendiarism, and various moral perversions are common in some chronic epileptics. The dementia, it is true, is tardy in its establishment, but it comes eventually if the individual lives long enough.
In some individuals there is a very early tendency to the development of mania; there is a certain periodicity about the explosions, and when established the excitement either precedes the attack by a few days or occurs shortly afterward. The violence is characteristically acute, and such insanity very often makes itself known in homicidal acts rather than in those of a suicidal character. Hypochondriasis is quite likely to follow continued epilepsy.
It is the rule for epilepsy to undergo decided modifications in the beginning of its course. The first attack may be simply eclamptic, without any peculiarities or definite character, and with recurrence there is a tendency to regularity and constancy in expression. Infantile convulsions, that may occur at any time after inconsiderable exciting causes, may eventually be confined to the early morning or night. So-called fainting attacks may precede petit mal, and headache may be the precursor of ill-defined seizures. So, too, the relation of grave and light attacks may vary. In the beginning there may be nothing but attacks of petit mal, while later these may be supplemented by severe fits, and even disappear entirely. So far as my own cases go, I find that nearly two-thirds of the entire number happen at night or in the early morning, while the others may occur in the day or at any other time, or by day and night. Besides the terms nocturnal and diurnal, we may use the word matutinal in relation to the time of attack. So far as the number of attacks is concerned, we find great irregularity. It is not always possible to count them, or even to recognize them, for the examples are numerous where nocturnal attacks have been undetected for years, and have finally been followed by fits during the daytime. I have cases who have seizures but once or twice a year, and others who have ten to forty or fifty daily. In some cases there may be eight or ten attacks of petit mal daily, and but two or three grave attacks during the week.
The statistics of Delasiauve and Leuret go to show that of 296 cases of epilepsy, the cases of general epilepsy were most common—that is to say, the attacks which occur both by day and night.
Delasiauve, Herpin, and others make delicate distinctions between the attacks, and the former grades the seizures beginning at accesses, and successively advancing to vertiges, accès intermédiares, and attaques or accès compléts.31In fact, these are but varying degrees of violence of the discharge, after all. We thus have light discharges and severe discharges at different times, or, as the habit is established, only the light or only the severe, the manifestation depending probably upon the number of discharging cells and the importance of the exciting cause.
31Traité de l'Épilepsie, etc., Paris, 1845, p. 55et seq.
When the attacks occur in alarming frequency, as they sometimes do, the condition is known as the status epilepticus. Leuret had a patient who had eighty in two hours, and Delasiauve reports the case of a young man fifteen years old who had twenty-five hundred in one month. They may be so numerous as to be apparently continuous. The patient remains in a state of coma (the status epilepticus), with very high temperature. If he be not restored, he sinks into a deeper coma, and all the signs of collapse manifest themselves. Bed-sores form, œdema of the lungs ensues, and the patient dies. Happily, this condition of affairs is rare.
Delasiauve calls attention to the fact that the first two or three attacks that usher in this state do not usually attract much attention, but the succeeding ones are so violent as to immediately suggest violent consequences. In one of my cases the attacks, when they had once become numerous, were readily excited by the least jarring, noise, or handling, just as we find in strychnine-poisoning or tetanus.
Irregular Forms.—There are occasional cases of psychical or masked epilepsy, the study of which is intensely interesting. Such forms are characterized by perverted consciousness and a low degree of volitional direction which may vary from automatism to the undoubted exercise of complex functions of the mind, though badly co-ordinated. Mesnet's soldier, when subject to a paroxysm and apparently unconscious, would perform a number of suggested acts in a rhythmical manner and with no subsequent knowledge of the previous event: when started off by the word of command to march, he would blindly go on, marking time when he met with an obstruction until stopped, or when a paper and tobacco were placed in his hands he would proceed to roll an unlimited number of cigarettes.
Two cases of a more complex exercise of certain intellectual powers, while others were dormant, came under my observation some time ago. One of them was a young man of twenty-three, who had had irregular epileptic seizures for some years. He went to bed one evening as usual, arose, and breakfasted with his family without creating any suspicion that he was at all unwell. He then went down to his place of business, and after his arrival was sent to a distant part of the city for some tool. On his return down town he stopped at a tobacconist's and became involved in a quarrel with one of the persons in the shop. A policeman was called, who, more intelligent than many of his class, immediately detected something queer about the man, arrested him, and afterward took him to Bellevue Hospital. There he remained three days, and suddenlyreturned to consciousness and a knowledge of his surroundings, but was entirely ignorant of his unfortunate experience. It is unnecessary to say his habits were perfectly good and he was not drunk at the time of the quarrel or arrest. His last recollection was that of going to bed the night before the day of his arrest.
Another case of unusual interest which came under my care, illustrating a phase of sensory epilepsy, is worthy of reproduction:
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 speechdifficulty. 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 8.15P.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 rememberperfectly 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.
SENSORYEPILEPSY.—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.32The notable examples of Sommers, Bergmann, Tagges, Guislain, and others belong to this category.
32I 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.