Volume 5 — DISEASES OF THE NERVOUS SYSTEM. (continued 23)

Patients complain of habitual dryness of the mouth and of an insipid, mildly astringent, sweetish, or faintly metallic taste; the tongue is coated, the breath fetid. There are nausea and occasional vomiting. Constipation is common. The patient is now liable to attacks of lead colic, joint trouble, and the specific palsy, or to cerebral disturbances of grave character. Of these affections, colic is the most frequent; the joint affection is next in order; the paralysis far less common, and the cerebral disturbances comparatively rare. Continued exposure results after a time in an intensification of the malnutrition, either with repeated attacks of colic or the other specific disturbances, or occasionally in their absence, although at length the positive lead dyscrasia is established, characterized by obstinate dyspepsia, constipation, and intensification of the curious earthy or yellowish color of the skin, which is succeeded by the pallor of profound anæmia. Loss of muscular power with transient œdema now shows itself. The patient becomes apathetic, irritable, and morose. As the condition of cachexia deepens muscular tremor occasionally shows itself. After a prolonged period of profound ill-health the patient succumbs to some complication, among the more frequent of which are tuberculosis, pneumonia, and chronic interstitial nephritis. Gout is a very common complication of chronic lead-poisoning.

2. Lead Colic; Colica saturnina; Colique de plomb; Bleikolik.—This affection is also known as painter's colic, Devonshire colic, colica pictonum, the last term being derived from Poitou. It occasionally develops abruptly without previous symptoms. In the majority of cases, however, the earlier disturbances of nutrition before pointed out precede the attacks, often by a period of weeks or months. An increased tendency to constipation with transient recurrent abdominal pains, occurring sometimes immediately after food, more frequently at irregular intervals, often characterizes the prodromic period. There is, moreover, an intensification of the other symptoms of chronic lead-poisoning. The appetite is decidedly impaired, so also are the powers of digestion; the foul condition of the mouth becomes more annoying, and the peculiar taste alluded to more persistent and more marked. Constipation occasionally alternates with diarrhœa. The colicky pains which constitute the attack are of variable intensity, but usually severe. They are of the nature of true colic. Pressure over the seat of pain almost invariably gives relief, particularly in severe attacks. During the remission pressure is grateful to the patient. The position of the pain varies. Its most common seat is in the region of the umbilicus. It may occupy the epigastric and hypochondriac regions or the lower part of the abdomen, extending from side to side above the level of the pubic bone. These colics are of some minutes' duration, recurring after short intervals, which are characterized by a certain amount of continuous cramp. Tenesmus is frequent. It may be accompanied by strangury or retention of urine, with pain in the course of the ureters, the spermatic cord, or in the penis. Shooting pains in the breast also occur. The abdomen is retracted, and scaphoid to such an extent in some instances that the prominence caused by the bodies of the vertebræ is apparent in the median line of the abdomen. It occasionally happens, however, that retraction does not occur, the belly, on the other hand, remaining prominent. As a rule, to which there are few exceptions, the bowels are constipated. This condition is often stubborn. In rare cases, however, the colic is accompanied by diarrhœa. Nausea is of common occurrence. Vomiting is sometimes violent and distressing, being ushered in by repeated attacks of retching. The vomited matters are usually discolored by bile, and icterus of slight degree usually occurs. The pulse is invariably diminished in frequency, sometimes as low as 40 or even 30, per minute. It is at the same time full and tense. The pulse-frequency is occasionally variable within comparatively brief periods of time. Respiration is increased in frequency during the attack. The urine is small in quantity and of high specific gravity, and frequently contains small amounts of albumen. It is sometimes entirely suppressed. Fever is absent, and the slight increase of temperature occasionally observed is attributable to the restlessness of the patient. The intellectual condition usually remains clear, but in violent cases it is characterized by excitement. The paroxysms of pain are more severe in the latter part of the day and during the night. The course of the attack is irregular. Relapses, which may occur in the course of some days or even weeks, are frequent. The duration of the attack does not, as a rule, exceed a week, but the illness is often protracted by repeated relapses, and in continued exposure to the cause the tendency to colic may become chronic. The termination of the attack is in most instances as abrupt as its onset, and with the cessation of the colic the associated symptoms, such as retraction of the abdomen, constipation, vomiting, and diminished frequency of the pulse, rapidly subside. Lead colic is not unfrequently associated with the other affections of chronic lead-poisoning. The prognosis is favorable, the mortality being about 2 per cent.

3. The Arthralgia; Arthralgia saturnina.—The tissues entering into the formation of the joints and the muscular masses contiguous to them are frequently the seat of intense paroxysmal pains, which appear more or less suddenly, either with or without prodromes, and run a course similar to that of lead colic, exhibiting violent exacerbations and occasional more or less complete remissions. These pains are accompanied by painful contractions of the muscles. They are somewhat relieved by pressure and friction and aggravated by exercise and exposure to cold. Swelling and redness over the affected joints, ligaments, and tendons are absent. These pains do not follow the course and distribution of the nerve-trunks, and lack the tender pressure-points of true neuralgias. They are more common in the lower extremities, the knee being especially apt to be affected. When they affect the upper extremity the elbow and shoulder suffer more frequently than the other joints. The flexor muscles are much more frequently attacked than the extensors. The muscles of the back, especially the great masses of long muscles in the lumbar region, whose function is to sustain the equilibrium of the body, are frequently implicated. The intercostal muscles and the muscles of the neck are also frequently affected. The small joints and the muscles of the hands and feet as a rule escape. The pain is tearing and burning in character, and very often severe. It is occasionally associated with tremor. The derangement of the alimentary canal, especially constipation, which is so characteristic of lead colic, is often absent. Fever does not occur.

A careful study of these cases establishes the fact that in a considerable proportion of them the morbid condition is myalgic. Whether it be due to simple muscular malnutrition or to some specific action of lead upon the muscle-substance is not determined. The analogy between these muscular pains and lead colic renders it probable that in a large majority of the cases the latter view is correct. These pains are frequently associated with the attack of lead colic; sometimes, however, they precede the colic, while at other times they follow it. After a series of remissions and exacerbations the pain often ceases as suddenly as it began. As in other lead affections, relapses are frequent. The prognosis is favorable.

The English physicians, and especially Garrod,⁶ Todd,⁷ and Bence Jones,⁸ have insisted upon the frequency of gout in chronic lead-poisoning. Murchison,⁹ Wilks,¹⁰ and others regard this association as causative. This view has not found general acceptance in France. Lancereaux¹¹ in particular, after a comparative study of the lesions of the kidney and of the joints in saturnine cachexia and the corresponding lesion of gout, concludes that the gouty kidney and the kidney of lead-poisoning have the same appearance, and are simply varieties of interstitial nephritis, and that the articular and arterial lesions in both affections are identical. So-called saturnine gout differs in no respect, either in its clinical or anatomical characters, from ordinary gout. It would therefore appear superfluous to describe saturnine gout. At the same time, it must be insisted upon that chronic lead-poisoning exercises an important influence in the production of gout—a causal relation due to the fact that lead diminishes the excretion of uric acid.

⁶ Medico-Chirurg. Transactions, 1854, vol. lvi.

⁷ Clinical Lectures, London, 1856.

⁸ Transactions of the Pathological Society, London, 1856.

⁹ Lancet, 1868.

¹⁰ British Medical Journal, 1875.

¹¹ “Nephritis et Arthrites saturnines: coincidence de ces Affections paralleles avec la Nephrite et l'Arthrite goutteuse,” Arch. gén. de méd., Decembre, 1881.

4. Lead Palsy; Lead Paralysis; Paralysie Saturnine; Bleilähmung.—This condition is fourth among the affections due to chronic lead-poisoning, both in frequency and in the order of succession. Nevertheless, like each of the others, it may be the first in the order of appearance after disturbances of nutrition which are in some rare cases of very slight degree. As a rule, however, the paralysis has been preceded by colic or by joint affection, or by both. Tanquerel¹² found that in 88 cases of lead palsy 25 had been preceded by an attack of colic, 15 by two attacks, 3 by ten attacks, 1 by fifteen attacks, and that in single cases as many as twenty, and even thirty, attacks of lead colic had occurred before the appearance of paralysis.

¹² Loc. cit.

The length of time over which habitual exposure to lead without the development of palsy may extend was found by the same observer to vary from eight days to ten, twenty, or even twenty-five years. One individual first suffered from paralysis after fifty-two years of exposure.

Without dwelling upon the sources of error in statistics of this kind, it must be conceded that they establish in a general way the extraordinary differences in the susceptibility of individuals. There are persons who every time they are attacked with colic, of whatever degree of severity, suffer also from paralysis. Others, on the contrary, suffer from repeated attacks of violent colic without the development of paralysis.

Lead palsy is an affection of adult life. Of 102 cases, 2 only occurred in individuals below twenty years of age.

Like the other specific lead affections except the encephalopathy, palsy is more common in the summer than at any other season.

Those who have once suffered are very liable to successive attacks. Tanquerel and Maréchal have observed many returns of paralysis, presenting the characters of the original attack, many years after the patient had withdrawn from exposure to lead.

Perverted sensations of the parts about to be affected, such as coldness, numbness, and hyperæsthenia, may precede the attack. Impairment of motor-power, manifested by feebleness, stiffness, or awkwardness and tremor, also appears in the prodromic period. This trembling consists in slight agitation of the muscles, rather than in well-marked rhythmical contractions. These precursors may indeed constitute the attack, which occasionally, and especially under treatment, terminates at this point. The prodromic symptoms are less severe during the day, while the patient is at work; at night they are aggravated. After some days they end in the characteristic paralysis. Colic is a common precursor. After the attack some stiffness of the muscles is experienced, which terminates by rapid loss of power or abruptly in actual palsy. Occasionally encephalopathy precedes the paralysis. It is rare that prodromes are wholly absent.

In the great majority of the cases the upper extremities and the extensor muscles are first attacked. If the paralysis be slight other muscles may escape. The characteristic form of lead paralysis consists in loss of power in the extensors of the hands and fingers, especially of the extensor communis, without implication of the supinator longus. The muscles affected are in the region of distribution of the musculo-spinal nerve. The deformity is known as wrist-drop. Next in order, the triceps and deltoid are most frequently attacked. The lower extremities commonly escape for a considerable time. When attacked, the extensor muscles of the feet and toes are the first to suffer.

Paralysis of the dorsal muscles occurs in rare instances. It gives rise to a peculiar stooping, uncertain, and tottering gait. Paralysis of the intercostal and laryngeal muscles was observed by Tanquerel. Paralysis of the muscles of the face or of those of the eye has never been observed in consequence of lead-poisoning. The loss of power never corresponds strictly to the distribution of the branches of a single nerve-track.

The paralysis, as a rule, affects both sides, and frequently the corresponding muscles of the two extremities. Sometimes, however, the affected muscles of the two sides are not the same, and it occasionally happens that the affection is limited to one side. In very rare cases, the arm and leg on the same side being paralyzed, the affection resembles hemiplegia.

Local paralysis may be limited to the extensor muscle of a single finger or may involve all the muscles of a limb. There may be slight impairment of power in the flexor muscles. The enfeeblement of certain flexor muscles, especially of those of the fingers, may, Naunyn suggests, be only apparent, the position of the hands being in pure extensor paralysis unfavorable to the exercise of the flexors and limiting their function.

Sensation is not, as a rule, affected in lead paralysis. Pains in the paralyzed muscles, in neighboring muscular masses, and in the structures about the joints often precede the attack of paralysis. Anæsthesia of the skin is rare. When present, it is usually of slight degree, and corresponds to the region of the paralysis. Deep anæsthesia has also been observed.

Atrophy of the paralyzed muscles is constant and rapid. It frequently reaches a high degree, causing characteristic deformities, which are rendered more marked by the fact that the adjacent non-paralyzed muscles preserve to a great extent their original state of nutrition. As the neuro-muscular lesion progresses other deformities arise, in consequence of derangements of the balance of force between opposing muscles and from other causes. Among these deformities are partial or complete dislocations of the more movable joints, as the shoulder and phalanges, with the formation of tumors, which, when they occupy the dorsum of the hand, might be carelessly mistaken for the nodosities of gout. It is to be noted that the latter are occasionally present as complications.

There is rapid diminution, and finally complete loss, of the reaction to the faradic current. With the galvanic current the reaction of degeneration is usually well marked. The reaction of degeneration may be demonstrated in the extensors of the limbs in lead-poisoning before wrist-drop has occurred.

The course of lead palsy is very variable. In a few cases it is progressive. When local emaciation or atrophy arrives at the last degree of marasmus, the skin seems glued to the bones, to such an extent are the paralyzed parts wasted; the muscles especially are so thinned that the contour of the bones is easily distinguished. If the paralysis attack the whole of the limb, then this organ, abandoned to its own weight, stretches the ligaments and permits the head of the bone to leave its cavity. In other rare instances the paralysis remains stationary for long periods of time, only to grow worse after each new attack of lead disease. Much more frequently lead paralysis disappears spontaneously or under treatment in the course of some days or weeks. The convalescence is gradual, and usually rapid, when the gravity of the lesion is considered; occasionally it is complete in the lapse of a few days.

The prognosis depends upon the degree of lead cachexia present, the possibility of withdrawing the patient from the exposure, and finally upon the degree and extent of the paralysis and of the atrophy. It is rendered unfavorable by the necessity of the prolonged exposure of the patient to lead, by a high degree of disturbance of the general nutrition of the patient; and by the fact of his having presented for a considerable period the evidences of lead disease, with occasional attacks of colic or arthralgia; by the complete loss of power and electrical reactions in the affected muscles; and, finally, by absolute wasting of the muscular masses. The prognosis is less favorable in relapses than in the primary attack.

Progressive muscular atrophy has been frequently observed in patients suffering from lead disease (Naunyn).

5. The Encephalopathy; Encephalopathia Saturnina.—This term was suggested by Tanquerel to designate collectively the various morbid cerebral phenomena produced by chronic lead-poisoning. It embraces, therefore, those affections due to the action of lead upon the central nervous system, and occasionally described as lead insanity, delirium, convulsions, epilepsy, coma, etc. It is, of all the disorders produced by lead, the most rare. Tanquerel met with seventy cases only. It occurs only in those individuals who are exposed to large quantities of lead, and in such a manner as to favor the absorption of the metal and its compounds by the digestive and respiratory tract. Hence house-painters and the workers in lead-factories supply the greater number of cases. The time of exposure elapsing before the manifestations of the special morbid action of the poison upon the nervous system show themselves varies from a few days to many years. In a large proportion of the cases the time has been less than one year.

Women are much less liable than men—a fact clearly due to the nature of their occupations even when involving exposure to lead.

The majority of the cases have occurred between the ages of twenty and fifty. Alcoholic habits, insufficient and unwholesome diet, privations, exposure, and an irregular life especially predispose those working in lead to the affection. Lead encephalopathy has developed with nearly equal frequency in warm and in cold weather. Relapses are frequent.

Among the prodromes are headache, vertigo, agitated and interrupted sleep marred by distressing dreams, and troublesome insomnia; derangements of the special senses, dimness of vision, alterations of the pupils, tinnitus aurium; dysphagia and a sense of constriction of the pharynx have also preceded the attack of cerebral disorder. It is usually preceded likewise, and often by a considerable lapse of time, by the other lead affections above described—namely, colic, arthralgia, and palsy. Psychical derangements—stupor, apathy, or excitement—also precede the attack. In a small number of cases lead encephalopathy has occurred abruptly without prodromes.

The symptomatology is exceedingly variable and irregular. Three forms, the delirious, the comatose, and the convulsive, have been described. These conditions may succeed each other in the same attack. The delirium is variable in kind. It is apt to be at first tranquil; after some time it becomes paroxysmal and furious. It is broken by intervals of somnolence. At length true sleep supervenes, and the patient awakes restored almost completely to his reason.

Coma may develop suddenly, even instantaneously. In a very few cases the comatose form has developed itself alone during the course of chronic lead-poisoning. As a rule, however, it is preceded by delirium or convulsions.

The convulsive form is the most common. The convulsions may be partial, involving the face or the one side of it, a single limb, or one side of the body. They may be general, without loss of consciousness. These incomplete attacks are apt to be followed, after a period of dulness or unconsciousness, by acute eclampsia. Epileptiform attacks may now follow each other in rapid succession, ending in more or less profound coma. The attacks are sometimes separated by intervals of uneasiness, restlessness, or delirium. These attacks continue several days. If they are very violent and frequent death may rapidly supervene. In favorable cases consciousness is gradually restored, or the patient may awaken suddenly from his drowsiness after some hours or a day.

These attacks are not preceded by an aura.

Amaurosis is among the more important of the symptoms produced by the action of lead upon the nervous system. It is usually accompanied by dilatation of the pupils. The amaurosis gradually disappears as the other symptoms subside, and with the improvement in sight the pupils contract. Recovery is frequently complete. Occasionally vision does not return with the improved general condition. Norris¹³ saw, in two cases of lead-poisoning due to the use of white lead as a cosmetic, marked choking of the discs in connection with severe cerebral symptoms. The terminal condition, when recovery does not take place, is that of nerve-atrophy.

¹³ See this System of Medicine, Vol. IV.

Albuminuria is common in this as in other lead affections. It may be of moderate amount and due to the rapid breaking-down of the blood-corpuscles which marks the exacerbation of the general condition. Albuminuria may be a direct consequence of the eclampsia. Finally, it may be due to coexisting nephritis.

The prognosis is in a high degree unfavorable.

MORBID ANATOMY AND PATHOLOGY.—There are no well-characterized anatomical lesions in chronic lead-poisoning. Lead has been found in almost every organ and tissue in the body. It exists in combination with the albumen of the tissues. The changes in the nervous system that have thus far been described are neither constant nor characteristic. The same is true of the lesions of the intestines. The paralyzed muscles are found to have undergone atrophy, with loss of the striæ and increase of connective tissue. The nerve-trunks are also the seat of atrophic degeneration.

The mode of action of lead is not yet determined. The view of Henle, that lead acts chiefly upon the unstriped muscular fibre, was at one time generally accepted, and served to explain many of the characteristic phenomena of the disease. On the other hand, there is much in the clinical history to support the opinion of Heubel, that its primary action is upon the nervous system. Whether the peripheral degeneration by which the paralysis is to be explained is primary or secondary to central degeneration in the anterior cornua of the gray matter of the spinal cord, is yet in dispute. Some of the forms of encephalopathy are doubtless due to the nephritis which is found in many cases of chronic lead disease, and are, in fact, symptoms of uræmia. In the great majority of the cases this is not the case. The morbid condition must be explained by the direct toxic action of lead upon the central nervous system. No theory adequate to account for all the cerebral manifestations has yet been suggested. Naunyn has pointed out the resemblance between the nervous and psychical derangements in lead encephalopathy and those which characterize chronic alcoholism, and suggests that lead, like alcohol, produces these effects not as a direct poison, but indirectly in consequence of abnormal nutrition of the whole system, brought about by the continued circulation of a foreign poisonous material in the blood.

Chronic Lead-Poisoning in Animals.—Animals exposed to lead under conditions favorable to the development of the lead affections in man suffer in like manner. Instances of this kind are of frequent occurrence in and about large lead-factories. The drinking of water containing lead also gives rise to these affections in animals. Horses, dogs, cats, and fowls have frequently suffered from lead colic. Horses used in lead-factories suffer from a form of laryngeal obstruction due to lead paralysis of the muscles of the larynx. Relief has followed tracheotomy and the introduction of a canula, and removal to an atmosphere free from lead has resulted in recovery. Cats who spend some time in red-lead workshops frequently are paralyzed. Even the rats in lead-factories become paralytic (Tanquerel).

DIAGNOSIS.—The diagnosis of the affections due to chronic lead-poisoning is, as a rule, unattended with difficulty. The malnutrition, anæmia, poor digestion, foul mouth, stubborn constipation, and the gingival line, considered in connection with the history of prolonged or habitual exposure to lead, would warrant the assumption that the relation between this poison and the symptoms is a causal one. When there is added colic, arthropathies, and paralysis, or the cerebral states having the characters above described, the assumption becomes a certainty. The absence of any one of the ordinary phenomena, such as the blue line or constipation, would still leave the clinical picture sufficiently full to justify the diagnosis. The reaction of degeneration, which is usually marked in saturnine wrist-drop, with the escape of the supinators, distinguishes it from pressure paralysis of the musculo-spiral.

TREATMENT.—A. Prophylaxis.—Free ventilation and scrupulous cleanliness constitute, in general terms, the most efficient safeguards for those whose occupations involve prolonged exposure to lead. Workmen employed in lead-factories and those otherwise exposed to lead should be compelled to wash their hands and change their outer clothing before eating; they should also bathe regularly every day on leaving work; under no circumstances should they be suffered to eat or sleep in or near the workshops. As all kinds of work in the manufacturing of lead preparations are not attended with equal risk, the workers should from time to time be transferred from one department to another or from in-door to out-door work. In order to prevent the constant rising of dust, the floors are to be kept constantly sprinkled or covered with moist sawdust. It is unnecessary to go into further details in regard to the hygiene of the subject. It is probable that towels and sponges worn over the mouth, or other forms of respirators, because of the inconvenience which they occasion and the false sense of security which attends their use, are of less value than has been generally supposed.

It appears scarcely needful to here insist upon the avoidance of cosmetics and hair-dyes containing lead, or upon the exercise of reasonable prudence in the matter of the manufacture, sale, and use of articles of food or drink which are liable either by accident or design to become adulterated with lead compounds.

The use of sulphuric-acid lemonade is a measure of prophylaxis of less real value than was at one time supposed, seeing that the sulphate of lead is in itself capable of producing the disease. Occasional doses of magnesia sulphate are of use where a tendency to constipation exists. Workmen who begins to show signs of chronic lead-poisoning should without delay abandon their work and seek some occupation free from its peculiar dangers.

The precautions against the use of water contaminated with lead have been pointed out under the heading Etiology.

B. Curative Treatment.—When the disease shows itself, no matter in what form, the primary indication is the discontinuance of exposure to lead. Chronic cases of malnutrition, constipation, functional nervous disorder, will occasionally be found upon careful search to depend upon long exposure to lead in some unsuspected way. The cause being removed, such cases often promptly recover.

Measures aimed first at the separation of the lead from the tissues, and then at its elimination from the body, constitute a rational treatment. Sulphur baths and the internal administration of sulphur may be employed with a view of converting the lead eliminated by the skin and mucous membranes into an insoluble sulphide, and thus preventing its resorption. Repeated laxative doses of castor oil will remove unabsorbed lead from the intestinal canal. The plan of treatment at present in general favor consists in the combined use of potassium iodide and magnesium sulphate. From five to twenty grains of the iodide are to be given in not less than six or eight fluidounces of water three times daily, the stomach being empty: two hours after each dose one or two drachms of the magnesium sulphate are to be taken; after this dose an ordinary meal.

This treatment is designed to dissolve the lead deposited in the tissues, and cause its elimination by the mucus of the alimentary canal in part, and to a slight extent also by the urine. The magnesium sulphate tends to remove such lead as finds its way into the alimentary canal thence with all possible rapidity. These measures, together with the removal of the patient from further exposure, exert of themselves a favorable influence upon the malnutrition and anæmia. Quinine, strychnine, iron, cod-liver oil may be advantageously administered as the toxic effects of the lead pass away.

The colic demands the administration of opium or its derivatives to relieve the pain, which is commonly excruciating. For this purpose the hypodermic injection of morphia is our most efficient remedy. Alum in doses of twenty to thirty grains every four or six hours is useful in lead colic.

For the relief of the local paralytic affections local as well as general treatment is necessary. Massage is of great use, especially when combined with passive movements. Galvanism, one large electrode being applied to the cervical vertebræ, the other to the extensor surface of the affected limbs, is followed by excellent results. Labile currents of fifteen or twenty cells should be thus applied, the poles being changed several times at each sitting. As the nutrition of the muscles improves faradic currents may be occasionally substituted. Persevering treatment is necessary to obtain the best results.

For the present relief of the arthralgia gentle frictions with or without anodyne liniments must be employed. The hypodermic use of morphine may become necessary in cases in which the pain is urgent. It is, however, here as elsewhere, to be if possible avoided. The tendency to recurrent joint-pains rapidly disappears as the poison is eliminated from the organism.

For the relief of the severe cerebral symptoms which are described under the term Encephalopathy special treatment is of little avail. All observers agree in recommending an expectant plan. The measures of treatment directed against the general condition, as above described, must be steadily continued. The influence of chronic lead-poisoning upon pregnancy is very deleterious. Constantine Paul¹⁴ and others have shown that the early death of the fœtus very constantly occurs. The prompt removal of women who have become pregnant, from all exposure to lead, and energetic medicinal treatment, are needed to obviate the danger of abortion.

¹⁴ Arch. gén. de Méd., vol. xv., 1860.

PROGRESSIVE UNILATERAL FACIAL ATROPHY.

BY CHARLES K. MILLS, A.M., M.D.

DEFINITION.—Progressive unilateral facial atrophy is a disease characterized by progressive wasting of the skin, connective tissue, fat, bone, and more rarely muscles of one side of the face.

SYNONYMS.—Progressive facial hemiatrophy, Neurotic atrophy of the face (Virchow), Facial trophoneurosis (Romberg), Prosopodismorphia (Bergson), Atrophy of the connective tissue of the face or Laminar aplasia (Lande).

HISTORY AND LITERATURE.—This disease has been known to the medical profession since 1825, when, according to Eulenburg, it was first described by Parry. The Index Catalogue contains references to thirty books and articles on the subject in various languages. Eulenburg gives a bibliography which contains thirteen references not given in the Index Catalogue. One personal case, which will be detailed later, has fallen under observation. In all, I have collected from fifty to sixty cases. The number of reported cases is slowly increasing; nevertheless, the disease must still be regarded as rare. Carswell,¹ according to Lasègue,² first arranged scientifically the various lesions which might arise in consequence of a retardation and arrest, or even inverted action, in nutrition, and at the same time that he assigned their causes pointed out the several forms of atrophy. Aran³ has written a contribution on the subject, furnishing valuable material for classifying atrophies. Romberg called general attention to the disease in his clinical researches, published in 1846. He described the disease under the name of a new form of atrophy of the face, and took the position that it was a primary trophoneurosis. Of contributions by American writers, a paper by Bannister⁴ is the most complete and valuable. This not only contains two fully and carefully reported cases, but also a thorough discussion of special symptoms and the pathology of the disease. The chapter of Eulenburg⁵ contains an excellent sketch of the disease, and is especially valuable for the discussion of the various theories as to its nature.

¹ Illustrations of the Elementary Forms of Disease, 1836.

² Archives générales de Médecine, May, 1852, p. 71.

³ Ibid., Sept. and Oct., 1850.

⁴ Journal of Nervous and Mental Disease, vol. iii., No. 4, Oct., 1876, p. 539.

⁵ Ziemssen's Cyclopædia of the Practice of Medicine, vol. xiv., 1877.

ETIOLOGY.—The disease is of much more frequent occurrence among females than males. Out of 25 cases which I have been able to classify, 9 were in men and 16 in women. It is an affection of comparatively early life. The following were the ages of 25 patients: under ten years, 7; from ten to twenty years, 11; from twenty to thirty years, 6; over thirty years, 1.

Graefe⁶ reports a case due to syphilis, in which there was also paralysis of the left oculo-motor externus and trigeminus. In one of Bannister's cases the patient had suffered repeatedly from slight frost-bites. Among the causes which seem to be thoroughly established traumatism holds the first place. The affection could be traced to injury in a fair number of the published cases. A case is reported by Maragliano⁷ of a child who had a fall in which she struck the left half of her face, and in consequence of which a circumscribed swelling appeared near the external angle of the left eye and remained for a few days. Shortly after this the left half of the face began to grow smaller than the right. Schuchardt⁸ speaks of a fall on the head which left a cicatrix on the right parietal bone below the coronary suture, the injury being followed by facial atrophy. Panas⁹ reports a case in which the wasting of the face followed a fracture of the lower jaw. In one of two cases reported by Bannister,¹⁰ a man, aged forty-two, is stated to have been thrown from a stage-coach, falling on his head, and not losing consciousness, but receiving a scalp wound over the coronal suture. Subsequent to this time he had cerebral symptoms, physical and mental, and unilateral facial atrophy developed.

⁶ Quoted by Rosenthal.

⁷ Note di Clinica Medica, Genoa, 1881, translated by Joseph Workman, M.D.—Alienist and Neurologist, vol. ii. No. 2, April, 1881, p. 146.

⁸ Quoted by Eulenburg.

⁹ Bull. Soc. de Chir. de Par., 1869, 1870, 2d S. ex., 198, and Gaz. des Hôpitaux, 1869.

¹⁰ Journ. Nerv. and Ment. Dis., Chicago, 1876, iii. pp. 539-560.

SYMPTOMATOLOGY.—Before discussing the symptomatology I will give brief notes of the following case, which has not been before reported, and which was observed by me while in charge of the Nervous Dispensary of the Hospital of the University of Pennsylvania several years since.

C——, aged seventeen, white, a mill-operative, had a good family history. Three years before coming under observation a white spot appeared in the skin over the right malar prominence, and since that time this side of the face had gradually atrophied. When this patient was first examined the skin had a mottled appearance. Close examination showed that the skin, connective tissue, and bone were decidedly wasted, and there seemed also to be some loss of substance in the muscles of the right side of the face. The mouth, however, could be closed properly, the lips showing no puckering or drawing to one side. The right half arches of the palate were atrophied. The uvula was drawn slightly but positively to the left. Careful examination of the membrane of the drum of the right ear showed it to be atrophied. An ophthalmoscopic examination of both eyes was made, but no changes were discovered. No changes were noted in the hair of the patient on the affected side. He was able to perform all movements with the facial muscles, but the creases and contours of the face in repose and in motion were less marked on the right side than on the left. The skin of the right side of the face seemed to be bound closely to the bone. The muscles on the same side responded well to the faradic and galvanic current. The patient had at times a peculiar sensation of stinging in the atrophied half of the face. He was treated with electricity, local massage, and tonics, and did not improve, but did not get worse while under observation, a period of several months.

At the Hospital of the University of Pennsylvania I also observed one well-marked case of unilateral atrophy of the tongue without atrophy of the face, of which, unfortunately, I have not preserved the notes. The patient was a middle-aged man with a syphilitic history. The atrophy in the reported cases has occurred much more frequently upon the left than upon the right side of the face.

Changes in the skin are among the most constant and striking phenomena. By some dermatologists the disease is regarded as a form of morphœa, the keloid of Addison. Morphœa as described by Duhring¹¹ occurs in the form of patches, rounded, ovoid, or irregular in outline, small or large, tough or leathery, smooth or shiny, and may also manifest itself in the form of atrophic pit-like depressions in the skin. The skin alterations differ somewhat in character; frequently one of the first signs of the disease is the appearance of a white spot on the cheek. This was the first noticeable phenomenon observed in the above case. After a time this spot or patch may change in color, becoming of a yellowish or brownish hue. A number of spots appearing and undergoing change, the skin after a time assumes a mottled appearance. In some of the reported cases a yellowish, or yellowish-gray, or brownish appearance is recorded as having been present. In these the observations were probably made after the affection had existed for some time, and in them the white spots or patches were probably at first present. In one of Romberg's cases, first recorded when the patient was nine and a half years of age, he noted the deposit of a yellowish-gray pigment which began at the median line and extended to the angle of the jaw across the lower half of the face and neck, divided here and there by portions of healthy skin. Discoloration, varying in intensity, also existed in irregular patches on the upper part of the face and on the forehead. The discolored tissues were glossy and greasy-looking. The skin adhering to the bone often gives the patient an aged appearance. Pruritus has been several times observed.

¹¹ Medical News, vol. xlv. No. 4, July 26, 1884, p. 85.

Changes take place in the hair. Sometimes it simply changes color; at other times it falls out or ceases to grow. The beard or hair of the head will thus be gray or white upon one side, or in one or two locations of one side and not of the other; or the hair may be absent or simply thinned out. Peculiar limitations in the extent to which the hair is affected are sometimes observed. In one of Romberg's cases, for example, complete absence of the eyelashes from the inner angle of the eye to the middle of the lids was noted; the hair was very thin, and in some parts altogether wanting, and the left eyebrow was almost entirely devoid of hair. The changes which take place in the color of the hair in some severe cases of chronic trigeminal neuralgia will be recalled in this connection. I have known of several instances in which a few locks of hair were turned gray or white on the neuralgic side. Anstie, author of the work on Neuralgia, was a sufferer from supraorbital neuralgia, and the eyebrow of the same side contained a white tuft. Facts of this kind serve to corroborate and emphasize the view that unilateral facial atrophy is a tropho-neurosis.

Anidrosis, or absence of perspiration, on the atrophied side of the face is a nearly constant phenomenon. In one of Bannister's cases, a printer, attention was first called to the patient by the fact that in working a hand printing-press in the hot weather he perspired only on the right side of his face. The left side remained perfectly dry, no matter how warm the temperature or hard the labor. This hemidrosis, or unilateral sweating, was confined only to the right side of the forehead, the right cheek, side of the nose, and lip. On the chin it encroached a little on the left. Under the chin and in the throat there was a little excess of perspiration on the right side; on other portions of the body no difference was noted. Nicati¹² considers a combination of symptoms, several of which were present in a recorded case—namely, lowered temperature, stoppage of perspiration, and wasting of the side of the face—as indicating a paralysis of the sympathetic nerve in what he calls its second or more advanced stage. In true facial hemiatrophy, however, other phenomena of such paralysis are wanting. Brunner¹³ gives a case in which perspiration, tears, mucous secretions, and temperature were all diminished on the affected side, and in which were also present exophthalmos and dilatation of the pupil. The cervical sympathetic in this case was irritated by a tumor. I agree with Bannister, however, that while we cannot exclude the possibility of the participation of the sympathetic in hemiatrophy of the face, the evidence is not sufficiently positive. Mechanical interference with the sweat-glands by pressure or otherwise has been suggested. The true view to take would seem to be that the perspiratory disorder is due to the same central or peripheral neurotic affection which gives rise primarily to the atrophy. Eulenburg says that the secretion of the sebaceous follicles is in most cases diminished or stopped on the atrophied side, while the sweat-glands often act in a normal manner; but his experience does not seem to correspond with that of the majority of other observers.

¹² Sur Paralysie du Nerf sympathetique-cervical, Lausanne, 1873, quoted by Bannister.

¹³ St. Petersburger Med. Zeitschr., ii., 1871, quoted by Bannister.

Seguin, Dreschfeld, and others have found no notable differences in surface temperature between the two sides of the face. In one of Bannister's cases the temperature was tested in both ears, and a difference of six-tenths of a degree Fahrenheit in favor of the ear of the unatrophied side was found, that of the left ear being 97.6°, and that of the right 98.2°. This examination was repeated on another occasion with nearly the same result.

Weakness of pulsation in the carotid of the affected side has been observed. Sometimes the power to blush is lost on the affected side, but this may return. In one instance the cheek, having been reddened by electrical stimulation, so remained for an hour or more.

Paræsthesiæ, such as pain, numbness, and stinging sensations, are not uncommon. A diminution of the general tactile sensibility of the skin of the affected side has been observed by Tanturri¹⁴ and by Vulpian,¹⁵ and perhaps by others, but anæsthesia is not commonly present.

¹⁴ Il Morgagni, 1872, quoted by Bannister.

¹⁵ L'Appareil vaso-moteur, ii. p. 430.

Eulenburg and Guttmann found atrophy of the muscles supplied by the motor branch of the fifth nerve—namely, the masseter and temporal. The changes which take place in the muscles of the affected side are, so far as can be judged, not of the character of fatty degeneration. Probably a general and uniform shrinkage of calibre of muscular fibres and bundles occurs. Certainly, this would seem to be true of the muscles supplied by the portio dura. The muscles on the atrophied side respond to both the faradic and galvanic currents, reactions of degeneration not being present. The absence of the reactions of degeneration in the facial muscles shows that normal muscular fibres remain. In one reported case the facial muscles seemed at first to react more readily than in normal conditions. The muscles of the upper lip sometimes appear to have wasted, preventing full closure of the mouth on this side. Fibrillary twitchings have been observed only very rarely. Voluntary control over the muscles of the affected side is not impaired.

In one of Bannister's cases the first upper molar of the left side sometimes ached, and the gum and bone were so wasted as to expose its roots for a considerable distance. Falling out of the teeth has been observed in a few other cases.

Atrophy of the tongue is associated with the facial wasting in a certain percentage of cases, and the uvula and soft palate are also sometimes wasted. On the other hand, atrophy of the tongue has been observed as an isolated phenomena.

Atrophy of the bones of the face is frequently present, and has been determined by careful measurements. It will show to a greater or lesser extent according to the age of the patient. When the disease arises after the bony development has been fully acquired, there will be little external evidence of osseous involvement; when it begins during the time of active growth of the bone, as it does not infrequently, the arrest in the skeleton will be very apparent. Thus in a number of cases beginning in early childhood bone atrophy has been a very marked feature.

Eulenburg says that the functions of taste, smell, hearing, and seeing are not interfered with in any of the reported cases; but Bannister reports impairment of the sense of taste in both of his cases. In one the whole left half of the tongue was involved, in the other only the left posterior third. In the first of these, in which the whole left half of the tongue was involved, both the glosso-pharyngeal and the chorda tympani nerves were affected. According to Bannister, the significance of this observation is that either the glosso-pharyngeal and fifth nerves are both involved, or that the taste-fibres for the base as well as tip of the tongue are derived from the last-named nerve. In this case, however, which was due to a severe injury, the headache, mental confusion, loss of hearing, etc. more probably indicated a widespread intracranial lesion, affecting to some degree the nucleus of the glosso-pharyngeal. In the other case, which was apparently a typical one of unilateral facial atrophy in its early stage, no history of traumatism was present, and the sense of taste, which was carefully tested several times, was seriously impaired over the left posterior third of the tongue.

Bannister discusses the probable cause of impairment of taste in each case, as follows: “The atrophy seemed to involve the region supplied by the second division of the fifth nerve as much as, or more than, the other divisions; and on this is situated the spheno-palatine ganglion which gives off the Vidian nerve, the upper division of which, the superior superficial petrosal nerve, is supposed by Schiff to contain the taste-fibres, which return from the lingual nerve through the chorda tympani. The usual theory of this disease is that it depends upon an affection of the trophic fibres contained in the fifth nerve or one or more of its divisions. Accepting this theory, Baerwinkel,¹⁶ noticing a case of unilateral atrophy of the face affecting only the second division of the fifth, and, as he held, indicating trouble with the spheno-palatine ganglion, its trophic centre, mentions the fact that there was no impairment of taste in the anterior portions of the tongue as rather against the hypothesis of Schiff as to the course of the taste-fibres. At the same time he gives two other observations of lesions of the trifacial and the seventh nerve that favor it. In our patient I should have expected a priori to have found the taste affected in the anterior portion of the tongue, if anywhere, but the reverse was the fact. It is difficult to suppose an accidental lesion of the glosso-pharyngeal coexisting with the one of the trigeminus that produces the atrophy when there are no more signs of nervous disorder than there were in this case. On the other hand, the taste-fibres of the glosso-pharyngeal are not generally supposed to have such connections with the fifth nerve as to be involved in its disease, while those of the chorda tympani, of the connections of which with the trigeminus there is much more physiological evidence, escape. The observation is a contradictory one, and I cannot at present explain it.”

¹⁶ Deutsch. Archiv f. klin. Med., xvii. 1, 1875.

So far as I know, ophthalmoscopic changes have never been noted. In a few cases enophthalmos, or sinking in of the eye, doubtless due to the disappearance of orbital fat as the disease advances, has been recorded.

Defects of hearing, and even partial deafness, have been reported in a very limited number of cases, making it questionable whether this symptom is a coincidence or a complication. In the case reported by me careful examination of the membrane of the tympanum by W. Ziegler of the ear department of the Hospital of the University of Pennsylvania showed atrophy of this membrane.

Eulenburg speaks of one case in which the external atrophy had reached as far as the larynx, and in which the pronunciation of the letter r was somewhat impeded. Beverly Robinson,¹⁷ reports a case accompanied with slight paralysis of the adductors of the vocal cords. The laryngeal paralysis in this case improved under treatment, which led Robinson to believe that the relation between the two affections might have been only one of coincidence.

¹⁷ Am. Journ. Med. Sci., October, 1878, p. 437.

Flascher¹⁸ has described a unique case of bilateral facial atrophy occurring in a woman twenty-three years old. When an infant she fell and injured her forehead, and soon afterward had an attack of measles without eruption. Shortly afterward the atrophy appeared. Tactile sensibility was diminished and the secretion of sweat absent. The masseter, temporal, and facial muscles and bones were atrophied. External strabismus of the left eye was present; the pupil of this eye was dilated, irregular, and non-reactile; sight was diminished, the optic disc atrophied. This case would appear to differ somewhat from true facial hemiatrophy. It was probably a peripheral nerve affection following measles.

¹⁸ Berliner klin. Wochenschr., 1880, No. 31.

Mitchell¹⁹ has reported a case of absence of adipose matter in the upper half of the body. I had an opportunity of examining this patient, who was exhibited at a meeting of the Philadelphia Neurological Society, and noticed that both sides of the face presented a striking similarity to the affected side in cases of unilateral atrophy. The muscles were apparently not affected. Mitchell suggests the possibility of separate centres capable of restraining deposits of adipose tissue.

¹⁹ Am. Journ. Med. Sci., July, 1885.

DURATION AND TERMINATION.—The disease pursues a slow but generally progressive course. In a few instances, after having attained a certain development, it seems to have remained stationary for many years. Nothing could better illustrate the slow progress or stationary character of this affection than the study which Virchow made in 1880 of a patient whose case had been described by Romberg more than twenty years before. After comparing the patient's present condition with that described in notes taken twenty-one years before, Virchow was not able to convince himself that any material alteration had occurred in the atrophic parts; in fact, he regarded the disease as having long ago become stationary. Cases reported by Tanturri, Baerwinkel, and others bear out the same view as to the stationary character of the affection in some cases.

COMPLICATIONS.—In a case of Virchow's, in addition to the facial atrophy the patient presented atrophy which, commencing in the distribution of some of the dorsal cutaneous nerves of the scapular region, affected the musculo-spiral nerve from its origin downward, most markedly in the forearm and head. Mendel²⁰ describes a case of facial hemiatrophy occurring in a monomaniac, the diagnosis being complicated by the somatic signs of degeneracy—congenital facial and bodily asymmetry. The patient was twenty-eight years old, and the disease could be traced back to the seventh year. In several reported cases the disease has followed or has been associated with epileptic seizures. Buzzard²¹ has published a case in which the affection came on in a girl aged thirteen in the course of a second attack of chorea. In another it followed typhoid fever. In a case reported by Parry²² left hemiplegia with temporary disturbance of intelligence preceded the disease by about two years. In another, observed by Axman and Hueter, the patient, a man aged thirty-two, had suffered from irregular contractures of the left masticator muscle, which commenced in the seventh year. These spasms were associated with an increased delicacy of sensation in the region of the left trigeminus. Neuralgia is sometimes a complication. In a case reported by Holland,²³ the patient for several years suffered from paroxysms of fronto-temporal neuralgia.

²⁰ Neurologisches Centralblatt, June 15, 1883, quoted in Journal of Nervous and Mental Disease, 1883, x. p. 571.

²¹ Transactions of the Clinical Society of London, vol. v., 1872, p. 146.

²² Eulenburg.

²³ American Practitioner, vol. xxxi. No. 182, Feb., 1885, p. 72.

PATHOLOGY.—Up to the present time, so far as I have been able to learn, not a single autopsy in a case of typical progressive unilateral facial atrophy has been made. Conclusions as to pathology can therefore only be drawn from an analysis and study of symptoms and from comparisons with other degenerative affections. Most authorities hold that it is neurotic in origin. Lande, however, regarded it as having its origin in the connective tissue, affecting the nutrition of the other tissues by interfering with the circulation.

Different views have been held by those who have believed in its neurotic origin. Moore regarded it as a form of progressive muscular atrophy attacking the muscles supplied by the portio dura, but what has already been shown with reference to the symptomatology is sufficient to show that this is an erroneous hypothesis. The muscles supplied by the facial nerve may diminish in volume, but they do not undergo a true degeneration.

Brunner attributed the affection to continued irritation of the cervical sympathetic by some continuing lesion. Irritation of such a lesion, according to him, causes vascular spasms, diminishes blood-supply, and thus leads to the gradual development of the atrophy. This view, to say the least, has not been made out.

Virchow holds that the affection is peripheral, and not central. He believes, however, that a primary process being once set up in the nerve-trunks, owing to inflammations of the surrounding tissues it may then extend upward to the spinal and basal ganglia. The disease, according to this view, has some analogy to herpes zoster. The manner in which nerve-districts are unequally attacked is similar to what is observed in the various forms of lepra and in morphœa. The doctrine of a tropho-neurosis is the one that is best upheld by clinical facts. In the first place, various arguments can be adduced to show that the disease is neurotic in origin. It is limited to one side of the face and to special nerve-distributions. Affections of sensibility in the domain of the trigeminal nerve are frequently present. In the case given under Symptomatology, although anæsthesia was absent, the patient complained of peculiar paræsthesia in the form of stinging sensation. Either trophic centres or trophic fibres, which have the same connection and course as the trigeminal, are affected by a degenerative process. Whether nuclei, ganglia, or peripheral fibres are primarily affected has not yet been satisfactorily determined.

The case of Romberg, observed by Virchow twenty years after, was considered by the latter to entirely overthrow the vaso-motor theory, owing to the condition of the blood-vessels. While the surrounding tissues from the skin inward were wasted and shrunken, the veins and arteries of the parts appeared absolutely unchanged, the larger even projecting above the surface of the skin. Under stimulation the vessel dilated as in a normal individual, appearing to take no part in the atrophy.

DIAGNOSIS.—The chief affections from which progressive unilateral facial atrophy are to be diagnosticated are congenital asymmetry of the face and head, facial paralysis, progressive muscular atrophy, and hypertrophy of the opposite side of the face.

Congenital asymmetry of the face is observed in institutions for the idiotic and feeble-minded. Such asymmetry, however, is usually associated with corresponding defects of other parts of the body, as of the head and limbs. In the nervous wards of the Philadelphia Hospital is now, for instance, a patient the entire right half of whose body is very decidedly wasted, the case being one of epileptic idiocy with paralytic and choreic manifestations which point to atrophy of the opposite half of the cerebrum. At the Pennsylvania Training-School for Feeble-minded Children want of development of one-half of the head is often observed, but in these and in other congenital cases the special changes in nutrition, in the skin, in the color and growth of the hair, and in sensation, are absent. In some cases of congenital asymmetry of the head atrophy of the face or limbs, if present, will be upon the opposite side of the body; and this is what might be expected, as arrested development of the cranial vault will probably correspond with arrested development of the cerebrum of the same side and bodily defects of the opposite side.

Eulenburg, Maragliano, and others refer to an acquired want of symmetry of the face sometimes observed in cases of wry neck or scoliosis with deviation of the spinal column. Eulenburg especially speaks of the so-called habitual scoliosis, where a curve in the dorsal region in one direction has a compensatory cervical curve, the side of the face which corresponds to the dorsal curve being often smaller than the other. This asymmetry is supposed to result from compression of the vessels and nerve-roots in the concavity of the cervical curve. Here, again, the absence of special nutritional changes will assist in the diagnosis. A study of the spinal curvature will also be of value. Indeed, only the most careless observation would allow this mistake in diagnosis to be made.

Facial paralysis—Bell's palsy of the usual type—would not be likely, except by a careless observer, to be confounded with the disease under consideration. When the muscles in the facial distribution are involved, it is only in a very slowly progressive deterioration, which may be due to mechanical causes, as compression by the contracting skin. Moore,²⁴ as already stated, was wrong in speaking of the morbid changes as entirely confined to the portio dura of the left side. It is the fat, the connective tissue, and the skin which are first and chiefly affected. In unilateral facial atrophy no true paralysis of the facial muscles is present; consequently, the forehead can be wrinkled, the eye closed, the mouth drawn up or down, or sideways, etc.; in short, all the movements of the face are possible. The opposite of this, it goes without saying, is present in general facial paralysis. The drawing of the face to the side opposite the paralysis in Bell's palsy is not here observed; the mouth is sometimes drawn upward slightly on the atrophied side. In facial paralysis of any severity reactions of degeneration are present. In unilateral facial atrophy, as in progressive muscular atrophy, the muscles respond both to faradism and galvanism. As minor aids in making the diagnosis, the absence of loss of taste in one-half of the tip of the tongue, and of deflection of the tongue and uvula, are in favor of facial atrophy.

²⁴ Dublin Journal of Medical Science, vol. xiv., August and Nov., 1852, p. 245.

It is possible that a case of progressive muscular atrophy might begin in almost any muscle or group of muscles in the body. In progressive muscular atrophy, however, the appearance of the skin is different; it is not mottled; it is not thin, as can be demonstrated by manipulation of it; it can be raised by the fingers; it is soft and flaccid. In progressive muscular atrophy also first one muscle and then another, just as likely as not at some distance from each other, are attacked by the wasting process; in unilateral facial atrophy the affection spreads uniformly and regularly over one-half of the face. According to Duchenne of Boulogne, when progressive muscular atrophy first appears in the face the orbicularis oris is the muscle most likely to be attacked, causing the lips to appear enlarged and pendent, and giving a stupid look to the patient. In unilateral facial atrophy this appearance is not present. Progressive muscular atrophy commonly begins at a later age than unilateral facial atrophy.

Hypertrophy of the opposite side of the face is suggested by Eulenburg as a possible source of mistake; but a little study, however, will suffice to exclude this affection.

The following points are given by Eulenburg with reference to vitiligo and porrigo decalvans: “In vitiligo we find the same white decoloration of the skin, the cicatricial feel, the turning gray and falling out of the hair, but not the loss of volume, which is the special characteristic of this disease. In porrigo decalvans inflammatory symptoms and œdema of the skin come first; the disease first appears in regular circular spots; the hairs fall out without previous loss of color; and finally, the disease is contagious, and fungi can be demonstrated (microsporon Audouini).”

PROGNOSIS.—The prognosis as to recovery is bad, but, as already shown, the disease may long remain stationary.

TREATMENT.—No well-authenticated case of cure under any method of treatment has yet been reported. Electricity, either in the form of galvanism or faradism, has been resorted to in a majority of cases. In most of these temporary benefit has been produced by the employment of these agencies. Baerensprung reports two cases as having been greatly benefited by faradism. In Moore's case it also produced temporary improvement. Carefully administered massage should be tried. With reference to friction or massage, however, it is well, as advised by Eulenburg, to be cautious, as excoriations are easily produced. Measures directed to building up the nervous centres are indicated—such medicinal agents, for instance, as the salts of silver, gold, zinc, and copper, arsenical preparations, strychnia, and iron.

DISEASES OF THE MEMBRANES OF THE BRAIN AND SPINAL CORD.

BY FRANCIS MINOT, M.D.

The membranes or envelopes of the brain and cord were formerly reckoned as three in number: the dura mater, lying next to the inner surface of the bones of the cranium and spinal column, forming an internal periosteum; the pia mater, which is in immediate contact with the brain and cord; and the arachnoid, which was described as a distinct membrane loosely attached to the pia mater, but more firmly united to the dura, and constituting a closed sac or serous cavity called the cavity of the arachnoid. This view is now abandoned by most anatomists, who regard the external arachnoid as simply a pavement epithelium covering the internal surface of the dura, and the visceral arachnoid as constituting the external layer of the pia. The term cavity of the arachnoid has, however, been generally retained for the sake of convenience; and it gives rise to no obscurity if we remember that by it we simply mean the space between the dura and the pia.

The diseases of the membranes which I shall consider are—congestion and inflammation of the dura, including hæmatoma; congestion and simple and tubercular inflammation of the pia; and meningeal apoplexy.

The membranes of the brain and those of the cord may be separately diseased, or both may be affected at the same time. Simultaneous inflammation of the cerebral and spinal meninges is apt to occur epidemically, and is supposed to be zymotic in its origin. This is described in another article. (See EPIDEMIC CEREBRO-SPINAL MENINGITIS, Vol. I. p. 795.)

Inflammation of the Dura Mater of the Brain.

SYNONYM.—Pachymeningitis. Either surface of the dura may be inflamed separately, and in many cases the symptoms are sufficiently definite to render it possible to diagnosticate the situation of the disease in this respect during life, especially when taken in connection with the causes. For this reason it is usual to speak of external and internal pachymeningitis. A peculiar form of the latter is accompanied by the effusion of blood upon the arachnoidal surface, which sometimes forms a tumor called hæmatoma. Congestion is also sometimes included in the diseases of the dura mater, but is rarely alluded to by writers on pathology or clinical medicine.

Congestion of the Dura Mater.

Except as the first stage of inflammation, congestion of the dura can hardly exist unless in connection with the same condition of the pia or of the brain. Its causes are either local, such as thrombosis of the sinuses, syphilitic or other growths in the membranes; or remote, the principal being obstructions to the general circulation, including valvular disease of the heart, emphysema and other affections of the lungs, renal disease, compression of the superior cava or jugulars by aneurism and other tumors, delayed or suppressed menstruation, etc. The symptoms cannot be distinguished from those of congestion of the pia or of the brain—namely, headache, a sensation of throbbing, pressure, or weight in the head, somnolence, etc. The absence of fever would serve to distinguish the affection from an inflammatory condition of the membranes or brain.

Inflammation of the External Surface of the Dura.

SYNONYM.—External pachymeningitis.

ETIOLOGY.—The most frequent causes of external pachymeningitis are injuries to the cranial bones from violence, caries and necrosis of the same, and concussion from blows on the head. Next in order of frequency, if not even more common, comes the propagation of inflammation from disease of the inner ear and of the mastoid cells. It is only of late years that this important subject has been brought prominently forward and the danger of ear diseases in this respect fully pointed out. The channels of communication, as indicated by Von Tröltsch¹ in his valuable article on diseases of the ear, are along the arteries and veins which pass from the skin of the meatus and the mucous periosteal lining of the middle ear to the contiguous bone, while the capillaries of the petrous bone are in direct connection with the dura mater, so that the vessels of the latter are in communication with the soft parts of the external and middle ear. The vessels of the ear and the membranes of the brain are also directly connected through the diploëtic veins of the temporal bone, which discharge into the sinuses of the dura, as well as through the venæ emissariæ, which, arising from the sinuses, pass through the bone and discharge their contents into the external veins of the head. Von Tröltsch also points out that the extension of an inflammatory process may occur along the sheath of the facial nerve, the canal of which (canalis Fallopii) is a branch of the internal auditory canal and is lined by the dura mater. The latter is separated from the mucosa of the tympanum only by a thin, transparent, and often defective plate of bone. Inflammation may also be transmitted from the scalp by means of the vessels which pass through the bones of the skull. In this way erysipelas and other diseases of the integument sometimes give rise to external pachymeningitis.

¹ “Die Krankheiten des Gehörorganes,” von Anton von Tröltsch, in Gerhardt's Handb. der Kinderkrankheiten, Tübingen, 1879, 5 B., ii. Abt., p. 150; also English translation of the same, by J. O. Green, M.D., New York, 1882, p. 107.

SYMPTOMS.—There are no known symptoms which are characteristic of inflammation of the external surface of the dura. In cases of death from other diseases an autopsy may show traces of previous inflammation, such as thickening of the membrane and its firm adhesion to the cranial bones, which were not manifested during life by other symptoms than those which accompany meningeal disease in general; and in some instances none at all were known to have occurred. At a variable time after the receipt of an injury to the head the patient may complain of headache, followed by a chill, with high fever, vomiting, vertigo, delirium, unconsciousness, convulsions, etc., arising from compression by the products of inflammation. The same phenomena may follow the transmission of inflammation to the dura from caries or other disease of the bones, or from otitis medea purulenta. These symptoms usually continue without interruption, though there is sometimes more or less complete remission of the pain, and the patient may recover his consciousness for a time, thus giving rise to fallacious hopes. In a large proportion of cases the disease extends to the inner surface of the membrane and to the pia, without any noticeable change in his condition other than coma. In chronic external pachymeningitis the principal symptom is pain in the head, which may persist for weeks or months without other manifestations except drowsiness. Sometimes, on the contrary, there is obstinate vigilance. Mental symptoms, such as loss of memory, hallucinations, dementia, or mania, are sometimes noticed, ending, as in the acute form, in coma.

PATHOLOGICAL ANATOMY.—In chronic cases often nothing is found but thickening of the membrane, which generally becomes firmly united to the inner surface of the cranium. Indeed, these appearances are not unfrequently observed, to a limited extent, when there has been no history of disease to account for them. In other chronic cases the connective tissue of the membrane is found to be in part ossified, and the osteophytes of the cranium sometimes found in pregnant women and in patients with rheumatic cachexia are supposed to be due to a chronic inflammatory process of the dura. The first changes observed in acute external pachymeningitis are increased vascularization, shown by red lines corresponding to the blood-vessels, with punctiform extravasations, swelling, and softening of the tissue. Later, there is thickening of the membrane from new formation of connective tissue, and exudation of lymph, and sometimes of pus, which latter may accumulate between the dura and the cranial bones, or in traumatic cases may escape through openings in the skull. The dura and the pia become united in most cases of acute inflammation. The lateral sinuses frequently contain thrombi, which, when ante-mortem, are firm in structure, reddish-brown in color, often closely adherent to the walls of the vessel, and may extend to other veins, sometimes reaching as far as the jugulars. When purulent inflammation of a sinus occurs, its walls are thickened, softened, and discolored, and the inner surface is roughened. The thrombus becomes more or less purulent or sanious, disintegrates, and the infecting particles are carried into the circulation, giving rise to embolism and disseminated abscesses of the lungs, kidneys, liver, or spleen. Other lesions, such as injury or caries of the cranial bones and purulent inflammation of the middle ear and of the mastoid cells, are frequently found in conjunction with pachymeningitis, to which they have given rise. When the disease of the dura extends to the pia, the adjacent portion of the brain is often found implicated in the inflammation. In traumatic cases the dura may be detached from the bone and lacerated to a greater or less extent.

DIAGNOSIS.—The existence of external pachymeningitis may be suspected from cerebral symptoms following traumatic injury of the skull, erysipelas, or suppurative otitis medea, but apart from the etiology it would not be possible to distinguish it from internal inflammation of the membrane, or even from leptomeningitis.

PROGNOSIS.—In cases of suspected external pachymeningitis the prognosis will depend upon the evidence of effusion of pus or blood between the dura and the skull, and the possibility of their removal. A large proportion of cases are fatal, especially those arising from caries of the cranial bones and from the propagation of disease from the middle ear, the mastoid process, or the external surface of the skull, the inflammation extending through the membrane to the arachnoid surface of the dura, and also to the pia mater. This would be shown by a high temperature, rapid pulse, pain, and delirium, followed by coma and perhaps convulsions. The frequency with which external pachymeningitis occurs in connection with diseases of the ear should put the physician on his guard in cases of otorrhœa or pain in the ear, that he may warn the patient or his friends of the possibility of danger, and may employ an appropriate treatment.

TREATMENT.—In all cases of injury to the skull or of severe concussion the possibility of subsequent external meningitis should be borne in mind. The patient should be confined to the bed and be carefully secluded from excitement. Cold applications should be made to the head, the diet should be simple and somewhat restricted, and the bowels kept free without active purging. If there be evidence or suspicion of the presence of pus or of blood between the dura and the skull, means should be taken to evacuate the effusion. In case of inflammatory symptoms leeches should be applied behind the ears, and a stimulating liniment or croton oil rubbed on the scalp. There is no specific treatment. Pain and sleeplessness must be relieved by opiates and sedatives, and the strength must be sustained by nourishing diet and stimulants.

Internal Pachymeningitis.

Internal pachymeningitis is of two kinds: 1st, simple inflammation, which may be accompanied by purulent exudation and by a corresponding affection of the pia mater: 2d, hemorrhagic inflammation or hæmatoma of the dura mater. Simple inflammation of the internal or lower surface of the dura, without the coexistence of external pachymeningitis is rarely found in the adult. The morbid appearances differ but little from that of the external form, and the causes and the diagnosis are also similar. In children, however, it is not uncommon, according to Steffen.² The pus may discharge itself spontaneously through the fontanels or the sutures, or caries of the cranial bones may open a passage for its exit. It has also been evacuated artificially when the symptoms have indicated its presence. In other cases more or less extensive firm adhesions between the dura and pia mater have shown the previous existence of inflammation, but it would be difficult to say in which membrane it began. In some instances no symptoms are observed during life; in others the coexistence of inflammation of the pia, effusion into the ventricles, etc. prevent an exact diagnosis.

² “Die Krankheiten des Gehirns in Kindesalter,” von Dr. A. Steffen, in Gerhardt's Handb., 5 B., i. Abt., 2te Hälfte, p. 380.

The TREATMENT does not differ from that of inflammation of the external layer of the pia.

Hæmatoma of the Dura Mater.

SYNONYM.—Hemorrhagic pachymeningitis.

DEFINITION.—A chronic inflammation of the dura mater, resulting in the formation upon its inner surface of successive layers of false membrane, into and between which there is usually an effusion of blood, the whole sometimes forming a large solid mass between the dura and the cavity of the arachnoid.

ETIOLOGY.—Hemorrhagic pachymeningitis never occurs in healthy persons except from traumatic causes. It is most frequently observed in advanced life, and especially in the male sex. In a large number of cases the blood is in an unhealthy condition, and hence the disease is seen in alcoholism, in scurvy, in acute articular rheumatism, and in the acute febrile affections, as typhoid fever, pleuro-pneumonia, and pernicious anæmia, of which last, according to Huguenin,³ it complicates one-third of the cases. It is frequent among the chronic insane. It is occasionally met with in young children, but with them its causes are mostly unknown; according to Steffen,⁴ they may consist in alterations of the blood resulting from insufficient nourishment or from the infectious diseases, and from abnormal blood-pressure, as in whooping cough, asthma, etc., as well as from blows on the head.

³ G. Huguenin, “Acute and Chronic Inflammations of the Brain,” in Ziemssen's Encylop., Am. translation, New York, 1877, vol. xii. p. 401 et seq.

⁴ Op. cit., p. 386.

SYMPTOMS.—Many cases of hemorrhagic pachymeningitis complicating other cerebral diseases can be distinguished by no special symptom from the original malady. Thus, in cases of chronic insanity its existence may not be suspected during the lifetime of the patient. Steffen quotes⁵ the case, reported by Moses, of a child seven months old who died of catarrhal pneumonia, and who exhibited no symptom which could suggest any disease of the brain or its membranes. The autopsy revealed a pachymeningitic cyst extending over the anterior half of the right hemisphere. When symptoms are present they vary in different cases according to the acuteness of the inflammatory process, the amount and situation of the effusion, and the participation of the pia mater and brain in the disease. In the beginning they are usually indefinite, headache being the most common and often the only complaint. This may continue for weeks without any other indication of disease, but it is frequently accompanied by tinnitus aurium, vertigo, muscular weakness, and contraction of the pupils. Wakefulness and restlessness at night and slight twitching of the facial muscles or of the limbs sometimes occur. There may be no change in the condition of the patient for a considerable length of time (weeks or months), or he may improve to some extent, owing to the absorption of the serous portion of the effusion or to the tolerance of the foreign body acquired by the brain. Sooner or later a fresh hemorrhage is followed by a recurrence, and usually an aggravation, of all the symptoms. Sometimes the patient becomes comatose, and dies speedily with apoplectic symptoms, but this is not common at an early period. The extravasation of blood generally takes place in small quantities at a time, without giving rise to unconsciousness or paralysis. After a period of uncertain duration, when the tumor has attained considerable dimensions a condition of somnolence may take place, the patient sometimes sleeping twenty-four hours or longer at a time. Distinct paralysis rarely occurs, though hemiplegia is sometimes seen, and the paralysis has been observed on the same side with the lesion. When symptoms of compression appear the pupils become dilated. Toward the end of life the patient is usually in a state of profound coma, the pulse is slow and irregular, and the sphincters are relaxed.

⁵ Op. cit., p. 394.

The distinctive features of internal pachymeningitis, therefore, so far as they are at present known, are those which would depend upon a primary cerebral irritation, followed by those of compression of the brain—persistent, often severe, headache, with contracted pupils, and occasionally motor and sensory disturbances; afterward, coma, dilated pupils, and involuntary discharges from the bowels and bladder.

The DURATION of the disease is exceedingly variable, extending from a few days to more than a year. According to a table made from carefully observed cases by Huguenin, 74 per cent. of patients die within thirty days; 18 per cent. live from one to six months; 4 per cent. live from six months to one year; and 4 per cent. live over one year.

PATHOLOGY.—A difference of opinion has existed among pathologists concerning the true nature of hemorrhagic effusions of the inner surface of the dura mater, especially as to whether the membrane in which they are enclosed is formed by a deposit of fibrin from the effused blood itself, or whether the disease consists essentially in an inflammation of the dura, followed by a pseudo-membranous deposit into which blood is afterward extravasated. The latter explanation, offered by Virchow, has been generally accepted, but Huguenin, whose opportunities for observing the disease were unusually good, believes that the first stage of hæmatoma is not the formation of connective tissue, but simply an extravasation of blood.

The disease begins with hyperæmia of the dura mater in the area of distribution of the middle meningeal artery, followed by the formation upon its inner surface of an extremely delicate membrane in small patches of a yellowish color and studded with innumerable bloody points. If the membrane be carefully raised, it is found to be connected with the dura by very delicate blood-vessels, and to contain an immense number of very wide and thin-walled vessels, much larger than ordinary capillaries.⁶ At a later stage we find the membranes to be made up of several distinct layers, of which the innermost exhibits the character of the primary deposit, being thin and abundantly supplied with blood-vessels, while those of older formation have become successively thicker and are composed of tough, lustrous fibres of connective tissue almost as dense as that of the dura mater itself. The delicate structure of the false membranes and of the vessels which they contain leads almost inevitably to the effusion of blood, which increases in quantity with the thickness of the whole growth, so that in an advanced stage it is not uncommon to find large pools of blood, partly clotted and partly fluid, constituting what is called hæmatoma of the dura mater. The small bloody points are within the substance of the false membrane; the extravasations are interstitial, the largest being always found between the youngest membrane and the older layers. In some cases serous transudations also occur between the false membranes. In still rarer instances no blood or serum is found between or beneath the layers. Occasionally small quantities of blood escape by leakage through the delicate membrane into the cavity of the arachnoid.

⁶ See Rindfleisch's Manual of Pathological Histology, New Sydenham Soc.'s translation, 1873, vol. ii. p. 302.

The usual situation of internal pachymeningitis is the vault of the cranium, near the median line, the upper or external layer of the tumor being in relation with the dura mater, to which it is adherent, and the lower or internal with the arachnoid and pia mater. If the effusion be extensive, it spreads downward over the frontal and occipital lobes of the brain, and laterally toward the fissure of Sylvius. In the majority of cases the disease occupies both sides of the median line symmetrically, but it may be limited to one side only. According to Huguenin, 56 per cent. of the cases embrace the surfaces of both hemispheres; 44 per cent., that of one only. Hæmatoma very rarely occupies the base of the cranium. The thickness and consistency of the deposit vary in different cases, depending upon the number of successive exudations, and hence are most marked in cases of the longest duration.

Hemorrhagic pachymeningitis is occasionally met with in young children, but much more rarely than in adults. The disease is usually more acute than in older persons, but the anatomical appearances are essentially the same.⁷

⁷ For an able description of the lesions of hemorrhagic pachymeningitis in children see the third edition of Rilliet and Barthez's Maladies des Enfants, by Barthez and Sanné, Paris, 1884, vol. i. p. 152.

PROGNOSIS.—The great majority of cases end fatally. A few instances of recovery are reported in which the diagnosis seems justified by the symptoms. Moreover, it is not very rare to find, on post-mortem examination of those who have died of other diseases, evidence of the former existence of hemorrhagic pachymeningitis. According to Steffen, while the prognosis of simple pachymeningitis in children is frequently good, owing to the favorable conditions for the exit of pus through the open fontanels and sutures, in hemorrhagic pachymeningitis recovery is rare.

DIAGNOSIS.—In a disease whose symptoms are so vague the diagnosis must of necessity be difficult and often impossible. The chief elements for the formation of the diagnosis are the slow and interrupted progress of the affection, persistent headache, tendency to prolonged slumber, and final coma; the absence of paralysis of the cranial nerves, of vomiting, and of general convulsions; the age of the patient, the coexistence of alcoholism, chronic insanity, or acute rheumatism, and traumatism. The diseases for which hæmatoma would be most likely mistaken are acute inflammation of the meninges, apoplexy from cerebral hemorrhage, tumor in the substance of the brain, and necrobiosis from thrombus or embolus. The absence of symptoms characteristic of these affections might lead to a probable diagnosis by exclusion, but in many cases the existence of the disease cannot be determined with certainty. In children it is most likely to be confounded with tubercular meningitis, from which it may be distinguished by the fact that it is most common under the age of two years, the other being rare before that period, and that the characteristic symptoms of tubercular meningitis (prodromic period, vomiting, irregularity of pulse, etc.) are wanting. But the tubercular disease in young infants may closely resemble hæmatoma, a profound coma replacing all other symptoms. Here the existence of tuberculous disease in the parents or other near relatives would point strongly to tubercular meningitis, while the absence of such antecedents would suggest hæmatoma.

TREATMENT.—If the disease be a complication of alcoholism, anæmia, scurvy, rheumatism, etc., the treatment appropriate to those affections should be employed, along with remedies addressed to the local disease. For the early symptoms of irritation, perfect rest in bed, cold to the head, bromide of potassium internally, sinapisms to the back of the neck, together with simple and nutritious diet, are the most efficient means. The bowels must be regulated, but purging is not necessary. The abstraction of blood from the head by leeches to the temples or behind the ears, or by cupping, is recommended by most authorities, especially if there be much heat of the head. Mercury may be tried, care being taken not to salivate the patient. It is obvious that this treatment, in order to be of any avail, must be instituted at the earliest stage of the disease. After the probable formation of the hæmatoma an effort should be made to promote its absorption, which sometimes actually takes place, as is proved by the autopsies of patients who, having previously suffered from this affection, have died from other causes. The iodide of potassium or the iodide of sodium should be employed perseveringly for this purpose, in the dose of from ten to thirty grains three times daily. The bromide should also be continued if necessary. The acne which sometimes accompanies the continued use of these remedies may be prevented or cured by the use of three to five drops of the liquor potassæ arsenitis, given once or twice daily after meals. Counter-irritation to the scalp by means of stimulating liniments or croton oil, and small blisters to the temples, are likely to do good. In alcoholic subjects the amount of wine or spirit taken should be regulated by allowing enough to support the strength, without too suddenly withdrawing the accustomed stimulant. In the stage of coma the treatment must be purely expectant. The state of the bladder must be regularly examined, and the catheter employed when necessary. Liquid nourishment should be carefully given as long as the patient is able to swallow.

Cerebral Meningeal Hemorrhage.

SYNONYM.—Meningeal apoplexy of the brain. Hemorrhage of or upon the membranes of the brain is closely connected with intracerebral hemorrhage. Both arise from similar causes, and the former may result directly from the latter. It is for convenience of arrangement that the two subjects are treated separately in this work.

ETIOLOGY.—The causes of meningeal apoplexy are predisposing and immediate. The most important predisposing cause consists in disease of the cerebral vessels, especially the arteries, which favors the formation of so-called miliary aneurisms, as demonstrated by Charcot and Bouchard in the case of cerebral apoplexy. Disease of the vessels in its turn arises from various conditions, among which alcoholism in adults holds a prominent place from its tendency to favor a fatty degeneration of the vascular walls. In subjects of purpura or hæmophilia (the so-called bleeders), in whom, from an inherent weakness of the capillary vessels or a deficiency of the fibrin of the blood, or both combined, there is a tendency to extravasation of the blood in various parts, hemorrhage into the arachnoid cavity may occur.⁸ A new-born child under my observation had bleeding at the navel and ecchymoses in various parts of the body. Suddenly it became comatose, and it died with signs of cerebral compression. There was no autopsy, but it seems probable that hemorrhage into the cavity of the arachnoid had taken place. A sister of the patient had also had spontaneous hemorrhage from the navel and from other parts shortly after birth, but recovered. Chronic general arthritis and gout also probably predispose to the affection, as well as old age, which is accompanied by atrophy of all the tissues. The disease is most frequently met with in the two extremes of life; according to Durand-Fardel,⁹ in adults the largest number of cases occurs between the ages of seventy and eighty years.

⁸ A case of this kind is cited in the article “Pathologie des Méninges” in Nouv. Dict. de Méd. et de Chirurg. pratiques, Paris, 1876, vol. xxii. p. 101.

⁹ Traité clinique et pratique des Maladies des Vieillards, par M. Durand-Fardel, Paris, 1854, p. 283.

The exciting causes comprise injuries to the head, both with and without fracture; strong muscular effort, as in lifting, straining at stool or in labor; powerful action of the heart in cases of hypertrophy. An interesting case is reported¹⁰ by S. G. Webber of Boston, in which the effusion was evidently caused by vomiting; a clot of blood covered the greater part of the posterior two-thirds of the right hemisphere. Sometimes meningeal hemorrhage may arise from the bursting of an intracerebral apoplexy into the arachnoid cavity, as in a remarkable case occurring in the practice of Morris Longstreth of Philadelphia, of bilateral effusion.¹¹ Outside the dura, corresponding with the left middle cerebral lobe, was a considerable amount of blood connected with a fracture of the skull, and on the right side a large quantity of blood in the cavity of the arachnoid, originating in the middle lobe, which was torn up. The patient had fallen in the street; he was stupid, there was no paralysis, active delirium came on, followed by coma and death in twenty-four hours. Here was cerebral apoplexy bursting into the cavity of the arachnoid on the right side, and causing the fall, which was the occasion of the fracture and hemorrhage on the left side.

¹⁰ Boston Med. and Surg. Journal, Jan. 17, 1884.

¹¹ Ibid., Dec. 28, 1882.

In young children, especially in the new-born, meningeal hemorrhage may follow difficult and instrumental labor, either from injury to the skull or from delay in the establishment of respiration, as in breech presentation, though it sometimes occurs in cases in which the labor has been normal. In a case of breech presentation under my care in 1873 the child, a female weighing nine pounds, did not cry or breathe for some minutes after birth, although delivery had not been much delayed. Soon afterward it was noticed that it did not move the right arm, although it moved the hand and the fingers. In the course of twenty-four hours, during which time it cried much more than usual, it became comatose, and remained so until its death, three days after birth. The whole surface was livid, and the child had two or three short convulsions. At the autopsy a clot about the size of a grape was found in the pia mater on the upper surface of the cerebellum, in the immediate vicinity of the pons Varolii. The brain was so soft that the amount of injury received by the cerebellum could not be exactly ascertained, but it was probable that the clot extended into the fourth ventricle.

Thrombus of the sinuses of the dura mater, and less frequently of the cerebral arteries, is the origin, in a considerable number of cases, of meningeal hemorrhage in children, in consequence of pressure upon the delicate vessels of the membranes caused by the obstructed circulation. Bouchut¹² reports an observation of hemorrhage produced in this way.

¹² E. Bouchut, Clinique de l'Hôpital des Enfants maladies, Paris, 1884, p. 263. See, also, Steffen, op. cit., p. 352.

SYMPTOMS.—In some cases the attack is preceded by indications of congestion, such as headache, vertigo, staggering, confusion of ideas, noises in the ears, feeling of weight in the head, delirium, stupor. At the time of the attack the patient frequently complains of severe pain in the head, just as in cerebral hemorrhage, and then falls to the ground with complete loss of consciousness. Sometimes the symptoms come on gradually. Hemiplegia occurs in a notable proportion of cases. Convulsions may occur at any time after the attack. In Webber's case, already referred to, the first symptom was sharp pain in the head and neck; this was followed by very severe headache and pain on motion of the head. Intelligence gradually diminished; on the sixth day there was almost no consciousness, and the patient died in about eight days. She had occasional spasms, in which both eyes were turned toward the left in extreme conjugate deviation, and the left side of the face was distorted. The spasms were followed by suspension of respiration for nearly a minute, cyanosis, and paralysis of the left hand and leg. Both the nature of the lesion and its seat were correctly diagnosticated during the patient's lifetime.

As a rule, the condition of unconsciousness continues up to the time of death, but in some cases there are intermissions during which the patient responds to questions more or less promptly. Death takes place at a period varying between a few hours and several days. Durand-Fardel¹³ reports a case in which the patient lived a month from the first attack, with preservation of the intellect and of motion. An inmate of the Home for Aged Women in Boston, eighty-eight years old, previously in good health, complained of severe pain in the head one morning before rising. She took her breakfast in bed, and immediately afterward vomited copiously. From that moment she became insensible, and remained so until her death, seven days and three hours afterward. During this time there was no stertor. No decided paralysis could be discovered, but there was some rigidity of the left arm. At the autopsy an effusion of blood was found in the arachnoid cavity extending from below upward on each side to a level with the top of the ear. There was a large amount of blood at the base of the brain, and both lateral ventricles were distended with bloody serum. The vessels were generally in an atheromatous condition. There was no laceration of the brain. The source of the hemorrhage could not be ascertained. In such a case the condition of the patient in respect to power of movement often cannot be ascertained with certainty, in consequence of the abolition of consciousness. Complete muscular resolution is most common when the effusion is bilateral, but when the hemorrhage is limited to one side more or less paralysis of the opposite limbs may exist. Should the blood make its way into the spinal canal, it might give rise to special symptoms, but this is not probable in view of the large amount of the cerebral effusion under the circumstances, which would produce complete insensibility or speedily cause death by pressure on the medulla.

¹³ Op. cit., p. 202.

The temperature of the body immediately after a copious cerebral or meningeal hemorrhage falls below the normal point, and remains so for several hours, after which it rises, its degree varying according to circumstances. In fatal cases the elevation is extreme, and remains so until death. If the patient recover, it gradually returns to the normal standard.

Vomiting is a frequent symptom at the beginning of the attack, just as in intracerebral hemorrhage, owing probably to compression of the pneumogastric by the effusion at the base of the brain. In Webber's case the vomiting was evidently the cause of the hemorrhage, and not its consequence, since it had been a frequent symptom for several days before the attack, and was probably due to the presence of a calculus in the pelvis of the right kidney, which was found at the autopsy, and there was no blood at the base of the brain.

PATHOLOGICAL ANATOMY.—The chief points of interest in the morbid anatomy relate to the seat and source of the effusion, the amount and condition of the blood, the state of the vessels and that of the brain, including the ventricles. In respect to the seat, the hemorrhage may occupy the space between the cranial bones and the dura mater; it may be found on the lower surface of the latter, in the arachnoid cavity, or in the meshes of the pia mater, the so-called subarachnoid space. Blood found upon the outer surface of the dura, between that membrane and the bones, is almost always the result of traumatic causes, such as blows or other injuries, with or without fracture, or of caries of the skull. If below the dura, but between that and the so-called parietal layer of the arachnoid, the lesion comes under the title of pachymeningitis interna, already described as an inflammatory disease of the dura with hemorrhagic effusion. The arachnoid cavity and meshes of the pia are by far the most common situations in which the blood is found in meningeal hemorrhage. The origin of the effusion is either the rupture of a capillary aneurism of one of the arterioles of the membrane or of one of the vessels themselves in consequence of atheromatous or other degenerations of its walls. On account of the minute size of the vessels it is seldom possible to discover the exact point at which the rupture took place. In rare instances the source of the hemorrhage is within the brain, the blood being forced through the cerebral tissue, either into the meshes of the pia or upon the surface of that membrane. The amount of hemorrhage varies according to conditions which are mostly unknown, but is probably dependent upon the size of the ruptured vessel and the conditions under which the accident occurs, such as muscular effort, cardiac action, etc. In some cases it is so small as to give rise to no definite symptoms, as is evident from post-mortem examinations of those who have died from other causes. In these cases there may be either a single effusion or several. The amount is largest when the arachnoid cavity is the seat of the extravasation. The blood, which may be either liquid or more or less coagulated, according to the time which has elapsed since the hemorrhage, is usually found upon the convexity of the hemispheres, most frequently on one side only, and oftenest on the left. But if the rupture have taken place at the base, it often ascends on each side, as in a case mentioned above. Where a large vessel has given way, its contents may cover a great part of the surface of the brain. The coagulum is found in a thin layer, which at the end of a few days is covered with a transparent envelope, evidently composed of a deposit or separation of fibrin. Should the patient survive long enough, this membrane may become organized, receiving vessels from the adjacent pia, and in turn become the seat of new hemorrhages, exactly as in the hæmatoma of internal pachymeningitis. In cases in which absorption of most of the fluid part of the effusion takes place, the membrane remains more or less dense and vascular, and usually contains a small quantity of recently-effused blood within its meshes. Small cysts, containing transparent or reddish-brown serum, are also occasionally observed enclosed between the layers of the membrane. In very young children, whose fontanels are not yet ossified, these cysts sometimes attain to a large size, containing several pints of fluid, which is more or less limpid from absorption of the coloring matter of the blood, constituting the so-called dropsy of the arachnoid.¹⁴ The convolutions of the brain are more or less flattened according to the amount of the effusion, and the cortical substance is correspondingly anæmic from pressure. The blood may make its way, if extravasated in large quantities, into the ventricles, over the medulla, into the spinal arachnoid cavity, or even into the central canal of the spinal cord. The arteries of the brain, especially at the base, are frequently in a state of atheromatous degeneration, and thrombi often occupy the sinuses of the dura mater.