BYCHARLES K. MILLS, A.M., M.D.
BYCHARLES 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 ANDLITERATURE.—This disease has been known to the medical profession since 1825, when, according to Eulenburg, it was first described by Parry. TheIndex Cataloguecontains references to thirty books and articles on the subject in various languages. Eulenburg gives a bibliography which contains thirteen references not given in theIndex 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,1according to Lasègue,2first 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. Aran3has 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 Bannister4is 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 Eulenburg5contains an excellent sketch of the disease, and is especially valuable for the discussion of the various theories as to its nature.
1Illustrations of the Elementary Forms of Disease, 1836.
2Archives générales de Médecine, May, 1852, p. 71.
3Ibid., Sept. and Oct., 1850.
4Journal of Nervous and Mental Disease, vol. iii., No. 4, Oct., 1876, p. 539.
5Ziemssen'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.
Graefe6reports 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 Maragliano7of 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. Schuchardt8speaks 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. Panas9reports a case in which the wasting of the face followed a fracture of the lower jaw. In one of two cases reported by Bannister,10a 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.
6Quoted by Rosenthal.
7Note di Clinica Medica, Genoa, 1881, translated by Joseph Workman, M.D.—Alienist and Neurologist, vol. ii. No. 2, April, 1881, p. 146.
8Quoted by Eulenburg.
9Bull. Soc. de Chir. de Par., 1869, 1870, 2d S. ex., 198, andGaz. des Hôpitaux, 1869.
10Journ. 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 tothe 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 Duhring11occurs 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.
11Medical 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 kindserve 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. Nicati12considers 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. Brunner13gives 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.
12Sur Paralysie du Nerf sympathetique-cervical, Lausanne, 1873, quoted by Bannister.
13St. 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 Tanturri14and by Vulpian,15and perhaps by others, but anæsthesia is not commonly present.
14Il Morgagni, 1872, quoted by Bannister.
15L'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 superiorsuperficial 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,16noticing 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.”
16Deutsch. 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 letterrwas somewhat impeded. Beverly Robinson,17reports 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.
17Am. Journ. Med. Sci., October, 1878, p. 437.
Flascher18has 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.
18Berliner klin. Wochenschr., 1880, No. 31.
Mitchell19has 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.
19Am. Journ. Med. Sci., July, 1885.
DURATION ANDTERMINATION.—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. Mendel20describes 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. Buzzard21has 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 Parry22left 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,23the patient for several years suffered from paroxysms of fronto-temporal neuralgia.
20Neurologisches Centralblatt, June 15, 1883, quoted inJournal of Nervous and Mental Disease, 1883, x. p. 571.
21Transactions of the Clinical Society of London, vol. v., 1872, p. 146.
22Eulenburg.
23American 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 halfof 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,24as 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.
24Dublin 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 regularlyover 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.
BYFRANCIS MINOT, M.D.
BYFRANCIS 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 EPIDEMICCEREBRO-SPINALMENINGITIS, Vol. I. p. 795.)
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.
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.
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 Tröltsch1in 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, inGerhardt'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 deathfrom 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.
PATHOLOGICALANATOMY.—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 theinflammation. 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.