Chronic inflammation of the pia mater rarely follows the acute form, but is generally secondary to other conditions, such as inflammation and tumors of the dura, tumors and abscess of the brain, disease of the vessels of the brain, suppurative otitis, and to constitutional diseases, especially alcoholism, syphilis, and pulmonary tuberculosis. It is one of the most common lesions found after death from general paralysis of the insane. As a distinct affection, unconnected with constitutional disease, it is extremely rare, though less so, according to Flint,19than the acute form. He cites a case in which the symptoms were intermittent. The patient, fifteen years old, died after a month's illness. The autopsy showed cerebral hyperæmia, lymph at the base of the brain, and distension of the ventricles with transparent fluid. There were no tubercles. In most cases in which the results of chronic meningitis are found after death the cortical substance of the brain is involved in the disease; hencethe difficulty in defining its symptoms, which are usually extremely vague, and not always distinctive of cerebral disease. The principal are pain in the head, vertigo, vomiting, impairment of the memory, mental apathy, drowsiness, and muscular weakness. The anatomical changes are thickening and opacity of the membrane by the deposit of lymph upon its surface and into the connective tissue, adhesions to the dura and to the cortical substances of the brain, together with hyperæmia of the latter. These appearances are usually distributed in irregular patches of greater or less extent.
19Austin Flint, M.D.,Principles and Practice of Medicine, 5th ed., Philada., 1881, p. 701.
TheDIAGNOSISof chronic meningitis is often obscure or impossible. Long-continued pain in the head, accompanied by vertigo, impairment of memory, drowsiness, mental apathy, etc., without paralysis, would be suggestive of it, especially if there were occasional intermissions of the symptoms. The probability would be greatly increased if the patient had a syphilitic or alcoholic history. The diagnosis should exclude tumor of the brain, chronic pachymeningitis, and chronic hydrocephalus, but as these diseases are often complicated with chronic meningitis, the distinction might be very difficult. As already stated, chronic meningitis is almost a constant lesion in general paralysis, as well as in other forms of chronic insanity, but there are no special symptoms by which its presence can be ascertained during life.
TREATMENT.—Our aim should be to relieve pain, diminish congestion, and favor absorption. Counter-irritation to the head and nucha by means of small blisters or croton oil should be employed with moderation. Bromide of potassium, or, if necessary, small doses of morphia, may be given if the pain be severe. Should there be symptoms of cerebral congestion, such as acute delirium, flushing, and heat of head, an ice-bag should be applied to the head and leeches behind the ears, or blood may be drawn from the temples or nucha by cupping. As an absorbent the iodide of potassium is much recommended, but it is not likely to be effectual, except in syphilitic cases. The bowels should be kept free, but without active purging. The general health of the patient should be promoted by suitable diet and regimen, by relief from excitement and fatigue, or by change of scene and of climate. For the treatment of chronic meningitis complicating syphilis, alcoholism, and tuberculosis, the reader is referred to the articles treating of those diseases.
BYFRANCIS MINOT, M.D.
BYFRANCIS MINOT, M.D.
DEFINITION.—Inflammation of the pia mater of the brain, with effusion of lymph and pus, caused by the deposit of miliary tubercles upon its surface or into its substance.
SYNONYMS.—Scrofulous meningitis, Granular meningitis, Basilar meningitis, Acute hydrocephalus, Dropsy of the brain.
HISTORY.1—It is only within a comparatively recent time that tubercular meningitis has been distinguished from other cerebral diseases. Up to the eighteenth century the term hydrocephalus was employed not only for the dropsical diseases of the head, including internal and external hydrocephalus, but also for meningeal inflammations, both simple and tubercular, and for congestion of the brain and of the membranes; the accumulation of water in the ventricles or between the membranes being looked upon as the disease, and not as one of its consequences. The term was even applied to external tumors, as cephalæmatoma and caput succedaneum. We owe the first accurate account of the symptomatology of acute hydrocephalus, or ventricular dropsy, to Robert Whytt of Edinburgh, whose remarkable monograph, entitledObservations on the Dropsy of the Brain, first published in 1768, after his death, was founded upon the study of 20 cases with 10 autopsies. No addition of importance has been made by later observers to his graphic description of the disease or to his rules for its diagnosis. Whytt, however, had no clear notion of its pathogeny, and it was not till 1815 that Gölis pointed out that acute ventricular dropsy was a secondary condition depending upon previous inflammation of the membranes or vessels of the brain.
1See W. Hughes Willshire's valuable paper, entitled “Historic Data on Scrofulous Meningitis,” inBrit. and For. Med.-Chir. Review, Oct., 1854.
In 1827, Guersant remarked that the inflammation of the meninges constituting acute hydrocephalus presented such peculiarities as led him to denominate it granular meningitis. He did not, however, connect the granular deposit with tubercle. This was left for Papavoine to effect, who in 1830 published two cases of tuberculous arachnitis, in one of which effusion into the ventricles, or hydrocephalus, existed. The meningeal granulations or tubercles were described with care, and their coincidence with tuberculous deposit elsewhere was remarked upon, as also the apparent occurrence of the former previous to the inflammatory action in the meninges, and in one case the existence of the tuberculous granules without the sequence of inflammation. The important pathological element of acute hydrocephalus thus clearly pointed out by Papavoinenow became apparent to observers, and obtained almost universal assent. The attention of the profession in this country was first called to it by W. W. Gerhard of Philadelphia in 1833, in an admirable paper published in theAmerican Journal of the Medical Sciences,2containing the reports of thirty-two cases with autopsies. In every case but two tubercles were found in other organs besides the meninges. In one of these two, gangrenous cavities were found in the lungs without tubercles, though perfectly characterized miliary tubercles existed in the membranes; in the other case the lungs were not examined with care, Gerhard not being present at the autopsy.
2Vols. xiii. and xiv., 1833-34.
Finally, the distinction between tubercular and simple meningitis was pointed out by Guersant in 1839, and clearly established by Barthez and Rilliet in 1843 in their systematic work on the diseases of children; and it was further elucidated by Rilliet in 1847.
ETIOLOGY.—The causes of tubercular meningitis are predisposing and exciting. Among the former are hereditary tendency to tuberculosis and to the so-called scrofulous diathesis; the previous existence of tubercle in any part of the body, especially in the lungs; and the presence of caseous degeneration in the bronchial, the mesenteric, and other glands, or in the parenchyma of various organs, as the lung, the testicle, the liver, the spleen, etc. The dependence of miliary tuberculosis of the pia upon previously-existing caseous or other inflammatory deposits in some part of the body is acknowledged by most modern pathologists. Seitz3states that out of 130 cases, with autopsies, of adults, upon which his work is based, such deposits were found in 93.5 per cent. General constitutional weakness, either congenital or resulting from grave disease or from overwork, from insufficient or unwholesome food, and from bad hygienic surroundings, also favors the deposit of tubercle in the meninges. Sometimes two or more predisposing causes exist at once. Thus, a child born of tuberculous parents may be fed with artificial diet instead of being nursed, or may live in a house whose sanitary condition is bad. Hence the disease is common among the poor, although by no means rare in the higher classes of society. In some cases it is difficult or impossible to assign any predisposing cause. A single child out of a numerous family may be stricken with the disease, while the rest of the children, as well as the parents and other ascendants, are healthy. For instance, while writing this article I had under observation a little boy six years old whose parents are living and healthy, with no pulmonary disease in the family of either. The only other child, an older brother, is healthy. While apparently in perfect health the child was attacked with tubercular meningitis, and died in seventeen days with all the characteristic symptoms of the disease. At the autopsy there was found much injection of the cerebral pia everywhere, a large effusion of lymph at the base of the brain and extending down the medulla, abundance of miliary tubercles in the pia and accompanying the vessels in the lateral regions of the hemispheres, lateral ventricles distended with nearly clear fluid, ependyma smooth, choroid plexuses covered with granulations, convolutions of brain much flattened. Careful investigation, however, will usuallyenable us to detect some lurking primary cause, either in the family predisposition or in the history of the patient himself.
3Die Meningitis Tuberculosa der Erwachsenen, von Dr. Johannes Seitz, Berlin, 1874, p. 317.
Season appears to have but little influence on the production of the disease. The largest number of cases is observed during winter and spring, owing doubtless to the influence of the temperature and weather, and to the exclusion from fresh air, in favoring the development of tubercle and the scrofulous diathesis. Males, both children and adults, are somewhat more frequently attacked than females.
In regard to the exciting causes it may be said that where a disposition to the deposit of tubercle exists, anything which tends to lower the vitality of the individual is likely to hasten the event. In infants with hereditary tendency to tubercle, an improper diet is especially liable to develop meningeal tubercle. In older children, besides unwholesome or insufficient food and unfavorable hygienic surroundings, the acute diseases common to that period of life, such as the eruptive fevers, intestinal disorders, whooping cough, etc., often act as immediate causes. Sometimes the development of the disease may be traced to over-stimulation of the nervous system by excessive study, often aided by imperfect ventilation or overheating of the school-room. Caries of the temporal bone from disease of the middle ear may act as an immediate cause of tubercular meningitis, although simple meningitis is of course a more frequent result of that condition. The disease has been known to follow injuries of the head from blows or falls. In a larger number of cases the exciting cause is not discoverable, especially when the meningeal affection is simply an extension of the disease from some other part of the body, as the lungs, the bronchial or mesenteric glands, etc. This is often the case, both in adults and in children, when tubercular meningitis complicates pulmonary consumption.
SYMPTOMS.—The disease is most frequently observed in children between the ages of two and seven years. It is much less common in adults, who are generally attacked between the ages of twenty and thirty years. In the majority of cases the invasion of the malady is preceded by a prodromic stage, usually occupying from a few days to several weeks, though sometimes extending over a considerably longer period. This stage probably represents the process of deposit of miliary tubercles in the pia mater before their presence has given rise to much structural change in the tissue. The characteristic symptoms of the prodromic stage consist chiefly in an alteration of the character and disposition of the patient, varying in extent in different cases. In general, it may be said that he becomes sad, taciturn, apathetic, irritable, indisposed to play, often sitting apart from his companions, gazing in a strange way into vacancy. There is diminution or loss of appetite and some emaciation. He is restless at night, is disturbed by nightmare, or grinds his teeth. The digestion is deranged. Usually there is constipation, but occasionally diarrhœa, or these conditions may alternate with each other. Squinting and twitching of the facial muscles are sometimes noticed. Headache may occur early in this stage, but it is usually observed later, and it then forms a prominent symptom. Vomiting is also frequent, usually not preceded by nausea, sometimes provoked by sudden movement, as in sitting up in bed, and is apt to occur when the stomach contains little or no food. These symptoms vary much in degree, and they are often so slightthat they pass unnoticed by the parents or friends. Occasionally the patient, if a child, will manifest a strange perversity or an unusual disobedience, for which he is perhaps punished under the belief that his misconduct is intentional. In older children and in adults delirium, especially at night, sometimes followed by delusions which may be more or less permanent, is frequent at this stage. The above symptoms often remit from time to time, and during the interval the patient may seem to have recovered his health. The prodromic symptoms are rarely altogether wanting in children, although they may have escaped notice from lack of opportunity of observation on the part of the physician. On the other hand, as Steffen4justly observes, the most characteristic symptoms may be present, and lead even an experienced observer to a confident diagnosis of tubercular meningitis during the early stage of a case of typhoid fever or of cerebral congestion without tuberculosis.
4“Meningitis Tuberculosa,” by A. Steffen, inGerhardt's Handb. der Kinderkrankheiten, 5 B., 1ste Abth., 2te Hälfte, p. 465.
For convenience of description it is customary to divide the disease proper, after the prodromal period, into three stages—viz. of irritation, compression, and collapse. In some cases it is not difficult to observe these divisions, but it must be borne in mind that in others the symptoms do not follow any regular sequence, so that no division is possible. In infants profound slumber may be the only morbid manifestation throughout the entire disease. Steffen records such a case, and I have seen two similar ones.
First Stage: The interval between the prodromic period and the first stage is usually so gradual that no distinction between the two can be detected. In other cases the disease is ushered in suddenly by some striking symptom, such as an attack of general convulsions, with dilated pupils and loss of consciousness. This is not often repeated, though partial twitchings of the limbs or of the muscles of the face may follow at intervals. In young children a comatose condition, with unequal pupils, is apt to take the place of these symptoms. The principal phenomena of the first stage are headache, sensitiveness to light and sound, vomiting, and fever. The latter varies much in intensity from time to time, but is not usually high, the temperature seldom rising above 103° F., and usually, but not always, higher at night than in the morning; but there is no characteristic curve. The pulse varies in rate, but is usually slow and irregular or intermittent. The respiration is irregular, with frequent sighing. The tongue is dryish and covered with a thin white coat. The bowels are costive. Delirium is frequent at night, and the sleep is disturbed, the patient tossing about and muttering or crying out. The eyes are half open during sleep. These symptoms become more marked from day to day. The pain in the head is more frequent and severe; the patient presses the hands to the forehead or rests the head against some support if sitting up. During sleep he occasionally utters a loud, sharp cry, without waking. There is increasing apathy, and some intolerance of light, shown by an inclination to turn toward the wall of the room or to lie with the face buried in the pillow. The appetite is lost, the constipation becomes more obstinate, the slowness and irregularity of the pulse persist. With the rapid emaciation the belly sinks in, so that the spinal column can be easily felt. Soon the child falls into a state of almostcontinual somnolence, from which, however, he can be awakened in full consciousness, and will answer correctly, generally relapsing again immediately into slumber. His restlessness diminishes or ceases altogether, and he lies continuously on the back with the head boring into the pillow. He becomes more passive under the physician's examination, in strong contrast to his previous irritability. At the end of a week or more from the beginning of this stage symptoms of irritation of some of the cerebral nerves begin to show themselves, in consequence of pressure from the increasing exudation at the base of the brain and into the ventricles. Strabismus (usually convergent), twitching of the facial muscles and grimaces, grinding of the teeth, or chewing movements of the mouth are noticed. The somnolence deepens into sopor, from which it becomes more and more difficult to arouse the patient, who gradually becomes completely insensible.
Notwithstanding the alarming and often hopeless condition which this assemblage of symptoms indicates, intervals of temporary amendment not unfrequently take place. The child may awake from his lethargy, recognize those about him, converse rationally, take his food with relish, and exhibit such symptoms of general improvement that the parents and friends are led to indulge in fallacious hopes, and sometimes the physician himself ventures to doubt the accuracy of his diagnosis. Such hopes are of short duration; the unfavorable symptoms always return after a brief interval. The duration of the first stage may be reckoned at about one week.
Second Stage: This period is not separated from the preceding one by any distinct change in symptoms. The patient lies in a state of complete insensibility, from which he can no longer be aroused by any appeal. The face is pale or of an earthen tint, the eyes are half closed. If the anterior fontanel be still open, the integument covering it is distended by the pressure beneath. Often one knee is flexed, the opposite leg extended; one hand applied to the genitals, the other to the head. Sometimes one leg or arm is alternately flexed and extended. The head is apt to be retracted and bores into the pillow. The pupils are dilated, though often unequal and insensible to light: the sclerotica are injected; a gummy exudation from the Meibomian glands forms on the edges of the lids. The patient sighs deeply from time to time, and occasionally utters a loud, piercing cry. Paralysis, and sometimes rigidity of one or more of the extremities, are often observed, and occasionally there is an attack of general convulsions. The pulse continues to be slow and irregular, the emaciation progresses rapidly, and the abdomen is deeply excavated. The discharges from the bladder and rectum are involuntary. The average duration of the second stage is one week.
Third Stage: No special symptoms mark the passage of the second stage into the third, which is characterized by coma, with complete resolution of the limbs. The constipation frequently gives place to moderate diarrhœa. The distended fontanel subsides, and often sinks below the margin of the cranial bones. A striking feature of this stage is a great increase in the rate of the pulse, the heart being released from the inhibitory influence of the par vagum in consequence of the complete paralysis of the latter from pressure. The pulse varies in rapidity from 120 to 160 or more in the minute. For the same reason the respiration alsoincreases in frequency, though not to the same degree. The eyelids are widely open; the pupils are dilated and generally motionless, even when exposed to a bright light. The eyes are rolled upward, so that only the lower half of the iris is visible; the sclerotica is injected from exposure to the air and dust. Convulsions may occur from time to time. Death terminates the painful scene, usually in from twenty-four to forty-eight hours, but sometimes the child lives on for days, unconscious, of course, of suffering, though the afflicted parents and friends can with difficulty be brought to believe it.
Certain points in the symptomatology of tubercular meningitis demand especial consideration.
I have already observed that the division of the disease into definite stages is purely arbitrary, and is employed here merely for convenience of description; in fact, few cases pursue the typical course. A period of active symptoms and another of depression can often be observed, but these frequently alternate. Stupor and paralysis may characterize the early stage, and symptoms of irritation, with restlessness, screaming, and convulsions, predominate toward the end. Certain characteristic symptoms may be wholly or in part wanting, such as vomiting, constipation, or stupor.
The temperature shows no changes which are characteristic of the disease. Throughout its whole course it varies from time to time, without uniformity, except that it usually rises somewhat toward night. It seldom exceeds 102° or 103° F., unless shortly before death, when it may rise to 104° F., or even higher, and may continue to rise for a short time after death.
During the premonitory stage the pulse offers no unusual characteristics. Its frequency is often increased, as is usual in any indisposition during the period of childhood, but it preserves its regularity. Toward the close of this period, and especially during the first stage of the disease proper, a remarkable change takes place. It becomes slow and irregular, the rate often diminishing below that in health. The irregularity varies in character; sometimes the pulse intermits, either at regular or irregular intervals. An inequality in the strength of different pulsations is also observed. These peculiarities of the circulation are due to the irritation of the medulla and the roots of the par vagum, by which the inhibitory function of that nerve upon the action of the heart is augmented. During the last period, on the other hand, the increasing pressure on the vagus paralyzes its function, and the heart, freed from its control, takes on an increased action, the pulse rising to 120 beats, and often many more, in the minute. Robert Whytt, in his interesting memoir,5dates the beginning of the second stage from the time that the pulse, being quick but regular, becomes slow and irregular; the change again to the normal frequency, or beyond it, marking the commencement of the third stage.
5“An Account of the Symptoms in the Dropsy of the Ventricles of the Brain,” in theWorksof Robert Whytt, M.D., published by his son, Edinb., 1768, p. 729.
In the early stage the respiration presents nothing abnormal, but when the pulse becomes slow and irregular the breathing is similarly affected. Sighing is very common in the prodromal period and first stage. Toward the end of the second stage the increasing paralysisof the respiratory centre gives rise to the phenomena known as the Cheyne-Stokes respiration, consisting of a succession of respiratory acts diminishing in force until there is a complete suspension of the breathing, lasting from a quarter to three-quarters of a minute, when the series begins again with a full inspiration. In general, the variations in the rate of the respiration follow those of the pulse, though the correspondence is not always exact.
In the early stage of the disease the pupils are usually contracted and unequal. They are sluggish, but still respond to the stimulus of light. At a later period they become gradually dilated, and react even more slowly to light or not at all, the two eyes often differing in this respect. Ophthalmoscopic examination frequently shows the appearance of choked disc and commencing neuro-retinitis. In rare cases tubercles are seen scattered over the fundus of the eye. They are about the size of a small pin's head, of a yellowish color, and of sharply-defined contour. Neuro-retinitis and choked disc are not, of course, pathognomonic of tubercular meningitis, and choroidal tubercles are so rarely seen as to be of little avail in diagnosis. In fact, they are less frequent in this disease than in general tuberculosis without meningitis. In twenty-six cases of tubercular meningitis examined by Garlick at the London Hospital for Sick Children they were found only once.6The effect upon the conjunctiva of the unclosed lids has been already described.
6W. R. Gowers, M.D.,Manual and Atlas of Medical Ophthalmoscopy, Philada., 1882, p. 148. See, also, Seitz,op. cit., p. 347; Steffen,op. cit., pp. 452 and 472; and “Tubercle of the Choroid,”Med. Times and Gazette, Oct. 21, 1882, p. 498.
The tongue is somewhat coated soon after the beginning of the disease, and the breath is offensive. The appetite is lost, and there is decided emaciation in many cases during the prodromic period. The thirst is usually moderate. Vomiting is one of the most constant symptoms during the first period, and its occurrence on an empty stomach is characteristic of tubercular meningitis. It is not usually preceded by nausea, and often takes place without effort, by mere regurgitation, the rejected fluid consisting chiefly of bile mixed with mucus. Although constipation is the most common condition in the early stage, and is often rebellious to treatment, yet in some cases diarrhœa is observed, which may mislead the physician in respect to the diagnosis. From the beginning of the second stage, and sometimes earlier, the discharges from the bowels and the bladder are involuntary.
DURATION.—The duration of tubercular meningitis, apart from the prodromic period, which often can hardly be determined, averages from two weeks to two weeks and a half. In exceptional cases death may take place in a few days or a week, and occasionally a patient may linger for several weeks,7the difference being apparently due to the rapidity of the tubercular deposit and of the resulting inflammation and exudation. The patient usually takes to his bed at the beginning of the first stage, but he may be up during a part of the day until the beginning of the second. In rare instances the child will be about, and even out of doors, until a few days before death.
7Such a case is reported by Michael Collins in the LondonLancet, March 8, 1884.
PATHOLOGICALANATOMY.—The essential lesion of tubercularmeningitis consists in a deposit of miliary tubercles in the pia mater of the brain, giving rise to inflammation of that membrane and exudation of serum and pus. In the early stage both surfaces of the pia are reddened and more or less thickened, and present an opaline appearance, while between them—that is, in the meshes of the pia—we find a colorless and transparent fluid which is effused in greater or smaller amount, resembling jelly when viewed through the arachnoid. These conditions are sometimes observable on the convexity of the hemispheres, but are much more abundant on the lateral surfaces, and especially at the base. More distinct evidence of inflammation is shown by the presence of a yellowish or greenish-yellow creamy deposit on the surface of the pia, consisting chiefly of pus, which is also much more abundant at the base than elsewhere, especially about the optic commissure, infundibulum, pons Varolii, and the anterior surface of the medulla. The cranial nerves may be deeply imbedded in the deposit, which often extends into the fissure of Sylvius, gluing together the adjacent surfaces of the lobes, and accompanies the vessels, forming narrow streaks along the sides of the brain up to the convexity.
The miliary tubercles or granulations consist of semi-transparent bodies, grayish or whitish in color, varying in size from that of the head of the smallest pin, indeed almost invisible to the naked eye, to that of a millet-seed (whence their name). Larger masses are frequently seen, formed by the aggregation of smaller granulations. The tubercles are usually found on the inner surface of the pia, always in the immediate neighborhood of the blood-vessels, which they accompany in their ramifications, and are also scattered, in greater or less numbers, throughout the purulent exudation from the surface of the pia. They are most abundant at the base of the brain, ascending the sides along the course of the vessels. Sometimes, though rarely, they are more abundant on the convexity. The total number varies; it is usually very large, but sometimes only a limited number exists, even in well-marked cases, and along with intense inflammation of the pia. The granulations are found in different degrees of development—sometimes all of them similar in color, size, and consistency, at others in various stages of fatty degeneration. The distribution may be symmetrical in the two hemispheres or irregular. Under the microscope (after suitable preparation of the part) the bacillus tuberculosus in considerable numbers may be found in the pia, in places adjacent to the arterioles.8
8See a case reported by Y. Dawson in the LondonLancet, April 12, 1884, in which tubercles were visible only by the microscope with numerous bacilli.
The ventricles of the brain are usually distended with a clear or opalescent, rarely bloody, fluid, the amount of which generally corresponds to the intensity and extent of the meningeal inflammation, although sometimes it is not above the normal quantity. The two lateral ventricles are affected in an equal degree; the third and fourth ventricles are more rarely implicated. According to Huguenin,9it is doubtful whether acute inflammation of the ependyma takes place in tubercular meningitis. Steffen also10says that the ependyma is not inflamed, and that it is not the seat of the deposit of tubercles. This latter statement is denied by other authorities, and Huguenin is inclined to believe that they may existin that membrane. In the following case, under my care, abundant granulations were found on the surface of the ependyma:
9G. Huguenin,op. cit., p. 499.
10Op. cit., p. 449.
Olaf M—— (male), æt. 8 years, born in Denmark, entered Massachusetts General Hospital Sept. 13, 1881. Maternal grandmother died of consumption; paternal grandfather lived to the age of ninety-five years. One brother had some disease of hip. Patient was the child of poor parents and lived in an unhealthy suburb of Boston. During the two preceding winters he had a bad cough. He was apparently well till four weeks before his entrance, when he complained of bellyache, and became listless, but he was out of doors ten days before he came to the hospital. It was noticed that he was sensitive to sound. No vomiting, no diarrhœa, no epistaxis, no cry; some cough. He had been somnolent, and was observed to swing his arm over his head while asleep. June 14, when first seen by me, he was lying on his back, unconscious, eyes half closed, pupils dilated, jaw firmly closed, much emaciated, belly retracted, left leg occasionally flexed and extended. No priapism. The optic discs were reddened. June 15, there is some intelligence, he answers questions; keeps one hand on the genitals. June 16, pupils contracted, does not swallow. June 18, left eye divergent, conjunctiva injected, whole surface livid, cries out occasionally. Died at midnight.
FIG. 30.
Spasm of flexors
Autopsy.—General lividity of surface, much emaciation. Much fine arborescent injection on outer surface of dura mater. Numerous Pacchionian bodies. Yellow matter beneath arachnoid along course of vessels on each side of anterior lobes. Abundant fine granulations along course of vessels on each anterior lobe, on upper margins of median fissure, along fissure of Sylvius, and on choroid plexuses. Very little lymph at base of brain. Six or eight ounces of serum from lateral ventricles, and abundant fine transparent granules over ependyma of both. Numerous opaque granulations in pia mater of medulla oblongata. Surface of right pleura universally adherent. Mucous membrane of bronchia much injected; a considerable amount of pus flowed from each primary bronchus. No tubercles in lungs nor in peritoneum. No ulcerations in intestines. No other lesions.
The choroid plexuses are generally involved in the inflammatory process, and are sometimes covered with yellow purulent exudations. As in the above case, large numbers of tubercles may be found in them, notwithstanding the opinion of Huguenin that their number is always small.
The substance of the brain in the vicinity of the tubercular deposit is generally found in a more or less œdematous condition, owing to the obstruction of the circulation resulting from compression of the vesselsby the tubercles and effused lymph. Softening, sometimes even to diffluence, not unfrequently occurs in the neighborhood of the deposit, probably from ischæmia (necrobiosis). If there be any considerable amount of exudation in the ventricles, the convolutions are flattened by compression against the cranial bones.
The above-described lesions are not confined to the brain, but may extend to the cerebellum, the pons, the medulla, and the spinal cord. If examinations of the latter were more frequent in autopsies of this disease, we should doubtless find, as has been done in some instances, that the membranes often show the characteristic alterations of tubercular meningitis, and even the presence of granulations in the cord itself. The lesions may extend throughout the cord, and are especially noticed in the dorsal region and in the vicinity of the cauda equina. Their presence explains some of the symptoms evidently due to spinal origin, such as retraction of the head with rigidity of the neck and of the trunk, contractions of the limbs, tetanic spasms, priapism, paralysis of the bladder and rectum, etc., which are common in simple spinal meningitis.
The deposit of miliary tubercles in the pia mater, with little or no accompanying meningitis, is met with in rare instances. The tubercles are few in number, but vary in dimensions, being sometimes united together in masses of considerable size, which are frequently encysted. Beyond thickening and opacity of the membrane, their presence seems to excite but little inflammatory reaction, but they are generally accompanied by ventricular effusion which by its pressure gives rise to characteristic symptoms.
The principal lesions found in other organs of the body consist of tubercle in various stages of development, caseous matter, diseases of the bones, etc. Miliary granulations are chiefly seen in the lungs, peritoneum, intestinal mucous membrane, pleura, spleen, liver, and kidneys. The bronchial and mesenteric glands often contain caseous masses, some of which are broken down and suppurating. The testicles sometimes present the same appearances. In adults, the most frequent lesion which is found external to the brain is pulmonary tuberculosis in a more or less advanced stage. Tubercles are also sometimes present in the eye. Angel Money11states that out of 44 examinations made at the Hospital for Sick Children, London, the meninges were the seat of gray granulations in 42. The choroid (one or both) showed tubercles 14 times (right 3, left 5, both 6), and 11 times there were undoubted evidences of optic neuritis. Twice the choroid was affected with tubercle when the meninges were free; in one of these instances there was a mass of crude tubercle in the cerebellum; in the other, although there were tubercles in the belly and chest, there were none in the head. So that 12 times in 42 cases of tubercles in the meninges there were tubercles in the choroid—i.e.about 31 per cent.
11“On the Frequent Association of Choroidal and Meningeal Tubercle,”Lancet, Nov. 10, 1883.
DIAGNOSIS.—In many cases tubercular meningitis offers but little difficulty in the diagnosis. Although the symptoms, taken singly, are not pathognomonic, yet their combination and succession, together with their relation to the age, previous health, and antecedents of the patient, are usually sufficient to lead us to a correct opinion. The prodromic periodof altered disposition (irritability of temper or apathetic indifference), headache, constipation, vomiting, and emaciation, followed by irregularity and slowness of the pulse, sighing respiration, sluggishness and irregularity of the pupils; the progress from somnolence to unconsciousness and coma; the sudden lamentable cry; the convulsions and paralysis; the return of rapid pulse and respiration in the last stage,—are characteristic of no other disease. Our chief embarrassment arises during the insidious approach of the malady, before its distinctive features are visible or when some important symptom is absent. Its real nature is then apt to be overlooked, and, in fact, in some cases it is impossible to decide whether the symptoms are indicative of commencing cerebral disease, or, on the other hand, are owing to typhoid fever, to a simple gastro-intestinal irritation from error in diet, to worms in the alimentary canal, to overwork in school, or to some other cause. Under these circumstances the physician should decline giving a positive opinion until more definite signs make their appearance. It must be remembered that very important symptoms may be absent in cases which are otherwise well marked. In all doubtful cases the family history should, if possible, be obtained, especially whether one or both parents or other near relatives have been consumptive or have shown symptoms of scrofula or tuberculosis in any form, and whether the patient himself has signs of pulmonary tuberculosis, of enlarged or suppurating glands, or obstinate skin eruptions. The presence or history of those conditions would add greatly to the probability of tubercular meningitis.
The diseases for which tubercular meningitis is most liable to be mistaken are acute simple meningitis, typhoid fever, acute gastro-intestinal affections, eclampsia of infants and children, worms in the intestines or stomach, the hydrencephaloid disease of Marshall Hall, and cerebro-spinal meningitis.
Acute meningitis is distinguished from the tubercular disease by its sudden invasion without prodromatous stage, by the acuteness and intensity of the symptoms, the severity of the headache, the activity of the delirium, the greater elevation of the temperature, and by its brief duration, which rarely exceeds one week. In those exceptional cases of tubercular meningitis in which the prodromal period is absent or not observed and the course is unusually rapid, it would be perhaps impossible to distinguish between the two diseases. A family history of tubercle, or the discovery of the granulations in the choroid by ophthalmoscopic examination, might save us from error under such circumstances. The great rarity of idiopathic simple meningitis should be remembered. Meningitis from disease of the ear sometimes resembles the tubercular affection, but the history of the attack, usually beginning with local pain and otorrhœa, will in most cases prevent any confusion between the two forms of disease.
The early period of typhoid often bears considerable resemblance to that of tubercular meningitis. Headache, languor, restlessness, and mild delirium are common to both. Typhoid can be distinguished by the coated tongue, the diarrhœa, the enlargement of the spleen, the tympanites, abdominal tenderness and gurgling, the eruption, and, above all, by the characteristic temperature-curve, which, if accurately observed, is conclusive. The course of typhoid fever is comparativelyuniform, while that of tubercular meningitis is often extremely irregular. It should not be forgotten that the two diseases may coexist.
The presence of worms in the alimentary canal may cause symptoms somewhat like those of tubercular meningitis, and the symptoms of the latter disease are occasionally erroneously attributed to those parasites. The administration of an anthelmintic, which should never be omitted in doubtful cases, will clear up all uncertainty.
Cerebro-spinal meningitis is usually an epidemic, and therefore not likely to be confounded with the tubercular disease. In sporadic cases it can be recognized by its sudden onset and acute character, by the eruption, and by the prominence of the spinal symptoms.
The so-called hydrencephaloid disease of Marshall Hall is a condition of exhaustion and marasmus belonging to infancy, caused by insufficient or unsuitable nourishment, by diarrhœa, and by the injudicious depletive treatment so much in vogue in former times, when the affection was much more common than at present. Some of its symptoms, such as sighing respiration, stupor, pallor, and dilated pupils, bear a certain resemblance to those of tubercular meningitis, though it would be more easily confounded with chronic hydrocephalus. The absence of constipation, headache, convulsions, and vomiting, and the favorable results of suitable nourishment and stimulants, serve to distinguish it from cerebral disease.
Eclampsia, or sudden convulsion, is common in infants and young children, and, since the occurrence of a fit may be the first or the most striking symptom in tubercular meningitis, it is important to ascertain its origin. In the majority of cases convulsions in children arise from some peripheral irritation, such as difficult dentition, worms in the alimentary canal, constipation, fright, etc., acting through the reflex function of the spinal cord, which is unusually sensitive in the early period of life. The absence of previous symptoms, and the discovery of the source of the irritation, with the favorable effect of its removal by appropriate treatment, will in most cases suffice to eliminate structural disease of the brain. In others we must withhold a positive opinion for a reasonable time in order to ascertain whether more definite symptoms follow. Convulsions also occasionally form the initial symptom of the eruptive fevers, especially scarlatina. Here the absence of prodromal symptoms, and the speedy appearance of those belonging to the exanthematous affection, will remove all sources of doubt. Convulsions, with or without coma, occurring in the early stage of acute renal inflammations, may simulate the symptoms of tubercular meningitis. An examination of the urine will show the true nature of the disease.
In addition to the above diseases there are some cerebral affections of uncertain pathology which resemble tubercular meningitis, but which are not generally fatal. As Gee justly remarks,12“Every practitioner from time to time will come across an acute febrile disease accompanied by symptoms which seem to point unmistakably to some affection of the brain, there being every reason to exclude the notion of suppressed exanthemata or analogous disorders. After one or several weeks of coma, delirium, severe headache, or whatever may have been the prominent symptom, the patient recovers, and we are left quite unable to say what has been thematter with him. To go more into detail, I could not do otherwise than narrate a series of cases which would differ from each other in most important points, and have nothing in common excepting pyrexia and brain symptoms. There is, generally, something wanting which makes us suspect that we have not to do with tubercular meningitis. Brain fever is as good a name as any whereby to designate these different anomalies; cerebral congestion, which is more commonly used, involves an explanation which is probably often wrong, and certainly never proved to be right.” No doubt such cases are occasionally cited as examples of recovery from tubercular meningitis.
12“Tubercular Meningitis,” by Samuel Jones Gee, M.D., inReynolds's System of Medicine, Philada., 1879, vol. i. p. 832.
PROGNOSIS.—Although there are on record undoubted instances of recovery from tubercular meningitis, yet their number is so small that practically the prognosis is fatal. It is safe to say that in almost all the reported cases of recovery the diagnosis was erroneous.13Even should the patient survive the attack, he is usually left with paralyzed limbs and impaired mental faculties, and dies not long afterward from a recurrence of the disease or from tuberculosis of the lungs or other organs.
13Hahn, “Recherches sur la Méningite tuberculeuse et sur le Traitement de cette Maladie” (Arch. gén. de méd., 4eSérie, vols. xx. and xxi.), claims to have cured 7 cases, but of 5 of them there is no evidence that they were examples of tubercular meningitis at all. The subject of the curability of tubercular meningitis is ably treated by Cadet de Gassicourt (Traité clinique des Maladies de l'Enfance, vol. iii., Paris, 1884, p. 553et seq.). His conclusion is that most of the alleged cures are cases of meningitis of limited extent, arising from the presence of tubercular tumors, syphilitic gummata, cerebral scleroses, and neoplasms of various kinds.
TREATMENT.—In view of the fatality of the disease, and of its frequent occurrence in childhood, the prophylactic treatment is of great importance. Every effort should be made to protect children whose parents or other near relatives are tuberculous or scrofulous, and who are themselves delicate, puny, or affected with any constitutional disorder, from tubercular meningitis, by placing them in the best possible hygienic conditions. Pure air, suitable clothing, wholesome and sufficient food, and plenty of out-of-door exercise are indispensable. Sedentary amusements and occupations should be sparingly allowed. Especial pains should be taken to prevent fatigue by much study, and school-hours should be of short duration. The hygiene of the school-room is of paramount importance, and if its ventilation, temperature, and light are not satisfactory, the child should not be permitted to enter it. The bed-chamber should be well ventilated night and day. A sponge-bath, cold or tepid according to the season or to the effect on the patient, should be given daily, followed by friction with a towel. The bowels must be kept regular by appropriate diet if possible, or by simple laxatives, such as magnesia or rhubarb. For delicate, pale children some preparation of iron will be useful. The choice must be left to the practitioner, but one of the best in such cases is the tartrate of iron and potassium, of which from two to six grains, according to the age, may be given three times daily after meals. Cod-liver oil is invaluable for scrofulous patients or where there is a lack of nutrition. A teaspoonful, given after meals, is a sufficient dose, and it is usually taken without difficulty by children, or if there be much repugnance to it some one of the various emulsions may be tried in proportionate dose. Along with this, iodide of iron will in many cases be found useful or as a substitute for the oil when the latter cannotbe borne. It is best given in the form of the officinal syrup, in the dose of from five to twenty drops. Change of air is useful in stimulating the nutritive functions, and a visit to the seashore or mountains during warm weather will often be followed by general improvement.
Since it is not possible to arrest the disease when once begun, the efforts of the physician must be directed toward relieving the sufferings of the patient as far as possible. In the early period the restlessness at night and inability to sleep will call for sedatives, such as the bromide of sodium or of potassium, in the dose of ten or fifteen grains at bedtime or oftener. This should be well diluted with water, sweetened if necessary. The addition of five to twenty drops of the tincture of hyoscyamus increases the effect. Sometimes chloral hydrate, either alone or combined with the bromide when the latter fails, will procure quiet sleep. From five to ten grains may be given at a dose, according to the age. Compresses wet with spirit and water or an ice-cap may be applied to the head if there be much pain in that region, or it may be necessary to give opium in some of its forms by the mouth, such as the tincture or fluid extract, in doses of from one to five drops. Constipation is best overcome by means of calomel in three- to five-grain doses, to which may be added, when necessary, an equal amount of jalap powder, or an enema of soapsuds may be administered. Active purging should be avoided. Liquid nourishment, such as milk, gruel of oatmeal, farina, or barley, beef-tea, broths, etc., must be given in moderate quantities at intervals of a few hours so long as the patient is able to swallow. Occasional sponging of the whole surface with warm or cool water, and scrupulous attention to cleanliness after defecation, especially when control of the sphincters is lost, will add to his comfort. He should occupy a large and well-ventilated chamber, from which all persons whose presence is not necessary for his care and comfort should be excluded. He should be protected from noise and from bright light, and should lie on a bed of moderate width for convenience of tending.
There is no specific treatment at present known which is likely to be of any benefit in this disease, any more than in tuberculosis of other organs than the brain. Common experience has shown that mercury, which formerly had so high a reputation in the treatment of cerebral diseases of early life, not only fails completely, but adds to the sufferings of the patient when pushed to salivation. The iodide of potassium is recommended by almost all writers, but, so far as I know, there is no proof that it possesses any virtue in meningeal tuberculosis. It has the high authority of Charles West,14however, who thinks the remedy is more encouraging than any other, and who mentions one instance in which recovery took place under its employment. He recommends that two grains be given every four hours to a child three years old, the bowels being kept free. Most authorities recommend much higher doses, such as ten or fifteen grains, three or four times daily.
14Op. cit., p. 102.
Counter-irritation to the head or back of the neck was formerly much employed, but is now generally abandoned, as giving rise to much discomfort without obvious beneficial effect. In the cases reported by Hahn, already alluded to under the head of Prognosis, recovery is attributed to the energetic application of tartar-emetic ointment to the scalp,producing extensive ulceration, which in one of them lasted more than ten months before cicatrization took place. A careful examination of the reports of these cases satisfies me that but two out of the seven were really examples of tubercular meningitis. How far the recovery in the successful cases is to be attributed to the treatment is very doubtful. Small blisters applied to the vertex or back of the neck are alluded to favorably by West, but he quotes no observations in which they were followed by benefit.