CHRONIC HYDROCEPHALUS.
BY FRANCIS MINOT, M.D.
SYNONYMS.—Dropsy of the brain, Dropsy of the head.
DEFINITION.—A gradual accumulation of serous fluid in the brain, occupying either the ventricles or the cavity of the arachnoid, or both, occurring chiefly in infants or very young children.
The term hydrocephalus, which was applied by the older writers to accumulations of serous fluid both within and also without the cranium, termed distinctively internal and external hydrocephalus, is now restricted to dropsical effusions either between the meninges or within the ventricular cavities. These may be acute or chronic, and they arise from the same conditions which are followed by the effusion of serum in other parts of the body; that is to say, from an alteration in the serous membrane lining a cavity, from an obstruction in the capillary circulation with increased tension in the larger vessels, from an altered condition of the blood, etc.
Chronic hydrocephalus is almost entirely confined to young children, and is probably due to an arrested development of the brain, as shown by its being usually congenital, by the dwarfed intellectual condition of the patient, and by its frequent association with spina bifida. The pathogeny of the disease is, however, still obscure. Whether the abnormal accumulation of serous fluid is to be ascribed to a chronic alteration of the ventricular walls or of the choroid plexuses, allied to inflammation, such as occurs in the pleura, for example, causing an increased secretion, or to a closure of the communication between the ventricles and the spinal cavity, as suggested by the late John Hilton, resulting in dropsy by retention, or to some other cause, is not yet determined. Hilton says:1 “In almost every case of internal hydrocephalus which I have examined after death I found that this cerebro-spinal opening [between the fourth ventricle and the spinal canal] was so completely closed that no cerebro-spinal fluid could escape from the interior of the brain; and, as the fluid was being constantly secreted, it necessarily accumulated there, and the occlusion formed, to my mind, the essential pathological element of internal hydrocephalus.” Sieveking, commenting upon Hilton's theory, says:2 “While giving these facts due weight, it must be pointed out that we are yet far from understanding either how the fluid is poured into the cerebral cavities or how it is removed, and that we do not positively know that the spinal canal has any better means of getting rid of an excess of fluid than the cerebral cavities have.” An arrest of the growth of the brain is supposed by some pathologists to account for ventricular as well as arachnoidal dropsy by the creation of a vacuum in the cavity of the cranium, which is filled by exudation of the more fluid portion of the blood from the vessels or of lymph from the lymphatics.
1 John Hilton, Lectures on Rest and Pain, etc., 2d ed., New York, 1879, p. 22.
2 Jones and Sieveking's Pathological Anatomy, 2d ed., London, 1875, p. 248.
ETIOLOGY.—That chronic hydrocephalus in young children is in a large proportion of cases an hereditary disease is shown by the fact that it is frequently congenital, that more than one child in a family is occasionally affected by it, and that while one child is hydrocephalic others may be idiotic. A scrofulous taint in the family history is noticeable in many cases, the evil effect of which is frequently enhanced by unfavorable sanitary conditions of life, especially by residence in dark, damp, ill-ventilated, and badly-drained apartments and by insufficient or unwholesome food. Hence the disease is more frequently met with among the poor than in the well-to-do classes of society. There is a difference of opinion as to whether it ever arises in consequence of external violence, such as a blow on the head, or of some strong impression, like fright or grief, acting on the mother of the child during pregnancy. The probability is that such causes would not be efficient except in cases where a predisposition to hydrocephalus had already existed. West3 mentions the case of a healthy child five months old who fell out of the arms of her nurse, and was taken with a fit the same day. She apparently recovered, but when a year old had frequent returns of convulsions. At the age of fifteen months the head began to enlarge, and it continued to increase in size until she was three years old, when she was attacked by measles, and died in a few days with convulsions and coma. The symptoms, both bodily and mental, were typical of hydrocephalus, and the diagnosis was fully confirmed by the autopsy.
3 Op. cit., p. 127.
The causes of chronic hydrocephalus in older subjects and in adults are, in addition to the above mentioned, chiefly mechanical. Any lesion which hinders the exit of venous blood from the cranium may be followed by dropsy of the arachnoid cavity or ventricles. The principal ones are tumors of the brain or its membranes, the effusion of lymph in the neighborhood of veins, thrombosis of the cerebral sinuses, compression of numerous small vessels by tubercular granulations, obstructions outside the cranium, including tumors of the neck, aneurisms, obstructive disease of the heart, emphysema and cirrhosis of the lungs, besides diseases giving rise to general dropsy, as the different forms of nephritis, marasmus, the various cachexiæ, etc.
SYMPTOMS.—When the disease is already somewhat advanced before birth, the head is often abnormally large, the cranial bones are separated, the fontanels are distended and fluctuating, and it occasionally happens that delivery of the distended head can only be effected after the fluid has been evacuated by puncture. Even when there is no abnormal increase in the size of the head, indications of cerebral disturbance are sometimes apparent from birth, such as strabismus or convulsions recurring with more or less frequency, along with signs of failure of the general health. In the course of some weeks, or, it may be, months, the attention of the parents is attracted to the prominence of the child's forehead, strongly contrasting with the comparatively diminutive size of the face. Soon an enlargement of the fontanels is perceived, the sutures between the cranial bones become broader, and the head assumes a globular shape from the pressure of the contained fluid. The separation of the bones at the vertex of the skull causes the os frontis to protrude forward, the parietals backward and outward, and the occipital backward and downward. The orbital plates of the frontal bone yield to the pressure of the fluid behind them, and from a horizontal position tend to assume a vertical one, protruding the eyeballs, which have a peculiar downward direction. The sclerotica is visible above the iris, and the latter is partially covered by the lower eyelid. The enlargement of the head is progressive, though not uniformly so, there being pauses of weeks or months during which it is arrested. If it be considerable, the cranial bones are usually thinned, so that the skull becomes translucent when opposite a bright light. When there is a wide separation of the bones there is an unusual development of ossa triquetra in the sutures.
In cases in which the ossification of the cranium is considerably advanced before the beginning of the disease, the enlargement of the head is apt to be correspondingly less. If all the sutures are consolidated, there may be no increase in size, and this is especially true of the mechanical dropsy of adult life. The dropsical effusion, which is then moderate in amount, finds room through compression of the brain-substance, and part of it escapes into the spinal canal.
The size of the hydrocephalic head varies considerably. Where there has been an early arrest of the disease it may be but slightly above the normal. On the other hand, the dimensions are sometimes enormous. In the Warren Museum of Harvard University is a cranium which measures 27½ inches in its greatest circumference, and 20½ inches from one auditory foramen to the other over the top of the head. Its internal capacity is 257 cubic inches. The patient, who died at the age of three years, was never able to sit up and support the head, or even to turn it from the left side, on which she continually lay; she never spoke, and seemed to have no intelligence. The skull of the celebrated James Cardinal measured 32¼ inches in its largest circumference.4 Even more remarkable instances are on record. The dimensions of the fontanels, particularly the anterior, usually correspond with those of the skull in general. During any strong muscular effort on the part of the patient the membrane covering them is seen to be bulged out by pressure from the fluid beneath. The enlargement is not always uniform; it may be in part or wholly confined to one side, owing to consolidation of the sutures of the opposite side, or because only one of the lateral ventricles is affected. The scalp is traversed by numerous distended veins. The hair is very scanty. The head has a soft, fluctuating feel, and the walls sometimes seem to crackle beneath the fingers, like parchment.
4 Reports of Medical Cases, etc., by Richard Bright, M.D., London, 1831, vol. i. p. 431.
In cases of moderate severity there may be few or no symptoms of active cerebral disturbance, such as convulsions or paralysis, but the child does not learn to talk, to walk, or to control the sphincters even at the age of three or four years, and the signs of imperfect mental development are evident. He is apt to be irritable or mischievous, and even when not actually idiotic is very backward in evincing signs of intelligence. There are, however, not a few exceptions to this, and some children with large hydrocephalic heads are intelligent and amiable. The cranium of a girl who died at the age of sixteen years is preserved in the Warren Museum. It measures 24¼ inches in circumference and 17¼ inches from one auditory meatus to the other over the vertex, and the bone is in no place more than one-eighth of an inch in thickness. The child was an inmate of a house of industry, where she was instructed in the usual branches of knowledge taught in our common schools, until at length, such was her capacity, she was entrusted with the teaching of the other pauper children, and she had an excellent character for intelligence and moral worth. She died of phthisis. On post-mortem examination “the brain was found floating, as it were, in a large collection of water.” As in other chronic diseases, there are often pauses, from time to time, of variable duration, in which there is some improvement in the condition of the patient, as well as a temporary arrest in the enlargement of the head. The growth of the child in stature is often retarded, and when life is prolonged the individual is more or less dwarfed. In some cases there is a considerable increase in the amount of adipose tissue, and the appetite is often voracious. L. Fürst reports5 the case of a hydrocephalic girl, sixteen years old, whose height (or, rather, length, for she was unable to stand) was 81 centimeters (nearly 32 inches), corresponding to the stature of a child between three and four years old. The periphery of the head measured 511/5 centimeters (20¼ inches). The anterior fontanel was still open. The age was verified by reference to the registry of birth at the police-office.
5 “Exquisite Wachsthumshemmung bei Hydrocephalus chronicus,” von Dr. Livius Fürst, Virchow's Archiv, June, 1884.
The symptoms of cerebral disturbance in chronic hydrocephalus are much less striking than one would expect, doubtless because the increase of pressure upon the brain is so gradual. Actual paralysis, especially of the limbs, is rare, but convulsions are not infrequent, as is the spasmodic constriction of the glottis known as laryngismus stridulus or spasmodic croup. A general state of uneasiness and restlessness is common. Vision is often impaired and sometimes wholly lost. Strabismus is frequently present, or there may be an involuntary rolling movement of the eyeballs. The pupils are often dilated and insensible to light. In consequence of the increased weight of the head, the child is unable to support it, and in most cases is compelled to keep the bed. Vomiting is common. The digestive functions are disturbed. Constipation is an almost constant symptom, and the sphincters are relaxed in the advanced stages of the disease. Although in some cases, as already stated, the child may grow fat, the reverse of this is the rule; there is usually progressive emaciation, especially of the lower extremities.
The duration of the disease varies much in different cases. The earlier the characteristic symptoms manifest themselves the more rapid is its course. Most children born hydrocephalic survive but a few days or weeks. In cases which are more slow in their development the patient may live some years, and in rare instances attain adult life. But his feeble vitality usually makes him an easy prey to the ordinary complaints of childhood, and a large proportion of cases succumb to inflammation of the lungs or of other organs, to diarrhœa, whooping cough, or the eruptive diseases. Most of those who escape these intercurrent maladies perish from defective nutrition, the result of malassimilation of food, or else are cut off by acute cerebral inflammation, with convulsions, etc.
PATHOLOGICAL ANATOMY.—It is rarely that a large amount of serum is found in the cavity of the arachnoid unless a free communication has been established between it and the ventricles by the destruction of cerebral tissue. In the pia the quantity may be more abundant than normal, filling the subarachnoid spaces, separating the convolutions from each other, and occasionally forming little sac-like elevations on the surface of the membrane; but it should be borne in mind that a certain amount of serous infiltration of the tissue of the pia is by no means rare in cases of death from various diseases, owing to the obstruction to the circulation during the last hours of life, and should not be considered pathological unless it exceed the usual limits. The amount of effusion into the ventricles varies between very wide limits, from some ounces to two or three pints. It may be transparent, or turbid from particles of cerebral tissue or epithelial or pus-cells, and is occasionally slightly tinged with blood. Its specific gravity is nearly identical with that of water, and it contains a trace of albumen. The effusion usually occupies the two lateral and the middle ventricles. Less frequently the dropsy is unilateral, in consequence, apparently, of an obliteration of the foramen of Monro. The tissues composing the walls of the ventricles are compressed and hardened. The ependyma of the walls and of the plexus is thickened and roughened by the formation of minute elevations, which the microscope shows to be composed of proliferated and sclerosed connective tissue. The brain in cases of large effusion is reduced to a membranous sac, flattened, with hardly any trace of its original structure at first sight apparent. In the case of James Cardinal, before alluded to, Bright says, “The brain lay at its base [of the skull] with its hemispheres opened outward like the leaves of a book.” Closer inspection shows that all the parts are present, although atrophied by pressure. The convolutions are flattened and the brain-substance is pale and softened. The cranial nerves are often softened and flattened.
In the dropsy of the head in adults which is the result of mechanical pressure or of cachexia the appearances are widely different. The effusion may occupy the cavity of the arachnoid, and even the space between the dura and the skull, as well as the ventricles. The amount of fluid is much less than in the chronic hydrocephalus of children. The ventricular walls present no signs of inflammatory changes.
DIAGNOSIS.—Chronic hydrocephalus is usually recognized without difficulty. The chief points of diagnostic importance are the progressive enlargement of the head, the separation of the cranial bones, with their peculiar change of position, as already described, and the evident signs of arrested intellectual development. If the head be but little enlarged, the case might be mistaken for that of chronic hypertrophy of the brain, but this is a very rare disease, and is not accompanied with defective mental development.
PROGNOSIS.—The elements of prognosis include the size of the head at birth, its rate of enlargement, the general condition of the child, both physically and intellectually, his hereditary antecedents, and the hygienic influences to which he may be subjected. A large proportion of children born hydrocephalic live but a short time; a few survive one or more years. The number of those who reach adult life is extremely small. The favorable indications are a tardy appearance of the dropsy and its slow progress, without marked evidence of defective mental and bodily development.
TREATMENT.—The treatment of chronic hydrocephalus is general and local, the first being the most important, although in many cases it is difficult to enforce it, from lack of intelligence and of means on the part of those in charge of the patient. Proper ventilation, good drainage, and cleanliness are essential. The child should be bathed daily, and should be protected against changes in temperature by suitable clothing. If his strength allow, he should be taken into the open air daily in fine weather. A wet-nurse should be provided for infants whose mothers are unable to suckle them. Older patients should take milk, cream, animal broths, farinaceous substances, etc., with wine or brandy. Tonics, especially cod-liver oil, hypophosphite of lime, and some preparation of iron or of the iodide of iron, are important, the choice being determined by the effect apparently produced. The internal and external employment of mercurial preparations, once in vogue, is now almost entirely abandoned by the best authorities. The evacuation of the fluid by puncture, followed by compression of the head by bandaging, has been occasionally resorted to, and in a few instances with success, but the cases in which it is indicated are rare. Thomas Young Thompson6 reports a case in which puncture was followed by recovery. The child, fourteen days old, fell, apparently without ill effects, but three weeks afterward a protuberance appeared on the crown of the head which continued to enlarge, and the signs of chronic hydrocephalus were unmistakable. In three months the circumference over the parietal eminences measured 20 inches, and a year afterward 24½ inches. In spite of energetic internal and external treatment the enlargement continued to progress, until the head was punctured, and about three hundred grammes of a clear, transparent fluid, free from albumen, were evacuated. Five weeks later a second puncture was made, and sixty grammes of a milky fluid withdrawn. The child recovered, and two years later was in good health, the head not being disproportioned to the rest of the body. West considers the cases in which the effusion is apparently external—that is, confined to the arachnoid cavity, rather than ventricular, and in which there are no indications of active cerebral disease—to be the most favorable for the operation. The proper situation for the puncture is the coronal suture, about an inch or an inch and a half from the anterior fontanel. A few ounces of fluid only should be withdrawn at a time, and compression should be carefully applied both during the escape of the fluid and afterward.
6 Med.-Chir. Transactions, vol. xlvii., 1864.
CONGESTION, INFLAMMATION, AND HEMORRHAGE OF THE MEMBRANES OF THE SPINAL CORD.
BY FRANCIS MINOT, M.D.
Congestion of the Spinal Membranes.
The blood-vessels of the spinal membranes communicate freely with the general circulation, and there is less opportunity for their obstruction than in the case of the meninges of the brain. Hyperæmia of the dura and pia mater is therefore seldom met with, except in connection with disease of the cord; and, indeed, but little is known on the subject, which is only alluded to as possible by authorities of the present day, although the affection was formerly supposed to be a common one, giving rise to various symptoms, such as numbness and formication of the extremities, muscular weakness, and even paraplegia—symptoms which are now known to be caused by structural diseases of the cord only. As Erb1 remarks, “It is hardly possible that any considerable hyperæmia of the meninges should exist without a similar condition existing in the cord also, as the vascular supply of both is the same.”
1 “Krankheiten des Rückenmarks,” von Wilhelm Erb, in Ziemssen's Handbuch, Leipzig, 1876; Am. trans., vol. xiii. p. 99.
ETIOLOGY.—Hyperæmia of the spinal membranes is found after death from convulsions, especially in cases of tetanus, hydrophobia, eclampsia, strangulation, poisoning from narcotics, etc., in which the effect is evidently due to asphyxia. An interesting case of extensive hyperæmia of the spinal membranes, as well as of those of the brain, complicating mania, is reported by M. R. G. Fronmüller.2 A girl of eighteen years, previously well, being accused of theft, fell into a state of melancholia, passing into mania, with frequent convulsions, screaming, etc. There was no spinal tenderness. The urine contained no albumen. The temperature was never elevated. No opisthotonos. The sphincters became relaxed, and she died at the end of about three weeks. The dura was found to be normal, but the pia mater of the brain, cerebellum, medulla oblongata, and cord was strongly injected. The brain and cord were normal; no ventricular effusion. Here the meningeal hyperæmia was doubtless caused by asphyxia resulting from the convulsions.
2 See Schmidt's Jahrbücher, 1883, No. 7.
Congestion of the spinal membranes has been attributed to sudden suppression of the menstrual flow or of hemorrhoidal bleeding, and to portal obstruction, but the evidence of this is very meagre. Exposure to cold and wet, as from sleeping on the damp ground, is an alleged and plausible cause.
The SYMPTOMS attributed to hyperæmia of the spinal membranes are pain in the back extending to the legs, with numbness and tingling of the toes, sensation of weight in the limbs, muscular weakness, appearing suddenly without fever and usually of transient duration. There is no evidence that it gives rise to paralysis. Considering that temporary congestion of the membranes must occasionally happen from convulsions, as in epilepsy, etc., it is remarkable that no symptoms attributable to it have been observed under these circumstances.3
3 See Epilepsy and other Chronic Convulsive Diseases, etc., by W. R. Gowers, M.D., London, 1881, p. 106.
MORBID ANATOMY.—We must be careful not to mistake post-mortem staining of the tissues from imbibition of the coloring matter of the blood—the result of decomposition aided by the position of the body—for true congestion. The latter is recognized by fine vascular arborization covering the surface of the dura or pia, often accompanied with small punctate hemorrhages. More extensive extravasations in the connective tissue, surrounding and between the membranes, are sometimes found. The spinal fluid is usually increased in amount, and often tinged with red. On account of the free vascular connection between the membranes and the cord the latter almost always partakes of the congestion.
DIAGNOSIS.—From what has been said it follows that simple hyperæmia of the spinal membranes can hardly be distinguished from that of the cord. (See the article on DISEASES OF THE SUBSTANCE OF THE BRAIN AND SPINAL CORD.) When the symptoms are unaccompanied by fever, are of very moderate severity, and of short duration, we may perhaps infer that the lesion is confined to the membranes.
The TREATMENT is the same as that for congestion of the spinal cord.
Acute Inflammation of the Spinal Dura Mater.
SYNONYM.—Acute spinal pachymeningitis.
Acute inflammation of the spinal dura mater is chiefly confined to the outer surface of the membrane (peripachymeningitis), and is almost always consecutive to either injury or disease of the vertebræ (fracture, dislocation, caries), to wounds penetrating the spinal cavity, or to suppurative disease in neighboring organs or tissues, which makes its way into the peridural space through the intervertebral openings. The symptoms are complex—in part caused by the original disease, and in part by the pressure of the products of inflammation exercised upon the nerve-roots, and even upon the cord itself. Pain in the back, corresponding to the seat of the disease, is rarely absent, and all movements of the trunk are extremely painful. When the exudation is sufficient to compress the nerve-roots, the pain will extend to the trunk and the limbs, and other signs of irritation, such as a feeling of constriction by a tight girdle, and tingling, numbness, and cutaneous hyperæsthesia in the limbs, will be observed, varying in situation according to the seat of the lesion. In some cases the compression of the cord may be sufficient to cause paraplegia. General symptoms will vary according to the complications of the case. Severe injury or extensive disease of the vertebræ will be accompanied with high fever; but if the external inflammation be moderate and the meningeal complication be limited in extent, the fever may be subacute.
MORBID ANATOMY.—The connective tissue between the dura and the bone is the seat of inflammatory exudation, usually purulent, of greater or less extent, and more abundant in the posterior than the anterior part of the spinal cavity, owing to the position of the patient. A more or less abundant exudation, either of pus or of dry caseous matter, is found upon the outer surface of the dura or infiltrating the connective tissue between it and the bony walls. The dura is thickened, and sometimes the exudation is seen upon its inner walls, but the pia is seldom involved in the inflammation. The cord may be compressed or flattened when the amount of exudation is large, and may in consequence show signs of inflammation in its vicinity. The spinal nerves likewise are sometimes compressed, atrophied, softened, and inflamed. The disease rarely occupies the cervical region, on account of the close union of the dura with the bones of that part; hence there is an absence of pain in the neck and of retraction of the head.
DIAGNOSIS.—The diagnosis is founded on the presence of general symptoms of spinal disease—pain in the back, but not extending to the neck, increased by movements of the trunk; cutaneous hyperæsthesia, tingling, or numbness in various parts of the surface of the body; paresis or paralysis of the lower extremities in severe cases; along with a history of vertebral disease or injury or of suppurative disease in the neighborhood of the spine. The history of the case will generally be sufficient to exclude myelitis, tetanus, or muscular pain (rheumatism, lumbago). From acute leptomeningitis the diagnosis must also be made by the history, but it should be borne in mind that the pia may be involved at the same time with the dura.
PROGNOSIS.—In complicated cases the prognosis is grave if the spinal symptoms are well marked and severe, especially when there is evidence of much pressure on the cord (paraplegia). If the signs of spinal irritation were moderate, the danger would depend upon the nature and extent of the external lesion.
TREATMENT.—This would be addressed mainly to the primitive disease. For the spinal symptoms the treatment would not differ materially from that of inflammation of the spinal pia mater.
Chronic Spinal Pachymeningitis.
This affection generally coexists with chronic inflammation of the pia. Like the acute inflammation of the dura, it is seen in connection with disease or injury of the vertebræ, and it may also arise from tumors of the membrane (chiefly syphilitic) and from myelitis. It is frequent among the chronic insane, and in them is sometimes associated with hemorrhagic effusions analogous to the hæmatoma of the cerebral dura mater. Chronic inflammation of the spinal dura is of unfrequent occurrence, and but little is known of its history and pathology. In a case reported by Wilks4 the membrane was thickened to nearly its whole extent, and in the cervical region presented numerous bony plates. The pia was also thickened at this part and adhered closely to the dura. The symptoms, which seemed chiefly due to disease of the cord from compression, were retraction of the lower limbs and violent jerking from excessive reflex action.
4 Transactions of the Pathological Society of London, vol. vii., 1856.
A special form of the disease, occupying chiefly the cervical region, was first described by Charcot.5 The membrane is thickened by a deposit of successive layers of fibrin, compressing the cord, which is flattened from before backward and inflamed. The nerve-roots are also more or less compressed. The course of the disease may be divided into two stages: First, that of irritation of the spinal nerves, with pain in the back part of the neck, extending to the head and along the upper limbs. The pain is permanent, but liable to exacerbations, and is accompanied with stiffness of the neck and a feeling of numbness and tingling, with muscular weakness of the arms. Sometimes the skin of the arms is the seat of trophic changes, as shown by the presence of bullæ or pemphigus. The second period is that of extension of the disease to the cord. The pain ceases, and is followed by paralysis or muscular atrophy, especially in the domain of the ulnar and median nerves, resulting in extension of the hand on the forearm, with flexion of the fingers toward the palm, giving rise to a claw-like appearance (main en griffe). In some cases an upward extension of the disease implicates the root of the radial nerve, and the hand then assumes a prone position from paralysis of the extensor muscles. The lower portion of the cord may also become involved, with similar results in the lower extremities. Although the disease is generally progressive, it is not always so, and Charcot cites one case in which great improvement took place in the course of some years, though not apparently in consequence of any special treatment.
5 Leçons sur les Mal. du Syst. nerv., par J. M. Charcot, Paris, 1875, vol. ii. p. 246. See, also, Maladies du Syst. nerv., par A. Vulpian, Paris, 1879, p. 127; and Clinique méd. de l'Hôpital de la Charité, by the same.
Acute Spinal Meningitis.
SYNONYM.—Leptomeningitis spinalis acuta.
By the term spinal meningitis is usually meant inflammation of both the arachnoid and the pia, the two being, as already stated, in reality one membrane. In connection with meningitis of the brain it constitutes a distinct disease which is usually epidemic, and probably zymotic in its origin. (See EPIDEMIC CEREBRO-SPINAL MENINGITIS.) Sporadic spinal meningitis is a rare disease. It may be acute or chronic.
ETIOLOGY.—Among the causes of acute spinal meningitis are injuries to the vertebral column, such as fracture and dislocation from falls, blows, etc.; wounds by stabbing or shooting; violent bodily effort, as in lifting heavy weights, etc. Other causes are local exposure to wet and cold, as from sleeping on the wet ground or standing long in water while at work. Like cerebral meningitis, it may complicate constitutional diseases, as rheumatism, pyæmia, etc., but less frequently than the former. Syphilitic inflammation of the spinal membranes is, however, not unfrequently met with, but chiefly of the chronic form. (See the article on SYPHILIS OF THE BRAIN AND SPINAL CORD.) In tubercular meningitis of the brain the pia mater of the cord is often implicated in the disease. Acute spinal meningitis is most frequently met with in young persons, and is more common in men than in women.
SYMPTOMS.—The onset of the disease is generally sudden, and it is rarely preceded by the usual inflammatory symptoms of chilly sensations, restlessness, headache, etc. A sharp rigor is the first indication of disease in most cases. This is followed by fever, with high temperature and a quick, full, hard pulse. Pain in the back follows, either confined to a limited region or extending throughout the whole extent of the spine, soon becoming acute, and aggravated by any movement of the trunk or even of the limbs. There is usually but little tenderness on pressure upon the spinous processes, and often none at all. The pain extends around the body and throughout the limbs. In the course of a few hours muscular contraction in the region of the back, and also of the limbs, is observed. When the disease is situated in the cervical region, the head is drawn backward, and cannot be moved without severe pain. If it occupies a considerable extent of the spine, the dorsal muscles become rigidly contracted and the body is arched forward (opisthotonos), as in tetanus, so that the patient may even rest upon his occiput and sacrum. Owing to the implication of the muscles of respiration, more or less dyspnœa is common, and may even cause death by asphyxia. There is no paralysis of the limbs, unless there be complication of myelitis, but the patient abstains as far as possible from any movement from dread of pain. Reflex irritability is exaggerated. Retention of urine and constipation are common, probably for the same reason. Hyperæsthesia of the surface of the body in limited areas is sometimes noticed, owing to the irritation of the posterior spinal roots. Provided the extent of the inflammation be comparatively limited, recovery is possible, though rare, at this stage of the disease.
When the course of the disease is unfavorable, symptoms of paralysis appear, from compression of the cord by the products of inflammation. The hyperæsthesia of the skin gives place to insensibility; the muscles lose their reflex function and their reaction to electricity; the bladder and rectum become paralyzed. The invasion of the medulla by the disease, which is announced by paralysis of the muscles of the œsophagus and of the tongue, is a fatal complication. The pulse and the respiration become rapid and irregular from compression of the vagus, the temperature rises to 106° or 108° F., and death ensues through asphyxia or failure of the heart.
The course of acute spinal meningitis is rapid. It sometimes terminates fatally within two or three days, and the average duration of fatal cases is about a week, but exceptionally the disease may last for several weeks or even months. Even then complete recovery may take place, though permanent effects are usually left behind, such as paresis or paralysis of the lower extremities, muscular contractions, muscular atrophy, etc., from injury to the nerve-roots or the cord.
The mind is generally clear in the early stage; afterward there may be delirium, especially along with cerebral complication.
MORBID ANATOMY.—It is rarely that the pia mater is the only tissue involved in the inflammation. Frequently the inner surface of the dura is the seat of a fine injection, with delicate false membranes, and the cord itself—at least its exterior portions—probably always participates more or less in the congestion. The pia is reddened and thickened, the surface showing small bloody extravasations, and the space between its two layers is the seat of a fibro-purulent deposit. The spinal fluid is turbid and flocculent. The seat and extent of the morbid appearances vary in different cases; they are always more abundant in the posterior than the anterior part of the cord, and may be confined to a limited space or extend throughout its whole length. It is remarkable that the region of the medulla oblongata is generally free or only slightly affected; but since bulbar symptoms are often prominent in grave cases, Leyden6 accounts for it by supposing that the exudation is washed away by the constant movement of the cerebro-spinal fluid. If the cord be involved in the inflammation, it is softened and injected, the nerve-sheaths are destroyed, and the axis-cylinders swollen in places. The nerve-roots show hyperæmia, infiltration of the interstitial tissue with round cells, and destruction of the nerve-sheaths.
6 Klinik der Rückenmarks-krankheiten, von E. Leyden, Berlin, 1874, vol. i. p. 407.
DIAGNOSIS.—Spinal meningitis is easily recognized in most cases by its sudden onset and the severity of its symptoms. The distinguishing symptoms are sudden and acute pain in the back, extending around the body and into the limbs, which is increased by every movement of the trunk, rigidity of the back, hyperæsthesia of the skin, retraction of the head, with difficulty of breathing and of swallowing. The diseases from which it must be distinguished are muscular rheumatism (so called), tetanus, and myelitis. In rheumatism of the dorsal muscles the pain is confined to the back, does not extend to the limbs, and is only excited by movement. The fever is moderate or absent; there are no symptoms of spinal complication, such as cutaneous hyperæsthesia, retraction of the head, paresis of the limbs, etc.; and the result is uniformly favorable. Tetanus is almost always due to some well-marked traumatic cause; the muscles of the jaw are usually first implicated (trismus); and the attacks of general muscular spasm are easily excited by peripheral irritation. Myelitis can be distinguished by the absence of pain in the limbs and by paraplegia, but it must be borne in mind that myelitis and spinal meningitis may coexist.
PROGNOSIS.—Acute spinal meningitis is always a grave disease, hence a guarded opinion should be given even in apparently favorable cases. General mildness of the symptoms, with no indication of extension to the medulla oblongata, would afford ground for encouragement. The unfavorable symptoms are those showing compression or inflammation of the cord, such as paresis, paralysis, twitching of the limbs, muscular contractions, cutaneous anæsthesia, etc. The extension of the disease to the medulla, as shown by difficulty of swallowing, speaking, or breathing, is almost necessarily fatal.
TREATMENT.—During the first stage of the disease an effort should be made to reduce the hyperæmia of the membranes by the local abstraction of blood. This is best effected by cupping along each side of the spine and by the application of leeches to the anus; the bleeding should be promoted by poultices. Free purging is likely to be of service, and is best obtained by means of ten grains each of calomel and jalap (for an adult), followed by saline laxatives. Counter-irritation to the back may be induced by the application of blisters or by painting the skin with a strong tincture of iodine (one or two drachms of iodine to an ounce of sulphuric ether). After the effusion of lymph and pus, as shown by symptoms of compression of the nerve-roots and cord, absorbents should be tried, of which the iodide of potassium, in doses of five to ten grains, four times daily, is most likely to be of benefit. Pain must be relieved by opium or morphia and chloral hydrate. The latter, either alone or combined with hyoscyamus and bromide of potassium, will be useful to allay spasmodic twitching, opisthotonos, or muscular contraction. The patient should be enjoined to lie on each side alternately, or on the face if possible, in order to equalize the hyperæmia of the membranes and cord. Liquid nourishment must be freely administered, such as milk, broths, gruel, etc., together with wine and other stimulants in case of exhaustion and threatening of collapse. During convalescence the patient should be carefully protected against cold and fatigue, and the strength must be supported by quinine and iron with suitable diet.
Chronic Spinal Meningitis.
SYNONYMS.—Chronic inflammation of the pia mater of the spinal cord, Chronic spinal leptomeningitis.
Chronic spinal meningitis may follow the acute form, or it may arise from chronic disease of the vertebræ or of the cord, especially myelitis and sclerosis. It is most apt to accompany sclerosis of the posterior columns, and it is often difficult to say in any particular case whether the meningeal affection preceded or followed that of the cord. Probably some cases of chronic myelitis, especially of the disseminated form, owe their origin to chronic meningitis.7 It has been thought to follow blows on the back, and also to arise from general concussion without traumatism, and has been considered as a frequent result of accidents from railroad collisions, etc. This view has been disputed by Herbert W. Page,8 who says: “Of the exceeding rarity of spinal meningitis as an immediate result of localized injury to the vertebral column we are well assured.... And we know of no one case, either in our own experience or in the experience of others, in which meningeal inflammation has been indisputably caused by injury to some part of the body remote from the vertebral column.” Chronic alcoholism and syphilis, especially the latter, predispose to the disease. In many cases no adequate cause can be assigned.
7 Leyden, op. cit., vol. i. p. 442.
8 Injuries of the Spine and Spinal Cord, without Apparent Mechanical Lesion and Nervous Shock, in their Medico-legal Aspects, London, 1883, p. 128.
SYMPTOMS.—Gowers9 remarks that a large number of symptoms formerly assigned to chronic spinal meningitis have nothing to do with that pathological state, but are now known to be owing to alterations within the cord which are frequently associated with it. The symptoms which are really due to the meningeal inflammation—namely, those arising from interference with the nerve-roots in their passage through the thickened membranes—do not differ essentially from those of chronic pachymeningitis of the spine; the principal are pain in the back, especially on movement, extending to the trunk and limbs, hyperæsthesia of the skin in various regions within the domain of the irritated sensory roots, with diminution or loss of the knee-jerk, and areas of anæsthesia due to their more complete compression. The compression of the motor roots gives rise to symptoms which are similar to those of disease of the anterior cornua, consisting chiefly of muscular wasting, with impairment of motion in corresponding regions, without fever.
9 The Diagnosis and Diseases of the Spinal Cord, by W. R. Gowers, M.D., 2d ed., Philada., 1881, p. 73.
The anatomical appearances, which are most pronounced in the posterior aspect of the cord, and diminish from below upward, are often of considerable extent. The membrane is thickened, opaque, and often adherent to the cord. On the inner surface are seen numerous small bodies composed of proliferating connective tissue.10 These, according to Vulpian, are chiefly found in the lower dorsal and lumbar region and on the cauda equina. The dura is very frequently involved in the inflammation; it is thickened, and its inner surface is adherent to the pia, often showing numerous miliary bodies similar to those found in the pia.
10 Leyden, op. cit., vol. i. p. 144; A. Vulpian, Mal. du Syst. nerv., Paris, 1879, p. 126.
DIAGNOSIS.—Chronic spinal meningitis so rarely exists apart from disease of the dura or of the cord that its separate diagnosis must be mainly a matter of conjecture. Leyden justly says we must usually be content to diagnosticate chronic meningitis without attempting any further distinction, except in cases which originate in disease of the vertebræ or of their neighborhood, and afterward penetrate into the spinal canal, and which would probably give rise to pachymeningitis. The diseases with which chronic meningitis of the spine is most likely to be confounded are posterior sclerosis (locomotor ataxia) and chronic degeneration of the anterior cornua (progressive muscular atrophy).11 From the former it is distinguished by the absence of ataxia; from the latter, by the irregular distribution of the symptoms; and from both, by the existence of limited areas of anæsthesia and of extensive spinal pain. It must be remembered that inflammation of the cord complicating that of the meninges, or pressure upon the cord by the thickened membranes may give rise to mixed symptoms. The latter is especially observed in syphilitic cases.
11 Gowers, op. cit., p. 74.
PROGNOSIS.—Simple chronic spinal meningitis is rarely if ever fatal. How far it is capable of amelioration or of cure is not, in the present state of our knowledge, known. When complicated with disease of the cord the prognosis will depend upon that of the latter. Syphilitic chronic meningitis is to a certain extent amenable to appropriate medication.
TREATMENT.—The treatment should have for its object (1st) to relieve pain; (2d) to arrest the progress of the inflammation, and especially to prevent it from extending to the cord; (3d) to promote the absorption of the exudation. For the first object the internal or hypodermic employment of morphia should be combined with external applications, such as fomentations, liniments, ice, etc. Counter-irritation by means of dry cupping, blisters, iodine, etc., with leeches, shampooing, and douches of hot water, may be of some use in retarding the progress of the inflammation, and should be aided by the administration of mercurials and the preparations of iodine. The biniodide of mercury would be useful for this purpose in the dose of one-sixteenth to one-eighth of a grain three times daily, its effect being carefully watched in order to prevent salivation. The same means will also aid in favoring the absorption of lymph. In syphilitic cases an appropriate specific treatment is indicated.
Spinal Meningeal Hemorrhage.
SYNONYMS.—Spinal meningeal apoplexy, Hæmatorrhachis.
Spinal meningeal hemorrhage may take place between the dura mater and the walls of the vertebral canal, or between the dura and the pia mater—i.e. into the arachnoid space.
ETIOLOGY.—Penetrating wounds, injuries to the vertebræ, fractures and dislocations, and violent blows are apt to give rise to more or less hemorrhage into the spinal canal. Violent convulsions, as in tetanus, epilepsy, uræmic eclampsia, hydrophobia, may be followed by meningeal hemorrhage, owing to the disturbance of the circulation from asphyxia; and excessive muscular effort, as in lifting heavy weights, etc., has been said to cause it. In cerebral meningeal hemorrhage and in effusion into the substance of the brain the blood sometimes escapes into the spinal canal. An aneurism of the aorta has been known to communicate with the canal; such a case was reported by Laennec.12 In certain diseases with hemorrhagic tendency, as purpura and scurvy, spinal extravasation is occasionally observed.13
12 Traité d'Auscultation médiate, 4th ed., Paris, 1837, vol. iii. p. 443.
13 A case of scorbutic spinal hemorrhage is reported in the British Med. Journal, Nov. 19, 1881.
SYMPTOMS.—These vary according to the conditions under which the hemorrhage is produced and to the amount of bleeding. In traumatic cases the signs of hemorrhage are often completely overshadowed by those of the injury of the vertebræ, the membranes, or the cord, and are undistinguishable. When a large amount of blood is suddenly introduced into the spinal canal, it usually gives rise, by pressure on the cord, to paraplegia, which, however, is sometimes of only short duration. Thus in Laennec's case the bursting of an aneurism into the spinal cavity was signalized by a sudden paraplegia, but in half an hour the power of movement returned, though sensation did not. The patient died in a few hours from hemorrhage into the left pleural cavity. The amount of blood in the spinal cavity is not stated, and, in fact, it would appear that the spine was not opened. When the amount of blood is smaller the symptoms indicate irritation of the spinal nerves and of the cord. Pain in the back is always present, extending into the limbs, and is sometimes severe. Its seat corresponds to that of the effusion. There may be a feeling of tingling and numbness in the lower extremities, with anæsthesia or hyperæsthesia of the skin and more or less paresis. A feeling of constriction around the waist or the chest is sometimes complained of. In slight effusions the symptoms may be limited to numbness and formication of the extremities, with slight paresis. There is rarely fever in the early stages, unless the amount of blood is sufficient to give rise to inflammatory conditions of the cord or membranes. If the extravasation be moderate it is generally absorbed, with relief to the symptoms, although slight numbness and paresis of the extremities may continue for a long time.
PATHOLOGICAL ANATOMY.—In hemorrhage outside the dura the loose cellular tissue between the membrane and the bony canal contains more or less coagulated blood according to the circumstances of the case, especially in the posterior region of the canal and covering the nerve-roots. The dura is reddened by imbibition of the coloring matter of the blood. When the amount of the effusion is large, as in traumatic and aneurismal cases, or where cerebral hemorrhage has extended into the spine, the cord may be compressed by it. Hemorrhage into the arachnoid sac, except in cases of violence, etc., is usually of limited amount, sometimes only in the form of drops of blood upon the surface of the dura or pia. When more abundant it may surround the cord more or less completely, but in most cases it is limited in longitudinal extent, being confined to the space of one or two vertebræ. The cord may be more or less compressed, reddened, and softened. In all cases the spinal fluid is discolored and reddened in proportion to the amount of the hemorrhage.
DIAGNOSIS.—When the complications are such that symptoms attributable to hemorrhage are not observed, the diagnosis of spinal hemorrhage is impossible. This may happen in the case of wounds and injuries of the vertebræ and of the passage into the spinal canal of blood from an apoplectic effusion of the brain. In the convulsions of tetanus, epilepsy, etc. the amount of the hemorrhage is rarely sufficient to give rise to distinctive symptoms. In idiopathic and uncomplicated cases the chief diagnostic marks are suddenness of the attack; pain in the back, usually at the lowest part; disturbances of sensation in the extremities (anæsthesia, formication, etc.); paresis or paralysis of the legs; the absence of cerebral or spinal inflammatory symptoms; and, in many cases, the favorable course of the disease. Sometimes an ostensible cause, such as scurvy, purpura, suppressed menstruation, or hemorrhoidal flux, will aid in the diagnosis. The disease for which spinal hemorrhage is most likely to be mistaken is acute myelitis, but this is not sudden in its onset, is accompanied with fever, and gives rise to paralysis both of motion and sensation, and to loss of control over the sphincters, to bed-sores, etc. Hemorrhage of the cord would be accompanied by paraplegia and loss of sensation in the lower extremities and slight tendency to spasmodic manifestations; it is fatal in the majority of cases, or else is followed by permanent paralysis. Hysteria might be confounded with spinal hemorrhage, but the history of the case and the transient duration of the symptoms would clear up all obscurity.
PROGNOSIS.—In traumatic cases the hemorrhage is usually only one element in the gravity of the situation, which depends chiefly upon the character and extent of the original injury. In idiopathic and uncomplicated cases the prognosis, which must always be doubtful, will vary according to the severity of the symptoms as corresponding to the amount of the effusion. The danger is greatest during the first few days; if there should then be diminution of the more important symptoms, an encouraging opinion may be given. The immediate effects may, however, be less grave than the remote, such as bed-sores, cystitis, etc. Except in the very mildest cases the patient is likely to be confined to bed for several weeks. In more severe ones the convalescence may be very protracted, and permanent lameness, etc. may result.
TREATMENT.—In the early stage absolute rest in bed, with cold applications to the back and moderate purging, should be employed. Large doses of ergot are recommended, but there is little evidence of benefit from this medicine. In traumatic cases no rules for treatment of the hemorrhage can be laid down. If the extravasation evidently depends upon a constitutional diathesis, as in purpura, scurvy, etc., the remedies appropriate for these diseases should be employed, especially tonics and astringents, such as the tincture of the chloride of iron, in doses of from fifteen to thirty drops three times daily, quinine, and the vegetable acids. Should there be evidence of blood-pressure from suppressed discharge, as in amenorrhœa, arrested hemorrhoidal flow, etc., leeches should be applied to the anus, and blood may be taken from the region of the spine by cupping. In the later stages an attempt may be made to aid the absorption of the effused blood by the administration of the iodide of potassium or the protiodide of mercury, and by the application of blisters or strong tincture of iodine to each side of the spine. Pain must be relieved and sleep obtained, when necessary, by means of opium, chloral hydrate, or other anodynes. Electricity, rubbing, bathing, etc. will be useful for combating the paralytic symptoms which may remain after the disease itself is relieved.