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 {743} 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
reports[5] 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 51-1/5 centimeters (20¼ inches). The anterior fontanel was
still open. The age was verified by reference to the registry of birth
at the police-office.
[Footnote 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 {744} 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 {745} 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 Thompson[6] 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.
[Footnote 6: _Med.-Chir. Transactions_, vol. xlvii., 1864.]
{746}
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 Erb[1] 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.”
[Footnote 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.
[Footnote 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
{747} 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]
[Footnote 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 {748} 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 Wilks[4] the membrane was thickened to nearly its
whole {749} 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.
[Footnote 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.
[Footnote 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 {750} 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.