if the child survive. These bilateral cases are mainly of prenatal and birth origin, only a small number arising after the first few months of infancy and practically none after the third year of life. The more extensive injury to the brain is attended almost of necessity by greater mental impairment and idiocy marks many cases. Microcephalia sometimes results or the skull may have so defective a shape that porencephalia and anencephalia may be suspected. Such children are either noticed to be rigid and inactive at birth, or after-birth injuries and convulsive manifestation develop rigidity and contractures during the early months of infancy. Frequently it is only when the child is found incapable of learning to sit up or to use its legs in efforts at walking that the paralytic state is recognized. Mental backwardness, slowness in the development of speech, and other indications. of injury to the highest brain functions are often neglected until the third or fourth year in the misguided hopefulness that the child will "outgrow it." The rigidity and spastic state is frequently so great that the legs and arms present a "lead pipe" resistance to passive move

ments. Voluntary efforts are hindered or defeated, and some of these children never get the hands to the face, much less their toes to their mouths. They are difficult to handle and to dress. Their arms and legs are as unmanageable as stiff-jointed manikins. They are "all thumbs" and awkwardness. The spasticity in the lower extremities, which tends to flex hips and knees and especially to adduct the thighs, holds the knees closely together, makes it difficult to dress and bathe them as infants and defeats locomotion later on. Placed on a chair, the lower limbs have a tendency to maintain a rigid horizontal position. If they are placed on their feet the legs cross, the heels can not be brought to the floor, and if steps are taken it is only by advancing the foot that is in front and then bringing up the one in the rear. As they grow older a tendency to equinovalgus or varus and genu valgum is induced, but by keeping the feet widely separated they are able to dodge one knee around the other. They thus advance in a shuffling, knee-rubbing, toe-scraping, laborious manner, to which the increased reflexes and ankle-clonus give a jerky, tremulous, spastic character.

In some cases more or less athetosis is present in all four members, and occasionally it invades the face and even the tongue, pharynx, and larynx. When the athetosis is general, and particularly when it involves the feet, these patients are rendered almost completely helpless physically.

Little's Disease.-French writers, of the Salpêtrière school especially, insist upon a clinical form of spastic diplegia to which they give the distinctive name of Little's disease, after the London surgeon who among the first called specific attention to this class of disorders. As these cases referred to present certain definite characters and a more hopeful future than the others, they merit separate mention. The initial condition is a premature birth or the birth at term of a

Fig. 91.-Case of Little's disease. Rigidities and crossleg from spasticity in a child weighing three pounds at birth.

markedly undeveloped child. This means a defective pyramidal tract in the spinal cord and brain. Such children usually weigh less than four pounds. Spasticity results in the territory of the lower neuron from the lack of control due to the undeveloped condition of the upper neuron. These children are rigid from birth, but the mental qualities are not necessarily diminished, and if they survive the tendency is to continuous improvement as the pyramidal tracts develop. This development is never complete, but goes on up to the age of full growth at about twenty-four years of age. The motor condition is one of pure spasticity and there is no tendency to athetoid movements or epileptic attacks. The gait remains more or less spastic through life, which may be a long one marked by ordinary or even brilliant mental activity.

1

Amaurotic Family Idiocy.-Sachs 1 proposes this clinical designation for a group of cases of which he has collected nineteen occurring in ten families, with three autopsies. These children are born at term and present healthy physical and mental development up to the age of four to ten months, when they become weak, lethargic, and stupid. Ocular symptoms soon appear and blindness due to optic atrophy develops. Nystagmus, ocular deviations, and pupillary stasis have been noted. Deafness may be added. The child falls into a marantic and idiotic condition and dies within a year or two. Convulsions are lacking, but spastic features and motor feebleness or actual palsies are usually present, with increased tendon reflexes. The post-mortem findings have shown convolutional simplicity and cortical cellular agenesis. As many as four cases have been observed in a single family, and the inherent anatomical lack in the brain is not explained by in

1 "New York Medical Journal," May 30, 1896.

herited syphilis, which may, however, produce a very similar clinical picture.

Epileptic Attacks. One of the most serious features of the cerebral palsies of children is the marked tendency to the development of epileptic attacks. These appear in fully one-half of all cases. They are due to the brain-lesion, and usually are most marked in the paralytic limbs, but with a tendency to spread and become generalized. True Jacksonian fits are but rarely encountered. In some cases the initial eclamptic attack is at once followed at regular intervals of weeks or mouths by these seizures. In others they do not appear for a period of years. They are always to be expected, and when established constitute a serious menace to the health and mental state of the patient. The ordinary tendency of epilepsy to induce dementia is exaggerated in these already defective brains.

Diagnosis. The diagnosis of these cases when the condition is well marked should present no difficulty. In the slighter grades the epileptic attacks or the choreoid movements are frequently mistaken and the organic basis overlooked. The combination of head symptoms and mental enfeeblement with the motor difficulties in diplegic cases should be sufficiently striking. The hemiplegic distribution of paralysis can tell but one story. In cases presenting paraplegic features it may require a careful investigation to locate the trouble in the brain. The history here, as elsewhere, is a valuable guide. In addition the preserved electrical reactions, the comparatively normal trophic conditions, and the exaggerated reflexes should readily exclude a cord-lesion. The teratological deficit which furnishes the cases of amaurotic family idiocy and the defective development of Little's disease must both be deciphered from the clinical data or the post-mortem findings.

Prognosis. The prospects in these diverse cases vary widely, but it may be positively stated that complete recovery never occurs and that a perfectly normal condition is never attained. In the cases of prenatal origin the prognosis must rest on the observed course of development during the first year of life. Improvement in motor control and the appearance of mental awakening are hopeful indications, but the majority of these cases are doomed to idiocy and more or less physical helplessness. The cases that conform to the type of Little's disease are exceptions. In these the prognosis for mental development is good and progressive motor improvement, up to a certain point, may be confidently expected. Amaurotic family idiocy, which is a congenital defect, is, at present, of absolutely bad prognosis.

In the cases of birth palsy the intensity of the convulsions, their persistence, and the apparent extent of brain injury, in a very rough way, guide the prognosis as to life. If, after a few months, spasticity is still absent and some voluntary motion occurs in the paralyzed limbs, the prospects are much improved. The question of mental development can only be decided by watching the course of events. In the acquired cases the prognosis is much the same as in the apoplexies of adults as far as motility is concerned. The return of motion in the paralytic limb and the absence of contractures permit hope of a fair degree of motor

restoration. Speech is usually restored, unless the frontal lobes have been injured and mentality impaired. The loss of sight and more especially of hearing is likely to prevent mental development and to induce idiocy. Whenever epileptoid attacks appear, they constitute a very serious feature and foreshadow dementia. They are somewhat more common in the late than in the congenital cases and in the hemiplegic than in the diplegic palsies.

Treatment. In the birth-palsy cases the nutrition of the child is the first problem, as suckling and swallowing are often impossible. The evidence of meningeal hemorrhage is usually so indefinite that trephining, which has been proposed, can rarely, if ever, be practised. The convulsions must be subdued by cold applications to the neck and head, and by sedatives, such as chloral, bromid, morphin, and by continued chloroformization, if necessary. The bowels should be thoroughly opened, preferably by means of calomel. The same measures are indicated as in older cases of acute hemiplegic convulsions and in convulsions generally.

In the later cases the management of the apoplectic and paralytic states is the same as for adults (see p. 208). From the first, the nutrition of the paralytic muscles and the tendency to contractures must be met by massage, passive movements, and faradism. As soon as contractures appear, the massage and electricity must be used only on the weaker muscles that is, as a rule, only on the extensors. The use of these measures to increase the strength of the overacting muscles may occasion decided harm. Unfavorable positions of the limbs must be obviated by mechanical measures, if necessary. Such means may do much to retard contracture, to hasten and increase voluntary control, and to favor growth in the paralytic members. Orthopedic appliances and tenotomies in neglected cases sometimes secure much permanent improvement.

Athetosis may be subdued to a considerable extent by fixation appliances or plaster-of-Paris splints. Hammond reported some cases temporarily much benefited by nerve-stretching, and Horsley has extinguished the athetosis by excising the corresponding brain-centers. In one such case the writer advised the removal of the cortical centers for a very unruly upper extremity, and the resulting palsy in the member was replaced finally by slight but useful voluntary control.

When idiocy is present it is open to educational methods only. The epilepsy that so frequently complicates these cases can be controlled to some extent by bromids or combinations of various sedatives with bromids. When the attacks are of a distinctly Jacksonian character, the question of operation will come forward. Every case must be considered by itself. As in many instances the condition is one of cerebral sclerosis, no operation can do good. If there be a cystic state, exploration and evacuation may be productive of much relief. In some cases the mere effect of the operation has been beneficial; in others it has acted by relieving pressure. Removal of cortical centers in Jacksonian fits of this class is very likely to merely displace the initial symptoms and to add to the traumatic conditions already present. Linear craniotomy

when the brain is diseased can only be condemned.

If it have any

place it is in the cases that present closed fontanels at birth with microcephalic heads, the possible result of early synostosis.

CHAPTER XII.

HYDROCEPHALUS.

HYDROCEPHALUS is a term that has been loosely used to designate any undue amount of watery fluid within the skull, and in such a sense is synonymous with dropsy of the brain. Tubercular meningitis, frequently called acute hydrocephalus by older writers, is not the condition in question. We are not now to consider the compensatory increase of cerebrospinal fluid occurring in the convolutional shrinkage of old age, or in the cerebral atrophy of dementia, or in porencephalic or anencephalic defects. The question does not pertain to the increase of fluid which marks acute meningitis or tubercular meningitis, or to the edematous state that frequently attends cerebral tumors. Attention has also been called in the proper place to ventricular distention resulting from tumors situated in the posterior cranial fosse, which mechanically block the venous return through the veins of Galen and the straight sinus. It is desired to restrict the term hydrocephalus to a congenital or acquired, acute or chronic condition marked by great increase in the amount of cerebrospinal fluid within the skull attended by compression of the brain. In some cases it is entirely ventricular, constituting internal hydrocephalus; in others it is subdural, constituting external hydrocephalus; but often both the subdural and the ventricular spaces are distended.

Some

Etiology. The causation of hydrocephalus is obscure. families seem to be marked by a hereditary tendency to it, as shown by several cases occurring in the same or succeeding generations. Hereditary syphilis has for long been thought to be a competent cause, but in many cases it can be excluded with a reasonable certainty. Its causal relation is recently supported by Heller1 and by Titomanlio 2 in careful studies. Alcoholism on the part of the parents is also supposed to have some part in producing the congenital variety, and is doubtless active in some of the late adult cases. Traumatism after birth has produced it, and it has been noted as a sequel of cerebrospinal meningitis. Quincke describes a form of acute serous meningitis the symptoms of which do not vary materially from those of the ordinary infectious variety, but which is marked by the rapid production of subdural hydrocephalus and great intracranial pressure.

Morbid Anatomy.-In congenital cases and in those occurring before the cranial bones are firmly united the head is enlarged, sometimes

* Trans. Section on Diseases of Children, International Med. Cong., Rome, 1894.