Acute Infantile Hemiplegia

Acute Infantile Hemiplegia

Acute Infantile Hemiplegia SIDNEY CARTER, M.D. ARNOLD P. GOLD, M.D. Acute hemiplegia in childhood, also known as acute infantile hemiplegia, infantil...

1MB Sizes 67 Downloads 512 Views

Acute Infantile Hemiplegia SIDNEY CARTER, M.D. ARNOLD P. GOLD, M.D.

Acute hemiplegia in childhood, also known as acute infantile hemiplegia, infantile acquired hemiplegia, Marie-Striimpell encephalitis, polioencephalitis, and hemiconvulsion and hemiplegia (H. H.) syndrome, is postnatally acquired hemiplegia in a child apparently neurologically unimpaired at birth. This definition clearly excludes those children with hemiplegia related to prenatal factors (congenital anomalies, chromosomal aberrations or prenatal infections) and perinatal factors ( prematurity, birth trauma or perinatal infections). Recognition of acquired hemiplegia in the neonatal and early infancy period is most difficult, for it is often confused with congenital disorders. Delineation of the syndrome is further complicated by the frequent failure to recognize congenital hemiplegia before 4 to 5 months of age. Relative acuteness of onset is the determining factor.

ETIOLOGY AND PATHOGENESIS

Infantile hemiplegia is not a disease but the nonspecific response of the central nervous system to multiple and varied causative factors. Hemiplegia is the only consistent clinical feature, while the mode of onset, the subsequent clinical course,- the prognosis and the method of management will in large part be dependent upon etiologic delineation. Knowledge of all etiologic factors will alert the physician to the possible occurrence of the hemiplegic syndrome and will be beneficial in establishing a dynamic diagnostic and therapeutic program once it has occurred. Infantile hemiplegia may be symptomatic or idiopathic. Symptomatic infantile hemiplegia results from a known etiology with a well defined clinical picture, while the idiopathic variety is of obscure origin. Controversy concerning etiology is due to inadequately documented clinical histories and the relative limitation of postmortem material that is available for study. Improved diagnostic methods have been instrumental in reducing the number of idiopathic cases. Pediatric Clinics of North America-Vol. 14, No.4, November 1967

851

4 852

SIDNEY CARTER, ARNOLD

P.

GoLD

Viral and bacterial infections, trauma, immunizations, systemic diseases, cerebral arteriovenous malformations, cardiac abnormalities and neoplasms and their ensuing complications are recognized etiologic factors and result in fairly well defined clinical entities. Trauma. Contusion and laceration of the brain, as well as epidural, subdural and intracerebral collections of blood, may complicate head injuries and result in a hemiplegic state. Internal carotid artery thrombosis results from blunt nonpenetrating trauma to the paratonsillar area and usually occurs as a result of falling on a foreign object carried in the mouth. In such cases there is usually a latent period of 3 to 24 hours between intraoral trauma and the onset of hemiplegia-this being the time necessary for the development of the thrombosis. Air embolism to the brain is usually arterial and may complicate cardiac catheterization and cardiac, thoracic and neck surgery. Fat embolism has a characteristic clinical picture and is usually associated with fractures of long bones. In 12 to 48 hours after a long bone fracture, the child presents with fever, respiratory distress and blood-tinged sputum. A few hours later, neurological manifestations develop, which may include hemiplegia. In addition, there may be petechiae of the skin, fat in the retinal vessels and free fat droplets in the urine. Status Epilepticus. Prolonged convulsions may result in neural damage with gliosis and decreased numbers of neurons. Most fatal cases of status epilepticus have neuronal changes that affect one cerebral hemisphere more than the other; in a few fatal cases hemiplegia precedes death. Arteriovenous Malformations. These vascular anomalies can produce hemiplegia by rupture with resultant formation of an intracortical clot; however, in rare instances hemiplegia may occur in the unruptured state by compression and destruction of brain parenchyma. Cardiac Disease. Cyanotic congenital heart disease is often complicated by cerebral thrombosis, and usually occurs in children less than 2 years of age. It is the result of increased viscosity of the blood or of hypoxia, and as noted by Tyler and Clark, this complication only occurred when a red blood cell count was above 8,000,000 or an arterial oxygen content was below 10 volumes per cent. A brain abscess rather than arterial thrombosis may be responsible for the hemiplegia, especially in children more than 2 years of age. Atrial fibrillation and other arrhythmias favor the formation of mural thrombi, and these may result in cerebral emboli. This phenomenon is is seen in cyanotic congenital heart disease and rheumatic fever. Paroxysmal auricular tachycardia may on rare occasions be complicated by cerebral embolic phenomena. The acute hemiplegia may develop while the paroxysmal auricular tachycardia is being converted to a normal sinus rhythm. Bacterial endocarditis, acute and subacute, can result in acute hemi-

ACUTE INFANTILE HEMIPLEGIA

853

plegia by the formation of emboli from the cardiac vegetations. This neurologic manifestation may be the initial sign in many children with endocarditis. Myxomas of the heart may present as unexplained sudden hemiplegia. Headaches, dizziness, nausea and vomiting may precede the hemiplegic state. Carotid artery occlusion is due to thromboembolic episodes from a myxoma of the left atrium. The presence of a cardiac neoplasm is suggested by a day to day change in the cardiac murmur, relentless progression of heart failure and lack of response to adequate digitalization. Infections. Encephalitis of viral origin is only occasionally complicated by hemiplegia, as it rarely involves only one hemisphere. Hemiplegia in such children usually is associated with generalized or multifocal seizures, fever, somnolence or coma. Pleocytosis, initially polymorphonuclear and subsequently lymphocytic, and an elevated cerebrospinal fluid protein content are usually found. The electroencephalogram is diffusely abnormal with slow wave activity. Postinfectious and postvaccinal encephalitis causes perivascular demyelination and may result in unilateral hemiparesis, seizures and coma. Measles and, less commonly, rubella, varicella, mumps and influenza may be responsible infectious agents. Postvaccinal complications may result from immunization against smallpox, rabies and pertussis. Bacterial meningitis may be complicated by cortical vascular thromboses with a predilection for one heInisphere. The hemiplegia is of acute onset and is associated with pyrexia and meningeal signs and often with papilledema. The cerebrospinal fluid is usually under increased pressure, with a polymorphonuclear pleocytosis, an elevated protein content and a deceased sugar concentration. Cerebral arteritis may have many causes, and many cases of hemiplegia of obscure origin are secondary to inflammatory disease. Infections of the ears, nose, throat and paranasal sinuses often have an associated cervical lymphadenopathy. The enlarged lymph nodes, by their proximity, involve the adventitia of the internal carotid artery in the region of the base of the skull, with subsequent development of an intravascular thrombosis. Other causes of arteritis include mucormycosis of the paranasal sinuses, occasionally seen with uncontrolled diabetes, meningovascular syphilis and idiopathic granulomatous arteritis (Takayasu's disease). These may involve the cerebral arteries with thrombus formation and resultant hemiplegia. Acute or chronic pulmonary infections give rise to emboli which may occlude a cerebral artery. Since the speed of occlusion is so rapid, no compensatory collateral circulation can be established, and a cerebral infarct results. The middle cerebral artery or one of its branches is the vessel most frequently occluded. Systemic Diseases. Children with lupus erythematosus, periarteritis nodosa, blood dyscrasias (sickle-cell disease, polycythemia and throm-

854

SIDNEY CARTER, ARNOLD

P.

GOLD

botic thrombocytopenic purpura) and homocystinuria may develop arterial thromboses. Dehydration and debilitating states may have complicating dural sinus and cerebral venous thromboses with resultant hemiplegia. Obscure Etiology. In the majority of children who develop acute hemiplegia, no precise cause can be ascertained. Careful history may be rewarding in some of these cases. Improved clinical, diagnostic and pathological techniques have frequently resulted in a more precise diagnosis. Arterial thrombosis secondary to local changes in the artery is most often incriminated. Changes in the intima with either multiple lipoid-containing intimal plaques ulcerating into the lumen or an isolated atheromatous plaque may result in vascular occlusion. Dissecting aneurysms may also occlude the lumen; this congenital defect of the intima permits the development of an intramural thrombus between the intima and media. Localized arteritis secondary to an upper respiratory infection and unrecognized direct trauma to the internal carotid artery in the paratonsillar area may result in arterial occlusion and be responsible for some of these obscure cases.

CLINICAL CONSIDERATIONS

Incidence. Acute infantile hemiplegia is not a rare condition, for over 200 cases were observed by Ford at the Harriet Lane Home. However, it is not as prevalent as in former years because of increased ability to treat infection. Approximately one third of children in most cerebral palsy clinics have hemiplegia; 25 per cent of these cases are acquired postnatally. The reported sex ratio shows marked variance and is dependent upon the etiologic factor responsible. Trauma, cerebral arterial thrombosis and carotid arteritis show a clear-cut male preponderance; Takayasu's disease (primary aortitis) is most frequently seen in girls. Both sexes are equally affected when all etiologic factors are considered or when no obvious etiology can be delineated. Birth and Family History. The birth history is statistically unimportant, since only a few involved children are of low birth weight or the result of abnormal pregnancies and deliveries. Family history is probably insignificant. Parental alcoholism, mental subnormality and instability may be prominent historical features; ineptness of care due to these factors is perhaps the underlying cause of frequency of this type of family history in acquired hemiplegia. Age of Onset. The age at which the hemiplegia appears may vary with the specific cause. Trauma and bacterial infections are most common during the preschool years. Cerebral occlusive vascular disease, associated with cyanotic congenital heart disease, is most frequently observed during the first 2 years of life, while a brain abscess in this condition occurs after this age. Irrespective of the cause, most children are symp-

855

ACUTE INFANTn.E HEMIPLEGIA

tomatic before 6 years of age, with the largest number before 3 years; acquired hemiplegia is infrequently observed after 12 years. Mode of Onset. The clinical picture will vary and in part be dependent upon the primary disease responsible for the hemiparesis. Neurologic manifestations, both in the otherwise healthy child and in the one with a precipitating condition, vary according to the rapidity of development and the site and extent of damage. Embolism produces a rapidly evolving clinical picture, with maximum involvement within a few minutes. Thrombosis, slower in development, may progress either intermittently or progressively during a period of hours or days. There may be a prodromal period of days to weeks, consisting of a febrile upper respiratory infection or frontal headaches contralateral to the hemiparesis. Three forms of onset are usually encountered. 1. Abrupt or Apoplectiform Onset. This is the most common mode of onset, with seizures, a febrile reaction of 101 to 103 F., coma and hemiplegia. Status epilepticus lasting several hours or days is the initial manifestation. These clonic seizures are usually focal or localized to one side, but they may progress to the contralateral side, being more intense on the initially involved side. Coma always supervenes and may remain beyond the cessation of the seizures. Hemiparesis, usually flaccid, is evident on cessation of the seizures and principally affects the face and upper extremity. Recovery of the lower extremity precedes and exceeds that of the upper extremity. Deep tendon reflexes may be diminished or even absent in the acute phase, but the plantar response is usually pathological. Hemianopia and aphasia are commonly associated with the hemiplegic state. 2. Acute Onset of Hemiplegia. The hemiplegia may occur suddenly without any seizure activity. There may be no change in the state of consciousness, or one of only short duration. The characteristics of the hemiplegia are similar to those in the apoplectiform mode of onset, and it may or may not be associated with hemianopia and aphasia. S. Intermittent or "Stuttering" Pattern. Transient episodes of weakness may be observed in some cases ·of carotid artery thrombosis. Decreased palpability of the contralateral internal carotid artery may be of diagnostic significance. Abnormalities of Motor Function. Hemiparesis, initially flaccid and occasionally with edema of the involved limbs, appears on cessation of convulsions. It is maximal at onset and there is usually more severe involvement of the upper extremity and face. Severity and duration of seizures appear to be related to the degree of ultimate motor impairment. No such correlation exists between the severity of the febrile reaction and degree of paralysis. Hemiparesis may disappear rapidly, slowly regress over a period of 3 to 4 months or persist indefinitely. Recovery in the lower extremity precedes and exceeds that in the upper extremity. Approximately 50 per cent of affected children will have some residual motor deficit. Spasticity supervenes about 2 weeks after the onset, and contrac0

856

SIDNEY CARTER, ARNOLD

P.

GoLD

tures may be first noted 1 to 3 months later. Persistent weakness 2 to 3 weeks after the onset is usually indicative of a residual motor deficit in later life. Hemiatrophy (dwarfing of the involved limbs) is related to parietal lobe involvement and is more likely to develop with paralysis of early onset. Hemiparesis occurs more frequently on the right side. There is no adequate explanation for this predominance, but the increased frequency may be more apparent than real, as a mild left hemiparesis might be interpreted as natural left-sided clumsiness in a child thought to be right-handed. Motor activities may be further complicated by the development of involuntary movements, which usually become evident months after the onset of the hemiplegia. Their presence is often dependent on whether purposeful activities are being performed by the hemiplegic limbs. Athetosis and choreiform movements imply basal ganglion involvement and frequently complicate voluntary or purposeful function. Hand movements, such as in grasping objects, may be seriously impaired by athetosis. Instead of assuming a flexed state, the fingers slowly and irregularly separate and hyperextend with associated wrist extension; in a fraction of a second the fingers and wrist suddenly flex, often with force. Associated movements develop without purposeful activity and are generalized or imitative (mirror movements). Generalized movements, usually a position of generalized flexion, occur with nonspecific motor activities and result in the frequently observed "positioning" of the hemiplegic limbs when walking. Imitative or mirror movements, most marked during periods of emotion, consist of involuntary movements in the hemiplegic extremity similar to voluntary ones in the normal limb. Reflex changes in the involved extremities include absent or hypoactive deep tendon reflexes during the flaccid phase and exaggerated responses, with occasional spread to the contralateral extremities, with the development of spasticity. Contractures of the involved limbs may again diminish the amplitude of the deep tendon reflexes. PatholOgical responses, especially a Babinski response, occur soon after the onset of the hemiplegia and may persist for an indefinite period of time. Abnormalities of Sensation. Sensory impairment is the result of parietal lobe involvement. Superficial modalities (touch and pain) are usually intact, but due to the child's age, this examination may be limited to only awareness of pin prick. In the older child, stereognosis, position sensation and two-point discrimination are often impaired, and this often correlates with coexisting hemiatrophy. Rehabilitation of the involved extremities is complicated by this parietal lobe involvement, for there may be a lack of awareness or neglect of the hemiplegic extremities and a disturbed body image. Abnormalities of Vasomotor Function. The majority of affected children show at least some alteration in vasomotor function. During the acute stage of a flaccid hemiparesis the involved extremities may be warmer and edematous, and the skin may be erythematous. The edema

ACUTE INFANTILE HEMIPLEGIA

857

and skin congestion disappear after a few days, and with the development of spasticity the affected extremity becomes colder when compared with the normal limb. Abnormalities of Cranial Nerve Function. Eye defects are relatively common and include homonymous hemianopia to the side of the hemiplegia, convergent strabismus and degrees of amblyopia. Less common eye problems are divergent strabismus and severe myopia. Facial paresis of the upper motor neuron type is frequently seen and is not necessarily related to the severity of the hemiparesis. Difficulty with swallowing and lingual paresis are occasionally complicating manifestations which may result in drooling, inability to swallow coarse food and a speech defect. Disorders of Speech. Speedi defects and retardation of speech development are primarily dependent upon the age of the child at the onset of the hemiplegic syndrome. Dysphasia, more commonly encountered in older children with a dominant hemisphere lesion, is rare in children younger than 4 years of age; unless associated with mental retardation, it is never observed with onset before the age of 2 years. Dysphasia frequently occurs at the time of the acute episode, especially when associated with seizures; subsequently, normal speech patterns return after a period of 2 to 21 days, particularly in younger children. Mental retardation is the most probable explanation for the child's failure to develop expressive language function. Speech defects, primarily articulation problems, are common above all in younger children. Epilepsy. Approximately 50 per cent of children with acute hemiplegia will subsequently develop recurring seizures, this complication being more frequent with moderate or severe hemiplegia. Seizures usually become clinically evident within a year after the hemiplegia, but this latent period may be years or even decades. All children with acute acquired hemiplegia must be considered seizure suspects for an indefinite period. Epilepsy may be limited to one type, or a mixed convulsive disorder may develop. Seizure types include focal motor or sensory limited to the paretic extremities; jacksonian, often preceded by an aura, with initial clonic movements involving the hemiparetic side and subsequent spread to the normally functioning extremities; generalized or grand mal; psychomotor; and, less commonly, minor motor seizures with myoclonic jerks. Seizures of the focal motor or generalized types may result in a transient or permanent increase in the severity of the hemiplegia if they are of long duration. Seizures associated with the hemiplegic syndrome may prove to be refractory to anticonvulsant medication. Minor motor spells are often associated with a significant intellectual deficit. Defects of Behavior, Learning and Intellect. The physical disability of most infantile hemiplegic children is compatible with normal school placement and is in itself rarely the indication for special school placement. Intellectual, perceptual and behavioral problems, the addi-

858

SIDNEY CARTER, ARNOLD

P.

GOLD

tional result of the brain lesion, often complicate the hemiplegic's function, and such placement is then advisable if not mandatory. Reliable assessment of intelligence is most difficult in the preschool child. Despite this serious limitation, the early delineation of intellectual and learning potential is highly desirable, so that the parents can establish realistic academic and vocational goals and a future educational program. Hemiplegic children usually have a mild intellectual deficit; a few are of superior intellect, and approximately 25 per cent may be overtly retarded. Severe intellectual impairment is most commonly observed when the hemiplegia is complicated by seizures refractory to anticonvulsants. Psychometric evaluation does not reveal any significant difference between right and left hemiplegics, and intellectual potential cannot be inferred from the extent of physical disability. Motor defects are largely responsible for the usually observed lower performance scores; in such cases, the verbal rather than the full scale should be utilized as the guide to intellectual potential. Perceptual disturbances with visuospatial problems further complicate the academic performance of most hemiplegic children. Spatial orientation is defective, with difficulty in differentiating right from left and foreground from background. Difficulties are also encountered in copying geometric forms and putting together simple jigsaw puzzles. These perceptual problems interfere with the learning of basic school work and may contribute to emotional problems. Behavior and personality problems are common. Children with acute hemiplegia may be passive and underactive but more typically are hyperkinetic with hyperactivity, short attention span, low frustration threshold, impulsivity and distractibility. The development of this hyperkinetic behavioral pattern following the acute insult to the central nervous sys~ tern is common in children with the hemiplegic syndrome. Initially the child is passive and may even be lethargic for periods of 1 to 5 months. At this time, the passiveness is gradually replaced by increasing activity with a short span of attention. The child's personality development is marked by these behavioral characteristics, which permit him to be easily led, rejected by his peers, antagonistic to parents and siblings, destructive and aggressive.

LABORATORY FINDINGS

Blood count, urinalysis and erythrocyte sedimentation rate are normal. The cerebrospinal fluid during the acute phase is characteristically normal; a slight to moderate pleocytosis and increase in protein content may be found toward the end of the first week. Electroencephalograms, rarely normal, may demonstrate a variety of electrical abnormalities. The amplitude of the background activity is usually reduced over the damaged

,i

859

ACUTE INFANTILE HEMIPLEGIA

hemisphere. Paroxysmal discharges may consist of diffuse generalized high-voltage activity to focal spike discharges, commonly localized to the temporal or temporoparietal region of the involved hemisphere. RadiologiC studies often have diagnostic significance. Early skull x-rays are characteristically normal, but after a period of years they may show the features described by Dyke, Davidoff and Masson (DDM syndrome ) -thickening of the cranial vault, overdevelopment of the frontal and ethmoid sinuses and elevation of the petrous pyramid of the temporal bone on the contralateral side. These skull x-ray changes are the result of underlying cerebral hemiatrophy and are most common when the hemiplegia has its onset before 3 years of age. Midline echoencephalograms establish the position of the third ventricle and other midline structures. Acute hemiplegia may be associated with a swollen cerebral hemisphere, and the echoencephalogram at this stage shows a shift of the midline structures away from the involved hemisphere. After a period of months to years, cortical hemiatrophy may occur and the midline trace is then shifted toward the involved side. Radioactive intracranial lesion localization tests are often normal, except when the acute hemiplegia is associated with an arteriovenous malformation. Angiography performed at any stage in the hemiplegic syndrome may be normal. However, this ancillary procedure may be invaluable in diagnosis. Occlusive disease of the internal carotid or one of the cerebral arteries secondary to thrombosis or embolism, and arteritis with irregularity or "beading" of the arterial lumen, may be documented in the early hemiplegic stage. After a few months, this contrast study may show recanalization of the previously occluded artery or the presence of collateral circulation. Pneumoencephalography is rarely indicated during the acute phase. This air contrast study may be normal, or the third ventricle may be shifted across the midline by a swollen cerebral hemisphere, which may be falsely interpreted as a neoplasm rather than as cerebral edema. After months or years, atrophy of the involved hemisphere supervenes and results in a small atrophic cerebral hemisphere with an enlarged lateral ventricle. DIAGNOSIS

Diagnosis of acute infantile hemiplegia is not difficult in the previously healthy child who develops the classical manifestations of seizures, fever, coma and hemiparesis. Acuteness of onset of the hemiplegia is the essential feature of this disorder. Etiologic delineation, often obscure, may be established with a careful history and knowledge of all possible predisposing conditions. It is imperative that all conditions requiring specific therapy be excluded; these include epidural, subdural and intra-

860

SmNEY CARTER, ARNOLD

P.

GoLD

cerebral hematomas, brain abscesses, bacterial meningitis and cerebral neoplasms. Hematomas are suggested by a history of trauma and the subsequent development of progressive hemiparesis, with symptoms and signs of increased intracranial pressure. Arteriography is the procedure of choice to document the presence of these lesions and those resulting from rupture of arteriovenous malformations. Brain abscesses, difficult to exclude, should always be considered in the presence of pulmonary sepsis or in children with cyanotic congenital heart disease after the age of 2. In such cases the hemiparesis is more insidious in onset; there may be evidence of increased intracranial pressure, and the electroencephalogram often shows a prominent slow wave focus over the abscess. Bacterial meningitis can be excluded only by examination of the cerebrospinal fluid. Insidious onset and gradual progression of the hemiparesis is characteristic of cerebral neoplasms and degenerative diseases. Symptoms and signs of increased intracranial pressure and focal changes in the appropriate contrast studies are diagnostic of brain tumors. Hemiparesis present since birth is often unrecognized during the first 3 to 5 months of life. "Sudden" awareness of this congenital hemiplegia should not be confused with the acquired hemiplegia of acute onset. The lack of a specific ictus in congenital hemiplegia clearly differentiates it. Diagnosis requires a detailed history, a thorough physical and neurologic examination and the judicious use of such ancillary procedures as plain skull x-rays, cerebrospinal fluid analysis, electroencephalography, echoencephalography, brain scanning and arteriography. Other appropriate laboratory tests include sickle cell and lupus erythematosus preparations, cardiovascular studies and bacterial and viral cultures.

PROGNOSIS

Prognosis is most directly related to the condition responsible for the infantile hemiplegic syndrome. The primary disease responsible for the hemiparesis may have non-neurologic complications that may be lifethreatening. Obscure or relatively benign entities can result in hemiplegic states with manifestations of varied severity. Prognosis in an individual child is dependent upon such factors as age of onset, severity of hemiparesis and associated neurologic manifestations. Twenty-five per cent of children with the acute infantile hemiplegic syndrome died during the early phase of the illness; these deaths often result from status epilepticus or from the primary precipitating disease. Approximately 75 per cent of the survivors manifest neurologic residua consisting of motor and sensory deficits, convulSions, mental retardation and disabilities of speech, behavior and learning. Residual hemiparesis is best correlated with early age of onset and severity of paralysis. Complete recovery is unlikely when the hemiplegia

ACUTE INFANTILE HEMIPLEGIA

861

occurs in infancy and when there is little or no improvement during the first week. Permanent hemiparesis frequently follows an onset complicated by status epilepticus and coma. Lower extremity function is characteristically better than that of the upper extremity. Independent gait patterns eventually develop in most children, but the upper extremity, and particularly the hand, may be permanently nonfunctioning. Parietal lobe involvement further complicates the motor deficit. Hemiatrophy, or dwarfing of extremities, may seriously affect the gait pattern, while astereognosis limits hand function. Visual field defects, often denied, rarely affect motor performance. Mental retardation can be expected in 30 to 50 per cent of surviving children. Intellectual deficits of varying degrees are most commonly found when associated with minor motor seizures or convulsive states that are refractory to anticonvulsants. Prognosis for speech recovery is good. Residual speech deficits, other than articulation problems, usually indicate an associated intellectual retardation in the younger child or involvement of the dominant cerebral hemisphere with aphasia in the school-age youngster. Expressive aphasia is rarely permanent at any age. Epilepsy, a common complicating problem, may seriously interfere with the child's performance. Approximately 50 per cent of affected children have seizures, which include focal, generalized; psychomotor and minor motor types. Focal seizures unassociated with loss of consciousness are most responsive to anticonvulsant therapy; minor motor spells, often associated with retardation, are the most difficult to control. Behavioral problems and learning disabilities eventually occur in 70 to 80 per cent of the children. Hyperkinesis and perceptual and conceptual difficulties are common and may be the most disabling of the complications.

TREATMENT A rational therapeutic program requires a knowledge of all possible etiologic factors, as well as a complete understanding of the pathologic process and its resultant alteration of function. Treatment to Prevent and Minimize Severity of Complications Prophylactic measures include prompt recognition and treatment of infections, trauma and seizures. Therapy during the acute phase includes antipyretics, parenteral fluids, antibiotics and anticonvulsants. Seizures must be promptly controlled by the parenteral use of phenobarbital or paraldehyde; at times ether or chloroform inhalation anesthesia is necessary. Phenobarbital is administered intravenously in quantities large enough to control all seizure activity without suppressing respiration. Relatively "large" doses of anticonvulsants are often necessary. A 2 year

862

SIDNEY CARTER, ARNOLD

P.

GOLD

old may require 200 mg. of intravenous phenobarbital administered during a 10 to 15 minute period to obtain the desired results. Paraldehyde as a 4 per cent solution is given intravenously as a rapid infusion until seizures are controlled. Maintenance anticonvulsant therapy must be continued indefinitely. Anticoagulation with heparin is reserved for those children with multiple embolic phenomena. Treatment Subsequent to the Onset of Hemiplegia Motor Problems. Early therapy of the hemiplegia is essential, as its proper application may well determine ultimate motor function. Management is guided by the stage of hemiparesis. Acute hemiparesis, usually flaccid, may be so severe as to require bed rest. At this phase, convalescence may be expedited by preventing the development of edema and contractures. Edema is minimized by gravitational drainage; the arm is placed in abduction with the hand higher than the elbow, which in turn is higher than the shoulder. External rotation of the hip and foot drop must be prevented with proper placement of footboards, sandbags along the thighs, and splints if necessary. Passive daily exercise of all joints through a full range of motion is essential. Sitting and standing should be encouraged as early as permitted by function and the state of consciousness. Developing spasticity requires a formal program of physical and occupational therapy. Night splints are used to prevent deformities. Ambulation may require braces. Short leg braces are usually prescribed, as quadriceps function is adequate in most cases of acute infantile hemiplegia. Expert nerve blocks with 2 to 3 per cent phenol are often effective in improving function of spastic muscles. After a period of years, it may become apparent that joint instability and muscle imbalance cannot be corrected. At this phase, orthopedic surgery with arthrodesis and tendon transplants may be indicated. Convulsions. Seizures complicating the onset or subsequent course are an unequivocal indication for indefinite maintenance therapy. Oral anticonvulsants are given as permitted by the child's general health. Phenobarbital, if not complicated by a paradoxical hyperkinetic response, is the initial drug. Failure to obtain seizure control may require the use of multiple anticonvulsants that include diphenylhydantoin (Dilantin), primidone (Mysoline), acetazolamide (Diamox), bromides and even new investigational drugs. Some children with acute infantile hemiplegia do not respond to drugs and frequently develop severe personality and behavior disorders. Surgical therapy (hemispherectomy) should be considered in a refractory seizure state refractory to at least a 2 year trial with anticonvulsants. This procedure is usually reserved for carefully selected patients and should only be performed in major medical centers. Complete seizure control and improved behavioral patterns are the results of successful surgery.

863

ACUTE INFANTILE HEMIPLEGIA

Behavioral Problems. Hyperkinetic behavior and its management is most difficult, as drug therapy is unpredictable and a particular compound may lose its effectiveness with time (see also p. 767). Parental understanding is essential in establishing a consistent approach to discipline and developing a well-structured home. Dextroamphetamine (Dexedrine) and methylphenidate (Ritalin) are the most effective drugs; the apparent paradoxic effect of these agents is most frequently observed in children demonstrating barbiturate-induced hyperactivity. Phenathiazine (Mellaril and Thorazine) and diphenhydramine hydrochloride (Benadryl) may also be effective. Counseling that includes the parents may be useful in the management of this most difficult complication of the hemiplegic syndrome. Speech Problems. Although there are only a few cases of expressive aphasia, many children have articulatory problems of varying severity (dysphonia, dysrhythmia and dysarthria). In the very young child, sucking, swallowing and chewing are major therapeutic movements, and these functions are frequently deficient in those children with impaired speech patterns. Tongue movements may be improved by placing jam or peanut butter on various areas of the lips; lollipops and ice cream cones can also be used to good advantage by moving them in a strategic manner. Nursery school placement of preschool children may stimulate the development of more normal speech. Effective formalized remedial speech therapy requires comprehension, and this therapeutic approach has only limited usefulness with the retarded. Educational and Employment Problems. Mental deficiency is the primary deterrent to intellectual accomplishment. However, care should be taken not to place all the blame for low academic and employment achievement upon mental deficiency, lest the treatment of other contributory factors be neglected. School placement must be individualized; its goal must be attainment of as great an independence as possible within the scope of limitations imposed by the hemiparesis, epilepsy, mental retardation, behavioral difficulties and specific learning disabilities. Early realistic vocational planning and training are essential. All too often the hemiparesis and its management are the primary concern, with little or no interest in the academic and occupational problems.

REFERENCES GENERAL

Bax, M., and Mitchell, R. (Eds.): Acute Hemiplegia in Childlwod. London, Spastics Society, Heinemann, 1962. Gold, A. P., Hammill, J. F., and Carkl, S.: Cerebrovascular Diseases. In Farmer, T. W. (Ed.): Pediatric Neurology. New York, Hoeber Medical Division, Harper & Row, 1964. Ingram, T. T. S.: Paediatric Aspects of Cerebral Palsy. Edinburgh, Livingstone, 1964.

864

SIDNEY CARTER, ARNOLD

P.

GOLD

ETIOLOGY

Banker, B. Q.: Cerebral Vascular Disease in Infancy and Childhood. I. Occlusive Vascular Diseases. J. Neuropath. Exp. Neurol., 20:127, 1961. Bickerstaff, E. R.: Aetiology of Acute Hemiplegia in Childhood. Brit. Med. J., 2:82, July 1964. Pitner, S. E.: Carotid Thrombosis Due to Intraoral Trauma-Unusual Complication of Common Childhood Accident. N. Eng. J. Med., 74:764, 1966. Shillito, J., Jr.: Carotid Arteritis: A Cause of Hemiplegia in Childhood. J. Neurosurg., 21:540, 1964. Swinyard, C. A., Swenson, J., and Greenspan, L.: An Institutional Survey of 143 Cases of Acquired Cerebral Palsy. Develop. Med. Child Neurol., 5:615, 1963. Tyler, H. R., and Clark, D. B.: Cerebrovascular Accidents in Patients with Congenital Heart Disease. A.M.A. Arch. Neurol. Psychiat., 77:483, 1957. CLINICAL MANIFESTATIONS

Gastaut, H., Poirier, F., Payan, H., Salamon, G., Toga, M., and Vigouroux, M.: H. H. E. Syndrome: Hemiconvulsions, Hemiplegia, Epilepsy. Epilepsia, 1:418, 1960. Greer, H. D., and Waltz, A. G.: Acute Neurologic Disorders of Infancy and Childhood. Develop. Med. Child Neurol., 7:507, 1965. Wisoff, H. S., and RothbaUer, A. B.: Cerebral Arterial Thrombosis in Children: Review of Literature and Addition of Two Cases of Apparently Healthy Children. Arch. Neurol., 4:258, 1961. TREATMENT

Gold, A. P., and Carter, S.: Pediatric Neurology. In Shirkey, H. (Ed.): Pediatric Therapy. St. Louis, C. V. Mosby, 1966. Khalili, A. A., et al.: Management of Spasticity by Selective Peripheral Nerve Block with Dilute Phenol Solutions in Clinical Rehabilitation. Arch. Phys. Med., 45:513, 1964. Muckart, R. D.: in Henderson, J. L. (Ed.): Cerebral Palsy in Childhood and Adolescence. Edinburgh, Livingstone, 1961. Novak, J.: The Rehabilitation of the Hemiplegic Patient. Penn. Med. J., 68:43, March 1965. White, H. H.: Cerebral Hemispherectomy in the Treatment of Infantile Hemiplegia: Review of the Literature and Report of Two Cases. Conlin. Neural., 21:1, 1961. 630 West 168th Street New York, N.Y. 10032