PEDIATRIC REHABILITATION PART I: CEREBRAL PALSY AND SPINAL CORD INJURIES GABRIELLA E. MOLNAR LAWRENOE T. TAFT

NOTE: Part II will appear as the February issue.

TABLE

OF

CONTENTS

CEREBRAL PAU~Y . . . . . . . . . . . . . . . . . . Early Signs and Recognition . . Clinical Types and Natural Course Associated Disabilities . . . . . . Management . . . . . . . . . Conclusions . . . . . . . . .

. of . . .

. . . . . . . . . . the Motor Disability . . . . . . . . . . . . . . . . . . . . . . . . . . .

SPINAL CORD INJURIES . . . . . . . . . . . . . . . . Clinical Course and Management . . . . . . . . . . . . Conclusions . . . . . . . . . . . . . . . . . . .

6 8 14 27 30 a0 46 47 53

is Associate Professor in the Departments of Rehabilitation Medicine and Pediatrics and Director, Pediatric Rehabilitation Service at the Albert Einstein College of Medicine of Yeshiva University, New York. Doctor Molnar received her M.D. degree from the University of Budapest. After several years in pediatric practice, she took her residency in physical medicine and rehabilitation and subsequently received further training in developmental pediatrics and pediatric rehabilitation at the Albert Einstein College of Medicine. IIer research interests include normal and abnormal motor development and the evaluation of treatment methods for childhood disahilities.

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is Professor and Chairman of the Department of Pediatrics at the College of Medicine and Dentistry of New Jersey, Rutgers Medical School. Doctor Taft received his M.D. degree from Downstate Medical School, New York and received his pediatric internship and residency at Bellevue Medical Center and Cornell-New York Hospital. He received further training in pediatric neurology and pediatric rehabilitation at Boston Children's Hospital and the Albert Einstein College of Medicine. Doctor Taft has a special interest in the diagnosis and management of developmental disabilities. A former presidgnt of the American Academy for Cerebral Palsy, Dr. Taft presently is a member of the board of directors of the United Cerebral Palsy Association. In addition, he is a recent appointee to the Developmental Disabilities Council.

R E H A B I L I T A T I O N M E D I C I N E is concerned with r e s t o r a t i o n of function. W h e n complete recovery is not a n t i c i p a t e d the a i m is to p r o m o t e m a x i m a l f u n c t i o n a l a d a p t i o n to a p e r m a n e n t disability. As the need to seek help for a child is c r e a t e d by a c u r t a i l m e n t of m o t o r function it would a p p e a r at first g l a n c e t h a t the interest of pediatric r e h a b i l i t a t i o n is focused on the physical disability alone. U n d o u b t e d l y , a g r e a t deal of professional a t t e n t i o n is directed at the m o t o r h a n d i c a p a n d this problem tends to r e m a i n the c e n t e r of p a r e n t a l anxiety. However, the effect of a childhood disability e x t e n d s beyond the i m p a i r m e n t o f m o t o r function. It also acts as a n i n i t i a t i n g factor in a cycle t h a t profoundly alters the n o r m a l course of child d e v e l o p m e n t , of f a m i l y d y n a m i c s a n d the m u t u a l i n t e r a c t i o n of these processes. 3

The situation is further complicated by the fact that some major handicapping conditions of childhood, such as cerebral palsy and meningomyelocele, are frequently associated with abnormalities of several organ systems. These problems can sometimes overshadow the physical disability in their functional or health threatening significance. In such instances the motor dysfunction must be considered in a proper context among the presenting multiple handicaps. The foregoing points are intended to illustrate that properly interpreted pediatric rehabilitation encompasses a study and remediation of deviant child development with respect to a motor disability and its organic, functional and behavioral sequelae. The ultimate outcome of the rehabilitation process is determined by an intricate interplay of these diverse influences. Only by attention to all can one hope to ensure optimal results. Rehabilitation medicine is one of the youngest medical specialties and it evolved initially in response to war casualties. A further impetus has been provided by the large number of adult and geriatric patients who suffer from permanent disabilities unrelated to the violent events of war and who require special methods of care not usually coordinated in the realm of any one existing branch of medicine. Gradually, and essentially because of the interest and efforts of a handful of people, rehabilitation medicine became concerned with the management of handicapped children. At first, the principles of rehabilitation and the various modalities of restorative and adaptive training, already well established but developed primarily for handicapped adults, were applied with minor modifications to the management of physically disabled children. Over the years, however, pediatric rehabilitation began to emerge on its own as it was recognized that this area requires special expertise within the broad field of rehabilitation medicine. Nevertheless, only recently has a true integration of pertinent aspects from pediatrics and rehabilita9tion medicine taken place. The rehabilitation process now reflects an understanding of normal child development and its deviations among handicapped children. An outstanding example of the evolution that occurred and is still occurring in pediatric rehabilitation is the changing approach to prosthetic management of children with congenital upper extremity deficiency. In the past these youngsters were provided with a prosthesis when they were between 3 and 5 years of age. This recommendation reflected the rationale that the child must learn to operate an artificial arm by traditional verbal conceptual methods of training applicable to adults. Results of delayed prosthetic training were poor and the reasons i'or this are described in a later section. Eventually, however, the time of prosthetic fitting was advanced to an earlier and earlier age un-

til, at present, fitting with a passive prosthesis takes place between 3 to 6 months of age. Training with an active device is attempted early in the second year and in most instances the device can be used successfully between 18 to 24 months. Though the changes in approach evolved empirically, in retrospect, the success of early prosthetic fitting should have been anticipated on theoretical grounds considering the nature of the learning process in the sensorimotor stage of intelligence. These examples serve to demonstrate the state of the art of pediatric rehabilitation and emphasize the need for clinical research. Specifically, there is a need to develop more accurate norms of expectation for activities that handicapped youngsters of various ages have to utilize, similar to the standards established by Gesell and by many other investigators for normal children. Another urgent priority for research is more objective evaluation of the effectiveness of different treatment methods, particularly those proposed to alleviate the neuromuscular dysfunction in cerebral palsy. Finally, the third area that requires attention is more precise prognostication. A search for early predictive indicators must continue in order to unravel the complex interaction between the many organic, emotional and social factors that influence the final outcome even in those children who are not considered multiply handicapped in the narrow sense of the term. A better understanding of these correlations should enable us to detect more subtle warning signs, to institute more vigorous and earlier preventive measures and to avert some unexpected social and vocational failure when these children reach adulthood. The following review is not complete, in that only some problems seen in pediatric rehabilitatioi~ practice are included. We have selected two physical disabilities of childhood on the basis of their frequency (cerebral palsy and spina bifida) and another two (spinal cord injuries and limb deficiencies) were chosen because they represent major, though less common, handicaps and pediatricians are unlikely to be acquainted with their current management. Limitations of space resulted in omission of a number of conditions, burns, arthritis and a variety of orthopedic and neurologic diseases, where rehabilitation plays an adjunctive role. In most instances a specialist in pediatric rehabilitation is consulted to guide the pediatrician in the management of a child when the diagnosis of a specific disability is fairly certain. Nevertheless, we have dwelt at some length on the diagnostic aspects of different conditions for two reasons: first, because of their importance for the pediatrician and secondly, because a functional diagnostic evaluation is necessary to provide guidelines for immediate and long-term goals of rehabilitation. The establishment of a

realistic framework of expectations is a prerequisite of intervention and determines the choice of treatment. Technical details of various therapeutic modalities used in rehabilitation are kept at a m i n i m u m and are discussed to demonstrate essential principles of diagnosis and management. Contributions to the t r e a t m e n t of handicapped children by other medical specialties are briefly mentioned because they constitute an integral part of m a n a g e m e n t and have to be coordinated in the plan of long-term rehabilitation.

CEREBRAL PALSY Cerebral palsy is the most frequent permanent physical disability of childhood. Though it is difficult to determine the exact incidence, various epidemiologic studies suggest t h a t it varies from 1.5 to 5 per 1000 live births or population under 21 years. 1-3 The estimated number of children with cerebral palsy in this country is about 400,000. The diagnosis of cerebral palsy includes a collection of syndromes with diverse etiology, pathology and clinical manifestations. It is defined as a nonprogressive damage to the i m m a t u r e brain causing neuromuscular disability and other symptom complexes of cerebral dysfunction2, 5 Prenatal impairment of the brain can occur in complications of pregnancy with a risk of anoxia to the fetus. 5-8 Intrauterine infections;poisonings and maternal metabolic diseases are also among the prenatal etiologic factors. G-s In the perinatal period, mechanical or other abnormalities of the birth process, cardiorespiratory and metabolic disturbances oi~ isoimmunization of the newborn m a y be the precipitating cause. 5-9 Often there are multiple prenatal and perinatal contributory factors since newborns with intrauterine damage are more likely to have a complicated perinatal course. 6 In 20-30% of cases with symptoms evident in infancy there are no apparent past pathologic eventsY It is conceivable, in these instances, t h a t an abnormal incident had occurred but was unnoticed or t h a t there is an unknown and undetectable, possibly genetic, developmental anomaly of the brain. P r e m a t u r i t y remains the most significant single etiologic factor. ~~ Prenatal and perinatal complications account for more t h a n two thirds of all cases. Postnatally acquired cerebral palsy can be the consequence of head trauma, toxicoses, vascular accidents or other nonprogressive neurologic conditions with permanent impairment of the cerebral motor structures2 -s, 13-~6Residual motor dysfunction following meningitides and encephalopathies also fulfills the criteria ofnonprogressive brain lesion once the acute stage has passed or the disease process has been successfully controlled.

In the pathomechanism of brain damage anoxia plays the most significant rolc. '7 Less frequently hemorrhage, vascular occulsion, cortical laceration, toxicosis or infectious destruction of neuronal tissues are responsible. 18. t9 These kinds of mechanism usually occur with particular etiologic events and are also often accompanied by secondary cerebral anoxia. The neuropathologic lesions of anoxic encephalopathy are complex and generally diffuse. In spite of their diversity the lesions tend to lack specificity in respect to cause. ~7 These facts make it difficult to establish a precise localization of neurologic lesions on the basis of etiology and clinical signs. There are, however, some notable exceptions. The syndrome of kernicteric athetoid cerebral palsy is clearly related to hemolytic disease of the newborn and consequent bilirubin encephalopathy with pigment deposits in the basal ganglia and in some cranial nerve nuclei2 The characteristic type ofcerebral palsy among premature infants is spastic diplegia caused by a hypoxic infarct or hemorrhage in the periventricular zone adjacent to the lateral ventricles. 2~ These lesions involve the pyramidal tracts descending through the region, particularly, their medially located fibers subserving the lower extremities. Although less consistently associatcd, hemiplegic cerebral palsy may be the result of a mechanical birth injury or cerebral vascular anomaly with thrombosis or hemorrhage of the middle cerebral artery2 9.-0'.-0-~ The combination of spasticity with rigidity or athetosis suggests cortical and basal ganglion damage. ''~9 Cerebellar hypoplasia was demonstrated by pneumoencephalograms in a number of children with ataxic cerebral palsy2 More recently, neonatal hypoglycemia has been implicated as a possiTABLE 1.-CLINICAL TYPES OF CEREBRAL PALSY AND THEIR APPROXIMATE FREQUENCY TYPE

Spastic Monoparesis t Iemiparesis Congenital (two thirds) Postnatal (one third) Diplegia (paraparesis) Triplegia Quadriparesis (tetraplegia, diplegia) Athetoid (dyskinctic, dystonic) Rigid Ataxic Tremor Atonic (hypotonic) Mixed Spastic-athetoid Rigid-spastic Spastic-ataxic

FREQUENCY (%)

50- 60 Rare 35- 40 10-20 Rare 20- 30 20-25 5-10 1-10 Rare Rare 10- 40 10- 30 5-'10 Rare

ble etiologic factor in the same clinical type? ' Severe quadriparesis with mental retardation and microcephaly following prenatal or neonatal anoxic episodes would suggest generalized cortical and cerebral atrophy. '7''9 Classifications of cerebral palsy are based on pathologic findings, '9 etiologic factors 5 or neurologic signs, s. 25 For the clinician a designation by clinical type according to the nature and distribution of neuromuscular dysfunction is most helpful. The onset of brain injury is noted as well by stating prenatal, perinatal or postnatal origin. Table 1 shows the different clinical types and their approximate frequency. The diagnosis of cerebral palsy should be established by appropriate neurologic evaluation even when past history leaves no apparent doubt as to the reasons for the abnormal findings. At times repeated confirmatory studies may be indicated, particularly when etiology is unclear, clinical signs are unusual or the course is atypical. Insidiously progressing degenerative diseases or the early onset of slowly growing brain tumors may imitate cerebral palsy and a number of such cases have been described in a rather recent clinical and neuropathologic study. '~ Therefore, progressive familial diseases with genetic implications or possibly treatable conditions must be ruled out before one would conclude that a static encephalopathy is present. EARLY SIGNS AND RECOGNITION

Although abnormalities of pregnancy, delivery and neonatal course place the infant at risk for sufferingcerebra] damage, correlations between specificetiologicfactors and chancesof having cerebra] palsy are complexand require further studies.26A direct relationship, however, is well established in prematurity between decreasingbirth weight and the incidenceof cerebra] palsy.'~ It is unclear why in some cases the abnormal perinatal episode and the subsequent clinical dysfunctionare apparently disproportionate in their severity. 2', 2s A low Apgar score at 5 min. has been suggested as a predictor of permanent brain damage though not necessarily of cerebral palsyY s, 29 In a recent study, among other prenatal, obstetrical and perinatal abnormalities, neonatal seizures and intracranial hemorrhage were defined as high risk factors for severe cerebral palsy with mental retardation? ~ The complexity of these correlations compounded by a lack of reliable neonatal neurologic signs preclude detection of cerebral palsy at birth. Diagnostic uncertainties of the neuromuscular dysfunction in the newborn period are related to the fact that cortical control of movements does not occur until later in infancy. 3' Therefore, impairments of volitional motion, such as paresis or dyskinesias, are not discernible at an early age and the neuro8

muscular dysfunction cannot be recognized until after 2 - 4 months. Though there may be a suspicion of cerebral palsy, it is only in some cases that the diagnosis can be definitely ascertained w i t h i n t h e first 6 months even by the most experienced examiner.6, 7, 32-~ Unequivocally abnormal neurologic signs at such an early age usually augur a rather severe damage. An exception is congenital hemiplegia, where functional limitations are slight b u t can be recognized by the evident a s y m m e t r y of extremity movements beginning around 4 to 6 months of age. 6, 3,4, Otherwise, the greatest likelihood of confirming a suspected diagnosis is in the second half of the first year when delayed motor development and other signs become quite discernible. However, mildly affected infants may show only subtle symptoms that could be overlooked until delay or abnormal performance of more advanced motor milestones such as standing or walking is noted. It should also be mentioned that in a few instances mild b u t deftnite aberrations observed in infancy can resolve spontaneously as a result of plasticity inherent in the immature central nervous system23, 42 There are several groups of early signs and symptoms that should arouse suspicion and alert one to the possibility of cerebral palsy. 1. Delayed gross motor development is a universal symptom.e-s, 34, 35, 43, 44 Though it usually includes lateness in all motor accomplishments the problem often does not attract attention until delay of sitting is evident. Discrepancy of motor achievements tends to increase with successive milestones and there is also a degree of relationship between the length of delay and severity of neuromuscular dysfunction. Delayed.motor development is a particularly suggestive sign when personal-social and language behavior are normal. 2. Abnormal motor performance also reflects the neuromuscular dysfunction of cerebral palsy. Preferential unilateral hand use is quite noticeable by 6 months of age in congenital hemiplegia although some parents erroneously interpret this symptom as early evidence of normal handedness. 6, 39-4~ Detectable hand dominance usually does not develop until 11/2 to 2 years of age; therefore, this observation should be viewed with suspicion. Abnormal crawl is seen in diplegia when forward progression is accomplished by arm movements only. 4~ Pelvis and lower extremities are hiked along in a nonreciprocal manner that led to the descriptive term of b u n n y hop. Asymmetrical crawl by using the unaffected arm and leg is a sign of hemiplegia. 4~ Some children with diplegia or hemiplegia do not crawl at all. Instead, they resort to scooting on their abdomen or buttocks and propel themselves only with the unaffected extremities. 7' 46 A persistent tendency to stand or walk on toes m a y indicate spasticity of the legs. 6. ~. ~ Incoordination or involuntary movements are signs of 9

Fig l.-Opisthotonus in a spastic-athetoid infant. Abnormal fisting of the hands.

dyskinetic cerebral palsy. Athetosis is first recognizable by facial grimacing and writhing movements of the tongue, fingers and toes.6, 7 In the ataxic type intention tremor on reaching, head titubation and truncal ataxia can be observed. Poor sucking and feeding difficulties with tongue thrust are symptoms of pseudobulbar palsy with spastic or athetoid incoorclination of the oropharyngeal musculature. 3. Alterations o f muscle tone are early signs though it may be difficult to detect mild aberrations. Abnormal tone is manifested as either decreased or increased resistance to passive movements. Fig 2.-Severe spastic quadriparesis with marked extensor spasticity around the hips in a 14-month-old infant.:-"

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Most children with cerebral palsy undergo an early stage of hypotonia before definitive neurologic signs appear. 6, 7, 47 Severity and duration of hypotonicity tend to be related to the clinical type and are discussed later. Hypertonia is characteristic of the spastic and rigid clinical types. G,7, 24, ~5 A tendency of opisthotonic postures is an indication of increased extensor tone (Fig 1)7, 4s and is often interpreted by mothers as precocious elevation of head and trunk or rolling over. There are other typical symptoms described by parents and easily reproduced by the examiner that aid in recognizing spastic hypertonicity. The infant feels stiff on handling and dressing. Difficulties on diapering suggest spasticity of hip adductors and lower extremities. An increased resistance is encountered when the infant is pulled to sit. In severe cases, rather than bending the hips the whole body becomes extended like a board and the infant cannot be brought to sitting (Fig 2). This finding is consistent with increased tone of hip extensors and is one of the earliest signs of spasticity. In athetosis there is a fluctuating tone 4s that can be felt when the examiner's hand is placed on the extremity or when it is moved passively. Sometimes, tone fluctuations are discernible more easily or prior to visible signs of athetosis. 4. Abnormal postures at rest or predictable postural changes when the infant is moved into different positions are quite typical of spastic cerebral palsy. In prone position, h i p s consistently higher than the t r u n k w h i l e arms and legs are flexed or drawn under the body is a sign of increased muscle tone from the earliest age2 9 In supine position, spastic hypertonicity is indicated by scissoring and extension of the legs, feet held in equinus attitude (Fig 3, A). This posture increases when the infant is suddenly lift-

Fig 2 . - T w o and one-half-' spastic quadriparesis. Abnm (A) increased in erect (B) po hands fisted. Legs in extens in equinus attitude.

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Fig 4.-Twenty-two-month-old child with severe hypotonicity. Posture of hands and feet suggestive of early athetosis. Fig 5 . - O n e and one-half-year-old athetoid child. Persistent obligatory asymmetrical tonic neck reflex, face arm extended, occiput arm in flexion, Athetoid posturing of left hand. Tongue thrust and deviation toward the side of head rotation.

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..:_ 12

j

Fig 6.--Persistent Moro reflex in a l~/2-year-old child with athetoid cerebral palsy.

ed into vertical suspension and is further aggravated by trying to make him bear weight. (Fig 3,B). 6, T,35, 4s There are gradations of this abnormal posturing depending on the degree of spasticity. In case of milder impairment it may be elicited only in vertical position and the postural abnormality is more pronounced around or confined to the distal joints. When such posturing is observed spastic paresis of the legs is confirmed. In the first few months of life infants tend to hold their arms abducted at the shoulders, elbows flexed, hands fisted when they are resting or sleeping in supine position. Persistence of this so-called infantile arm posture on awakening, if constant, after 4 - 5 months or consistent fisting after 6 months are signs of spasticity in the upper e x t r e m i ties (Fig 3). 6' ~,35, 4s In milder cases fisted hands m a y be most evident. In congenital hemiplegia the affected arm is adducted and held along the torso with the elbow in pronation and slight flexion, hand closed. 6. 7.35.4s The posture resembles t h a t of Erb's palsy but difference in muscle tone offers a diagnostic clue. The severely hypotonic child lies with his legs in a frog position (Fig 4). 48 5. Reflex abnormalities at a later age consist of the usual signs ofspasticity, such as hyperreflexia and ankle clonus. In addition, on fast passive movements a stretch reflex can be elicited in m a n y muscle groups. One of the most readily evoked among these signs is an adductor catch, i.e., resistance to passive abduction, when the hips are suddenly separated by the examiner. Persistent and predominant primitive infantile reflexes offer the earliest clues of suspicion or diagnosis. 6, 7, 35, 36, 43, 4s, 50, 51 Obligatory tonic neck reflex at a n y age or its presence in a nonobligatory form after 6 months signifies an impairment of the motor system (Fig 5). 50The Moro (Fig 6), plantar and palmar grasp reflexes (Fig 7) are some other infantile reflexes t h a t tend to become hyperactive or persist.6, 7, 4s, 50, 53-5~ Positive supporting reaction of the legs is also 13

Flg 7.-Palmar grasp reflex in 18-month-old with spastic quadriparesis.

predominant and contributes to tone increase on weight bearing mentioned earlier2 8, 50 Primitive reflex abnormalities are not restricted to any one clinical type of cerebral palsy. Persistence of infantile reflexes may be helpful for the differential diagnosis of delayed motor development caused either by cerebral palsy or by mental retardation. In the later case primitive reflexes disappear at the usual expected age. 52 CLINICAL TYPES AND NATURAL COURSE OF THE MOTOR DISABILITY At an early age neurologic signs show characteristic stages of successive change. This phenomenon of the natural course enhances the diagnostic problems and sometimes makes determination of the exact clinical type uncertain within the first 1 or 2 years of life. The changing neurologic picture is related to a continuing, although defective, maturation of the central nervous system already referred to in the discussion of early recognition. SPASTIC TYPES OF CEREBRAL P A L S Y . - I n

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spastic cerebral palsy,

which is the most common of all clinical types, generalized hypotonia or decreased tone in the affected extremities is an early neurologie manifestation. 6. 7 This stage can last from a few weeks or months to as long as 1 year. The degree of hypetonicity varies from mild to severe. Though there are no hard and fast rules, it seems that both marked hypotonia of long duration or a reversal of hypotonicity to unequivocal evidence of spastieity before 6 months tend to carry a poor prognosis. After the first appearance of hypertonia signs of spasticity will usually increase until a plateau is reached. 7 This and the subsequent evolution of volitional control depends on the extent of damage to the motor structures. Mildly affected children continue to show progressive improvement for years and acquire increasing coordination as a result of maturation in spite of an evident spastic paresis. On the other hand, when the motor system is profoundly damaged the child makes virtually no progress and functional achievements remain arrested on a low level. From a clinical behavioral viewpoint the nature of movement disorder in spastic cerebral palsy may be analyzed as consisting of the following features: (1) hypertonicity, (2) uneven tone distribution, (3) persistent primitive reflexes, (4) lack or delay of normal postural control and (5) spastic paresis. 4~ Muscle tone is increased and is abnormally distributed largely under the influence o f t h e persistent primitive reflexes and of body position in space described in some detail under early diagnostic signs. Protective and equilibrium reactions that contribute to the automatic control of posture and balance, and are required as background for coordinated motion, are absent or faulty and develop later than expected. In addition, the presence of spastic paresis implies that voluntary movement control and coordination are impaired. As a consequence, the child is more or less unsuccessful in overcoming by his own volition the habitually assumed stereotyped pathologic postures. In fact, active attempts of motion increase the abnormal postures and are accompanied by an overflow of mass movement responses to other parts of the body, particularly in more severe cases. Having a defective postural mechanism and insufficient control of willed motion the child is unable to attain or has an impairment of fine and gross motor skills. It is easy to understand the mechanism by which these neurologic abnormalities give rise to secondary museuloskeletal complications. From a kinesiologie standpoint muscle groups with predominant hypertonicity are virtually in a state of continuous reflex contraction. At first, this is a reversible process without permanent change in the muscle tissue itself and if spastieity is alleviated by some means, the abnormal postures decrease, as indeed at this stage they tend to subside while the child is asleep. 15

However, when the longstanding neurologic abnormality perpetuates this situation respective muscle groups become tight and then shortened as a result of permanent physicochemical changes in their viscoelastic properties. On the other hand, muscles antagonistic to those with excessive hypertonia, though they m a y contract intermittently, are overstretched and eventually develop a weakness of disuse. Next, ligamentous and capsular joint contractures occur and these deformities are consistent with the abnormal postures. In a growing child, the spastic muscle imbalance leaves its m a r k on the osseous skeleton. Configuration of articular surfaces, epiphyses and other bony structures are inevitably affected when spasticity is severe. This sequence of events completes the cycle from a neurologic abnormality to skeletal deformities and can lead to progressive functional deterioration as the neuromuscular deficit is compounded by mechanical limitations of mobility.

SPASTIC HEMIPLEGIA.--Hemiplegic cerebi'al palsy is most frequent within the spastic group and among all clinical types. Congenital hemiplegia is rarely recognized at birth. Approximately half of the cases are diagnosed by 6 months of age and all but the most mildly affected ones by 1 year. 4~The early hypetonic stage is usually short-lived and mild, passing before the problem is realized.6, 39, 40 Discrepancy in the use of upper extremities, apparent early handedness, abnormal crawl, persistent fisting of one hand, (Fig 8) standing or walking on the toes, all on the same side are typical signs. 56 Early motor milestones are usually delayed by 4 - 6 months. All hemiplegic children walk eventually, at the latest by 3 years, unless there is a profound intellectual deficit.6, ~, sT As a rule, the motor deficit is'less severe in the leg. Sometimes, it m a y be so slight that a functional diagnosis of monoplegia is designated, in spite of hyperactive deep tendon reflexes in the leg

Fi9 8.-Ten-month-old infant, congenital right hemiparesis, right arm not used, hand tightly fisted.

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and a present Babinski sign. The affected hand is used for assistive function and to an extent that depends on the severity of spasticity and presence or absence of parietal lobe dysfunction. Parietal lobe syndrome is found in approximately 50% of cases and is an almost unique feature of hemiplegic cerebral palsy. ~s It consists of impairment of cortical sensory modalities. Most commonly, stereognosis, two point discrimination and position sense are defective or absent. It is only around 4 - 5 years of age that this deficit can be reliably proven by objective examination. However, one would suspect it when there is a neglect and excessively poor assistive use of the affected hand that seems to be disproportionately great for the degree of spasticity. It may also be quite evident on close observation that the child looks at the extremity every time it is used, in order to substitute visual clues for the absent sensory feedback from the hand. Growth disturbances of the paretic extremitiesp 9 thought to be a t r o p h i c , albeit poorly understood, effect of the parietal lobe, occur most often in the presence of cortical sensory deficit (Fig 9). Both muscular and bony structures are underdeveloped and there may be a delay in the appearance of ossification centers, r176 rarely longer than 1 year. The size and length discrepancy becomes noticeable between 12 and 18 months and continues to increase until 6 to 8 years of age. It is generally greater in the arm than in the leg, but seldom exceeds 1 inch in the lower extremity. The course of development and final outcome is so predictable in congenital hemiplegia t h a t one can almost plot it out for the parents as soon as the diagnosis is made. A youngster with this type of cerebral palsy will walk, he will do most activities with one hand and his handicap will be cosmetic rather than functional. In postnatally acquired hemiplegia the neuromuscular deficit and prognosis of motor function are similar to those of the conFig 9.-Underdeveloped left hand, congenital hemiparesis.

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genital type: Cortical sensory deficit occurs in this group as well, when the damage extends to the parietal lobe. The extent and frequency of associated extremity hypoplasia decreases in relation to the amount of growth already completed at the onset of cerebral insult. SPASTIC QUADRIPARESIS.-Compared to the relative uniformity of motor development and functional outcome in hemiplegic cerebral palsy, children with spastic quadriparesis show considerable variations in the severity of neuromuscular deficit, clinical course and ultimate prognosis. 6. 7 This group has the highest incidence of severe disability among the spastic types. Also, since it is more frequent than some other clinical types with unfavorable outlook, spastic quadriparetics comprise the largest number of severely disabled children in the total population of cerebral palsy. These are, however, statistical chances and the fact is that about one fourth of the spastic quadriparetic children are only mildly affected and have minimal or no functional limitations in ambulation, self-care and other activities (Fig 10). Approximately half of the group is moderately impaired and impeded in skills necessary for complete community independence but can attain a degree of selfsufficiency to function in a sheltered situation. The remaining

Fig lO.-Eight-year-old mild spastic quadriparetic girl. Slightly abnormal posture. Independent ambulator, no significant limitation of hand function.

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one fourth are severely damaged and require almost total care. Delay of motor milestones is generally proportionate to the degree of motor deficit and provides some guide for prognostication. When early hypotonia is replaced by spasticity, extensor tone prevails for some length of time. 7 This may or may not change to predominant flexor hypertonicity or gradual acquisition of active mobility.7 It has been suggested that successive evolution of these stages is related to the degree of damage to the motor system and unfavorable prognosis is expected when the extensor stage is prolonged or fails to subside. ~In quadriparetic cerebral palsy spasticity is greater in the lower extremities. An asymmetrical impairment of the two sides of the body is not unusual (Fig 11). Sometimes, one upper extremity may be functionally spared though minimal signs of spasticity are evident On neurologic examina-

Fig 11.-Seven-year-old spastic quadriparetic boy with greater neurologic impairment on the right side. Not ambulatory. Typical posture of hip adduction and compensatory knock knee.

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tion. In these cases a functional descriptive diagnosis of triplegia m a y be preferred. By agreement, the term double hemiplegia is used when the arms are more affected. This type is rare and has a generally unfavorable prognosis.

SPASTIC DIPLEOIA.--Paraparesis and diplegia are used interchangeably to designate spasticity in the legs. This is the most common form of cerebral palsy in premature infants. 6' Early hypotonia is confined to the lower extremities and definite signs of spasticity appear within the second half of the first year. There is no significant limitation of upper extremity function though hyFi9 12.--Six-year-old boy, moderate spastic diplegia. No independent balance; ambulation with walkerette. Typical posture of hip and knee flexion and compensatory kyphosis.

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perreflexia in the arms m a y indicate a minimal neurologic involvement. For this reason, some authors prefer to include diplegia in the group of spastic quadriparesis which leads to some confusion in the interpretation of findings and results of different clinical surveys. 6 Neuromuscular dysfunction of the lower extremities causes late a t t a i n m e n t of gross motor milestones, sitting, standing and walking, while development of hand skills is generally appropriate for age. 6z After a shorter or longer interval of delay most diplegic children walk. 63 Some require the aid of crutches or other assistive devices (Fig 12). In mild cases abnormal signs are limited to distal joints of the legs. MUSCULOSKELETAL COMPLICATIONS IN SPASTIC TYPES.--In all types of spastic cerebra] palsy a number of prevalent deformities and musculoskeletal complications are encountered24'65 Elbow flexion and pronation contractures, wrist and finger flexion deformities are characteristic in the upper extremities.66 Marked fixed deformities are frequent with severe spasticity. When some degree of active function is preserved serious contractures are less likely to develop. Although one usually finds slight limitations of range of motion in the presence of moderate or mild spasticity these do not necessarily interfere with functional ability provided that their progression is prevented. In spasticity of the lower extremities heelcord tightness or contracture with equinus position of the foot is most common and it represents the most significant deformity in hemiplegiaY Sequelae of spasticity around the hips are flexion and adduction contractures. The abnormal joint alignment interferes with proper standing posture and walking. Even more serious are its consequences, coxa va]ga and anteversion of the femoral neck,.that can lead to sub]uxation and eventual dislocation of the hips.ks This initiates a chain of events, pain and increase of spasticity, both leading to deterioration of function from ambulatory to wheelchair-bound or from a sitting to bed-ridden existence. Scoliosis and its complications will follow as a result of abnormal anatomical alignment of hips and pelvis after dislocation has occurred23 Hip flexion contraetures are reflected by a lordotic posture. Femoral anteversion causes internal rotation of the hips and a gait with toeing-in. Around the knees flexion contractures tend to develop due to hamstring spasticity.'~ It should be realized that the position of any large joint in the leg influences the alignment of others, as well as the posture of pelvis and of the trunk. Consequently, an initial single deformitywill eventually lead to pathologic postures or contractures of a number ofjoints and body parts. An abnormal posture and gait require higher energy consumption; therefore, they are more tiring and decrease the endurance for ambulation.

ATHETOID CEREBRAL PALSY.--In the past athetoid cerebral palsy was usually the consequence of isoimmunization of the 21

newborn. When this occurs resulting lesions are discrete, and are confined to the basal ganglia, the eighth and other cranial nerve nuclei. The characteristic clinical syndrome is athetosis, dysarthria, high frequency hearing loss or deafness and conjugate upward gaze palsy2 Intelligence is often preserved and seizures are rare. Since exchange transfusion became a routine practice the relative frequency of this athetoid cerebral palsy has declined. However, perinatal anoxia can also cause athetoid cerebral palsy either directly or by predisposition to bilirubin encephalopathy.7~, 72 In these cases a more diffuse brain damage is more likely to occur with other manifestations of cerebral dysfunction, e.g., mental retardation, rarely seen in primary bilirubin encephalopathy. A newborn with kernicterus shows increased tone and opisthotonic posture. 7,9 These initial signs are followed by a stage of hypotonia at about 2 - 3 months of age. Hypotonia is also an early

Fig 1 3 . - O n e and one-half-yearold child with athetoid cerebral palsy. Involuntary movements of hands and feet.

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symptom of athetoid cerebral palsy of other etiology. It is usually more marked and prolonged as a prodromal stage of athetosis than it is of spasticity. 6, 7 Persistence of Moro and tonic neck reflexes is frequent in this clinical type. 6, ~, 48, 73 Athetoid movements make their appearance around 18 months or sometimes as late as 3 years. They are accompanied by increasing tone that shows rhythmic fluctuation. At first, writhing involuntary movements are most evident in small muscle groups and around distal joints (Fig 13). Later they involve whole extremities, particularly in severe cases. The involuntary movements tend to change in their quality as time goes on, resembling dystonic, choreiform or other types ofdyskinesia and, essentially, it is only the speed and distribution that distinguishes these extraneous movements (Fig 14). Intensity of involuntary movements increases under emotional stress and around adolescence. Face and oropharyngeal musculature are affected, resulting in drooling and dysarthria. Unlike most spastic types of cerebral palsy, in athetoids it is not unusual to find a greater impairment of the upper extremi-

Fig 14.-Fourteen-year-old child with dystonic-athetoid cerebral palsy. Dystonic trunk sway. Right hand stabilized against the hip to control extraneous movements.

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ties. Deformities rarely develop. In those exceptional instances when they do occur, it is in adolescents who have dystonia as predominant dyskinesia causing more stationary postures. Otherwise, the continuous uncontrollable movements seem to favor the maintenance of joint mobility. A T A X I C CEREBRALPALSY--Relative frequency of ataxic cerebral palsy varies greatly in different clinical surveys reflecting a rather prevalent disagreement among clinicians regarding the nature and diagnosis of this clinical type. ~s Both hypotonia and motor delay are usually severe. 74 Tone remains decreased throughout life. Cerebellar coordination tends to improve as the children get older and nearly all walk eventually, a comforting thought for all concerned in view of the very slow development these children exhibit in the first 3 - 5 years of life. All ataxic cases should be watched very carefully since some prove to be a progressive disease rather than a static lesion. If other neurologic signs appear eventually, especially spasticity, the diagnosis of cerebral palsy is definitely untenable. In some instances these changes may occur as late as in the teens.

MIXED TYPES.-- Among the mixed clinical types a combination of spasticity and athetosis or rigidity most often are encountered.6. 7, ~5 The distribution of neurologic signs is quadriparetic and many of these children are severely disabled. As a rule when spasticity is associated with athetosis musculoskeletal complications develop later on account of involuntary movements. When they appear they are similar to and as serious as those described in spastic quadriparesis. A combination of spasticity and ataxia is seen in some instances after traumatic postnatal head injuries.l~, 16In most large pediatri~ rehabilitation clinics one also sees occasional cases with these neurologic signs of very early onset. One would be inclined to make the diagnosis of cerebral palsy when there is also a history suggestive of cerebral anoxia. In our experience these children, almost without exception, have a degenerative neurologic disease although deterioration may not be evident for many years. When the etiology of spasticity and ataxia is an apparently arrested hydrocephalus, signs of possible progression must be looked for continuously. RIGID, ATONIC AND TREMOR TYPES. - - T h e remaining three clinical types are rare. Both the rigid and atonic types have very poor prognosis 7, 7G Rigidity is also preceded by hypotonia which is usually s e v e r e and long lasting. Markedly decreased tone is a permanent sign in the atonic type to an extent that the child can be moved like a rag doll. '7 Deformities occur in both types though the mechanism is different in each. Lack of active movements contributes in both types. However, in rigidity, deformities are a consequence of increased tone whereas in the atonic 24

type position of contractures is determined by the effect of gravitational forces in the absence of tonic support (see Fig 4). Cerebral palsy with tremor as the leading clinical sign is a rarity. Unlike in the ataxic type, fine or coarse tremor is seen at rest and on m o v e m e n t s ) The outcome of motor accomplishments is favorable and musculoskeletal complications do not occur. COURSE AND PROGNOSIS OF MOTOR F U N C T I O N . - It is evident from this review of the natural course of the motor deficit in different types that cerebral palsy is a static condition only in terms of the central nervous system lesions. Clinical manifestations undergo considerable changes over the long years of growth and development. In the first few years of life these changes are reflected by alterations in neurologic signs as maturation of the damaged neuromotor system proceeds. At some point in time the remaining inherent developmental potential begins to surface and acts as a positive force toward new functional achievements. It is also part of the natural course that once definitive neurologic signs have appeared they may increase in their intensity for some time or they m a y be overcome to some degree if there is a sufficient maturational reserve. Changing clinical manifestations at a later age are essentially related to the musculoskeletal sequelae of a long-standing neurologic abnormality and are particularly likely to appear in spasticity. Unless properly attended, these complications can diminish the favorable effect of natural development. Difficulties of early recognition and early identification of clinical types have already been discussed. There is also considerable uncertainty surrounding the issues of early prognostication. Unfortunately, there is no simple or absolutely reliable method or sign to determine in many cases what residual maturational potential is left with the brain injury. Therefore, it is not an easy task to predict on what level of function the positive influence of development and negative effect of the cerebral insult will arrive at an ultimate balance. Prognostication in the two extreme situations, very mild or profound impairment, should present no problem. However, elimination of these cases still leaves a large group of children in need of more objective prognostic indicators. A particular concern for parents and physicians is whether or not a child would walk. A number of guidelines were found useful in this respect, one being the clinical type of cerebral palsy. 63' 77 All hemiplegic children walk and a favorable prognosis can be expected in the ataxic type. 6 Those with rigidity and atonia, on the other h a n d , d o not attain ambulationT" ~6 The outlook is more variable in diplegia, quadriparesis, spastic-athetosis and athetosis. In these clinical types accomplishment of sitting by 2 years has a nearly absolute correlation with later ambulation, while lack of sitting by 4 years excludes the potential for walking with the same degree of reliability. 6' 63 However, only about half of

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Pediatric rehabilitation. Part 1: cerebral palsy and spinal cord injuries.

PEDIATRIC REHABILITATION PART I: CEREBRAL PALSY AND SPINAL CORD INJURIES GABRIELLA E. MOLNAR LAWRENOE T. TAFT NOTE: Part II will appear as the Febru...
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