Cerebral palsy

Barry S. Russman, M.D., received his undergraduate degree from Harvard University in 1959 and graduated from Tufts Medical School in 1963. The pediatric internship and residency were served at Strong Memorial Hospital from 1963 to 1965, followed by a second year of pediatric residency training (PL-3) at Children’s Hospital of Philadelphia. After two years in the Air Force as a general pediatrician, he received his neurological training from 1968 to 1971 at the University of Pennsylvania Hospital and Children’s Hospital of Philadelphia. He has been Chief of the pediatric neurology servke at Newington ChildrenS Hospital since 1971 and is Professor of Pediatrics and Neurology at the University of Connecticut Health Center and Chief of the Division of Neurology at the University of Connecticut Medical School. Dr. Russman’s interests have been in the evaluation and care of the child with cerebral palsy, muscular dystrophy, learning disability, and the chronic epilepsies. He has served on the advisory council for the State Department of Education of Connecticut from 2973 to 1983. He has also served on the Committee on Children with Disabilities (COCD) for the American Academy of Pediatrics from 1982 to 1988. His current research interests include the use of transcutaneous stimulation and partial dorsal root rhizotomy for the child with spastic cerebral palsy. Further, he is involved with two projects under the auspices of the Muscular Dystrophy Association: studying the natural course of disease of children with spinal muscular atrophy and evaluating children with Duchenne’s muscular dystrophy. Curr

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James R. Gage, M.D., received his M.D. from Northwestern University Medical School in 1964. Following internship at Hennepin County General Hospital in Minneapolis, he spent two years in the navy as a general medical oficer. He then returned to Minneapolis for residency in orthopedic surgery. Following residency he held teaching appointments at Gillette Children’s Hospital and the Veterans Administration Hospital in Minneapolis. In 1976 he joined the full-time stafs of Newington ChildrenIs Hospital. Currently he is an Associate Professor of Orthopedics at the University of Connecticut and directs the Cerebral Palsy Service and the Kinesiology Laboratory at Newington Children’s Hospital. 70

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CEREBRAL

PALSY

Cerebral palsy is “characterized by aberrant control of movement or posture of a patient, appearing early in life (secondary to a central nervous system lesion, damage, or dysfunction), and not the result of a recognized progressive or degenerative brain disease.“’ In addition to motor deficits, the patient may suffer from other manifestations of cerebral dysfunction, including mental retardation, epilepsy, sensory deficits (hearing or visual loss), learning disabilities, and emotional problems. The diagnosis is established by a history that the motor disability is nonprogressive. Physical examination localizes the problem to the central nervous system and not to the motor unit (anterior horn cell, peripheral nerve, nerve-muscle junction, muscle). Efforts to establish an etiology should be made. Understanding the etiology, together with the specific type of cerebral palsy (spastic, dyskinetic, etc.1 can lead to a prognosis and rational treatment program. The pediatrician’s role is to provide primary health care, support the emotional needs of the family, and help with the coordination of the subspecialty treatment recommendations. Cerebral palsy often has been referred to as a “wastebasket” term. A complete understanding of the meaning and implication of the diagnosis will erase this attitude. HISTORICAL

REVIEW

The term cerebral palsy became prominent as a result of the work of Little, an English surgeon in the 1860s. He described a specific type of cerebral palsy, spastic diplegia, for many years referred to as Little’s disease (see section on Classification). In addition, he is responsible for suggesting that birth anoxia was the cause of cerebral palsy. In his 1897 classical text, Infantile Cerebral Palsy,” Sigmund Freud emphasized the existence of associated problems such as mental retardation, epilepsy, and visual disturbances. During the early 20th century, most cerebral palsy research and publications were concerned with treatment. The gamut of treatCur-r

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ment programs ranged from inhibiting movement with braces to facilitating it with various stimulation techniques.3 Much of the confusion and disagreement regarding treatment and physiology of cerebral palsy related to the lack of a classification system. Moreover, an acceptable classification system that would allow study of similar patients had not yet been developed. In 1956 a classification system finally was established and is still used.4 Further, epidemiologic studies were undertaken in Sweden, Western Australia, and the United States.12”-11 A major study, developed in the United States, the National Collaborative Perinatal Project (NCPP), deserves special mention as it is the only prospective one. Twelve hospitals in the United States enrolled over 50,000 pregnant mothers the first time they presented themselves for prenatal care. Each mother’s pregnancy, labor, and delivery were monitored using a carefully designed protocol. The child was evaluated in the delivery room, at 1 day of age, 3 days of age, and at periodic intervals until 7 years of age. Included in these evaluations were developmental, neurologic, and psychological examinations. Although the last enrolled child reached the age of 7 in 1973, publication of data is still ongoing. Much of the information presented in the following sections of this monograph will be summaries of the studies from the NCPP, as well as from the Swedish and Western Australian reports. CLASSIFIkATION

The current classification system was developed in 1956 by the American Academy of Cerebral Palsy,4 (Table 1). The system is a clinical one based on the physiology of the motor dysfunction and the number of limbs involved. The system establishes an orderly approach to describing a patient’s disability. However, it does not afford insight into the etiology or pathology of the problem. A complete description of the patient’s condition should include: (11 etiology or the risk factors if they can be identified (Tables 2 and 3); (2) associated problems (Table 4); and (3) functional capacity (Table 5). PHYSIOLOGIC

(MOTOR

GROUPING)

The classification presented (see Table 1) groups types of cerebral palsy by their physiologic and anatomical characteristics. Spasticity is defined as increased stretch reflex determined by passively flexing and extending muscle groups across a joint. A satisfactory, reproducible system of grading muscle tone has never been developed. Most physicians describe the tone as being normal, increased, or 72

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TABLE

1.

Cerebral

Palsy

Classification-Motor

7 Topographic*

Spastic Diplegia-legs > arms Quadriplegia-all 4 extremities, equally involved Hemiplegia-one-sided involvement, usually arm Double hemiplegia-arms involved Dyskinetic Hwyperkinetic or choreoathetoid

> leg

> legs

Dystonic Ataxic Mixed *Adapted diatrics

fmm

Minear

1956; l&841.

WL: A classification Used by permission.

of cerebral

p&y.

Pe-

decreased. Associated with spasticity are enhanced deep tendon reflexes, usually associated with clonus and extensor plantar responses. However, the latter are sometimes difficult to elicit in the infant and even in the older child with spastic cerebral palsy. Dyskinesias are defined as abnormal motor movements that are most obvious when the patient initiates a movement. The motor patterns and posture of patients with dyskinesias are secondary to inadequate regulation of muscle tone and coordination.” When the patient is totally relaxed, usually in the supine position, a full range of motion and diminished muscle tone are found. Dyskinetic patients are subdivided into two subgroups: the hyperkinetic or choreoathetoid children have purposeless, often massive involuntary movements with motor overflow, that is, the initiation of a movement of

TABLE

2.

Possible

Etiologies

of Cerebral

Palsy*

Prenatal Genetic syndromes Congenital malformations In utero infections Perinatal Meningitis ? Apgar ? Grade *Adapted cedents ysis of adapted GW, et weight modern tdogy

Cum

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score < 3 at 20 min IV intraventricular hemorrhage

from Nelson KB, Ellenberg JH: Anteof cerebral palsy: I. Multivariate analrisks. N Engl J Med 1986; 315:Sl. Also from Kitchen WIT, Doyle LW, Ford al: Cerebral palsy in very low birthinfants surviving to two years with p&natal intensive care. Am J Perina1987; 429.

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73

TABLE

3.

Possible Cerebral

Risk Factors Palsv*

Associated

With

Prenatal Maternal mental retardation Maternal epilepsy Maternal hyperthyroidism Severe toxemia Incompetent cervix Third-trimester bleeding Perinatal Vaginal bleeding at time of admission Placental complications iprevia, abruptio) Breech presentation Low birth weight Apgar <3 at 10 min *Adapted from Nelson KB, Ellenberg JH: Antecedents of cerebral palsy: I. Univariate analysis of risks. Am J Dis Child 1985; 139:1031. Also adapted from Nelson KB, Ellenberg JH: Antecedents of cerebral palsy: Multivariate analysis of risk. N Engl J Med

1986;

315:Sl.

one extremity leads to movement of other muscle groups. Patients in the dystonic group have abnormal shifts of general muscle tone induced by movement. Typically, these children assume and retain abnormal and distorted posture in the same stereotyped patterns. Both types of dyskinesias may occur either together or independently in the same patient. Dystonia may be confused with spasticity, inasmuch as passive range of motion in both situations may be difficult. Examining the patient in the supine position commonly will not alter the muscle tone in the spastic patient, but usually will reveal low tone in the patient with dystonia. It is important to distin-

TABLE 4. Incidence in Specific

of Mental Retardation and Tvpes of Cerebral Palsy’ IQ < 50 I%1

Dyskinesia Diplegia Hemiplegia Quadriplegia

Seizure

Epilepsy Disorder (9%)

30

27

33

31

39

67

64

56

*From Gibbs FA, Gibbs EL, Perlstein MA, et al: Electrw encephalographic and clinical aspects of cerebral palsy. Pediatrics 1963; 3~73-84. Used by permission.

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TABLE Severity

5. of Cerebral Gross

Mild Moderate

Severe

Palsy* Motor

Independent walker Crawl or supported walk No locomotion

Fine

Motor

Unlimited function Limited function No function

Comitive >70 50-70

<50

(IQ)

Speech

Overall

>2 words

Independent function Needs assistance

Single

words

Severely impaired

Total

care

‘Adapted fmm Minear WL: A classification of cerebral palsy. Pediatrics 1956; 18:841. Also adapted from Veelken N, Hagberg B, Bagberg G, et al: Diplegia cerebral palsy in Swedish term and preterm children: Differences in reduced optimal&y relations to neurology and pathogenetic factors. Neuropediatrics 1983; 14:ZO.

guish the specific physiologic abnormality as far as possible because the etioloa, treatment, associated problems, and prognosis are different. (See sections on Diagnosis and Treatment.1 Ata)tic patients have a disturbance of the coordination of voluntary movements due to muscle dyssynergia. They commonly walk with a wide-based gait and have a mild intention tremor (dysmetria). A subgroup in the ataxia category is called the dysequilibrium syndr0me.l’ These children have dysmetria and a pronounced difficulty in maintaining posture and equilibrium. They tend to be hypotonic for the first several years of life prior to the development of useful functions such as walking and using small objects, such as blocks, spoons, etc. Mental retardation is almost invariable in this group. Children placed in this category should be considered to have an autosomal recessive disease. Attempts to delineate this syndrome further, including lysosomal enzyme studies, etc., have been unsuccessful. Preferably, the mildest form of ataxia would be labeled apra)tia, rather than ataxic cerebral palsy.‘” Children in this category have normal coordination by a standard neurologic examination but have an impairment in the ability to carry out purposeful gross motor acts such as hopping and skipping, as well as fine motor acts (cutting, buttoning, and writing). If the treatment program will not include orthopedic care, and if the motor limitations are minimal, it is preferable to label the child as being apraxic rather than as having cerebral palsy. The mixed group is the fourth category in the physiologic or motor classification system. Patients in this category commonly have mild spastic@, dystonia, and/or athetoid movements. Ataxia may also be a component of the motor dysfunction in patients placed in this group. Curr

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ANATOMKX

GROUPING

Diplegia refers to involvement of all four extremities, but the legs are more dysfunctional than the arms. Quacfriplegia refers to dysfunction of all four extremities; in some children one upper extremity might be less involved; the term triplegia then would be substituted. Hemiplegia refers to individuals with unilateral motor dysfunction, and in most children the upper extremity is more severely involved than the lower. Finally, an unusual situation may occur in which the upper extremities are much more involved than the lower; the term double hemiplegia is affixed to this group. Although this classification system is widely used, there are many problems with it. Blair and Stanley showed that the interobserver diagnostic agreement did not exceed 55% even after training in the use of the classification system.‘” Improved diagnostic precision is necessary if future studies investigating etiology, treatment, and prognosis are to be compared among various researchers studying children with cerebral palsy. PREVALENCE,

RISK

FACTORS,

CAUSES

Epidemiologic studies of the cerebral palsy population are important for the following reasons: (1) Knowing the prevalence of disabil-

ities in the population

at any one time makes it possible

to plan

programs and facilities. (2) Different types of cerebral palsy can be studied with respect to risk factors. This information allows for the development of hypotheses regarding the etiology of cerebral palsy, which then can be tested. (3) Prospective studies of children born with cerebral palsy can shed light on the benefits of or problems created by the ability to provide sophisticated care to the very sick newborn.’ However, the published epidemiologic studies on cerebral palsy must be reviewed critically. Was the study performed prospectively or retrospectively? Was a total population studied or just the

children

at a specific

hospital?

The years studied

must be consid-

ered as medical technology changes rapidly. (4) Finally, the criteria used for and the process of making the diagnosis must be described and understood.

Table 6 summarizes

the incidence

of cerebral

palsy by physiologic

and anatomical categories found in the NCPP. From a population of approximately 38,000 who completed all evaluations through the age of 7 years, 202 children had cerebral palsy of at least a mild degree (5 per 1,000 of those who completed the study; 3 per 1,000 who entered the study); 32% had diplegia; 29% had hemiplegia; 24% had quadriplegia; and 14% had either dyskinesia or ataxia.15 More than half the children with cerebral palsy in the NCPP population were born at term. Sixty-three percent of them were appropriate by 76

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weight for gestational age, and 15% were small for gestational age (Table 71.‘” Although only 10% of the children with cerebral palsy weighed less than 1,500 gm at birth, the risk of developing cerebral palsy in this group was found to be extremely high (90 per 1,000). This compared to 3 per 1,000 children who were born appropriate for gestational age and weighed more than 2,500 gm. Twelve percent developed cerebral palsy as a result of a postnatal event (Table 8). The Swedish and Western Australian studies compared the incidence of cerebral palsy over different time periods, revealing some interesting trends. The Swedish studies show that the incidence from 1959 through 1968 was 1.88 per 1,000 live births.“,” The number then fell from 1967 through 1970 to 1.44 per 1,000 births, and in the most recent analysis, from 1975 through 1977, the incidence rose to

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TABLE

8.

Incidence of Cerebral by Gestational Age’ Term birth Pretenn birth Postnatal onset

Palsy

60%-65% 35%40% 12%

‘From Ekrlherg JH, Nelson KH: Birth weight and gestational age in children with cerebral palsy 01 seizure disorder. Am J Uis Child 1978; 1333:1044. Used by permission.

1.63 per 1,000 live births. An increase in the number of children with cerebral palsy born prematurely as well as those born at term with dyskinetic cerebral palsy seems to account for the recent change. In England Pharoah et al. compiled all cases of children with cerebral palsy from the years 1966 through 1977.l” Of the 68.5 cases that were identified, 27% were spastic hemiplegia; 21% were diplegic; 27% were quadriplegic; 4% were dyskinetic/ataxic; the remainder had a mixed pattern. The prevalence ranged between 1.18 to I.97 per 1,000 births. A significant upward trend in prevalence was noted in the group with low birth weights. The authors questioned whether this increase could be attributed to the increased survival of infants in neonatal intensive care units. An alternate explanation is the improvement in the completeness of data collection. In order to determine whether the increased survival ability of infants is a result of the care provided by modern neonatal intensive care units, follow-up studies of infants with low birth weights among geographically delineated groups are needed. This will avoid the bias associated with selective admission to hospitals with neonatal intensive care units. In summary, some studies suggest that the incidence of cerebral palsy may be rising and the greatest increase appears to be in the population of children who were born prematurely. Detailed epidemiologic data, collected prospectively, are necessary to confirm these impressions. These data not only will provide insight into the changing incidence and prevalence of the disease, but also they should provide an understanding of the causation of cerebral palsy. Finally, the data will help to identify prenatal, perinatal, or postnatal events that may be used to predict whether an infant is likely to develop cerebral palsy. It must be emphasized that these events are termed risk j&ctors. They should not be identified as causative factors. Since the initial description of cerebral palsy by Little, the dogma has persisted that labor and delivery complications are the main 78

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causes of cerebral palsy. This dogma has had a, serious impact on the medical-legal climate. The NCPP was developed to study this issue in depth. It was anticipated that once these “complications” had been identified, corrective measures could be introduced that, hopefully, would prevent the development of cerebral palsy in future populations. The study, in fact, identified very few specij?c causes. Several risk factors were identified, but most significant and surprising were the conclusions that many of the heretofore suspected “causes” or “risk” factors did not correlate with the future development of cerebral palsy. A univariate analysis of risks associated with the future development of cerebral palsy identified separate maternal, labor, and delivery characteristics.17 Such maternal factors as level of maternal education, marital status, parity, paternal age, pregnancy spacing, smoking history, and intercourse frequency were no? associated with an increased risk of the child developing cerebral palsy. Unexpectedly, a history of maternal diabetes and the length of time to become pregnant also were not predictive of future cerebral palsy. On the other hand, in this particular analysis, maternal mental retardation, epilepsy, and hyperthyroidism prior to the pregnancy were significantly associated with the development of cerebral palsy in the child. Pregnancy problems that were identified as relative risk factors associated with future cerebral palsy included severe toxemia and incompetent cervix, when associated with premature birth. Third trimester bleeding, but not first or second trimester bleeding, was also a significant factor. Kidney and bladder infections, radiation exposure, and hyperemesis gravidarum were not associated with increased risk. Risk factors identified during the labor and delivery periods included vaginal bleeding at the time of admission and placental complications such as abruptio, premature rupture of the membranes, chorionitis, and breech presentation. However, many of these risk factors were significant only in a baby weighing less than 2,500 gm at birth. In addition, some of the risk factors, such as oxytocin augmentation, cord prolapse, or breech delivery, were relevant only if they were associated with low Apgar scores. A multivariate analysis of the same data on prenatal and perinatal risk factors provided even further insight into the correlation of predelivery problems and the development of future cerebral palsy.” Maternal mental retardation and severe proteinuria occurring late in pregnancy were definitely correlated with the development of future cerebral palsy. Breech position but not breech delivery was also associated significantly with future cerebral palsy. Most importantly, these analyses concluded that children of most mothers with risk factors did not, in fact, develop cerebral palsy. Curr

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Also, although the single most significant risk factor for the development of cerebral palsy was low birth weight, most infants with low birth weights did not develop cerebral palsy. As noted in the introduction to this section, the NCPP study was designed to provide answers about the causation(s) of cerebral palsy. The expectation of jkding a strong correlation with perinatal asphy)tia and complications during labor and delivery yielded a strong negative answer. In the study 202 children were identified as having cerebral palsy at age 7; only 40 had at least one clinical marker of asphyxia such as a low Apgar score or having more than 5 minutes pass prior to taking the first breath spontaneously.19 Of these 40, only 17 developed cerebral palsy. Further, only 20% of children with Apgar scores less than 3 at 5 minutes developed cerebral palsy. It was also found that obstetric complications could not be considered a cause of the brain damage if the 5-minute Apgar score was more than 7.‘” Anoxia secondary to birth trauma was not found to be a common cause or even a common risk factor in most cases of cerebral palsy. Kitchen et al.” described the outcome of children who weighed between 500 and 1,500 gm at birth. They found that cerebral palsy developed in 52 (7.7%) of 675 live-borns at 2 years of age.“’ Of these children with cerebral palsy, 70% had one or more of the commonly recognized perinatal risk factors, such as low birth weight, low Apgar score, etc. However, the identical rate of risk factors was present in the infants with very low birth weights who did not have cerebral palsy. Finally, intraventricular hemorrhage correlated very poorly with development of future cerebral palsy. The Swedish studies were performed retrospectively and, therefore, must be interpreted with caution.“-” The data, nevertheless, corroborate the findings of the NCPP study and the Western Australian studies. Of the babies with low birth weights, 6% had obvious prenatal causes of cerebral palsy including cerebral malformations, maternal disorders, and placental infarctions. Twenty-three percent of the infants with low birth weights had combined prenatal and perinatal risk factors including asphyxia, cerebral hemorrhage, and hyperbilirubinemia. Fifty-one percent had perinatal risk factors alone. Of the infants born at term who went on to develop cerebral palsy, 24% had prenatal risk factors; 20% had both prenatal and perinatal risk factors; and 19% had perinatal risk factors alone. The Swedish studies also correlated the anatomical and physiologic abnormalities with prenatal and perinatal risk factors and looked as well at whether the child was born preterm or at term. The weights of children born with spastic diplegia were almost uniformlx appropriate for gestational age; however, 55% were born preterm.-- Further, there was a low proportion of prenatal risk factors among this group of infants. The diplegic children born at term had 80

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a much more complex situation, including a much higher frequency of both prenatal and perinatal risk factors. These included toxemia, placental infarction, and evidence of intrauterine asphyxia, including meconium staining. The dyskinetic syndromes have been carefully analyzed by the Swedish groups.5-7 Their analyses are based on retrospective data and, again, the conclusions from the analyses must take this into consideration. They related the cause of the dyskinesias to perinatal difficulties, including asphyxia. Their conclusions are based on the observation that the majority of children with dyskinetic cerebral palsy were born at term and yet had evidence of asphyxia at birth including delayed onset of respiration, meconium staining, etc. The relationship of hyperbilirubinemia to athetoid cerebral palsy is a well-established entity. As a result of measures taken to prevent hyperbilirubinemia, it has become a rare cause of dyskinetic cerebral palsy in recent years. Unfortunately, the NCPP did not examine this specific question prospectively. This would help resolve some of these issues. Recent studies reporting analyses similar to those of the NCPP have corroborated the NCPP’s findings. In a retrospective study, Stanley and English reviewed the risk factors for cerebral palsy in a population of infants weighing less than 2,000 gm at birth.“” Of the 512 children who were alive at 5 years of age, 4.1% had cerebral palsy. They found that the majority of children with low birth weights who developed cerebral palsy had perinatal profiles that were no different from children with low birth weights who did not suffer cerebral palsy, that is, the incidence of asphyxia, infection, and gestational age were similar between the two groups. Finally, children with certain genetic syndromes may present initially as having motor disabilities secondary to central nervous system dysfunctionsZJ (Table 9). Many of these entities may appear to be nonprogressive initially. It is obvious that these syndromes must be considered during the evaluation of a patient with motor difficulties. A review of the epidemiologic data regarding the risk factors that relate to future cerebral palsy leads to the following conclusions: 1. IJnder some conditions, it is possible to establish a specific etiology of cerebral palsy, namely genetic syndromes, congenital mal.formations, and in utero or perinatal central nervous s-vstem hfec-

tions. 2. As pointed out by Stanley and English, the main contribution from the NCPP study is a veiy important negative finding, that cerebral palsy s”yndromes overall are not “causally related to perinatal problems, particularly asphyxia.““’ It may well be that developmental malformations of the fetal brain are responsible for the perinatal Curr

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TABLE

9.

Genetic Syndromes That Could Be Confused With Cerebml Palsy (Childhood Motor Dvsfunctioni* Ataxia telangiectasia (Progressive by second decade] Behr’s Syndrome Dysequilibrium syndrome Familial spastic paraplegia (progressive by second to third Hereditary microcephaly Lesch-Nyhan Suyndrome Marinesco-SjGgren Suyndrome ‘Frorrl

Fisher

RL, Russman

decade)

BS: Genetic

dromes associated with cerebral p&y. thop 1974: 99:~. llsed by permission.

syn-

C/in Or-

and postnatal problems that, heretofore, have been ascribed to poor management of the labor and delivery. This concept, in fact, was suggested by Freud in his treatise on infantile cerebral palsy: “Since the abnormal process of birth frequently produces no effect, one cannot exclude the possibility that, despite Little’s anamnesis, diple@a might be of congenital origin. Dificult birth, in certain cases, is

merely a symptom of the fetus.“’

of deeper eficts

that influence

the development

3. There is no specific identifiable cause of most cerebral palsy syndromes. 4. Although several risk factors for the development of cerebral palsy have been clearly identified, the majority of children born with known risk factors will not develop cerebral palsy. DIAGNOSIS The child must have an obvious motor deficit in order for the diagnosis of cerebral palsy to be considered. The chief complaint usually is a concern that the child is not reaching motor milestones at the normal time. A careful history must establish that the child is not losing function, assuring that the patient does not have a progressive disease, such as a neurodegenerative process, spinal cord tumor, or other neurologic disorder. This history, combined with a neurologic examination establishing that the patient’s motor deficit is due to cerebral abnormality, leads to the diagnosis of cerebral palsy. Cerebral palsy is easily diagnosed in a child who is not developing motor skills, whose muscle tone is generally increased, and who is not regressing. However, the child who is not developing normally 82

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1989

and who has normal or decreased muscle tone presents a more difficult problem. Persistent primitive reflexes or the lack of development of the protective reflexes at the expected time are important findings of the neurologic examination, suggesting corticospinal tract impairment. For example, the Moro reflex should be unobtainable after 6 months of age (Fig 1). The asymmetric tonic neck response should never be obligatory when the patient is placed in the appropriate position, that is, the infant should “break” the tonic neck posture spontaneously after 15 to 30 seconds, and it should be unobtainable after 6 months of age (Fig 2). The side protective reflexes should be evident after 5 months of age and the parachute reflex is obtained after 10 months of agez5 (Figs 3 and 4). Hand preference is another important observation in the physical examination. A child should not cross the midline when reaching for an object until after I year of age and should not show clear hand preference until 18 to 24 months of age. The development of handedness prior to this time suggests a neurologic lesion such as hemiplegia (Figs 5 and 6) or a brachial plexus injury. The diagnosis of cerebral palsy in the hypotonic child can be difficult. Serial examinations may be necessary to confirm the diagnosis. If infantile reflexes have disappeared at the appropriate time, and if protective reflexes have developed normally, a motor unit dis-

FIG 1. Moro reflex. Test position: semireclined with head midline. Stimulus: allow head to drop 20 to 30 degrees. Response: abduction of arms with extension of the elbows, wrists, and fingers. Onset: at 28 to 32 weeks’ gestation. Disappears: at 4 to 6 months of age. Cut-r

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FIG 2. Asymmetrical tonic neck reflex. Test posItIon: supine with head midline. Stimulus: passive or active full neck rotation Response: Extension of arm and leg on face side. Onset, birth with stronger response In legs; response in arms strongest at 1 to 2 months of age. DISappears’ at 4 to 6 months of age.

ease such as spinal muscular atrophy or a congenital myopathy should be considered. In these instances performance of an electromyogram, nerve conduction velocity studies, and a muscle biopsy may be helpful. In addition to the maternal and perinatal risk factors that should arouse concerns about the diagnosis of cerebral palsy in a child, certain neonatal and postnatal problems have been identified as early warning signals. Neonatal findings established by the NCPP as predictors of future cerebral palsy include Apgar scores-especially 84

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1989

at 5 minutes-of less than 3, neonatal seizures, and apneic episodes.26 It is important to reiterate that only 20% of children who had Apgar scores of less than 3 and who survived to age i’ had cerebral palsy.27 In addition to the neonatal problems, several neurologic findings on the fourth month examination were found to correlate with the future diagnosis of cerebral palsy. These included abnormalities of motor behavior, muscle tone, motor milestones, and movement. The most reliable sign was increased muscle tone of the neck, arms, or legs. If muscle tone was normal at 4 months of age,

FIG 3. Side protective response. Test posltion: place child in sitting posltion, with legs extended and hands free. Stimulus: a sudden lateral shift of trunk. Response: arms extend to side to prevent fall. Onset, at 5 to 6 months of age. Disappears. persists throughout ilfe Gwr

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85

FIG 4. Parachute reflex. Test position: support Infant in a vertical positlon. Stimulus: sudden of upper body downward to flat surface. Response: arms extend forward to prevent Onset: at 7 to 10 months of age. Dtsappears: persists throughout life.

tip fall.

even if results of the neurologic examination were considered to be mildly abnormal, the risk for future cerebral palsy was minimal. Some children with cerebral palsy will appear to lose motor function, especially during the early second decade of life. This usually relates to the development of contractures, excessive weight gain (resuiting in increased bulk on a weakened skeletal frame), or lack of motivation. The latter is especially noted in those patients who are barely able to ambulate. Finally, a hypotonic child may develop spasticity or a movement disorder. This change in muscle tone may lead to pseudoregression. The “loss” of function will be secondary to this 86

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1989

problem and not the result of a progressive cerebral disorder. A diagnosis of pseudoregression should not be made, on the other hand, until appropriate studies have been obtained, ensuring that a progressive disease has not been missed. Ancillary neurodiagnostic evaluations (such as imaging studies, x-ray films, electroencephalograms) are not necessary to confirm the diagnosis of cerebral palsy, which is a clinical one. However, they can be helpful to establish an etiology and/or suggest a prognosis. Several studies have demonstrated a correlation between the severity of the anatomical abnormality as determined by a computed tomography (CT) scan and the extent of the motor disability, cognitive deficits, and the presence of epilepsy.“x-30 Pederson et al. found abnormalities in 67% of patients with spastic diplegia, namely, cortical atrophy.” Cohen and Duffrrer, in their review of CT findings in 52

FIG 5. A 2-year-old Curr

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child Pediatr,

who

demonstrated

February

1989

clear

hand

preference

prior

to age

1 year 87

FIG 6. The child shown in Figure 5 who subsequently hand This was noted only while the child was

developed performing

dyston~c a task.

posturing

with the left

hemiplegic patients, noted a correlation between size of the cortical atrophy or porencephalic cysts with retardation and the presence of seizures.2Y A recent personal experience also demonstrated the value of imaging studies. During the course of a cerebellar stimulator study, the CT scan revealed hypotrophy of the cerebellum in a patient who subsequently was rejected as a candidate for this procedure.“’ Upon being informed of the rejection, the parents expressed relief rather than disappointment. For 14 years they had believed that dropping of the infant at 5 days of age, even though there was no change in his behavior and no loss of consciousness, was the etiologic explanation for cerebral palsy in their child. Imaging studies should not be ordered routinely. Specific questions about a particular patient’s problem should dictate the ordering of such studies. The cost/benefit ratio of the study should be considered. 8s

Curr

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1989

Ultrasonography is an imaging procedure that can be utilized in children whose anterior fontanelle is still open. The results of this procedure record on film anatomical structures including ventricular size and cysts. Periventricular cysts that are usually preceded by echodense lesions, when larger than 3 mm, are predictive of future cerebral palsy.“” In fact, these cysts are more predictive of future cerebral palsy than grade I or II intraventricular hemorrhages without ventricular dilatation.“” Another type of laboratory procedure that has recently become available to those involved with the care of children with cerebral palsy is gait analysis. Since this procedure is used as part of a treatment plan, its use will be discussed in the treatment section of this monograph. The obtaining of studies during the initial evaluation, as well as the ongoing care of the child with cerebral palsy, should be dictated by specific questions whose answers will enhance care. The cost/ benefit ratios of all studies should be carefully considered.

ASSOCIATED

PROBLEMS

An electroencephalogram should be ordered when a seizure disorder is suspected. The presence of “epileptic discharges” will help to confirm a clinical suspicion of epilepsy. On the other hand, the presence of “epileptic discharges” alone does not make a diagnosis of epilepsy; the clinician makes that diagnosis by observing an episode or obtaining a history consistent with a diagnosis of epilepsy. The presence of one brain dysfunction should alert the examiner to search for manifestations of other central nervous system abnormalities. Epilepsy, mental retardation, learning disabilities, vision difficulties, strabismus, dysarthria, and hearing loss occur with higher frequency in the cerebral palsy population compared to control groups.34 The incidence of mental retardation and epilepsy seen in the specific types of cerebral palsy has been summarized (see Table 4J.“” This does not mandate, however, that every child with cerebral palsy must see an ophthalmologist, audiologist, or have an electroencephalogram or a psychological assessment. Hemiplegic Cerebral Palsy-Epilepsy occurs more frequently in this group of patients than in those with spastic diplegia or quadriplegia, probably because of the focal brain insult. Usually, seizures develop in the first 2 years of life.‘!’ However, the electroencephalographic abnormalities have little predictive value for cognitive development. Levine et al. reviewed CT scans and electroencephalographic readings and correlated these findings with intellectual ability in 41 hemiplegic patients.“” The size of the lesion, independent of location, correlated with lowered IQ. Patients with congenital Curr

PrnblPediatr,

February1989

I39

lesions performed at a higher level verbally than those with acquired lesions, refuting tenets about brain plasticity. In addition to epilepsy, patients with hemiplegic cerebral palsy experience other problems that are unique. Homonymous hemianopsia is found frequently in this group of patients. Preferential classroom seating should be considered for these children. For example, a child with a right homonymous hemianopsia should sit on the right side of the room so the normal left peripheral vision can be used. Hemiplegic children may have a major stereognostic deficit in addition to the motor disability. The prognosis for useful function of the involved hand relates more to this sensory abnormality than to spastic@. A movement disorder, such as dystonic/athetoid posturing, may also limit hand use. Asymmetry of hand growth in this group of patients is related not to disuse, but rather to the sensory loss (see Figs 5 and 6). To substantiate this point, it will be noted that the involved foot is invariably smaller than the normal foot and yet the patient is ambulatory, confirming that a “trophic” factor and not disuse is a more likely explanation for the size disparity.“7 Diplegia-Most

studies

have

noted

ate to severe retardation

in children

were

a more

born

at term

and

a higher

incidence

with diplegic

favorable

prognosis

of moder-

cerebral palsy who for

the

preterm

patient.38 The Western Australian studies challenge these statistics, presenting data that children with diplegia who weighed less than 1,500

gm

at birth

later

showed

a high

incidence

of moderate

to se-

vere retardation.” The NCPP analyses do not provide data regarding this issue. Obviously, this problem is of concern for many reasons, including counseling of parents and planning for facilities to provide appropriate

programs

Dyskinesia.-Seizures

for children are less

with common

cerebral

palsy.

in this

group

but

mental

retardation, especially in the dystonic cerebral palsies, occurs in about 50%. As previously noted, the dyskinetic population of cerebral palsy patients can be divided into the hyperkinetic (choreoathetosis) and rigid groups (dystonia). The pathology associated with this type of cerebral palsy presumably is subcortical (in the basal ganglia),

although

definitive

studies

are

lacking.

Nevertheless,

cho-

reoathetoid patients tend to have average to above-average intelligence. However, in addition to the abnormal limb movements, most of these patients suffer from severe dysarthrias. The children cannot express themselves verbally and they also have difficulty with the use of communication devices such as boards because of the motor deficits. With the development of modern technology, including the ability to trigger computers with nonlimb movements such as eyeblinking, communication will be more readily available for these so

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children in the future. In any case, it is important to keep in mind that these children are not usually retarded. The dystonic group of dyskinetic patients, on the other hand, frequently includes the patients who fall into the retarded range. Nevertheless, these patients also should be carefully monitored before assuming that the IQs are abnormally low. The associated problems in this particular group of patients have never been carefully analyzed. COMMON

HEALTH

PROBLEMS

There are few data regarding the occurrence of common health problems in children with cerebral palsy. For example, the frequency of pneumonias, urinary tract infections, and otitis media is unknown. On the other hand, three health problems have been studied in detail, namely drooling, nutrition, and incontinence. Drooling may be responsible for severe skin irritations, but of greater significance is its unpleasant cosmetic effect.3B4Z Most studies of drooling in cerebral palsy suggest that oral motor dysfunction rather than hypersalivation is the cause of the problem. Fluoroscopy studies show ineffective and inadequate swallowing mechanisms. Many programs have been tried over the years attempting to help the children with this dysfunction, but none is universally successful. Anticholinergic medication has been used; however, because of side effects, the patients usually reject this therapy after a time. Surgical intervention, including reposition of the salivary ducts and dividing the cordi tympani, has sometimes been effective. However, the operation is not always successful and undesirable side effects can occur, such as increased difficulty in swallowing food. During the last ten years, behavior modification programs to help the individual control drooling have been effective. Rapp developed a device that provided auditory cues when the child drooled excessively.39 These cues effectively helped the small children in the study group control their drooling up to 6 months after the auditory signal was taken away. Recently, Koheil et al. demonstrated that electromyography (EMG) biofeedback and behavior techniques were effective.40 The EMG auditory feedback provided the signal to the patient that he/she was having difficulty controlling oral motor function. Dunn and associates in a single case study design demonstrated that a patient could be taught self-control procedures with nonvocal positive reinforcement techniques.4’ The criteria for success in the three studies included: (1) a mental age of more than 2 or 3 years, despite the fact that chronological age was as great as age 16; (2) motivation to control drooling; and (3) an understanding by the patients that drooling was socially unacceptable. Cum

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Poor nutrition also may be a major problem.““,44 Several early studies cited poor weight and height gain in children who were severely spastic or athetoid. This poor growth has been thought to be associated with oral motor dysfunction. This raises several questions: how to provide adequate and appropriate nourishment; and, if nourishment is provided, will the malnourished cerebral palsy child gain weight and height? Finally, will the adequately nourished child be more “healthy”? Recent studies have shown that either tube feeding or gastrostomy feeding resulted in a significant increase in weight gain and, in some patients, a significant increase in height. Criteria for using this type of nutritional support have yet to be established. The adverse effects of poor nutrition on the natural course of disease of patients with cerebral palsy are poorly understood. For example, are these children more prone to infection and skin breakdown? Will improving the child’s nutritional status minimize or prevent further pneumonias? Further studies are needed to answer these questions. A third health problem commonly encountered in patients with cerebral palsy is bladder dysfunction. Unfortunately, the literature provides little information on the incidence of this problem. Nevertheless, in addition to one’s clinical experience, the few published studies suggest that the problem is significant. In a recent review of 50 patients with cerebral palsy iboth children and adults1 referred to a urologic service, 28% complained of enuresis; 26% complained of stress incominence; 18% complained of urgency; and 6% noted dribbling.“” More than 36% of the patients had more than one symptom. However, only 4 of 45 patients who underwent cystometrogram were noted to have a neurogenic bladder. Are the bladder difficulties related to lack of sphincter control? Are the problems more prevalent in patients who are retarded? Is it a more frequent problem for children whose primary deficit is spasticity or dyskinesia? Future studies are also needed in this area. TREATMENT GENERAL

TREATMENT

PRINCIPLES

Prior to discussing specific treatment programs, tant general principles should be stated.

some very impor-

1. Long-term treatment objectives must be defined, taking into consideration not only the patient’s motor deficits, but also his cognitive abilities, social skills, emotional status, vocational potential, and, most important, the availability of family support. Will the patient be able to accomplish his daily living needs? Will the patient be independent in all areas or just some? Will the patient need pub92

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lit or private transportation to reach his place of employment? Will leisure activities be accessible? These questions should be considered at all times as the treatment program is being developed. 2. The effects of the patient’s growth and development on his problem, with and without the proposed treatment, should be evaluated. 3. Valid alternatives that look at risk/benefit ratios and humane/ ethical dilemmas and that might include nontreatment should be considered. 4. The goals of a treatment program will vary with the individual’s age. The treatment of a child with cerebral palsy is best accomplished by a team of knowledgeable professionals with various specialties.“” The team will establish the diagnosis, eliminate progressive diseases, and, if possible, identify specific genetic syndromes. A typical team might consist of a physician trained in the evaluation and treatment of developmentally disabled children. A knowledgeable orthopedist should be the second physician-member of the team. This professional will monitor contractures, subluxed or dislocated hips, and scoliosis, deformities that can interfere with function as well as a comfortable lifestyle. Other members of the team, depending upon availability, usually include a physical therapist, occupational therapist, speech/language pathologist, clinical nurse specialist, social worker, educator, and psychologist. INFANCY AND

TODDLER AGE

The diagnosis of cerebral palsy in most patients is established during the first 2 years of life. At this time, the patient should become involved in a physical and/or occupational therapy program. Since cerebral palsy is not one disease, there are a variety of therapy programs, as might be anticipated. Earlier programs emphasized passive range of motion and use of braces to prevent contractures as well as to inhibit abnormal muscle function, a program developed by Phelps.” Once these objectives were met, it was expected that the normal muscles would provide the necessary functions for the child to attain his milestones. This approach has been unsuccessful as the assumptions have been shown to be incorrect. In the 1940s, Deaver promoted a program that emphasized functional abilities rather than movement patterns. Extensive bracing was utilized to prevent abnormal motor movements from interfering with function. At about the same time, Fay developed the prototype of what became known as the Doman-Delacato or “patterning” program. Several studies over the years and, most recently, a study by Sparrow and ZiglerP7 demonstrated that this approach to the treatment of cerebral palsy is Curr

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93

ineffective. The American Academy of Pediatrics has issued a position statement regarding this “treatment,” emphasizing the lack of validity.4X Other therapeutic approaches in vogue at that time included the providing of a sensory input, including stroking, icing, and heat, to promote a motor output, a program developed by Rood.3 In the late 1950s and 196Os, the Bobaths developed the neurodevelopmental treatment program (NDT) for this patient population4’ The methods utilized in this program attempt to inhibit abnormal infantile reflexes such as the tonic neck and Moro reflex and to promote, i.e., facilitate, more normal movement patterns such as the righting reflex. To date, there have been no well-designed studies showing the benefit of this approach. Published studies suggest that, for some patients, certain therapy programs may make a difference in the patient’s outcome. In a 1962 retrospective review, Paine noted that individuals receiving intensive physical therapy had fewer contractures compared with those who did not receive a therapy program, but that both treated and untreated patients required a similar number of surgical procedures.50 In 1973, Wright and Nicholson reported that treatment programs did not affect range of motion or influence the retention or loss of developmental reflexes.5’ On the other hand, some possible benefit was found for those children who had quadriparesis in the first year of life. Finally, Scherzer et al. found a strong trend toward improvement in motor status and social motivation after treatment among those children with cerebral palsy who had a normal IQ compared with findings in a control group.” Many studies of therapy programs, on the other hand, have reached negative conclusions regarding the benefit of such programs on the eventual motor outcome of the patient5” However, when reviewing the benefits of any program, more than the motor outcome must be considered. If a program offers only physical therapy with the implication that a child’s motor development will improve significantly, several questions should be raised about the value of such a program. Many authors have suggested that stimulation programs for the child with cerebral palsy have beneficial effects on the general well-being and social advancement of the child, as well as on the happiness of the patient and parents. These assumptions need to be validated. The psychological impact of rearing a disabled child can be devastating. This subject has been the focus of several studies. Recently, in a study addressing the issue of psychological stress in mothers whose children are disabled, Breslaw et al. concluded that the specific diagnosis did not cause as much stress as expected among mothers; however, the dependency of the disabled child on the mother to accomplish activities of daily living was significantly cor94

Curr

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1989

related with maternal stresss4 Specifically, the neurologically handicapped child, such as a child with cerebral palsy who needed a great deal of care, including feeding, toileting, dressing, and help with mobility, caused a great deal more distress to the mother in comparison with distress resulting from a child with an illness such as cystic fibrosis. A therapy program might be extremely helpful in these cases, not necessarily to stimulate development but rather to offer parents easier ways to work with their child. As discussed by Bax, if agreement is going to be developed as to what constitutes an appropriate therapy program, future studies will need:55 I. 2. 3. 4.

Clearly defined populations Recognizable pathology Identification of all problems, not just motoric Consideration of the child’s life situation

PRESCHOOL,

SCHOOL-AGED,

AND

ADOLESCENT

deficits

PATIENTS

As the child with cerebral palsy approaches school age, the goals of the therapy programs begin to shift from enhancing motor development and minimizing contractures toward helping the child cope with the expectations of the classroom. Sitting properly and moving about the environment (including the use of a wheelchair) are gross motor needs that may require therapy.56 Use of the small muscles for fine motor function such as writing, cutting, etc. may need to be enhanced. Most important is a therapy program to help the child communicate, either with speech or communication devices. Dressing, feeding, and toileting (activity of daily living [ADL]) skills are important needs that should be incorporated into the educational/ “treatment” program. The occupational therapist is usually the person to work with the patient toward these ends. Medical,

Neurosurgical,

and

Orthopedic

Zntervention

The major neuromuscular problems in cerebral palsy that might necessitate medical, neurosurgical, and/or orthopedic intervention are : 1. Loss of selective motor control and dependence on primitive reflex patterns for ambulation. 2. Abnormal muscle tone (spastic@ or dystonia) that is strongly influenced by body posture an&or position and/or movement. 3. Relative imbalance between muscle agonists and antagonists that, with time and growth, leads to fixed muscle contracture and bony deformity. 4. Impaired body balance mechanisms. Curr

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95

Since cerebral palsy is the result of a brain injury/dysfunction, several of these problems may not be treatable and others can only be partially remedied. Any treatment approach should be based on the functional abnormality of the patient. Once the treatment is completed, it should be critically evaluated so as to optimize the outcome and to prevent perpetuation of errors in future cases. The motor deficits can be analyzed in four very distinctive ways. 1. Loss of Selective Motor Control and Dependence on Primitive Reflex Patterns for Ambulation.-A remedy does not exist that can significantly alter selective motor loss, such as lack of control of lower extremity muscle, whether the problem is secondary to spasticity or dystonia. Physical and occupational therapy programs can provide help. As already discussed, various schools of therapy promote programs that superficially vary greatly but, nevertheless, have certain common principles. These include development of sequence learning, normalization of tone, training of normal movement patterns, inhibition of abnormal patterns, and prevention of deformity. Schools use different stimuli and facilitation techniques to accomplish this. In general, since a child learns motor control in a cephalocaudal direction, the therapist will work in the same way by first trying to establish trunk control and then working toward control of the lower extremities. The therapist should serve as a coach to the parents who carry out much of the actual treatment on a daily basis at home. Realistic expectations must be articulated firmly. Rather than cautiously attempting to correct a dysfunction that cannot be corrected, the therapist should help the patient develop compensation techniques, recognizing that the severity of the disability mitigates against the development of “normal” motor control. 2. Abnormal Muscle Tone ture and/or Position and/or

That

is Strongly

Injluenced

by Body

Pos-

Movement.-Since muscle stretch is the stimulus that produces muscle growth in a child, abnormal tone in certain muscle groups is likely to be the root cause of the relative imbalance between muscle agonists and antagonists that, with time and growth, leads to muscle contracture.“’ Surgical lengthening of muscles treats the effect but not the primary cause. Recently, Peacock and Arena have advocated selective, partial dorsal root rhizotomy as a method of deafferenting the muscle spindles and thereb-v obtaining a true reduction in muscle tone.“” This procedure is carried out on the dorsal roots of L-Z through S-l bilaterally and, according to Peacock and Arena, will produce a permanent reduction of tone without significant!y altering sensation or strength. If effective, this procedure will represent a major advance in the treatment of cerebral pa1s.v. Appropriate use of orthotics (braces, can modifi/ tone to some de-

36

Curr

ProbI

Pediatr,

February

1989

gree. For example, a hinged ankle-foot orthosis with a plantarflexion stop may be very useful to prevent excessive extensor thrust. In addition, it will control foot position throughout the gait cycle, which may also provide a significant reduction in extensor tone. Physical therapy also attempts to lower tone, mainly through positior?ing and/or facilitation techniques; however, the effects of facilitation therapy on tone are relatively transitory. Medication has also been used to alter muscle tone. The three that are used most often include: (1) Diazepam (Valium) appears to act on the central nervous system at the level of the limbic system, i.e., the thalamus and hypothalamus. It is also a mild sedative. (21 Baclofen (Lioresal) acts through the inhibition of monosynaptic and polysynaptic reflexes at the spinal cord level. It is an analog of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA) but does not appear to have any central action on spasticity although it can act as a central nervous system depressant.“” (31 Dantrolene (Dantrium) acts on skeletal muscle beyond the myoneural junction probably by inhibiting the release of calcium ions from the sarcoplasmic reticulum.“” Dantrolene can be hepatotoxic in selected individuals and liver function tests must be monitored at regular intervals when the drug is used. Although diazepam appears to work well to control acute postoperative spasm, none of the three drugs seems to be particularly beneficial in the long-term control of spastic@. Recently Barbeau et al. described the effect of cyproheptadine (Periactin) in patients with spinal cord injuries.“’ It appears that when the corticospinal tracts are interrupted, the serotonin receptors in the cord distal to the transection become hypersensitive. Cyproheptadine, in addition to being a mild antihistamine, is a serotonin blocker and when used chronically may significantly reduce spasticity in many of these patients. Furthermore, its side effects are minimal and are related to its action as an antihistamine. The major side effects are drowsiness and h-yperphagia. Studies using this medication in a cerebral palsy population have not been performed. The above medications are used to diminish spastic@ when it is believed that with such a reduction, the care of the child will be easier (perineal care for example), or the function of the child will improve (sitting more easily). Commonly, the physical or occupational therapists will make the request. In the mid-1970s, chronic cerebellar stimulation, accomplished by the surgical implantation of electrodes on the superior surface of the cerebellum, was proposed as a way of decreasing spasticity, which, in turn, would allow functional improvement in patients with spastic cerebral palsy.“2 A subsequent study, controlled for placebo effect, could not confirm the initial findings.3’ A recent publication suggests that this form of therapy can decrease spastic@ but not necessarily Curr

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Pediatr,

Fehruq

1989

97

improve function.63 A second nemosurgical procedure that has been proposed to alter either tone or movement disorders is stereotaxic thalamotomy.64 To date, this procedure has been effective in decreasing hemiparetic tremors only. Transcutaneous electrical stimulation of the scalp is another modality that has been proposed to reduce spastic@. A recent preliminary study found this to produce greater improvement in the patients’ motor function than had been predicted with physical therapy alone.65 Logan found improved function in a group of spastic cerebral palsy children using this technique.66 Whether transcutaneous electrical stimulation will become a part of available techniques in the treatment of the spastic cerebral palsy patient remains to be seen. 3. Imbalance Between Muscle Agonists and Antagonists.-Static contracture of muscle secondary to spasticity is a common problem for which surgical lengthening of the musculotendinous unit is frequently performed. Fixed muscle contractures are almost never seen in patients with pure dyskinesias; however, when they do occur, surgical intervention is considered-but with extreme caution. Given the absence of a constant kinematic baseline, the results of surgery are difficult to predict. Furthermore, if the agonist is surgically weakened in the dyskinetic patient, the result is often a postoperative deformity that favors the antagonist. For example, if the adductors are lengthened or released, a fixed abduction deformity of the hips is likely to occur postoperatively. Consequently, if at all possible, it is best to avoid surgery in athetoid patients. They are unlikely to benefit and are very likely to be worse off as a result of the intervention. As with most medical problems, prevention is preferable to remedy; specifically, it is much better to prevent fixed muscle contractures than to remedy them once they have occurred. In a child with spasticity, fixed muscle contractures come about with growth. A child whose bones are growing stretches his muscles daily during normal activities and thus maintains muscle growth in proportion to bone growth. The inability of the child with cerebral palsy to stretch spastic muscles adequately favors static muscle contracture of certain muscle groups. Anything that will normalize or, at least, reduce tone, will help to prevent contracture. Even if tone cannot be normalized (reduced), spastic muscles will still grow providing that they receive adequate stretch. Thus, a program to prevent muscle contracture should logically be designed that will reduce or normalize tone, apply adequate stretch to muscles that need to be elongated, and remove the stimulus of stretch from the muscles that need to be shortened. Unfortunately, this is easy to conceive but difficult to accomplish. 98

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Methods for normalizing tone have already been discussed: physical therapy programs, medication, partial dorsal root rhizotomy, cerebellum stimulation, transcutaneous stimulation. In general, a program of physical therapy should be started early in conjunction with a good home maintenance program. Parents should be taught to place their children in positions that prevent the deformities and favor recovery, e.g., prone lying to stretch the hip flexors and long sitting to elongate the hamstrings. Night-splinting is often effective, but it must not interfere with normal nocturnal movements or the child will not tolerate the splints. For example, an Ilfield abduction splint will provide stretch to the hip adductors while still allowing the hips to move in the sagittal and transverse planes. This splint can often be used in conjunction with ankle-foot orthoses that maintain the triceps surae on stretch but will still allow the child sufficient freedom to turn in bed. If hamstrings and heelcords are contracted, a knee immobilizer on one limb and an ankle-foot orthosis on the contralateral limb, reversing them every other night, can be effective. In that way the child is not overly encumbered by the splinting. Inhibition casts, as advocated by Sussman, are useful in controlling dynamic spastic&y and hence maintaining length of the posterior tibial musculature; however, the long-term benefit of their use has not been established.“’ Occasionally, as advocated by Bleck, alcohol “washes” (the injection of alcohol into the spastic muscles to decrease spastic@ for a short period of time) of contracted muscles can be used to regain adequate control of an early static deformity.“’ This will usually result in approximately 6 weeks of relative weakness of the treated muscle or muscle group and during this time an appropriate program of therapy, casting, or bracing can be carried out to regain muscle length without resorting to surgery. Surgical lengthening of tendons and/or muscles is probably the most effective way of restoring balance once static contracture of muscle has developed. Unfortunately, the lengthened muscle is also weakened. At the present time there is no way to strengthen the antagonist. In general, lengthening is not a problem with isometric muscles (stabilizers) since they are usually weak in spastic cerebral palsy and rarely require elongation. In most cases, eccentric muscles (decelerators and shock absorbers) can be lengthened without significant loss of function. However, lengthening is a major problem with concentric muscles (accelerators) since these muscles are necessary to initiate movement of the part on which they are acting. Since a hip flexion contracture is usually present in spastic cerebral palsy, the iliopsoas is a prime example of this dilemma. If lengthening of the musculotendinous unit is sufficient to correct the contracture, the iliopsoas is often weakened to the point that the patient has difficulty in initiating hip flexion. In this particular problem, perCurr

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1989

99

forming an intramuscular lengthening of the tendinous portion of the psoas and accepting some contracture of the iliacus in exchange for better muscle strength is recommended. Rang and associates and Bleck have both argued cogently that the overall result is much better if all contracted muscles are lengthened simultaneously rather than staging the procedures.57’“” Not only is morbidity lessened by accomplishing all surgery during the course of a single procedure, but also, by simultaneously balancing all major lower extremity joints, much better function is possible. Since many of the muscles that require lengthening are biarticulate, surgical lengthening of muscles to correct imbalance at any one joint is likely to cause imbalance at the joint above or below. For example, if the hamstrings are lengthened in a patient with knee flexion contractures, the result is likely to be better extension at the knee, but at a cost of increased flexion contracture at the hips. This occurs because the hamstrings, in addition to being knee flexors, are also hip extensors. Thus hamstring lengthening also lessens hip extensor power, and in the presence of spastic hip flexors, the hips will move into a more fixed flexion deformity. It is useful to think of the low back, hip, knee, and ankle as four weights on the corners of a suspended balance board. Unless weight is subtracted or added evenly at all four corners, the board will tip. Unfortunately, the more muscles that are lengthened at one time, the more likely the possibility of making a judgment error. Therefore, the muscle “imbalance must be precisely defined. In addition, primary abnormalities must be differentiated from adaptive or “coping” mechanisms. A simple example of this would be a circumduction gait in a child who does not have sufficient knee flexion for foot clearance during swing. This is a simple example, but the coping mechanisms can be extremely subtle. Unfortunately, if the surgeon focuses on the coping mechanism rather than on the primary abnormality, the patient will often be worsened by the procedure rather than helped. The differentiation between primary and secondary abnormalities often cannot be accomplished without d-vnamic gait analysis. GAIT ANALYSIS.-Gait analysis is a method by which the walking pattern of an individual is examined in detail (Fig 71. It is based on the gait cycle that is the basic unit of walking. The gait cycle begins at initial contact when the foot strikes the ground and ends when the same foot strikes the ground again. As such, it consists of a period of stance, when the foot is on the ground, and a period of swing, when the foot is off the ground. In order for normal walking to occur, more than 30 major muscles in each lower extremity must act precisely with respect to both timing and power during each gait cycle. 100

Curr

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1989

Heel strike

FIG 7. The normal gait cycle. Normal gait consists of a serves of repetitive cycles that begrn wrth heelstrike and end when the same heel strokes the ground again. It consrsts of two major periods-stance phase that constitutes 60% of the cycle and swing phase that constrtutes 40% During stance phase there are two periods of double support when both feet are In contact with the ground, each of which occupies approximately 10% of the cycle. The first period occurs just after heelstrike and the second just before toe off. During a gart cycle, more than 30 major muscles in each lower extremity have to turn on and off synchronously.

Computerized gait analysis has made rapid progress in recent years and presently several commercial systems are available. The modern gait analysis laboratory usually consists of three major measurement systems: motion analysis, force plate, and electromyography (Figs B-10). The software outputs of such a laboratory integrate these three measurement parameters to provide information regarding the specific abnormalities at each of the major joints of the lower extremity throughout the gait cycle as well as the electromyographic activity of the muscles controlling the joint. With the aid of this information, a much more precise definition of the gait abnormality is possible. Treatment can then be tailored specifically to the child’s abnormality.‘” Furthermore, more extensive treatment is possible at one time with less risk of error, e.g., surgical lengthening of all contracted musculature at hips, knees, and ankles bilaterally during the course of a single operation. Following convalescence, postoperative gait analysis allows accurate assessment outcome. 4. Impaired Body palsy will invariably In spastic diplegia child who only has ally be able to walk Cum

Probl

Pediatr,

February

Balance Mechanisms.-The child with cerebral have abnormalities of balance to some degree. posterior balance is affected most severely. A disturbances in posterior equilibrium will usuwithout the use of external aids. If anterior bal1989

101

merls Wtannel

1

FIG 8. Floor plan of a gait analysis laboratory. The modern gait analysis major measurement parameters: (1) dynamic electromyography these ftgures), (2) force plates to measure ground reaction forces, surements.

laboratory has (not demonstrated and (3) motion

three In mea-

ante is also affected, crutches will be necessary for ambulation. Children with deficiencies in lateral equilibrium will usually require a walker or, if the lateral equilibrium reactions are severely deficient, may be unable to walk independently. Again, the deficiencies in equilibrium are secondary to an irreparable neurologic lesion and 102

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1989

are lifelong. However, a good physical therapy program in early childhood may help the child to improve his equilibrium responses to some degree. If there is instability in the stance phase of gait, appropriate orthotics and/or surgery can be used to provide a stable plantigrade foot that will, in turn, have a beneficial overall effect on balance. Independent ambulation without crutches demands that the mass of the head and upper trunk be maintained over the base of support. When fixed contractures at the hips, knees, and/or ankles prevent this, surgical lengthening of contracted musculature may allow independent ambulation if the child’s equilibrium reactions are adequate. TREATMENT

PROTOCOL

FOR SPASTIC CEREBRAL PALSY

It is now possible to formulate a rational treatment protocol for the neuromuscular deficits, based on the pathophysiology of cerebral palsy. Even though each patient is unique and treatment must be individualized, the method of approach can be similar. There are five major tenets of treatment: I. Avoid surgery to improve ambulation until after the gait has matured. In the meantime, maximize function with appropriate orthotics, physical therapy, and a home maintenance program. Various modalities that alter muscle tone may be attempted, including med-

GAIT ANALYSIS

PROCEDURE

Camera 2 Q

FIG 9. Force Curr

plates Probl

to measure Pediatr,

ground

February

1989

reactlon

forces 103

FIG 10. Motion measurements (a series of computer cameras that track the X.Y,Z coordinates of up to 32 body markers at rates of from 30 to 200 times per second). Video cameras are usually set up alongside the motion cameras to provide a permanent record of the walk and to allow the operator to monitor each camera view.

ication, transcutaneous stimulation, and selective posterior rhizotomy. The latter two modalities should be considered experimental at this time. 2. When the gait is mature, usually sometime between the ages of 6 and 10, perform gait analysis. Utilize these data in conjunction with the clinical examination to determine an appropriate course of treatment. This is usually some combination of surgery and orthotits. 3. If surgery is elected, try to avoid staging. Lengthen and/or transfer all muscles necessary to obtain balance during the course of a single surgical procedure. If this is not possible, stage two procedures close together so that only one extended period of recovery is necessary. 104

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4. Following surgery, minimize casting and remobilize the patient rapidly. Maintain an active physical therapy program as long as the gait is improving (usually about 12 months). Prevent recurrence of contractures throughout the remaining years of growth with appropriate night-splinting and a good home maintenance program designed to stretch tight musculature adequately. 5. Reanalyze the patient’s gait once it has stabilized (usually 9 to 12 months postoperatively). Carefully and critically evaluate the treatment hypotheses and the treatment outcome. PROGNOSIS When the diagnosis of cerebral palsy is first established in a nonambulator, the first question asked is usually, “Will my child walk?” Criteria for predicting independent walking have been developed (Table 1O).71 If, by the age of 1, the patient still has persistent primitive reflexes and the protective reflexes have not developed, it is unlikely that the child will ever. ambulate independently. Further, if the child has severe dyskinesias or falls into the dysequilibrium category, ambulation will not be achieved. Even though cerebral palsy is a result of a nonprogressive central nervous system lesion, the child who is a marginal ambulator, upon entering the early teens, may lose walking ability because of contractures, excess weight gain, or lack of motivation. Those involved with the care of cerebral palsy patients must be alert to these potential problems and take preventive measures early. As cerebral palsy is commonly associated with mental retardation, parents also express concerns about the child’s cognitive development. Data from the analyses of large series help address this issue.

TABLE

10.

Prognosis

for Independent

1. 2. 3. 4. 5. 6. 7.

Walking*t

Asymmetrical tonic neck reflex Suymmetrical tonic neck reflex More reflex Neck-righting reflex Extensor thrust lsee Fig 5J Foot placement reaction Parachute reaction (see Fig 7)

(see Fig 1)

‘From Heck El2 Locomotor prognosis in cerebral palsy. Dev Med Child Neural 1975; 17:18. Used hy permission. ti\t age 1, 1 point is given if an.v of Nos. l-5 is found; 1 point if either 6 01‘ 7 is not found. 0 = good pmgnosis; 1 = guarcled; 2 = poor prognosis.

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The quadriplegic patient who has epilepsy almost certainly will be, at best, educably mentally retarded. Ninety percent of patients with dysequilibrium syndrome are also retarded. In most patients, a prognosis about intellectual development must be deferred pending the development of language, as this skill is correlated with intellectual development. Therefore, in the questionable situations, a prognosis cannot and should not be rendered until after the patient is age 2. Further, in the athetoid patient who might have a severe dysarthria, a prognosis about intelligence should be postponed until schoolage. An examiner experienced with the severely disabled dyskinetic population should perform the evaluation, as the patient may be a poor examinee because of the motor disability and the scores might be misleading or incorrect. As the child matures, changes in muscle tone and function may occur that might raise concerns about the diagnosis.72 For example, the hypotonic infant and toddler may commonly develop spasticity or athetoid movement. Not only may the muscle tone change, but the disability may lessen or disappear entirely. An analysis of the data from the NCPP showed that 118 children diagnosed as having mild cerebral palsy at age 1 showed no motor disability at age 7.73 However, as a group, they had a higher incidence of learning difficulties and afebrile seizures. Obviously, even the child who improves over time is at risk for the associated problems. Finally, the examiner must be able to discuss issues of life style with the “parents. Communication is the most important skill required by a human being. Without this ability, even with a normal intellect, the child is “locked in.” The technical advances being made, now and even more so in the future, will allow a more positive outlook for even the most severely disabled patient with cerebral palsy. PROGNOSIS ABOUT

VOCATION

The goal of any treatment program is to maximize the child’s strengths and minimize the weaknesses. This obviously involves an intensive treatment program that has already been, discussed. Education is also critically important for the individual with cerebral palsy. The educational program must be geared not only to develop academic skills, but also to assure that the patient will be employed, or better employed, than what one might have predicted during the early years of development. Studies regarding vocational status of individuals with cerebral palsy, in the 19.50s and 196Os, have indicated that employability is related to cognitive skills, self-care, independence, severity of the physical disability, educational level attained, and mobility in the community. As pointed out by O’Grady et al., the studies heretofore 106

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have been retrospective.74 In the San Francisco area, 97 students with cerebral palsy between the ages of 7 and 16 were evaluated in the 1960s and 1970s and predictions were made as to their future employability. In 1983, 60 of the 76 individuals over age 18 were contacted. At the time of the survey, only 17 were employed, although 39 had been employed at some time. Employment was related to the severity of the disability and the cognitive skills of the patients. A positive correlation was found between employment and a patient with mild cerebral palsy, that is, an individual with normal or nearnormal intelligence and minimal physical disability. Further, unemployment was correlated with those individuals who had severe physical disabilities and/or who were retarded. An accurate prediction for patients with cerebral palsy who were in the middle range of intelligence, severity of handicap, and self-help abilities, was quite difficult. For those individuals who did better than predicted, family support and personal determination were believed to be paramount in their ability to attain their specific status. Furthermore, the development of technology helped at least one individual who was predicted to be unemployable, but who was working as an office computer assistant. Other positive factors identified in the present investigation as being important to vocational status were an integrated education and a community-based assessment program. The group of patients who had never been employed were older, but presumably were less likely to have benefited from changes in the educational system and the newer technology. One has to conclude from this recent study, as well as from those in the past, that employability is not related solely to the individual’s disability, but rather to other factors including family support, educational programs, technology, and community-based programs. CONCLUSIONS

Cerebral palsy is a term used to describe a patient who has a nonprogressive brain lesion leading to a motor deficit. That the motor disability may change over the years does not obviate the diagnosis. Associated problems, including seizures, mental retardation, language disorder, and speech deficits, as well as a strabismus, must be evaluated and treated appropriately. There are many causes of cerebral palsy, including genetic diseases and embryologic abnormalities. Most often, a specific cause cannot be identified. Risk factors, however, can be identified in about 30% to 40% of cases. Risk factors alert the clinician to anticipate the presence of cerebral palsy in a patient; they should be considered separate from causation. Cerebral palsy is an acceptable term as long as it is used appropriately and as long as the issues associated with this term are carefully explained to the parents. Cerebral palsy ranges in severity from Curr

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minimal limitations, requiring no treatment, to total care and intensive treatment. The public commonly associates the “total care” patient with the term cerebral palsy. Consequently, presenting the diagnosis to the parents must be carefully articulated, emphasizing the various degrees of impairment. If one anticipates a treatment program including physical therapy and potential orthopedic intervention, the term is appropriate and should be used. REFERENCES 1. Nelson KB, Ellenberg JH: Epidemiology of cerebral palsy, in Schoenberg BS (cdl: Advances in Neurology. New York, Raven Press, 1978, pp 421-435. 2. Freud S: Zr$tntile Cerebral Paralysis, Russin LA (Vans). Miami, University of Miami Press, 1968. 3. Weiss H, Betts HB: Method of rehabilitation in children with neuromuscular disorders. Pediatr Clin North Am 1967; 14:lOOS. 4. Minear WL: A classification of cerebral palsy. Pediatrics 1956; 18:841. 5. Brun A, Kyllerman M: Clinical pathogenetic and neuropathological correlation in dystonic cerebral palsy. Eur J Pediatr 1979; 131:9X 6. Kyllerman M, Bager B, Bensch J, et al: Dyskinetic cerebral palsy: I. Clinical categories, associated neurological abnormalities and incidences. Acta Paediatr &and 1982; 71:551. 7. Kyllerman M: Dyskinetic cerebral palsy: II. Pathogenetic risk factors and intra-uterine growth. Acta Pediatr &and 1982; 71:551. 8. Hagberg B, Hagberg G, Olow I: Gains and hazards of intensive neonatal care: An analysis from Swedish cerebral palsy epidemiology. Dev Med Child Neurol 1982; 24:13. 9. Hagberg B, Hagberg G, Olow I: The changing panorama of cerebral palsy in Sweden: IV. Epidemiological trends 1959-1978. Acta Paediatr Stand 1984; 73:433. 10. Dale A, Stanley FJ: An epidemiological study of cerebral palsy in Western Australia, 1956-1975: Spastic cerebral palsy and perinatal factors. Dev Med Child Neural 1980; 22:13. 11. Blair E, Stanley FJ: An epidemiological study of cerebral palsy in Western Australia, 1956-1975: III. Postnatal etiology. Dev Med Child Neural 198%; 24575. 12. Sanner G: The dysequilibrium syndrome: A genetic study. Neuropediatrics 1973;4:403. 13. Gubbav SS: The Clumsy Child. Philadelphia, WB Saunders Co, 1975, p 39. 14. Blair E, Stanley F: Interobserver agreement in the classification of cerebral palsy. Dev Med Child Neural 1985; 27:615. 15. Ellenberg JH, Nelson KB: Birth weight and gestational age in children with cerebral palsy or seizure disorder. Am J Dis Child 1979; 133:1044. 16. Pharoah POD, Cooke T, Rosenbloom I, et al: Trends in birth prevalence of cerebral palsy. Arch Dis Child 1987; 62379. 17. Nelson KB, Ellenberg JH: Antecedents of cerebral palsy: I. Univariate analysis of risks. Am J Dis Child 1985; 139:1031. 18. Nelson KB, Ellenberg JH: Antecedents of cerebral palsy: Multivariate analysis of risk. N Engl J &fed 1986; 315:81. 19. Nelson KR, Ellenberg JH: Apgar scores as predictors of chronic neurologic disability. Pediatrics 1981; 68:36. 10.9

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4.2. Sochaniwskyj AE, Koheil RM, Bablich K, et al: Oral motor functioning, frequency of swallowing and drooling in normal children and in children with cerebral palsy. Arch Phys Med Rehabil 1986; 67:866-874. 43. Patrick .I, Boland M, Stoski D, et al: Rapid correction of wasting in children with cerebral palsy. Dev Med Child New-01 1986; 29:734. 44. Shapiro B, Green .I, Krick .I, et al: Growth of severely impaired children: Neurological versus nutritional factors. Dev Med Child Neural 1986; 28:729. 45. McNeal DM, Hawtrey CE, Wolraich MG, et al: Symptomatic neurogenic bladder in a cerebral palsied population. Dev Med Child New-01 1983; 25:612. 46. Russman BS: Comprehensive management of children with muscular disorders. Pediatr Ann 1984; 13:103. 47. Sparrow S, Zigler E: Evaluation of a patterning treatment for retarded children. Pediatrics 1978; 62:137-150. 48. American Academy of Pediatrics: Policy statement: The Doman-Delacato treatment of neurologically handicapped children. Pediatrics 1982; 76:810. 49. Bobath B: The very early treatment of cerebral palsy. Dev Med Child Neurol 1967; 9:373. 50. Paine RS: On the treatment of cerebral palsy: The outcome of I77 patients, 74 totally untreated. Pediatrics 1962; 29605. 51. Wright T, Nicholson J: Physiotherapy for the spastic child: An evaluation. Dev Med Child New-01 1973; 15:146-163. 52. Scherzer AL, Mike V, Ilson .I: Physical therapy as a determinant of change in the cerebral palsied infant. Pediatrics 1976; 58:47-X2. 53. Simeonsson RJ, Cooper DH, Scheiner AP: A review and analysis of the effectiveness of early intervention programs. Pediatrics 1982; 69:635. 54. Breslaw N, Stanch KS, Mortimer EA: Psychological stress in mothers of disabled children. Am J Dis Child 1982; 136:682. 55. Bax M: Aims and outcomes of therapy for the cerebral-palsied child. Dev Med

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66. Logan M: Improved Mechanical Efficiency in Cerebral Palsy Patients Treated with Noninvasive Cortical Electrical Stimulation. Presented at American Academy of Cerebral Patsy and Developmental Medicine. Boston, October 1987. 67. Watt J, Sims D, Harckham F, et al: A prospective study of inhibitive casting: As an adjunct physiotherapy in the cerebral palsied child. Orthop Transactions 1984; 8:llO. 68. Bleck EE: Orthopedic Management in Cerebral Palsy. Oxford, England, MacKeith Press, 1987, p 98. 69. Bleck EE: Orthopedic Management in Cerebral Palsy. Oxford, England, MacKeith Press, 1987, p 190. 70. Gage JR, Fabian D, Hicks R, et al: Pre- and postoperative gait analysis in patients with spastic diplegia: A preliminary report. J Pediatr Orthop 1984; 4:715. 71. Bleck EE: Locomotor prognosis in cerebral palsy. Dev Med Child Neurol 1975; 17:18. 72. Hanson RA, Berenberg W, Byers RK: Changing motor patterns in cerebral palsy. Dev Med Child Neural 1970; 12309. 73. Nelson KB, Ellenberg JH: Children who “outgrew” cerebral palsy. Pediatrics 1982;

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1985:

February

DM, Kohn JG, et al: Vocational predictors status of 60 young adults with cerebral

compared palsy. Dev

27:775.

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