Initial Assessment of a Child with Cerebral Palsy
coagulation disorders, infectious conditions, or genetic disorders.     anomalies of brain development, and is more useful...

                                                                              Screening for othe...
Pediatric neurology consultation                                   REFERENCES
In evaluation and management of a child with...
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Initial Assessment of a Child with Cerebral Palsy


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Initial Assessment of a Child with Cerebral Palsy

  1. 1. C M E F E AT U R E Initial Assessment of a Child with Cerebral Palsy LEON G. EPSTEIN, MD Cerebral palsy (CP), defined as a nonprogressive disorder of posture or movement due to a lesion in the developing brain, occurs annually in about 10 000 infants born in the U.S.1 To help pediatricians assess a child with this common disorder, this review will highlight the latest findings on pathogenesis of CP, summarize the new recommendations for diagnostic testing, and discuss the prognostic implications of various types of brain injury responsible for CP. Educational Objectives Diagnosis Etiology At the conclusion of this CP is usually recognized early in life when a The brain abnormality resulting in CP may activity, participants will be child fails to reach motor milestones at the have been caused by various antenatal, perinatal able to: appropriate chronological age. Diagnosis is based and postnatal events. In the past, the most on detailed history and physical examination, common cause of CP in the term or near term • Describe the underlying including a neurological evaluation. infant was thought to be intrapartum hypoxic- causes of cerebral palsy ischemic injury, often called perinatal asphyxia. Since CP is distinguished from degenerative • Direct the initial assessment It is now known that hypoxia-ischemia is not brain disorders, medical history must establish of a child with cerebral palsy the only cause of neonatal encephalopathy, and that the child is not losing previously acquired that CP is rarely caused by hypoxia-ischemia. • Recognize when a referral to function. However, some neurological disorders, Epidemiological studies have established that a pediatric neurologist is due to slow progression of symptoms, may antenatal factors account for as much as 70% of required in the evaluation initially be mistaken for CP (eg, dopa-responsive acute neonatal encephalopathy.3 and management of a child dystonia, hereditary spastic paraplegia, ataxia with cerebral palsy telangiectasia). These conditions may be suggested Furthermore, not all cases of neonatal by unusual complaints, a history of progressing encephalopathy result in CP. Mild encephalopathy deficits, characteristic abnormalities on is invariably associated with favorable prognosis, neuroimaging studies, or a family history of and normal outcome is seen in about 75%-80% childhood neurologic disorder with associated CP. infants with moderate encephalopathy.4 All infants with severe encephalopathy stringently During examination, pediatricians should check defined have a poor outcome.4 developmental reflexes, muscle tone, deep tendon reflexes, and plantar responses. According to the essential criteria developed by Attention should be paid to reflexes that are the American College of Obstetricians and retained for abnormally long periods, which is Gynecologists,3 in order to attribute CP to common for children with CP. The Moro reflex, intrapartum hypoxia, the child born at 34 or for example, tends to be seen beyond the usual more weeks of gestation must have had the 6 months in children with CP. However, clinical signs of severe or moderate neonatal children with CP may develop hand preference encephalopathy, defined as seizures, abnormal much earlier than usual (age 3). tone, poor feeding, and a depressed level of consciousness within the first 24 hours of life. It is also useful to classify the clinical type of CP, Computed tomography (CT) scan and since this has implications regarding etiology electroencephalogram (EEG) if obtained will be and associated conditions, as discussed in more abnormal. In addition, evidence of a metabolic detail below. In one study, 44% of the CP acidosis in fetal umbilical cord arterial blood population were spastic diplegic, 33% spastic obtained at delivery (pH<7 and base deficit≥12 hemiplegic, and 6% spastic quadriplegic. Even mmol/L) is needed, as well as exclusion of other less common presentations include ataxic and identifiable etiologies such as trauma, dyskinetic CP.2 14 I Fall 2004 The Child’s Doctor
  2. 2. coagulation disorders, infectious conditions, or genetic disorders. anomalies of brain development, and is more useful in Finally, the only forms of CP attributable to acute hypoxic determining whether the brain injury was antenatal, perinatal, or intrapartum events are spastic quadriplegic and, less commonly, postnatal. Periventricular white matter damage (eg, periventricular the dyskinetic type. leukomalacia) is frequently found in children with CP who were preterm births. A child with CP who was a preterm birth and Spastic quadriplegia, however, may result from causes other than suffered severe cerebral hypoperfusion during early fetal life, intrapartum hypoxia. Also, purely dyskinetic or ataxic CP, however, will usually have injury that is mostly in the deep gray especially with an associated learning difficulty, commonly has a matter and brain stem nuclei. In term births, severe reduction to genetic etiology and is rarely caused by intrapartum or cerebral blood flow more commonly causes injury to subcortical peripartum asphyxia. white matter and cerebral cortex.7 Spastic hemiplegia and spastic diplegia are almost always due to Genetic and metabolic testing: Since the introduction of antepartum events, the latter occurring almost exclusively in neuroimaging, congenital brain malformations have been preterm babies.5 Generally, infants born very prematurely or with discovered in some children with CP (12% on MRI and 7% on low birth weight are at higher risk for CP, compared to term or CT).6 These malformations include lissencephaly, schizencephaly, near-term infants. and pachygyria, which have been associated with specific genetic CP with antenatal origins is often due to infections during disorders (eg, lissencephaly/Miller-Dieker syndrome/ pregnancy (eg, rubella, cytomegalovirus, toxoplasmosis), stroke, chromosome 17p13.3, Zellweger syndrome). Also, some toxemia, or placental abruption.6 Other antenatal factors metabolic disorders may appear as CP. These disorders include associated with CP include intrauterine growth restrictions, glutaric aciduria (type 1), Lesch-Nyhan syndrome, 3- multiple pregnancies, coagulation disorders, antepartum methylglutaconic aciduria, pyruvate dehydrogenase deficiency, bleeding, congenital or genetic anomalies, and infertility and female carriers of ornithine transcarbamylase deficiency. If a treatments.3 In addition to hypoxic-ischemic encephalopathy, brain malformation is found in a child with CP, or if other causes of CP with perinatal onset include kernicterus and neuroimaging does not show a specific structural abnormality, trauma.6 Postnatal causes of CP include brain infections (eg, genetic or metabolic testing is warranted. bacterial meningitis, viral encephalitis), progressive hydrocephalus, Coagulopathies: Diagnostic testing for coagulation disorders or brain injury from car accidents, falls, or child abuse.6 should be considered only in children with spastic hemiplegic CP, since neuroimaging shows high incidence of unexplained Diagnostic testing cerebral infarction in these children. Coagulation disorders may The role of diagnostic testing is to clarify the etiology and include Factor V Leiden deficiency, the presence of anticardiolipin suggest the prognosis based on the type and severity of or antiphospholipid antibodies, and Protein C or S deficiency. confirmed brain injury. In the recently published guidelines from the American Academy of Neurology and the Child Neurology Epilepsy and EEG: Although epilepsy accompanies cerebral palsy Society,6 neuroimaging is recommended as the initial diagnostic in 45% of cases,6 routine EEG is not useful during initial study in children with CP. See images 1-4 on page 16 for evaluation, since it does not help to determine the etiology of examples of neuroimaging studies used as diagnostic tools in CP CP. An EEG is recommended only when epileptic features are evaluation. The guidelines do not recommend routine use of present. Epilepsy is seen more often in children with spastic metabolic and genetic testing, coagulation studies, or EEG. quadriplegia or hemiplegia, compared to children with other However, since 70% of children with CP have associated types of CP. conditions,1 early screening is advised for potential cognitive Children with CP and epilepsy are classified as having delay, vision and hearing impairments, speech and language symptomatic epilepsy. Children with epilepsy associated with CP disorders, and swallowing disorders. are more likely to have neonatal seizures and seizures within the MRI or CT: The AAN/CNS review of research concluded that first year of life, status epilepticus, and a need for polytherapy. magnetic resonance imaging (MRI) is preferable to CT, since These children are more likely to have partial seizures and are less MRI was abnormal in 89% of children with CP, compared to likely to become seizure free. In children with CP and epilepsy, 77% of cases with abnormal CT findings. MRI also is more favorable factors associated with a seizure-free period of 1 year or sensitive than CT in detecting periventricular leukomalacia, more include normal intelligence, single seizure type, other perinatally acquired lesions, and subtle congenital monotherapy, and spastic diplegia.8 Children’s Memorial Hospital Fall 2004 I 15
  3. 3. CEREBRAL PALSY (cont.) Screening for other associated conditions: Screening for conditions commonly found in children with CP must be included in the initial evaluation. Cognitive delay occurs in 52% of children with CP, and it is more likely to be present if neuroimaging studies are abnormal than if they are normal or have minor abnormalities.9 Also, type and etiology of CP are associated with specific disorders. For example, mental impairment tends be more severe in children with spastic quadriplegia than in children with spastic hemiplegia or diplegia. Visual problems, found in 28% of cases, are more likely in children whose CP was caused by periventricular leukomalacia.6 Hearing impairment occurs in 12% of children with CP, and is more frequent when Image 1: MRI axial T2 Image 2: MRI axial T1 CP is related to very low birth weight, kernicterus, neonatal sequence of a 14-month-old sequence of a 14-month-old meningitis, or severe hypoxic-ischemic insults.6 girl with a right hemiparesis. boy with microcephaly and There is an abnormal delayed motor development. Prognostic implications of neuroimaging findings hyperintense signal in the The MRI demonstrates Neuroimaging is helpful in determining prognosis. Outcomes are white matter of the right enlarged ventricles and a hemisphere indicating an old large porencephalic cyst in the less favorable in children with bilateral injury involving cortical (antepartum) infarct. left frontal lobe with a thin and subcortical structures usually associated with a spastic rim of residual cortex. There quadriparetic type of CP. Neuroimaging findings of atrophy, is also thickened and abnormal gray matter configuration, and marked leukomalacia simplified cortex (pachygyria) usually imply severe impairment.10 in the right frontal lobe. In very premature infants, selective vulnerability of the periventricular white matter to ischemic injury produces diplegic CP with greater motor deficit in the lower extremities compared to the upper extremities. In term infants, white matter lesions with internal capsule involvement are associated with abnormal motor outcome, whereas moderate white matter changes with normal internal capsule are correlated with normal motor function.11 Extensive bilateral cerebral and basal ganglia lesions, associated with spastic quadriplegia and the most adverse outcome, commonly signal epilepsy and severe cognitive, motor, visual and auditory impairments.11 Mild basal ganglia lesions, however, are found in dyskinetic type of CP and indicate minor neuro-motor Image 3: CT scan of an Image 4: CT scan of a abnormalities and sometimes normal cognitive development.11 18-month-old girl born 5-month-old boy with a Children with focal ischemic injury (stroke) limited to 1 prematurely who sustained severe spastic quadriparesis an intraventricular and acquired microcephaly hemisphere will usually have a residual hemiparesis, but are likely hemorrhage. She has a left associated with an to have normal or nearly normal cognitive function. The MRI hemiparesis and a mild intrapartum hypoxic-ischemic will show loss of brain parenchyma and compensatory increase in degree of spastic diparesis. injury. The scan shows the size of the ventricles and subarachnoid spaces on the involved The CT scan demonstrates marked diffuse atrophy and side. Partial seizures with or without secondary generalization are enlargement of the right encephalomalacia with lateral ventricle. secondary enlargement of the also common with this type of injury. ventricles and sulci. Although neuroimaging studies help to assess prognosis for a child’s CP, more substantial prognostic information must be * Note that for all images the left side of the brain appears on derived by considering imaging data in combination with clinical the right side of the image. evaluation. 16 I Fall 2004 The Child’s Doctor
  4. 4. Pediatric neurology consultation REFERENCES In evaluation and management of a child with cerebral palsy, consultation with a pediatric neurologist should be considered [1.] Boyle CA, Yeargin-Allsopp M, Doernberg NS, et al. Prevalence of when there are questions about accuracy of the diagnosis, to selected developmental disabilities in children 3-10 years of age: the Metropolitan Atlanta Developmental Disabilities Surveillance. MMWR determine the underlying cause, or if there are unusual clinical 1996;45:1–14. features to suggest that the child may have a progressive rather [2.] Hagberg B, Hagberg G, Beckung E, et al. Changing panorama of than a static condition. A pediatric neurologist may also help cerebral palsy in Sweden. VIII. Prevalence and origin in the birth year to inform parents about initial evaluation, interpretation of period 1991–94. Acta Paediatr 2001;90:271–277. neuroimaging studies, and prognosis. Severe cases can be [3.] American College of Obstetricians and Gynecologists Task Force on referred to a pediatric neurologist for regular evaluations on Neonatal Encephalopathy and Cerebral Palsy. Neonatal Encephalopathy annual or semiannual basis, and for coordination of therapy and Cerebral Palsy: Defining the Pathogenesis and Pathophysiology. with physical, occupational and speech therapists, and with Washington, DC: ACOG; 2003. orthopedic specialists. Finally, a referral is needed when there [4.] Volpe JJ. Neurology of the Inborn. 4th ed. Philadelphia, PA: WB are associated neurological problems, in particular seizures, Saunders; 2001. which may be difficult to control. [5.] Nelson KB, Grether JK. Potentially asphyxiating conditions and spastic cerebral palsy in infants of normal birth weight. Am J Obstet Conclusion Gynecol 1998;179:507-513. CP is common and will be seen in most pediatric practices. [6.] Ashwal S, Russman BS, Blasco PA, et al. Practice Parameter: There are multiple causes of CP and the pediatrician should Diagnostic assessment of the child with cerebral palsy: Report of the Quality Standards Subcommittee of the American Academy of Neurology not assume that minor events in the intrapartum period are and the Practice Committee of the Child Neurology Society. Neurology responsible without careful consideration of the criteria for 2004;62(6):851-863. neonatal encephalopathy and a thorough investigation for [7.] Barkovich AJ, Hallam D. Neuroimaging in perinatal hypoxic other causes. There are medical-legal implications to casual ischemic injury. Ment Retard Dev Disabil Res Rev 1997;3:28-41. comments or written notes regarding the etiology of CP even [8.] Wallace SJ. Epilepsy in cerebral palsy. Dev Med Child Neurol if these are not well founded. Neuroimaging, in particular 2001;43:713-717. MRI, is the most useful diagnostic tool for children with CP. [9.] Schouman-Claeys E, Picard A, Lalande G, et al. Contribution of Neurological consultation is most often justified to help computed tomography in the aetiology and prognosis of cerebral palsy in establish the type of cerebral palsy and to discuss prognosis children. Br J Radiol 1989;62:248-252. with the child’s parents. ■ [10.] Yokochi K, Hosoe A, Shimabukuro S, et al. Gross motor patterns in children with cerebral palsy and spastic diplegia. Pediatr Neurol 1990;6:245. [11.] Mercuri E, Barnett AL. Neonatal brain MRI and motor outcome in school age children with neonatal encephalopathy: A review of personal experience. Neural Plasticity 2003;10(1-2):51-57. LEON G. EPSTEIN, MD Derry A. and Donald L. Shoemaker Professor of Pediatric Neurology, Division Head, Neurology, Children’s Memorial Hospital; Deputy Director for Clinical Research, Children’s Memorial Research Center; Associate Director of General Clinical Research Center, Feinberg School of Medicine, Northwestern University; Chicago, Illinois lepstein@childrensmemorial.org Children’s Memorial Hospital Fall 2004 I 17