Evaluation of a child with cerebral palsy
Evaluation of a child with cerebral palsy (CP) requires a multidisciplinary approach with a team of professionals comprising of a pediatrician or pediatric neurologist, occupational therapist, a physiotherapist, child psychologist, and a social worker. The assessment is necessary to confirm the diagnosis, determine the cause, assess the motor function and associated problems.
The diagnosis of CP is clinical but selected investigations may be required for ascertaining the cause. Evaluation includes assessment for common medical problems of childhood particularly nutritional disorders and assessment of family functioning. Additional disabilities are common.
Routine assessment of vision and hearing is required in children with CP. Since CP is a changing disorder, some limitations may not be evident early in life but manifest in the school age or later. The evaluation of a child with CP is an ongoing process and should be a part of continuing care as the child grows from infancy to adolescence.
Cerebral palsy (CP) a heterogenous group of persistent disorder of movement and posture caused by non-progressive defects or lesions of immature brain, is the most common cause of childhood physical disability. The incidence of CP in developed world is 2-2.5/1000 live births.[1] Evaluation of a child with CP requires a multidisciplinary approach with a team of professionals comprising of a pediatrician or pediatric neurologist, occupational therapist, a physiotherapist, child psychologist, and a social worker. Evaluation of a child with CP is necessary to confirm the diagnosis, determine the severity of the problem and its cause. The team of professional need to work together in close co-operation of parents and teachers to help the child reach his maximum potential.
Early detection
Accurate and early diagnosis of CP is important for medical and social reasons. Given the availability of limited resources it becomes critical to accurately identify, as early as possible, those most in need of services. The periodic evaluation of “at risk” infants is a an effective strategy for early diagnosis. The probability of CP increases with increasing prematurity, multiple pregnancies, and adverse perinatal events. Abnormal neurological examination in infants helps in identifying children at risk for subsequent motor difficulties. In preterm infants, presence of atypical features e.g. coarse jitters, dominant asymmetric tonic neck reflex (ATNR), paucity of movements, hypotonia/hypertonia predict major motor dysfunction.[2] Ultrasound abnormalities especially grade 2-4 leukomalacia together with early visual and neurocognitive assessment data is a good predictor for later neurodevelopment.[3] All such patients who have risk factors or have abnormal neurological signs should be followed closely for motor development. Ellenberg and Nelson report that a combination of low birth weight and hypertonus at 4 months is highly predictive of CP at 7 years.[4] A cluster of certain behavioral symptoms and deviation from normal motor patterns are suggestive of CP [Table - 2].
It is often difficult to diagnose CP in the first half of infancy except in severe cases. This difficulty is compounded by the fact that some normal babies may show abnormal signs that clear up spontaneously. Pediatricians are often in a dilemma as to whether the parents should be informed of the suspicion of CP. In such a case it is best not to give the diagnostic label, but to inform the parents that the child’s development needs to be assessed frequently.
Classification
Cerebral palsy can be classified according to pathology of brain injury e.g. vascular, infective, inflammatory or traumatic; or according to the timing of brain injury such as prenatal, perinatal or postnatal. The modified Swedish clasification is the widely used classification which is based on the tone, number and distribution of the affected limbs[5] [Table - 3]. The changing nature of symptoms and signs make the clinical classification difficult in the first years of life as the pattern of movement and tone may change completely.
Ascertaining the Cause of Cerebral Palsy
CP in developing countries is most often attributed to birth asphyxia and birth trauma and indeed birth asphyxia is still a leading cause of CP in India. However, the other important causes include developmental abnormalities, intrauterine and postnatal infections, iodine deficiency disorders, prematurity and metabolic disorders. Identifying the actual cause is important, since it avoids needless blame on obstetric management and the feeling of guilt in parents.
A consensus statement issued by International Cerebral Palsy Task Force suggested a set of criteria which should be present to pinpoint intra-partum hypoxia as the cause of CP. The essential criteria suggested were - evidence of metabolic acidosis in intrapartum or very early neonatal blood samples, early onset of moderate to severe neonatal encephalopathy in infants >34 weeks gestation and cerebral palsy of spastic quadriplegic or dyskinetic type.[6] The role of perinatal events in the causation of CP are better appreciated in the neonatal period. It may be difficult to pinpoint the exact cause when the child presents in infancy with CP. In patients delivered in hospital, the intrapartum events would have been recorded and help in ascertaining the cause. In patients delivered at home it may be very difficult to ascertain the exact cause. However it may be noted that neonates with significant birth asphyxia are likely to have abnormal behavior in the first 24 hours following birth and are unlikely to accept breastfeed.
Factors pointing to antenatal origin of CP are microcephaly at birth, history of preterm birth or twinning, presence of malformation, history of CP in a sibling, severe intrauterine growth retardation, early imaging evidence of long standing neurological disease such as porencephalic cyst or ventriculomegaly.[6] On the other hand, presence of cerebral edema on ultrasonography in neonatal period indicates recent insult. In areas endemic for iodine deficiency disorders, deaf mutism, mental retardation and spasticity with predominant involvement of proximal lower limbs is suggestive of neurological cretinism.[6]
Differential diagnosis
Diagnosis of CP is made purely on clinical grounds. While it is desirable to diagnose this condition as early as possible, it is almost impossible to diagnose CP under the age of 4 months and in less affected ones even upto the age of 8 months. In the early infancy when the child is in hypotonic phase, neuromuscular conditions like myopathies may cause diagnostic confusion. Careful observation reveals that limb movements are preserved and there is predominantly axial hypotonia in children with CP. Global delay in development is often seen in children with severe malnutrition but the diagnosis is clearly apparent in such cases. Patients with mental retardation without motor deficits are often given a diagnosis of Cerebral palsy because the patient presents with delayed milestones. Children with mental retardation may have hypotonia but do not have abnormal motor patterns or postures; and have significantly more deficits in speech and language and social adaptive domains as compared to motor deficits. Neurodegenerative conditions which have onset in early infancy such as Tay-Sach disease, Krabbe’s disease, and Metachromatic Leucodystrophy can mimic CP. The progressive course of these conditions can be ascertained on the basis of history, and relevant investigation can confirm the diagnosis. Dopa-responsive dystonia and organic aciduria like glutaric aciduria may look like dystonic CP.[7]
Aneja S. Evaluation of a child with cerebral palsy. Indian J Pediatr [serial online] 2004 [cited 2007 Oct 13];71:627-634. Available,
References
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Aneja S
Department of Pediatrics, Lady Hardinge Medical College & Associated Kalawati Saran Children Hospital, New Delhi, India
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