Etiologic yield of cerebral palsy: a contemporary case series

Abstract

Cerebral palsy is an established symptom complex that results from heterogeneous etiologies. Our understanding of the relative contribution of underlying etiologies to the occurrence of cerebral palsy is largely derived from studies lacking systematic neurologic evaluation or the application of contemporary imaging modalities. Throughout a 10-year inclusive period, the case records of all consecutive patients diagnosed with cerebral palsy in a single pediatric neurology practice were reviewed with reference to clinical features and diagnostic yield. A total of 217 cases of cerebral palsy were identified (129 male, 88 female): 77 (35.5%) spastic quadriplegic, 68 (31.3%) spastic hemiplegic, 39 (18%) spastic diplegic, five (2.7%) spastic monoplegic, 12 (5.5%) mixed, 12 (5.5%) ataxic-hypotonic, two (0.9%) dyskinetic, two (0.9%) Worster-Drought syndrome. Overall etiologic yield was 82.0%, varying according to type of cerebral palsy: 50% dyskinetic, 59% diplegia, 80% monoplegia, 80.9% hemiplegia, 90.9% quadriplegia, 91.7% ataxic hypotonia, and 100% mixed/Woster-Drought. The top five etiologic entities identified were periventricular leukomalacia, 24.9%; intrapartum asphyxia, 21.7%; cerebral dysgenesis, 17.1%; intracranial hemorrhage, 12.9%; and vascular, 9.7%. Although a single etiology was apparent in 144 (66.4%) of the cases, multiple etiologies were believed to be contributory in 34 (15.6%) of the cases. The etiologic profile varied according to such features as the type of cerebral palsy, gestational age, and the source (high-risk neonatal population or not) of the patients. Features of the child's cerebral palsy, such as microcephaly, neonatal difficulties, prior or coexisting epilepsy, and high-risk source, were found to be predictive of eventual etiologic yield. This contemporary evaluation of cerebral palsy etiologic yield suggests that it is much higher than previously reported and varies, depending on key clinical features.