UNILATERAL CEREBELLAR APLASIA

We describe three children with unilateral cerebellar aplasia (UCA). D eliveries at term and neonatal periods were uneventful. Pregnancy was normal in one and complicated by mild bleeding (in second and fourth m onth respectively) in two Instances. Presenting signs were delayed mot or development with marked contralateral torticollis (n = 1), hemipleg ia (n = 1) and unusual head nodding (n = 1). Neuroradiological investi gations revealed complete aplasia (n = 1) and subtotal aplasia (n = 2) of one cerebellar hemisphere with only a residual wing-like structure below the tentorium. There was contralateral underdevelopment of the brainstem. The infant with hemiplegic cerebral palsy had an additional supratentorial periventricular parenchymal defect, contralateral to t he cerebellar hypoplasia. In view of Literature reports, describing si milar neuroradiological or neuropathological findings in asymptomatic individuals, it is doubtful whether UCA is responsible for our patient 's problems. In our cases UCA has presumably resulted from a prenatal destructive lesion, possibly an infarct, but the timing and exact natu re are unknown.