Pontocerebellar Hypoplasia in Extreme Prematurity: Clinical and Neuroimaging Findings

The involvement of the cerebellum in unfavorable outcomes of extreme prematurity is increasingly recognized. Evidence implicates both cerebellar injury and cerebellar growth failure, which, along with supratentorial lesions, aggravate motor and developmental outcomes. We describe clinical and neuroradiologic findings of 12 extremely premature patients with acquired pontocerebellar hypoplasia (mean follow-up, 4 years). Patients’ neuromotor outcomes involved combined motor abnormalities (spasticity, dystonia, and ataxia), whereas 25% were ambulatory by age 4 years. All patients exhibited developmental delays of variable degrees. One patient died at age 7.5 years. The possible etiopathogenesis, presentations, sequelae, and differential diagnoses of acquired pontocerebellar hypoplasia are discussed.