Cerebellar atrophy occurs in the inherited neurodegenerative syndrome Ataxia Telangiectasia (A-T) but the trajectory of cerebellar atrophy across childhood and its relationship to clinical status is unknown. We report cerebellar and fourth ventricular volumes (normalized to intracranial volume) from 24 children with A-T and 24 matched controls. Cerebellar volume declined linearly with increasing age in the A-T group with a divergent trajectory of age-related cerebellar volume change compared to normally-developing children. Fourth ventricular volume increased with age in children with A-T and correlated with clinical status, and may provide an imaging marker of neurological status in childhood A-T.
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