Prospective study of activities of daily living outcomes in children with cerebellar atrophy

Aim: The aim of this study was to identify clinical and radiological predictors of activities of daily living (ADL) outcomes in children with cerebellar atrophy. Method: Over a period of 5 years, we evaluated 44 participants (25 males, 19 females) children with confirmed cerebellar atrophy using magnetic resonance imaging (MRI). The median age at the time of assessment 9 years; range 16mo-18y. Participants were grouped according to whether the cerebellar atrophy was isolated or associated with other radiological abnormalities. Severity of cerebellar atrophy was graded using qualitative and quantitative scoring systems. A standardized ADL assessment was used to characterize functional outcomes. The characteristics of the participants were analysed using descriptive statistics. Results: The mean age at symptom onset was 20 months (range birth-10y). The group with isolated cerebellar atrophy had better outcomes than the group with cerebellar atrophy associated with other radiological abnormalities, with a mean total ADL score difference of 8.0 points (95% confidence interval 1.8-14.2 points, p=0.01). Age at onset of cerebellar atrophy before 2 years of age, progression of cerebellar atrophy on magnetic resonance imaging, presence of seizures, and decreased size of transverse cerebellar hemisphere diameter were all associated with worse outcomes. Interpretation: We present a prospective study of clinical and radiological predictors of ADL outcome in children with cerebellar atrophy. This information may be useful in the diagnosis and future management of this complex group of disorders.