Introduction

Dentatorubral-pallidoluysian atrophy (DRPLA) is a rare autosomal dominant neurodegenerative condition characterised by gradual onset of a variable combination of ataxia, chorea, epilepsy and dementia1 and is due to a (CAG)n expansion in the atrophin-1 gene.2 The pathological abnormalities consist of atrophy of the dentatorubral and pallidoluysian systems.3 Previously reported changes on brain MRI in DRPLA include atrophy of the affected areas and high-signal lesions on T2-weighted imaging in the cerebral white matter, brainstem and thalamus.4 Susceptibility-weighted imaging (SWI) has not been reported in DRPLA.