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- Dentatorubral-pallidoluysian atrophy
- susceptibility-weighted imaging SWI
- Parkinson's disease
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.
Three male subjects over three generations were symptomatic (figure 1).
Competing interests None.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
Data sharing statement Complete neuroimaging is available from the corresponding author.