The brain MRI of our patients revealed symmetricalhyperintense lesions of the basal ganglia, mainly putaminaand caudate nuclei, associated with severe and rapid neurodegeneration, which led to the diagnostic workup formitochondrial disease. The cerebellum was initially normal.Cerebellar atrophy, which is a common finding in SCA28,was only observed during follow-up in one of the five patients reported in this study.