MSA-C presenting with leukencephalopathy

We present a 55-year-old female with cerebellar signs including gait ataxia, coordination impairment, oculomotor in combination with neurogenic bladder dysfunction indicating multiple system atrophy of the cerebellar type without clinical signs of parkinsonism. MRI scans revealed cerebellar atrophy but no pathologies within basal ganglia or mesencephalon. 2-year follow-up scans showed severe leukencephalopathy. Leukencephalopathy with axonal spheroids (LENAS) presenting as MSA-C has been described before. In contrast, biopsy in our case did neither uncover axonal loss nor demyelination but oligodendrocytic accumulation of synuclein restricted to the white matter that has not yet been reported in the literature.