Background: Spinocerebellar ataxia type 31 (SCA31) is not usually associated with dementia, and autopsy in a patient with both conditions is very rare.
Case presentation: An 87-year-old male patient presented with ataxia and progressive dementia. Genetic testing led to a diagnosis of SCA31. Fifteen years after his initial symptoms of hearing loss and difficulty walking, he died of aspiration pneumonia. A pathological analysis showed cerebellar degeneration consistent with SCA31 and abundant argyrophilic grains in the hippocampal formation and amygdala that could explain his dementia.
Conclusions: This is the first autopsy report on comorbid argyrophilic grain disease with SCA31.
Keywords: Argyrophilic grain disease; Autopsy; Case report; Spinocerebellar ataxia type 31.