Neuroimaging features of progressive ataxia and palatal tremor.

A 56-year-old man complained about a 6-year-history of gait disturbance and bilateral tinnitus. His neurological family history was unremarkable. Physical examination disclosed gait ataxia, slowly horizontal saccadic pursuit and a 2Hz frequency palatal tremor. Neuroimaging unveiled inferior olivary complex hypertrophy bilaterally and mild cerebellar atrophy (Figure), highly suggestive of progressive ataxia and palatal tremor (PAPT). PAPT represents a rare idiopathic neurodegenerative disease characterized mainly by progressive cerebellar ataxia, variable bulbar dysfunction and symptomatic palatal tremor1 with the peculiar neuroimaging finding of hypertrophy and variable hyperintensity of the inferior olivary complex2.