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. Neuro imaging 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 tremor[1] with the peculiar neuroimaging finding of hypertrophy and variable hyperintensity of the inferior olivary complex[2].