Progressive ataxia and palatal tremor

A previously healthy 72-year-old man presented with 1 year of progressive ataxia and slurred speech. There was no notable family history and no history of alcohol abuse or neurotropic drug use. Examination showed bilateral hypometric saccades, a 2–3 Hz palatal tremor with face and neck involvement, and truncal ataxia (video at [Neurology.org/cp][1]). There was no pendular nystagmus. MRI brain 6 months after the onset of symptoms showed high signal abnormality in the inferior olivary nuclei of the anterior medulla and mild superior vermal atrophy (figure, A and B). His clinical presentation and neuroimaging were consistent with a diagnosis of progressive ataxia and palatal tremor (PAPT), a rare idiopathic neurodegenerative disease with bulbar features, palatal tremor, and cerebellar ataxia. PAPT imaging features include a typical hypertrophic olivary appearance on MRI, sometimes with cerebellar degeneration.1,2 [1]: http://cp.neurology.org/lookup/doi/10.1212/CPJ.0000000000000298