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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). 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
Footnotes
Funding information and disclosures are provided at the end of the article. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/cp.
Supplemental data at Neurology.org/cp
- Received May 26, 2016.
- Accepted July 11, 2016.
- © 2016 American Academy of Neurology
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