Abstract
Background Progressive ataxia and palatal tremor (PAPT) is characterized by adult-onset ataxia and palatal tremor typically due to a lesion affecting the Guillain-Mollaret Triangle. This pathway consists of the ipsilateral inferior olivary nucleus and red nucleus, as well as the contralateral cerebellar dentate nucleus. PAPT may be idiopathic or as a result of stroke, tumor, infection or demyelination. Familial causes of PAPT have also been rarely described.
Case A 70 year-old European man presented with a one year history of progressive gait unsteadiness. There were no ocular or bulbar symptoms, hearing loss, tinnitus, vertigo or vomiting. Past medical history was significant for controlled hypertension and an alcohol intake of 21 standard drinks per week. Family history was unremarkable. Examination of his palate revealed oscillatory movements of his soft palate at a frequency of 2 hertz without the sensation of ‘ear clicks’. There was moderate truncal and gait ataxia but no limb ataxia. MRI demonstrated increased T2 and FLAIR hyperintensities in bilateral medullary olives consistent with hypertrophic olivary degeneration. Genetic testing was requested for glial fibrillary acid protein, POLG mutation and spinocerebellar ataxia panels to exclude familial forms of PAPT.
Conclusion We report a patient with the clinical syndrome of PAPT and corresponding MRI findings suggestive of bilateral hypertrophic olivary degeneration. Descriptions of this are limited to case series in the literature. Palatal tremor should be carefully observed for in all patients presenting with ataxia as it can often be asymptomatic.
