Vietnamese patient with progressive ataxia and palatal tremor syndrome

Abstract

Background: We present clinical and MRI features of progressive ataxia and palatal tremor (PAPT). Case Report: A 67 year-old gentleman visiting from Vietnam presented with intermittent stroke-like episodes consisting of facial weakness, dysarthria, and osillopsia. He reported gradually worsening ataxia and dysequilibrium over 4 years. Examination revealed small amplitude nystagmus towards the right, impaired VOR to the left, palatal tremor, left-sided dysmetria, and an unsteady gait. MRI of the brain demonstrated increased T2/FLAIR signal within the inferior olive. Contrast enhanced and diffusion sequences were normal. MRA was normal. Electrocardiography, telemetry, and echocardiogram were normal. CSF was normal. Glial fibrillary acidic protein (GFAP) and vitamin E levels were normal. Genetic testing for hereditary forms of ataxia including spinocerebellar ataxia was not completed. Conclusion: Palatal tremor is commonly classified into symptomatic or essential subgroups. Symptomatic palatal tremor is frequently caused by a lesion in the triangle of Guillain and Mollaret leading to hypertrophic olivary degeneration. A subgroup of symptomatic palatal tremor form a syndrome of PAPT. Published details of cases of PAPT are sparse and the disorder appears to be mainly sporadic. Common features include progression of ataxia, olivary degeneration, gaze-evoked nystagmus, and internuclear ophthalmoplegia. There is no known effective treatment for progressive ataxia, which is the most disabling symptom of PAPT.