Syndrome of progressive ataxia and palatal myoclonus: a case report
Phanthumchinda, K.
Journal of the Medical Association of Thailand 82(11): 1154-1157
1999
ISSN/ISBN: 0125-2208 PMID: 10659552 Document Number: 7715
A 46-year old man presented with progressive cerebellar ataxia for 5 years. Physical examination revealed palatal and tongue myoclonus, cerebellar gait, limb ataxia and spasticity of the lower extremities. The imaging studies including CT-scan and MRI of the brain revealed progressive pancerebellar atrophy and bilateral hypertrophic degeneration of inferior olives. The clinical course was slowly progressive. Various medications included anticonvulsants, benzodiazepines and antispasticity failed to abolish the abnormal palatal movement and ataxic syndrome. The syndrome of progressive ataxia and palatal myoclonus is a rare and unique neurodegenerative syndrome. The pathogenesis and treatment are still unknown.
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