J Med Assoc Thai 1999; 82 (11):1154

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Syndrome of Progressive Ataxia and Palatal Myoclonus : A Case Report
Phanthumchinda K Mail

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 ncurodegenerative
syndrome. The pathogenesis and treatment are still unknown.
Key word : Palatal Tremor, Palatal Myoclonus, Cerebellar Ataxia

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