Myoclonic Epilepsia Partialis Continua and Friedreich Ataxia

Abstract

Clinical features and pathologic findings of slowly progressive spinocerebellar disease were found in a woman with no family history of the disorder. There was a history of infrequent seizures until pregnancy when she developed a continuous myoclonus, intractable to medication and with features of rhythmicity and asymmetry, suggesting epilepsia partialis continua. The patient died after aspiration. Pathologic findings included atrophy of the dentate nucleus and superior cerebellar peduncle and loss of Purkinje cells. Possibly, the release (disinhibition) of epileptic phenomena was generated by massive damage to the cerebellum.