A form of progressive myoclonic epilepsy
Clinical features:
Myoclonus, dementia, ataxia
Neuropathy, choreoathetosis
Choreoathetosis appears when myoclonus is suppressed & vice versa
Pathology:
Autonomic terminals: Axonal spheroids
A form of progressive myoclonic epilepsy
Myoclonus, dementia, ataxia
Neuropathy, choreoathetosis
Choreoathetosis appears when myoclonus is suppressed & vice versa
Autonomic terminals: Axonal spheroids