Spinocerebellar ataxia with epilepsy

Orpha code: 254881OMIM code: 607459

Definition

A rare, mitochondrial DNA maintenance syndrome characterized by cerebellar ataxia, sensory peripheral neuropathy, myoclonus, epilepsy, progressive cognitive impairment, late-onset ptosis and external ophthalmoplegia. Liver failure may also occur, most often in association with the use of antiepileptic drug sodium valproate.

Disease data
Classification

Disease

Synonyms
MSCAE
Mitochondrialna ataksja rdzeniowo-móżdżkowa z padaczką
MSCAE
SCAE
Mitochondrial spinocerebellar ataxia with epilepsy
SCAE
ORPHA code
254881
OMIM code
607459
ICD10 code
E88.8
ICD11 code
5C53.21

No additional description.

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