ICD-11 classes
08 Diseases of the nervous system
Movement disorders
8A03 — Ataxic disorders
8A03.1 — Hereditary ataxia
8A03.14 — Hereditary episodic ataxia

ICD-11 8A03.14 — Hereditary episodic ataxia

Autosomal dominant disorders associated with intermittent episodes of cerebellar dysfunction, with normal functioning or minimal ataxia and nystagmus between episodes. The two major subtypes include EA1 and and EA2. EA1 is caused by a mutation of the KCNA1 gene coding and characterized by episodes triggered by exercise and muscle myokymia. EA2 is caused by a mutation in CACNA1A gene and involves more prolonged attacks of ataxia (lasting hours to days), and interictal residual ataxia with nystagmus.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.