Ataxia - oculomotor apraxia type 1 AOA1 ICD10:G11.3 Ataxia-oculomotor apraxia type 1 (AOA1) is a form of autosomal recessive cerebellar ataxia (ARCA; see this term) characterized by a progressive cerebellar ataxia associated with oculomotor apraxia, choeroathetosis and severe peripheral neuropathy. OMIM:208920 orphanet Mitochondrial disease with peripheral neuropathy Mitochondrial oxidative phosphorylation disorder due to nuclear DNA anomalies Autosomal recessive cerebellar ataxia due to a DNA repair defect Oculomotor apraxia or related oculomotor disease