Preferred Label : Autosomal dominant ataxia;
ICD-11 definition : Autosomal dominant cerebellar ataxia (ADCA) describes a clinically and genetically
heterogeneous group of neurodegenerative diseases characterized by a slowly progressive
ataxia of gait, stance and limbs, dysarthria and/or oculomotor disorder, due to cerebellar
degeneration in the absence of coexisting diseases. The degenerative process can be
limited to the cerebellum (ADCA3) or may additionally involve the retina (ADCA2),
optic nerve, ponto-medullary systems, basal ganglia, cerebral cortex, spinal tracts
or peripheral nerves (ADCA1) or may describe a cerebellar syndrome with the occurrence
of epilepsy (ACDA4).;
ICD-11 synonym : Autosomal dominant cerebellar ataxia;
CISMeF acronym : ADCA;
Origin ID : 782552318;
Automatic exact mappings (from CISMeF team)
- Validated automatic mappings to NTBT
Autosomal dominant cerebellar ataxia (ADCA) describes a clinically and genetically
heterogeneous group of neurodegenerative diseases characterized by a slowly progressive
ataxia of gait, stance and limbs, dysarthria and/or oculomotor disorder, due to cerebellar
degeneration in the absence of coexisting diseases. The degenerative process can be
limited to the cerebellum (ADCA3) or may additionally involve the retina (ADCA2),
optic nerve, ponto-medullary systems, basal ganglia, cerebral cortex, spinal tracts
or peripheral nerves (ADCA1) or may describe a cerebellar syndrome with the occurrence
of epilepsy (ACDA4).
