ICD-11 code : 8A44.2;
Preferred Label : Alexander disease;
ICD-11 definition : Alexander's disease is a neurodegenerative disorder encompassing different clinical
forms: the infantile form (birth to 2 years), the most common, is characterized by
its early onset and severe evolution with progressive megalencephaly (sometimes hydrocephaly),
retarded psychomotor development or mental deterioration, pyramidal signs, ataxia
and convulsive seizures. The juvenile forms start in school-aged children and associate
spastic paraplegia and progressive bulbar signs. Adult forms are heterogeneous and
difficult to diagnose.;
ICD-11 synonym : Alexander's leukodystrophy;
Origin ID : 2023359698;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
Alexander's disease is a neurodegenerative disorder encompassing different clinical
forms: the infantile form (birth to 2 years), the most common, is characterized by
its early onset and severe evolution with progressive megalencephaly (sometimes hydrocephaly),
retarded psychomotor development or mental deterioration, pyramidal signs, ataxia
and convulsive seizures. The juvenile forms start in school-aged children and associate
spastic paraplegia and progressive bulbar signs. Adult forms are heterogeneous and
difficult to diagnose.
