Alexander disease (Q41170): Difference between revisions

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Latest revision as of 07:13, 13 August 2026

Alexander's disease is a neurodegenerative disorder encompassing different clinical forms: the infantile form (birth to 2 years), the most common, is characterised 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.

Language	Label	Description	Also known as
default for all languages	8A44.2
English	Alexander disease	Alexander's disease is a neurodegenerative disorder encompassing different clinical forms: the infantile form (birth to 2 years), the most common, is characterised 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.