Alexander disease (Q41170): Difference between revisions
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| description / pt-br | description / pt-br | ||
Doença de Alexander é um transtorno neurodegenerativo que abrange diferentes formas clínicas. A forma infantil (do nascimento aos 2 anos), a mais comum, é caracterizada por início precoce e evolução grave com megalencefalia (algumas vezes hidrocefalia), atraso do desenvolvimento psicomotor ou declínio cognitivo, sinais piramidais, ataxia e crises convulsivas. A forma juvenil inicia-se na fase escolar da criança, e se associa a paraplegia espástica e sinais bulbares pogressivos. A forma adulta é heterogênea e de díficil diagnóstico. | |||
| description / en | description / en | ||
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. | |||
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 |
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| 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. |
