Dementia due to prion disease (Q40248): Difference between revisions
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Demência devida a doença priônica é uma doença neurodegenerativa primária causada por um grupo de encefalopatias espongiformes resultantes do acúmulo anormal de proteínas priônicas anormais no cérebro. Podem ser esporádicas, genéticas (causadas por mutações no gene da proteína priônica), ou transmissíveis (adquiridas de um indivíduo infectado). O início é insidioso e há uma rápida progressão dos sintomas e comprometimento caracterizado por déficits cognitivos, ataxia, e sintomas motores (mioclonia, coreia, ou distonia). O diagnóstico tipicamente é feito baseado em estudos de imagem cerebral, presença de proteínas características no líquor, EEG, ou testes genéticos. | |||||||||||||||
| description / en | description / en | ||||||||||||||
Dementia due to prion disease is a primary neurodegenerative disease caused by a group of spongiform encephalopathies resulting from abnormal prion protein accumulation in the brain. These can be sporadic, genetic (caused by mutations in the prion-protein gene), or transmissible (acquired from an infected individual). Onset is insidious and there is a rapid progression of symptoms and impairment characterised by cognitive deficits, ataxia, and motor symptoms (myoclonus, chorea, or dystonia). Diagnosis is typically made on the basis of brain imaging studies, presence of characteristic proteins in spinal fluid, EEG, or genetic testing. | |||||||||||||||
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| Property / Canonical URI: https://id.who.int/icd/entity/1206955889 / rank | |||||||||||||||
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CID11:6D85.5 | |||||||||||||||
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dki-india-6D85.5 | |||||||||||||||
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| Property / Knowledge Architect: https://pauloleads.com.br/cases-publicos/ / rank | |||||||||||||||
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13 August 2026
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| Property / Collection date: 13 August 2026 / rank | |||||||||||||||
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| Property / Linked ICD 10: F02.8 / rank | |||||||||||||||
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Latest revision as of 05:58, 13 August 2026
Dementia due to prion disease is a primary neurodegenerative disease caused by a group of spongiform encephalopathies resulting from abnormal prion protein accumulation in the brain. These can be sporadic, genetic (caused by mutations in the prion-protein gene), or transmissible (acquired from an infected individual). Onset is insidious and there is a rapid progression of symptoms and impairment characterised by cognitive deficits, ataxia, and motor symptoms (myoclonus, chorea, or dystonia). Diagnosis is typically made on the basis of brain imaging studies, presence of characteristic proteins in spinal fluid, EEG, or genetic testing.
| Language | Label | Description | Also known as |
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| default for all languages | 6D85.5 |
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| English | Dementia due to prion disease |
Dementia due to prion disease is a primary neurodegenerative disease caused by a group of spongiform encephalopathies resulting from abnormal prion protein accumulation in the brain. These can be sporadic, genetic (caused by mutations in the prion-protein gene), or transmissible (acquired from an infected individual). Onset is insidious and there is a rapid progression of symptoms and impairment characterised by cognitive deficits, ataxia, and motor symptoms (myoclonus, chorea, or dystonia). Diagnosis is typically made on the basis of brain imaging studies, presence of characteristic proteins in spinal fluid, EEG, or genetic testing. |
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CID11:6D85.5
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dki-india-6D85.5
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Concluído
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13 August 2026
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