Familial Alzheimer-like prion disease (Q91713): Difference between revisions
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Doença priônica familiar semelhante a Alzheimer é uma forma extremamente rara de doença priônica caracterizada pelas características neuropatológicas da doença de Alzheimer, incluindo comprometimento da memória e depressão, relacionada à proteína príon anormal (PrP) causada por uma variante patogênica no gene PRNP. Os pacientes apresentam um curso prolongado e atípico (ausência de mioclonia ou ataxia), diferente de outras formas de doença priônicas com patologia grave de emaranhado neurofibrilar e altos níveis de amiloidose cerebral. | |||||||||||||||
| description / en | description / en | ||||||||||||||
Familial Alzheimer-like prion disease is an exceedingly rare form of prion disease characterized by the neuropathological features of Alzheimer disease including memory impairment and depression, related to abnormal prion protein (PrP) caused by a pathogenic variant in the PRNP gene. Patients present with a prolonged, atypical course (absence of myoclonus or ataxia) unlike other forms of prion disease with severe neurofibrillary tangle pathology and high levels of cerebral amyloidosis. | |||||||||||||||
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| Property / Canonical URI: https://id.who.int/icd/entity/1297025427 / rank | |||||||||||||||
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CID11:ID_1297025427 | |||||||||||||||
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dki-india-ID_1297025427 | |||||||||||||||
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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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Latest revision as of 18:44, 15 August 2026
Familial Alzheimer-like prion disease is an exceedingly rare form of prion disease characterized by the neuropathological features of Alzheimer disease including memory impairment and depression, related to abnormal prion protein (PrP) caused by a pathogenic variant in the PRNP gene. Patients present with a prolonged, atypical course (absence of myoclonus or ataxia) unlike other forms of prion disease with severe neurofibrillary tangle pathology and high levels of cerebral amyloidosis.
| Language | Label | Description | Also known as |
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| default for all languages | ID_1297025427 |
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| English | Familial Alzheimer-like prion disease |
Familial Alzheimer-like prion disease is an exceedingly rare form of prion disease characterized by the neuropathological features of Alzheimer disease including memory impairment and depression, related to abnormal prion protein (PrP) caused by a pathogenic variant in the PRNP gene. Patients present with a prolonged, atypical course (absence of myoclonus or ataxia) unlike other forms of prion disease with severe neurofibrillary tangle pathology and high levels of cerebral amyloidosis. |
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CID11:ID_1297025427
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dki-india-ID_1297025427
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Concluído
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13 August 2026
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