Classical sporadic Creutzfeldt-Jakob Disease (Q85587): Difference between revisions
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A doença priônica humana mais comum, tipicamente caracterizada por demência rapidamente progressiva, ataxia de marcha, mioclonia, mutismo acinético e distúrbio visual. A imagem por ressonância magnética do cérebro, em sequências de imagem ponderada por difusão (DWI), revela hiperintensidade irregular dos córtices cerebrais e núcleos da base. O líquido cefalorraquidiano mostra proteínas 14-3-3 e tau aumentadas. Existem descargas síncronas periódicas (PSD) no eletroencefalograma (EEG). Esta é a DCJ clássica ou típica e sua neuropatologia é caracterizada por encefalopatia espongiforme que consiste em perda neuronal, vacuolização do neurópilo e astrogliose. | |||
| description / en | description / en | ||
The most common human prion disease, typically characterised by rapidly progressive dementia, ataxic gait, myoclonus, akinetic mutism, and visual disturbance. Magnetic resonance imaging of the brain, on diffusion-weighted imaging (DWI) sequences, reveals patchy hyperintensity of the cerebral cortices and basal ganglia. Cerebrospinal fluid shows increased 14-3-3 and tau proteins. There are periodic synchronous discharges (PSD) on electroencephalogram (EEG). This is the classical or typical sCJD and its neuropathology is characterised by spongiform encephalopathy consisting of neuronal loss, neuropil vacuolation, and astrogliosis. | |||
Revision as of 10:56, 15 August 2026
The most common human prion disease, typically characterised by rapidly progressive dementia, ataxic gait, myoclonus, akinetic mutism, and visual disturbance. Magnetic resonance imaging of the brain, on diffusion-weighted imaging (DWI) sequences, reveals patchy hyperintensity of the cerebral cortices and basal ganglia. Cerebrospinal fluid shows increased 14-3-3 and tau proteins. There are periodic synchronous discharges (PSD) on electroencephalogram (EEG). This is the classical or typical sCJD and its neuropathology is characterised by spongiform encephalopathy consisting of neuronal loss, neuropil vacuolation, and astrogliosis.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | ID_1855860109 |
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| English | Classical sporadic Creutzfeldt-Jakob Disease |
The most common human prion disease, typically characterised by rapidly progressive dementia, ataxic gait, myoclonus, akinetic mutism, and visual disturbance. Magnetic resonance imaging of the brain, on diffusion-weighted imaging (DWI) sequences, reveals patchy hyperintensity of the cerebral cortices and basal ganglia. Cerebrospinal fluid shows increased 14-3-3 and tau proteins. There are periodic synchronous discharges (PSD) on electroencephalogram (EEG). This is the classical or typical sCJD and its neuropathology is characterised by spongiform encephalopathy consisting of neuronal loss, neuropil vacuolation, and astrogliosis. |
