Variant Creutzfeldt-Jakob disease

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Variant Creutzfeldt-Jakob disease

Synonyms: Variant MCJ | vCJD

A rare acquired human prion disease characterized by a progressive invariably fatal neuropsychiatric disorder resulting from transmission via consumption of products from prion-diseased cows or via blood transfusion from an affected individual. Patients typically present early psychiatric symptoms (such as depression anxiety apathy withdrawal and delusions) as well as persistent painful sensory symptoms ataxia myoclonus chorea or dystonia and dementia. Brain MRI often shows bilateral FLAIR hyperintensities involving the pulvinar thalamic nuclei. Neuropathological examination reveals spongiform change and extensive deposition of abnormal prion protein with florid plaques throughout the cerebrum and cerebellum.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on Data version March 2024

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