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8E03 Variably protease sensitive prionopathy

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

A disease of the brain, caused by a mutation(s) in prion protein genes. This disease is characterised by deposition of abnormal prions in the brain leading to behavioural and mood changes, speech deficits, and progressive motor impairments. Confirmation is by pathological examination of the brain or identification of protease-sensitive prion proteins in a brain sample.

synonyms

• Variably protease sensitive prionopathy
• VPSPr - [Variably protease-sensitive prionopathy]

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