Catalog
Variably protease sensitive prionopathy
Link to WHO Definition
Variably protease sensitive prionopathy – http://id.who.int/icd/entity/172957869
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.