Variably protease sensitive prionopathy

Description

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.

Index terms

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

Exclusions from above levels

05 – Endocrine, nutritional or metabolic diseases
18 – Complications of pregnancy, childbirth and the puerperium
19 – Certain conditions originating in the perinatal period

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22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

8E00 – Sporadic Creutzfeldt-Jakob Disease
8E01 – Acquired prion disease
8E02 – Genetic prion diseases
8E0Y – Other specified human prion diseases
8E0Z – Human prion diseases, unspecified

Foundation entity

Variably protease sensitive prionopathy – http://id.who.int/icd/entity/172957869

Part of: 08: Diseases of the nervous system