ICD·index Chapters ENESDE
6D85.5 category

Dementia due to prion disease

Definition

Dementia due to prion disease is a primary neurodegenerative disease caused by a group of spongiform encephalopathies resulting from abnormal prion protein accumulation in the brain. These can be sporadic, genetic (caused by mutations in the prion-protein gene), or transmissible (acquired from an infected individual). Onset is insidious and there is a rapid progression of symptoms and impairment characterised by cognitive deficits, ataxia, and motor symptoms (myoclonus, chorea, or dystonia). Diagnosis is typically made on the basis of brain imaging studies, presence of characteristic proteins in spinal fluid, EEG, or genetic testing.

Coding note

This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere. When dementia is due to multiple aetiologies, code all that apply.

Also indexed as

Dementia due to FFI - [fatal familial insomnia]Dementia due to GSS - [Gestmann-Staussler-Sheinker syndrome]Dementia due to Gerstmann-Staussler-Sheinker syndromeDementia due to KuruDementia due to SFI - [sporadic fatal insomnia]Dementia due to fCJD - [familial Creutzfeldt-Jakob disease]Dementia due to familial Creutzfeldt-Jakob diseaseDementia due to fatal familial insomniaDementia due to iCJD - [iatrogenic Creutzfeldt-Jakob disease]Dementia due to iatrogenic Creutzfeldt-Jakob diseaseDementia due to prion diseaseDementia due to sporadic Creutzfeldt-Jakob diseaseDementia due to sporadic fatal insomniaDementia due to vCJD - [variant Creutzfeldt-Jakob disease]Dementia due to variant Creutzfeldt-Jakob disease

Nearby in Dementia due to diseases classified elsewhere