6D85.5 Dementia due to prion disease

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

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.

postcoordination

synonyms

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

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