ICD-11 MMS

06 Mental, behavioural or neurodevelopmental disorders

Neurocognitive disorders  (6D70-6E0Z)

Dementia  (6D80-6D8Z)

6D85 Dementia due to diseases classified elsewhere

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

Add Stem and/or Extension codes to form a cluster code which adds detail to the condition.

Associated with  - multiple selections are allowed
Has severity  - only one may be selected
Has causing condition  - a selection is required  - multiple selections are allowed

synonyms

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