6D85.1
Dementia due to Huntington disease
Demência devida à doença da Huntington
CategoryDefinition
Dementia due to Huntington disease occurs as part of a widespread degeneration of the brain due to a trinucleotide repeat expansion in the HTT gene, which is transmitted through autosomal dominance. Onset of symptoms is insidious typically in the third and fourth decade of life with gradual and slow progression. Initial symptoms typically include impairments in executive functions with relative sparing of memory, prior to the onset of motor deficits (bradykinesia and chorea) characteristic of Huntington disease.
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.
Diagnostic Criteria
- All diagnostic requirements for Dementia are met.
- Dementia is presumed to be attributable to underlying Huntington Disease, as demonstrated by neuropsychological test data, neuroimaging data, genetic testing, medical tests, family history, and/or clinical history.
- Dementia Due to Huntington Disease occurs as part of a widespread degeneration of the brain due to a trinucleotide repeat expansion in the HTT gene, which is transmitted through autosomal dominance.
- Onset of symptoms is insidious typically in the third and fourth decade of life with gradual and slow progression.
- Initial symptoms typically include impairments in executive functions with relative sparing of memory, prior to the onset of motor deficits (bradykinesia and chorea) characteristic of Huntington Disease.
Note: A diagnosis of 8A01.10 Huntington Disease should also be assigned.
Inclusions
- Dementia in Huntington chorea
Index Terms
Dementia due to Huntington diseaseDementia in Huntington chorea