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The worldwide prevalence is estimated to be 2.7 per 100,000.
The genetic basis of Huntington's disease is a fully penetrant autosomal dominant expansion of the CAG trinucleotide, often called a CAG repeat in the huntingtin gene.
A CAG repeat length of 36 or more is invariably associated with Huntington's disease, with longer repeat lengths associated with early age at onset.
The criteria are met for major or mild neurocognitive disorder.
There is insidious onset and gradual progression.
There is clinically established Huntington’s disease, or risk for Huntington’s disease based on family history or genetic testing.
The neurocognitive disorder is not attributable to another medical condition and is not better explained by another mental disorder.