How Early Does Anosognosia Begin in Huntington's Disease?

Individuals with Huntington's disease (HD) commonly experience anosognosia, a lack of awareness of disease-specific deficits. However, how early anosognosia begins in the course of HD remains unclear. To characterize the onset of anosognosia in HD by exploring its presence/absence at various stages of disease. The Anosognosia Scale was given to 60 gene carriers (stratified by the HD Integrated Staging System (HD-ISS), Total Functional Capacity (TFC), CAG-Age-Product (CAP), and composite UHDRS (cUHDRS) score) as well as their study partners. Differences between informant and patient scores of ≥6 points were considered evidence of anosognosia. 18% of participants in HD-ISS stage 0/1, 22% with TFC = 13, 37% who would be eligible for clinical trials (CAP score 400-500), and 23% with a cUHDRS ≥14 displayed anosognosia. We conclude that anosognosia appears early in HD. This lack of awareness should dissuade against over-reliance on self-report instruments in HD clinical research.