Awareness of Chorea in Huntington’s Disease

Abstract

BACKGROUND: Anosognosia, or unawareness of illness of deficits, has been observed in Huntington's disease (HD) in relation to motor and cognitive signs and symptoms. Most studies of awareness in HD have used self-report questionnaire methodology rather than asking patients to report on their symptoms in real-time. The two studies in which patients were asked about their chorea in real-time had small sample sizes and only examined patients early in disease progression. OBJECTIVE: To examine awareness of chorea in real-time in HD patients across a broad range of disease progression. METHODS: Fifty HD patients across motor and cognitive impairment severity were asked if they noticed any involuntary movements after completing a simple working memory task used to elicit chorea. A movement disorders specialist rated the presence or absence of chorea while the patients completed the task. Disagreement between the patient and movement disorders specialist's ratings was considered to be an indicator of unawareness. RESULTS: Approximately 46% of patients who exhibited chorea did not report chorea. Eighty-eight percent of participants who acknowledged chorea did not report chorea in all parts of the body that chorea was observed. CONCLUSIONS: HD patients demonstrate unawareness of chorea across cognitive and motor sign severity.