AWARENESS OF INVOLUNTARY MOVEMENTS IN HUNTINGTON-DISEASE

Objective: To determine why patients with Huntington disease are appar ently unaware of their involuntary movements. Design: Correlative stud y using a subjective report questionnaire of physical symptoms and obj ective measures of neurologic and cognitive dysfunction. Patients: For ty patients with Huntington disease attending a regional Huntington di sease clinic. Results: Patients were poor at reporting experiential sy mptoms of involuntary movements. There was no relationship between sel f-report of these symptoms and objective indices of motor dysfunction or severity of cognitive impairment. Patients could, however, report s econdary consequences of their movement disorder, which correlated hig hly with nonchoreic indices of motor dysfunction. Conclusions: Patient s with Huntington disease have impaired subjective experience of chore a. Denial of symptoms is likely to have a physiological basis and is n ot a secondary consequence of patients' cognitive impairment or a psyc hological defense against a debilitating disease.