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Awareness of involuntary movements in Huntington disease
J S Snowden1, D Craufurd, H L Griffiths
1Department of Neurology, Manchester Royal Infirmary, England.
Objective:
To determine why patients with Huntington disease are apparently unaware of their involuntary movements.
Design:
Correlative study using a subjective report questionnaire of physical symptoms and objective measures of neurologic and cognitive dysfunction.
Patients:
Forty patients with Huntington disease attending a regional Huntington disease clinic.
Results:
Patients were poor at reporting experiential symptoms of involuntary movements. There was no relationship between self-report of these symptoms and objective indices of motor dysfunction or severity of cognitive impairment. Patients could, however, report secondary consequences of their movement disorder, which correlated highly with nonchoreic indices of motor dysfunction.
Conclusions:
Patients with Huntington disease have impaired subjective experience of chorea. Denial of symptoms is likely to have a physiological basis and is not a secondary consequence of patients' cognitive impairment or a psychological defense against a debilitating disease.