Emotion recognition and experience in Huntington's disease: is there a differential impairment?

Findings on affective processing deficits in Huntington's disease (HD) have been inconsistent. It is still not clear whether HD patients are afflicted by specific deficits in emotion recognition and experience. We tested 28 symptomatic HD patients and presented them with pictures depicting facial expressions of emotions (Karolinska-Set) and with affective scenes (International Affective Picture System; IAPS). The faces were judged according to the displayed intensity of six basic emotions, whereas the scenes received intensity ratings for the elicited emotions in the viewer. Patients' responses were compared with those of 28 healthy controls. HD patients gave lower intensity ratings for facial expressions of anger, disgust and surprise than controls. Patients' recognition deficits were associated with reduced functional capacity, such as problems with social interactions. Moreover, their classification accuracy was reduced for angry, disgusted, sad and surprised faces. When judging affective scenes for the elicitation of happiness, disgust and fear, HD patients had a tendency to estimate them as more intense than controls. This finding points to a differential impairment in emotion recognition and emotion experience in HD. We found no significant correlations between emotion experience/recognition ratings and CAG repeats, symptom duration and UHDRS Motor Assessment in the patient group.

Impaired ideomotor limb apraxia in cortical and subcortical dementia: a comparison of Alzheimer's and Huntington's disease.

BACKGROUND: Although ideomotor limb apraxia is often considered to occur only in dementia with cortical involvement like Alzheimer's disease (AD), it is also frequently seen in dementia with subcortical degeneration like Huntington's disease (HD). METHODS: To assess the occurrence of ideomotor limb apraxia, 46 patients with HD (27 men) and 37 patients with AD (16 men), matched for cognitive performance, were assessed with an apraxia test battery containing tests of the imitation of meaningless hand and finger gestures, the performance of meaningful gestures and of pantomimic movements. RESULTS: There was a high frequency of ideomotor limb apraxia in both AD and HD patients. For the assessment of hands' imitation 13.5% of the AD patients and 41.3% of the HD patients were apraxic, for fingers' imitation 21.6% (AD) and 41.3% (HD) were apraxic, for gestures 27.0% (AD) and 32.6% (HD), and for the assessment of pantomimic movements 24.3% (AD) and 52.2% (HD) showed apraxia. In the AD patients, disease severity was related to the occurrence of apraxia. CONCLUSIONS: Ideomotor limb apraxia is a common sign in both groups of patients, occurring in a high percentage. For particular neuropsychological deficits, including ideomotor limb apraxia, a division of dementia in a subcortical and cortical subtype seems to be clinically not meaningful.