Emotion recognition in Huntington's disease and frontotemporal dementia

• Published in: Neuropsychologia 2008-09, Vol.46 (11), p.2638-2649
• Main Authors: Snowden, J.S., Austin, N.A., Sembi, S., Thompson, J.C., Craufurd, D., Neary, D.
• Format: Article
• Language: English
• Subjects: Adult; Adult and adolescent clinical studies; Affect; Aged; Anger; Biological and medical sciences; Choice Behavior - physiology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Degenerative disease; Dementia - physiopathology; Disgust; Emotions - physiology; Facial Expression; Female; Humans; Huntington Disease - physiopathology; Male; Medical sciences; Middle Aged; Nervous system (semeiology, syndromes); Nervous system as a whole; Neurology; Neuropsychological Tests; Organic mental disorders. Neuropsychology; Pattern Recognition, Visual; Photic Stimulation - methods; Psychology. Psychoanalysis. Psychiatry; Psychopathology. Psychiatry; Reaction Time - physiology; Recognition (Psychology) - physiology; Statistics, Nonparametric
• ISSN: 0028-3932; 1873-3514
• DOI: 10.1016/j.neuropsychologia.2008.04.018

A well-documented feature of Huntington's disease (HD) is disproportionate impairment in the ability to recognise the emotional expression of disgust. However, this finding has been challenged by studies that report no differential disgust impairment and attribute apparent differences across emotions to task difficulty. The present study sought to shed light on disparities in findings through a comparative study of emotion recognition in HD and frontotemporal dementia (FTD). Ten HD, 12 FTD patients and 12 healthy controls were administered 10 tasks assessing facial and vocal recognition of emotions and comprehension of emotion terms. The findings were not consistent with either the ‘selective disgust impairment’ or ‘task difficulty’ view. Both HD and FTD groups were impaired compared to controls, deficits in HD being less severe. Impairments in FTD were elicited for all emotions whereas in HD they were demonstrated predominantly for negative emotions of fear, disgust and anger. Consistency in performance, despite varying task demands, excluded an explanation in terms of item difficulty, and was in keeping with the notion of distinct neural substrates for processing of negative emotions. Contrary to the notion of disproportionate disgust impairment, the most severe deficits in HD were elicited for anger, a finding that may have relevance for the poor anger control that is the hallmark of HD. The data raise the possibility that linguistic influences and conceptual complexities of the emotion of disgust may contribute to the variable finding of selective disgust impairment in HD.