Is non-recognition of choreic movements in Huntington disease always pathological?

Clinical experience and prior studies suggest that Huntington disease (HD) patients have low insight into their motor disturbances and poor real-time awareness (concurrent awareness) of chorea. This has been attributed to sensory deficits but, until now, concurrent awareness of choreic movements has...

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Bibliographic Details
Published in:Neuropsychologia 2013-03, Vol.51 (4), p.748-759
Main Authors: Justo, Damian, Charles, Perrine, Daunizeau, Jean, Delmaire, Christine, Gargiulo, Marcela, Hahn-Barma, Valérie, Naccache, Lionel, Durr, Alexandra
Format: Article
Language:English
Subjects:
Adult
Adult and adolescent clinical studies
Aging - psychology
Anosognosia
Attention - physiology
Awareness
Biological and medical sciences
Brain - pathology
Chorea
Chorea - psychology
Cognition - physiology
Concurrent awareness
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Depression - psychology
Female
Heterozygote
Humans
Huntington disease
Huntington Disease - genetics
Huntington Disease - psychology
Image Processing, Computer-Assisted
Insight
Intelligence - physiology
Linear Models
Magnetic Resonance Imaging
Male
Medical sciences
Memory, Short-Term - physiology
Middle Aged
Movement - physiology
Movement Disorders - psychology
Nervous system (semeiology, syndromes)
Nervous system as a whole
Neurology
Neuropsychological Tests
Organic mental disorders. Neuropsychology
Psychiatric Status Rating Scales
Psychology. Psychoanalysis. Psychiatry
Psychopathology. Psychiatry
Recognition (Psychology)
Self Concept
Sex Characteristics
Surveys and Questionnaires
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Summary:Clinical experience and prior studies suggest that Huntington disease (HD) patients have low insight into their motor disturbances and poor real-time awareness (concurrent awareness) of chorea. This has been attributed to sensory deficits but, until now, concurrent awareness of choreic movements has not been compared to the degree of insight that presymptomatic carriers of the HD gene and healthy control subjects have into non-pathological involuntary movements. To further investigate loss of insight into motor dysfunction in HD patients, we administered a video-recorded interview and 4experimental tasks to 68 subjects from the TRACK-HD cohort, including 28 high-functioning patients in early stages of HD, 28 premanifest mutation carriers and 12 controls. All underwent full neurological and neuropsychological evaluations and 3T MRI examinations. Subjects were asked to assess the presence, body location, frequency, practical consequences and probable causes of motor impairments, as well as the presence and body location of involuntary movements during 4experimental tasks. The accuracy of their judgments, assessed by comparison with objective criteria, was used as a measure of their insight into motor disturbances and of their concurrent awareness of involuntary movements. Insight was poor in early HD patients: motor symptoms were nearly always underestimated. In contrast, concurrent awareness of involuntary movements, although also poor, was essentially indistinguishable across the 3groups of subjects: non-pathological involuntary movements were as difficult to perceive by controls and premanifest carriers as was chorea for early HD patients. GLM analysis suggested that both concurrent awareness and perception of practical consequences of movement disorder had a positive effect on intellectual insight, and that mental flexibility is involved in concurrent awareness. Our results suggest that low insight into motor dysfunction in early HD, although marginally modulated by cognitive factors, is mainly non-pathological, and parallels a general tendency, shared by healthy subjects, to neglect self-generated involuntary movements in real time. This tendency, combined with the paucity of functional consequences of incipient chorea, could explain the difficulty of its discovery by the patients. ► Anosognosia for motor trouble was systematic in early HD patients. ► Anosognosia correlated with symptom severity, mental flexibility deficit and striatal atrophy. ► Awaren
ISSN:0028-3932
1873-3514
DOI:10.1016/j.neuropsychologia.2012.12.005