title: Unawareness of deficits in Huntington's disease.
abstract: People with Huntington's disease (HD) may show reduced awareness of physical and mental changes in themselves. This article reviews the evidence for loss of awareness (anosognosia) in an attempt to elucidate its characteristics and possible underlying mechanisms. It is shown that defective awareness occurs across domains. People with HD may under-report the presence or severity of involuntary movements, under-estimate cognitive impairment and deny behavioural change. Nevertheless, awareness is not all or none. Moreover, it may be affected differentially for different symptom domains and emerge at different stages of disease, raising the possibility of distinct contributory mechanisms. Findings of an inverse relationship between insight and severity of disease suggest that cognitive impairment, in particular executive dysfunction, may be an important contributory factor. Evidence has accrued to support this argument. However, cognitive impairment cannot fully account for patients' lack of awareness of involuntary movements. Findings that patients accurately report consequences but not the experience of involuntary movements, and better acknowledge their presence when watching videotapes of themselves suggests that physiological factors play an important role. The putative role of denial as a coping mechanism is discussed. Recognition by clinicians of deficient self-awareness is crucial because of its implications for diagnosis and optimal clinical management of HD.
authors: Department of Neurology, St. Adalbert Hospital, Gdansk, Poland Department of Neurological and Psychiatric Nursing, Medical University of Gdansk, Gdansk, Poland.
authors: Emilia J
authors: EJ
authors: Sitek
authors: Cerebral Function Unit, Greater Manchester Neuroscience Centre, Salford Royal NHS Foundation Trust, Salford, UK Institute of Brain, Behaviour and Mental Health, Faculty of Medical and Human Sciences, University of Manchester, Manchester, UK.
authors: Jennifer C
authors: JC
authors: Thompson
authors: Institute of Human Development, Faculty of Medical and Human Sciences, University of Manchester, Manchester, UK Manchester Centre for Genomic Medicine, Central Manchester University Hospitals NHS Foundation Trust and Manchester Academic Health Science Centre, Manchester, UK.
authors: David
authors: D
authors: Craufurd
authors: Cerebral Function Unit, Greater Manchester Neuroscience Centre, Salford Royal NHS Foundation Trust, Salford, UK Institute of Brain, Behaviour and Mental Health, Faculty of Medical and Human Sciences, University of Manchester, Manchester, UK.
authors: Julie S
authors: JS
authors: Snowden
mesh_terms: D000377:Agnosia / Q000209:etiology* / Q000503:physiopathology* / Q000523:psychology; D001364:Awareness / Q000502:physiology*; D006801:Humans; D006816:Huntington Disease / Q000150:complications* / Q000503:physiopathology* / Q000523:psychology
keywords: Huntington disease; anosognosia; awareness; chorea