Psychopathology in Verified Huntington's Disease Gene Carriers

• Published in: The journal of neuropsychiatry and clinical neurosciences Vol. 19; no. 4; pp. 441 - 448
• Main Authors: van Duijn, E, Kingma, E.M, van der Mast, R.C
• Format: Journal Article
• Language: English
• Published: Washington, DC American Psychiatric Publishing, Inc 01.10.2007; American Psychiatric Press
• Subjects: Adult and adolescent clinical studies; Anxiety - epidemiology; Anxiety - etiology; Anxiety - psychology; Biological and medical sciences; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Depression - epidemiology; Depression - etiology; Depression - psychology; DNA Repeat Expansion - genetics; Heterozygote; Humans; Huntington Disease - epidemiology; Huntington Disease - genetics; Huntington Disease - psychology; Irritable Mood - physiology; Medical sciences; Mental Disorders - epidemiology; Mental Disorders - genetics; Mental Disorders - psychology; Nervous system (semeiology, syndromes); Nervous system as a whole; Neurology; Neuropsychological Tests; Obsessive-Compulsive Disorder - epidemiology; Obsessive-Compulsive Disorder - etiology; Obsessive-Compulsive Disorder - psychology; Organic mental disorders. Neuropsychology; Psychiatric Status Rating Scales; Psychology. Psychoanalysis. Psychiatry; Psychopathology. Psychiatry; Psychotic Disorders - epidemiology; Psychotic Disorders - etiology; Psychotic Disorders - psychology; Reproducibility of Results; Nervous system diseases; Gene; Central nervous system disease; Huntington disease; Genetics; Degenerative disease; Psychopathology; Genetic determinism; Genetic disease; Cerebral disorder; Extrapyramidal syndrome
• ISSN: 0895-0172; 1545-7222
• DOI: 10.1176/jnp.2007.19.4.441

Huntington's disease is characterized by motor, cognitive, and neuropsychiatric symptoms. This study reviews original research on psychopathology in Huntington's disease that uses standardized instruments in verified gene carriers. Frequently reported neuropsychiatric symptoms are depressed mood, anxiety, irritability, and apathy, with prevalences of 33% to 76%. Obsessive-compulsive symptoms and psychosis occur less often with prevalences of 10% to 52% and 3% to 11%, respectively. Available research provides little insight into the true prevalences of psychopathology in Huntington's disease due to small sample sizes, use of different methodologies, and lack of comparison groups. Future research requires larger cohorts stratified to disease stage, consistent methodologies, and adequate comparison groups.