Neuropsychiatric aspects of Huntington’s disease.
Open accessAbstract
OBJECTIVE: Neuropsychiatric symptoms are common in Huntington's disease and have been considered its presenting manifestation. Research characterising these symptoms in Huntington's disease is variable, however, encumbered by limitations within and across studies. Gaining a better understanding of neuropsychiatric symptoms is essential, as these symptoms have implications for disease management, prognosis, and quality of life for patients and caregivers. METHOD: Fifty two patients with Huntington's disease were administered standardised measures of cognition, psychiatric symptoms, and motor abnormalities. Patient caregivers were administered the neuropsychiatric inventory. RESULTS: Ninety eight per cent of the patients exhibited neuropsychiatric symptoms, the most prevalent being dysphoria, agitation, irritability, apathy, and anxiety. Symptoms ranged from mild to severe and were unrelated to dementia and chorea. CONCLUSIONS: Neuropsychiatric symptoms are prevalent in Huntington's disease and are relatively independent of cognitive and motor aspects of the disease. Hypothesised links between neuropsychiatric symptoms of Huntington's disease and frontal-striatal circuitry were explored. Findings indicate that dimensional measures of neuropsychiatric symptoms are essential to capture the full range of pathology in Huntington's disease and are vital to include in a comprehensive assessment of the disease.
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Individuals with these disorders report an increase in anxiety.
“Although it is not clear whether abnormal developmental expression of NCoR directly impacts these disorders, it is interesting to note that individuals with these disorders report an increase in anxiety (46 -48).”
From The Nuclear Receptor Corepressor Has Organizational Effects within the Developing Amygdala on Juvenile Social Play and Anxiety-Like Behavior (2010) · cites this paper as [48]
The Neuropsychiatric Symptom Prevalence section reports anxiety in 51.9% of the Huntington’s disease sample. But the Procedure section says caregivers, not the individuals themselves, reported symptoms, and the paper does not establish an increase in anxiety over time or against a comparison group. Page numbers are unavailable in our copy. (LLM, conf=0.88)
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Cites
Kok, J. S., Berg, I. J., & Scherder, E. J. A. (2013) · Dementia and Geriatric Cognitive Disorders Extra · doi:10.1159/000353441 · Cited by 20,589 · see citing works
MACDONALD, M. (1993) · Cell · doi:10.1016/0092-8674(93)90585-e · Cited by 8,366 · see citing works
The Neuropsychiatric Inventory: Comprehensive assessment of psychopathology in dementia
Cummings, J. L., Mega, M., Gray, K., Rosenberg-Thompson, S., Carusi, D. A., & Gornbein, J. (1994) · Neurology · doi:10.1212/wnl.44.12.2308 · Cited by 7,568 · cited 1× · see citing works
“Previous studies have used an array of data collection methods, however, and standardised uniform assessment of psychiatric features in Huntington's disease is lacking. The purpose of the current study is to use the well established neuropsychiatric inventory (NPI”
MethodDistinct cognitive profiles of cortical and subcortical dementia in advanced illness
Paulsen, J. S., Butters, N., Sadek, J. R., Johnson, S. A., Salmon, D. P., Swerdlow, N. R., & Swenson, M. R. (1995) · Neurology · doi:10.1212/wnl.45.5.951 · Cited by 97 · see citing works
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Research works printed but not linked: 26
- Caine ED, Shoulson I. Psychiatric syndromes in Hunting- ton's disease. Am J Psychiatry 1983;140:728-33.
- Cummings JL. Behavioral and psychiatric symptoms associ- ated with Huntington's disease. In: Weiner WJ, Lang AE, eds. Behavioral neurology of movements disorders. New York: Raven Press, 1995:179-86.
- Folstein SE, Folstein MF. Psychiatric features of Hunting- ton's disease: recent approaches and findings [review]. Psy- chiatric Developments 1983;1:193-205.
- Shiwach R. Psychopathology in Huntington's disease patients. Acta Psychiatr Scand 1994;90:241-6.
- Dewhurst K, Oliver JE, McKnight AL. Socio-psychiatric consequences of Huntington's disease. Br J Psychiatry 1970;116:255-8.
- Gutekunst C, Li S, Yi H, et al. Nuclear and neuropil aggre- gates in Huntington's disease: relationship to neuropathol- ogy. J Neurosci 1999;19:2522-34.
- Cha JH, Kosinski CM, Kerner JA, et al. Altered brain neu- rotransmitter receptors in transgenic mice expressing a portion of an abnormal human Huntington disease gene. Proc Natl Acad Sci USA 1998;95:6480-5.
- Nauta WJ, Mehler WR. Projections of the lentiform nucleus in the monkey. Brain Res 1966;1:3-42.
- Swerdlow NR, Koob GF. Lesions of the dorsomedial nucleus of the thalamus, medial prefrontal cortex and pedunculopontine nucleus: eVects on locomotor activity mediated by nucleus accumbens-ventral pallidal circuitry. Brain Res 1987;412:233-43.
- Cummings JL. Frontal-subcortical circuits and human behavior. Neurological Review 1993;50:873-80.
- Vonsattel JP, Difiglia M. Huntington's disease [review]. J Neuropathol Exp Neurol 1998; 57:369-84.
- Middleton FA, Strick PL. Basal ganglia and cerebellar loops: motor and cognitive circuits. Brain Res Rev 2000;31: 236-50.
- Di Maio L, Squitieri F, Napolitano G, et al. Suicide risk in Huntington's disease. J Med Genet 1993;30:293-5.
- Cummings JL, Mega M, Gray K, et al. The neuropsychiat- ric inventory: comprehensive assessment of psychopathol- ogy in dementia. Neurology 1994;44:2308-14.
- Folstein MF, Folstein SE, McHugh PR. Mini-mental state. A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 1975;12:189-98.
- The Huntington Study Group. Unified Huntington's disease rating scale: reliability and consistency. Mov Disord1996;11:136-42.
- Litvan I, Mega MS, Cummings JL, et al. Neuropsychiatric aspects of progressive supranuclear palsy. Neurology 1996; 47:1184-9.
- Litvan I, Paulsen JS, Mega MS, et al. Neuropsychiatric assessment of patients with hyperkinetic and hypokinetic movement disorders. Arch Neurol 1998;55:1313-19.
- Levy ML, Miller BL, Cummings JL, et al. Alzheimer disease and frontotemporal dementias. Behavioral distinctions. Arch Neurol 1996;53:687-90.
- Kaufer D. Beyond the cholingeric hypothesis: the eVect of metrifonate and other cholinesterase inhibitors on neu- ropsychiatric symptoms in Alzheimer's disease. Dement Geriatr Cogn Disord 1998;2:8-14.
- Riso LP, Klein DN, Anderson RL, et al. Concordance between patients and informants on the personality disorder examination. Am J Psychiatry 1994;151:568-73.
- Paulsen JS, Stout JC, Delapena J, et al. Frontal behavioral syndromes in cortical and subcortical dementia. Assessment 1996;3:327-37.
- Levy ML, Cummings JL, Fairbanks LA, et al. Apathy is not depression. J Neuropsychiatry Clin Neurosci 1998;10:314- 19.
- Andrew SE, Goldberg YP, Hayden MR. Rethinking genotype and phenotype correlations in polyglutamine expansion disorders. Hum Mol Genet 1997;6:2005-10.
- Leroi I, Michalon M. Treatment of the psychiatric manifes- tations of Huntington's disease: a review of the literature [review]. Can J Psychiatry 1998;43:933-40.
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Basal-ganglia 'Projections' to the Prefrontal Cortex of the Primate
Middleton, F. A. (2002) · Cerebral Cortex · doi:10.1093/cercor/12.9.926 · Cited by 451 · see citing works
Bacalman, S., Farzin, F., Bourgeois, J. A., Cogswell, J., Goodlin-Jones, B. L., Gane, L. W., Grigsby, J., Leehey, M. A., Tassone, F., & Hagerman, R. J. (2006) · The Journal of Clinical Psychiatry · doi:10.4088/jcp.v67n0112 · Cited by 175 · see citing works
Huntington’s disease: Clinical presentation and treatment
Novak, M. J. U., & Tabrizi, S. J. (2011) · International Review of Neurobiology · doi:10.1016/b978-0-12-381328-2.00013-4 · Cited by 137 · see citing works
O'Callaghan, C., Bertoux, M., & Hornberger, M. (2014) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp-2012-304558 · Cited by 131 · see citing works
(2018) · Handbook of Clinical Neurology · doi:10.1016/b978-0-444-63233-3.00017-8 · Cited by 122 · see citing works
Mazarakis, N. K., Cybulska-Klosowicz, A., Grote, H., Pang, T., Van Dellen, A., Kossut, M., Blakemore, C., & Hannan, A. J. (2005) · The Journal of Neuroscience · doi:10.1523/jneurosci.4320-04.2005 · Cited by 115 · see citing works
Anderson, K. E., van Duijn, E., Craufurd, D., Drazinic, C., Edmondson, M., Goodman, N., van Kammen, D., Loy, C., Priller, J., & Goodman, L. V. (2018) · Journal of Huntington's Disease · doi:10.3233/jhd-180293 · Cited by 108 · see citing works
Anxiety in Huntington’s Disease
Dale, M., & van Duijn, E. (2015) · The Journal of Neuropsychiatry and Clinical Neurosciences · doi:10.1176/appi.neuropsych.14100265 · Cited by 83 · see citing works
Prognostic importance of apathy in syndromes associated with frontotemporal lobar degeneration
Lansdall, C. J., Coyle-Gilchrist, I. T. S., Vázquez Rodríguez, P., Wilcox, A., Wehmann, E., Robbins, T. W., & Rowe, J. B. (2019) · Neurology · doi:10.1212/wnl.0000000000007249 · Cited by 66 · see citing works
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Fritz, N. E., Boileau, N. R., Stout, J. C., Ready, R., Perlmutter, J. S., Paulsen, J. S., Quaid, K., Barton, S., McCormack, M. K., Perlman, S. L., et al. (2018) · The Journal of Neuropsychiatry and Clinical Neurosciences · doi:10.1176/appi.neuropsych.17080173 · Cited by 56 · see citing works
Self-citationJessen, H. M., Kolodkin, M. H., Bychowski, M. E., Auger, C. J., & Auger, A. P. (2010) · Endocrinology · doi:10.1210/en.2009-0594 · Cited by 53 · see citing works
Disordered Decision Making: A Cognitive Framework for Apathy and Impulsivity in Huntington’s Disease
Morris, L., O'Callaghan, C., & Le Heron, C. (2022) · Movement Disorders · doi:10.1002/mds.29013 · Cited by 36 · see citing works
- Authors
- Paulsen, J. S.
- Affiliations
- University of Iowa (US)
- Corresponding author
- Paulsen, J S
- ORCID
- Paulsen, J S
- Venue
- Journal of Neurology, Neurosurgery & Psychiatry
- Publisher
- BMJ
- Published
- 2001-09-01
- Volume
- 71
- Issue
- 3
- Pages
- 310-314
- ISSN
- 0022-3050 (ISSN-L)
- PMC
- PMC1737562
- OpenAlex
- W2133909979
- Cited by
- 486
- References
- 4
- Citations by year
- 2026: 7 · 2025: 17 · 2024: 20 · 2023: 16 · 2022: 12 · 2021: 19 · 2020: 28 · 2019: 25 · 2018: 11 · 2017: 18 · 2016: 23 · 2015: 33 · 2014: 33 · 2013: 26 · 2012: 28
- FWCI
- 3.94
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Journal of Neurology Neurosurgery & Psychiatry (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (5r29mh055331-06, 1r03mh059430-01)
- U.S. Public Health Service (P04 10951-G)
- National Institute on Aging (K08AG100784, T32AG000214, T32AG00214)
- National Institute of Mental Health (R03 MH59430-01, 1K02 MH01579-01, 5R29MH553 31-05, K02MH001579)
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Social Sciences › Psychology › Clinical Psychology › Obsessive-Compulsive Spectrum Disorders
- Health Sciences › Medicine › Neurology › Neurological disorders and treatments
- Keywords
- Huntington's disease, neuropsychiatric symptoms, dysphoria, agitation, irritability, apathy, anxiety, cognitive impairment, chorea, caregiver burden, symptom severity
- MeSH terms
- Adult, Aged, Cognition, Educational Status, Female, Frontal Lobe, Humans, Huntington Disease, Male, Mental Status Schedule, Middle Aged, Motor Skills, Nervous System Diseases, Neuropsychological Tests, Psychomotor Agitation, Severity of Illness Index, Visual Cortex, Prevalence, Neurocognitive Disorders
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