Preview

Neurology, Neuropsychiatry, Psychosomatics

Advanced search

Mental disorders in Huntington’s disease

https://doi.org/10.14412/2074-2711-2012-2508

Full Text:

Abstract

untington’s disease (HD) is one of the most severe and fatal hereditary diseases of the nervous system. The paper outlines the history of studies of the disease, its key features and development mechanisms in terms of recent advances in molecular biology. The neuropsychiatric manifestations of HD, which are a major disabling factor, are characterized. Mental and emotional-volitional disorders are systematized; possible methods for correction of the clinical manifestations of HD are presented. A special emphasis is made on the fact that cooperation between different specialists and with a patient’s relatives is of importance in achieving the best treatment results. The authors present their experience in studying the psychopathological manifestations of HD at the Neurology Research Center, Russian Academy of Medical Sciences, and the results of an integrated study of cognitive and neuropsychological characteristics of at-risk asymptomatic HD gene carriers, by using a battery of psychological tests and the newest methods of electroneurophysiology and neuroimaging, and conclude that there is a preclinical disease stage. The authors’ experience with pharmacotherapy for neuropsychiatric manifestations and cognitive deficit in HD patients is described.

About the Authors

S. A. Klyushnikov
N.N. Burdenko Institute of Neurosurgery, Russian Academy of Medical Sciences, Moscow
Russian Federation


E. N. Yudina
N.N. Burdenko Institute of Neurosurgery, Russian Academy of Medical Sciences, Moscow
Russian Federation


S. N. Illarioshkin
N.N. Burdenko Institute of Neurosurgery, Russian Academy of Medical Sciences, Moscow
Russian Federation


I. A. Ivanova-Smolenskaya
N.N. Burdenko Institute of Neurosurgery, Russian Academy of Medical Sciences, Moscow
Russian Federation


References

1. <div><p>Huntington G. On chorea. Med Surg Rep (Philadelphia) 1872;26:317−21.</p><p>Bates G., Harper P., Jones L. Huntington's Disease. New York: Oxford University Press, 2002;558.</p><p>Folstein S.E. Huntington’s disease: a disorder of families. Baltimore: The Johns Hopkins University Press, 1989;251.</p><p>Gusella J.F., Wexler N.S., Conneally P.M. et al. A polymorphic DNA marker genetically linked to HD. Nature 1983;306:234−8.</p><p>Wexler N.S., Lorimer J., Porter J. et al. Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset. Proc Natl Acad Sci USA 2004;101:3498−503.</p><p>The Huntington's Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 1993;72:971–83.</p><p>Иллариошкин С.Н., Иванова-Смоленская И.А., Маркова Е.Д. и др. Анализ экспансии тринуклеотидных повторов как нового механизма мутации при хорее Гентингтона: теоретические и прикладные аспекты. Генетика 1996;32:103–9.</p><p>Warby S.C., Montpetit A., Hayden A.R. et al. CAG expansion in the Huntington disease gene is associated with a specific and targetable predisposing haplogroup. Am J Hum Genet 2009;84:351–66.</p><p>Andrew S.E., Goldberg Y.P., Kremer B. et al. The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington’s disease. Nature Genet 1993;4:398−403.</p><p>Langbehn D.R., Hayden M.R., Paulsen J.S. CAG-repeat length and the age of onset in Huntington disease (HD): a review and validation study of statistical approaches. Am J Med Genet B Neuropsychiatr Genet 2010;153B:397–408.</p><p>Aziz N.A., Jurgens C.K., Landwehrmeyer G.B. Normal and mutant HTT interact to affect clinical severity and progression in Huntington disease. Neurology 2009;73:1280–5.</p><p>Illarioshkin S.N., Igarashi S., Onodera O. et al. Trinucleotide repeat length and rate of progression in Huntington's disease. Ann Neurol 1994;36:630−5.</p><p>Ross C.A. When more is less: pathogenesis of glutamine repeat neurodegenerative diseases. Neuron 1995;15:493−6.</p><p>Ross C.A., Tabrizi S.J. Huntington's disease: from molecular pathogenesis to clinical treatment. Lancet Neurol 2011;10:83–98.</p><p>Paulson H.L. Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold. Am J Hum Genet. 1999;64:339–45.</p><p>Иллариошкин С.Н. Конформационные болезни мозга. М.: Янус-К, 2003;248 с.</p><p>Клюшников С.А., Иванова-Смоленская И.А., Никольская Н.Н. и др. Этические проблемы медико-генетического консультирования на примере хореи Генгтингтона. РМЖ 2000;2:326.</p><p>Cowan C.M., Raymond L.A. Selective neuronal degeneration in Huntington's disease. Curr Top Dev Biol 2006;75:25–71.</p><p>Albert M.L. Subcortical dementia. In: Alzheimer’s disease: Senile Dementia and Related Disorders. New York: Raven Press, 1978;7:173–80.</p><p>Brandt J., Folstein S.E., Folstein M.F. Differential cognitive impairment in Alzheimer's disease and Huntington's disease. Ann Neurol 1988;23:555–61.</p><p>Burns A., Folstein S.E., Brandt J. et al. Clinical assessment of irritability, aggression, and apathy in Huntington and Alzheimer disease. J Nerv Ment Dis 1990;178:20–6.</p><p>Яхно Н.Н. Актуальные вопросы нейрогериатрии. В кн.: Достижения в нейрогериатрии. Под ред. Н.Н. Яхно, И.В. Дамулина. М.: ММА, 1995;9–29.</p><p>Arango-Lasprilla J.C., Rogers H., Lengenfelder J. et al. Cortical and subcortical diseases: do true neuropsychological differences exist? Arch Clin Neuropsychol 2006;21:29–40.</p><p>Rosenblatt A. Neuropsychiatry of Huntington's disease. Dialogues Clin Neurosci 2007;9:191–7.</p><p>Клюшников С.А. Диагностика хореи Гентингтона на доклинической стадии и при атипичных вариантах заболевания (клинические и молекулярно-генетические сопоставления). Автореф дисс … канд мед наук. М., 1998;28 с.</p><p>Brandt J., Shpritz B., Codori A.M. et al. Neuropsychological manifestations of the genetic mutation for Huntington's disease in presymptomatic individuals. J Int Neuropsychol Soc 2002;8:918–24.</p><p>Duff K., Paulsen J.S., Mills J. et al. Mild cognitive impairment in prediagnosed Huntington disease. Neurology 2010;75(6):500–7.</p><p>Shiwach R. Psychopathology in Huntington’s disease in patients. Acta Psychiatr Scand 1994;90:241–6.</p><p>Paulsen J.S., Ready R.E., Hamilton J.M. et al. Neuropsychiatric aspects of Huntington’s disease. J Neurol Neurosurg Psychiatry 2001;71:310–4.</p><p>Shoenfield M., Myers R.H., Cupples R.A. et al. Increased rate of suicide among patients with Huntington’s disease. J Neurol Neurosurg Psychiatry 1984;47:1283–7.</p><p>Lipe H., Schultz A., Bird T.D. Risk factors for suicide in Huntingtons disease: a retrospective case controlled study. Am J Med Genet 1993;48:231–3.</p><p>Mayberg H.S., Starkstein S.E., Peyser C.E. et al. Paralimbic frontal lobe hypometabolism in depression associated with Huntington’s disease. Neurology 1992;42:1791–7.</p><p>Baxter L.R., Mazziotta J.C., Pahl J.J. et al. Psychiatric, genetic, and positron emission tomographic evaluation of persons at risk for Huntington’s disease. Arch Gen Psychiatry 1992;49:148–52.</p><p>Mendez M.F. Huntington’s disease: update and review of neuropsychiatric aspects. Int J Psychiatry Med 1994;24:189–208.</p><p>Craufurd D., Thompson J.C., Snowden J.S. Behavioral changes in Huntington’s disease. Neuropsychiatry Neuropsychol Behav Neurol 2001;14:219–26.</p><p>Cummings J.L., Mega M., Gray K. et al. The Neuropsychiatric inventory: comprehensive assessment of psychopathology in dementia. Neurology 1994;44:2308–14.</p><p>Marder K., Zhao H., Myers R. et al. Rate of functional decline in Huntington’s disease. Huntington Study Group. Neurology 2000;54:452–8; correction, 54:1712.</p><p>Anderson K., Louis E.D., Stern Y. et al. Cognitive correlates of obsessive and compulsive symptoms in Huntington’s disease. Am J Psychiatry 2001;158:799–801.</p><p>Cummings J.L., Benson D.F. Psychological dysfunction accompanying subcortical dementias. Annu Rev Med 1988;39:53–61.</p><p>Lyketsos C.G., Rosenblatt A., Rabins P.V. Forgotten frontal lobe syndrome or “executive dysfunction syndrome.” Psychosomatics 2004;45:247–55.</p><p>Rosenblatt A., Anderson K., Goumeniouk A.D. et al. Clinical management of aggression and frontal syndromes in Huntington’s disease. In: Mental and Behavioral Dysfunction in Movement Disorders. Totowa, NJ: Humana, 2002:427–41.</p><p>Лурия А.Р. Высшие корковые функции человека и их нарушения при локальных поражениях мозга. 2-е изд. М., 1969;504 с.</p><p>Хомская Е. Д. Нейропсихология. 4-е изд. СПб.: Питер, 2005;496 с.</p><p>Pennington B.F., Ozonoff S. Executive functions and developmental psychopathology. J Child Psychol Psychiatry 1996;37(1):51–87.</p><p>Drayton S.J., Davies K., Steinberg M. et al. Amantadine for executive dysfunction syndrome in patients with dementia. Psychosomatics 2004;45:205–9.</p><p>Rosenblatt A., Leroi I. Neuropsychiatry of Huntington's Disease and Other Basal Ganglia Disorders. Psychosomatics 2000;41:24–30.</p><p>Biglan K., Shoulson I. Juvenile-onset Huntington disease: a matter of perspective. Arch Neurol 2007;64(6):783–4.</p><p>Tabrizi S.J., Langbehn D.R., Leavitt B.R. et al. Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data. Lancet Neurol 2009;8:791–801.</p><p>Rupp J., Blekher T., Jackson J. et al. Progression in prediagnostic Huntington disease. J Neurol Neurosurg Psychiatry 2010;81:379–84.</p><p>Whitwell J.L., Josephs K.A. Voxel-based morphometry and its application to movement disorders. Parkinsonism Relat Dis 2007;13(Suppl. 3):S406–16.</p><p>Kassubek J., Juengling F., Ecker D., Landwehrmeyer B. Thalamic atrophy in Huntington’s disease co-varies with cognitive performance: a morphometric MRI analysis. Cerebral Cortex 2005; 15:846–53.</p><p>Peinemann A., Schuller S., Pohl C. et al. Executive dysfunction in early stages of Huntington’s disease is associated with striatal and insular atrophy: a neuropsychological and voxel-based morphometric study. J Neurol Sci 2005;239:11–9.</p><p>Lee S.T., Chu K., Park J. et al. Memantine reduces striatal cell death with decreasing calpain level in 3-nitropropionic model of Huntington's disease. Brain Res 2006;1118(1):199–207.</p><p>Anitha M., Nandhu M.S., Anju T.R. Targeting glutamate mediated excitotoxicity in Huntington's disease: neural progenitors and partial glutamate antagonist - memantine. Med Hypotheses 2011;76(1):138–40.</p><p>Ondo W.G., Mejia N.I., Hunter C.B. A pilot study of the clinical efficacy and safety of memantine for Huntington's disease. Parkinsonism Relat Dis 2007;13(7):453–4.</p><p>Hjermind L.E., Law I., Jønch A. et al. Huntington's disease: effect of memantine on FDG-PET brain metabolism? J Neuropsychiatry Clin Neurosci 2011;23(2):206–10.</p><p>Иллариошкин С.Н., Клюшников С.А., Брылев Л.В. и др. Превентивная нейропротекция при нейродегенеративных заболеваниях: использование антагонистов глутаматных рецепторов (обзор литературы и собственный опыт). Неврол журн 2006;5:47−54.</p><p>Pisani A., Bernardi G., Ding J. et al. Re-emergence of striatal cholinergic interneurons in movement disorders. Trends Neurosci 2007;30(10):545–53.</p><p>Никольская Н.Н., Федин П.А., Иванова-Смоленская И.А. и др. Клинико-нейрофизиологическая оценка эффективности семакса при лечении хореи Гентингтона. В сб. V Российский национальный конгресс «Человек и лекарство». Тез. докл. М., 1998:336.</p></div><br />


For citation:


Klyushnikov S.A., Yudina E.N., Illarioshkin S.N., Ivanova-Smolenskaya I.A. Mental disorders in Huntington’s disease. Neurology, Neuropsychiatry, Psychosomatics. 2012;4(2S):46-51. (In Russ.) https://doi.org/10.14412/2074-2711-2012-2508

Views: 462


Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.


ISSN 2074-2711 (Print)
ISSN 2310-1342 (Online)