Abstract
In 1872 beschreef George Huntington (figuur 1) hoe hij, zijn vader en grootvader, allen arts, generaties patiënten uit één familie zagen met eenzelfde soort ziektebeeld, waarvan de eerste verschijnselen zich op volwassen leeftijd openbaarden: ‘The movements gradually increase when muscles hitherto unaffected take on the spasmodic action, until every muscle in the body becomes affected (excepting the involuntary ones)… As the disease progresses the mind becomes more or less impaired, in many accounting to insanity, while in others mind and body gradually fail until death relieves them of their suffering’ (Huntington, 1872). Hij noemde de ziekte chorea, van het Griekse woord choreia (dans).
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Almqvist E.W., Bloch M., Brinkman R., Craufurd D., Hayden M.R. (1999). A worldwide assessment of the frequency of suicide, suicide attempts, or psychiatric hospitalization after predictive testing for Huntington disease. Am. J. Hum Genet.,64(5):1293-304.
Bachoud-Lévi A.-C., Hantraye P., Peschanski M. (2002). Fetal neural grafts for Huntington's disease: a prospective view. Mov.Disord., 17, 439-444.
Brown R.G., Marsden C.D. (1988). ‘Subcortical dementia’: the neuropsychological evidence. Neuroscience, 25, 363-387.
Craufurd D., Thompson J.C., Snowden J.S. (2001). Behavioral changes in Huntington's disease. Neuropsychiatry, Neuropsychol., and Behav. Neurol., 14, 219-226.
Cummings J.L. (1986). Subcortical dementia. Neuropsychology, neuropsychiatry and pathophysiology. British Journal of Psychiatry, 149, 682-697.
De Boo G.M., Tibben A., Lanser J.B.K., Jennekens-Schinkel A., Hermans J., Maat-Kievit J.A., Roos R.A.C. (1997). Early cognitive and motor symptoms in identified carriers of the gene for Huntington's disease. Arch Neurol, 54, 1353-1357.
De Marchi N., Mennella R. (2000). Huntington's disease and its association with psychopathology. Harvard Rev. Psychiatry, 7, 278-289.
Duisterhof G.M.D., Trijsburg R.W., Niermeijer M.F., Roos R.A.C., Tibben A. (2001). Psychological studies on Huntington's disease: making up the balance. J. Med. Genet., 38:852-861.
Farrer L.A. (1986). Suicide and attempted suicide in Huntington disease: implications for preclinical testing of persons at risk. Am. J. Med Genet.;24(2):305-11.
Folstein S.E. (1989). Huntington's disease, a disorder of families. Baltimore, The John Hopkins University Press.
Girotti F., Marano R., Soliveri P., Geminiani G., Scigliano G. (1988). Relationship between motor and cognitive disorders in Huntington's disease. J Neurol., 235, 454-457.
Gusella J.F., Wexler N.S., Conneally P.M., et al. (1983). A polymorphic dna marker linked to Huntington's disease. Nature, 306, 234-238.
Harper P.S. (1996). Huntington's disease. 2e druk, Londen (ed. Saunders).
Huntington G. (1872). On chorea. Med. Surg. Rep., 26, 320-321.
Huntington Disease Collaborative Research Group (1993). A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell, 72, 971-983.
Huntington Study Group (1996). Unified Huntington's Disease Rating Scale: reliability and consistency. Mov. Disord., 11, 136-142.
iha/wfn. International Huntington Association and the World Federation of Neurology Research Group on Huntington's Chorea (1994). Guidelines for the molecular genetics predictive test in Huntington's disease. Jl Med. Genet.,31(7):555-9.
Kieburtz K., MacDonald M., Shih C., et al. (1994). Trinucleotide repeat length and progression of illness in Huntington's disease. J. Med. Genet., 31, 872-874.
Lauwrence A.D., Sahakian B.J., Hodges J.R., et al. (1996). Executive and mnemonic functions in early Huntington's disease. Brain, 119, 1633-1645.
Lawson K., Wiggins S., Green T., Adam S., Bloch M., Hayden M.R. (1996). Adverse psychological events occurring in the first year after predictive testing for Huntington's disease. The Canadian Collaborative Study Predictive Testing. J. Med. Genet., 33(10):856-62.
Maat-Kievit J.A., Losekoot M., Roos R.A.C. (2001). Van gen naar ziekte; het hd-gen en de ziekte van Huntington. Ned. Tijdschr. Geneeskd., 145 (44), 2120-2123.
Meiser B., Dunn S. (2000). Psychological impact of genetic testing for Huntington's disease: an update of the literature. J. Neurol. Neurosurg. Psychiatry.,69(5):574-8.
Morris M. (1995). Dementia and cognitive changes in Huntington's disease. In: Advances in Neurology, Vol. 65, (eds. Weiner WJ, Lang AE) 187-200. Raven Press.
Podoll K., Caspary P., Lange H.W., Noth J. (1988). Language functions in Huntington's disease. Brain, 1111, 1475-1503.
Quinn N., Brown R., Craufurd D., et al. (1996). Core Assessment Program for Intracerebral Transplantation in Huntington's disease (capit-hd). Mov. Disord., 11, 143-150.
Roos R.A.C., Hermans J., Vegter-Van der Vlis M., van Ommen G.J.B., Bruyn G.W. (1993).Duration of illness in Huntington's disease is not related to age at onset. J. Neurol. Neurosurg. Psychiatry, 56, 98-100.
Shoulson I., Fahn S. (1979). Huntington's disease: clinical care and evaluation. Neurology, 29, 1-3.
Van Vugt J.P.P., Van Hilten J.J., Roos R.A.C. (1996). Hypokinesia in Huntington's disease. Mov. Disord., 11, 384-388.
Wechsler D.A. (1945). A standardized memory scale for clinical use. J. Psychology, 19, 87-95.
Wechsler D.A. (1955). Wechsler Adult Intelligence Scale. New York Psychological Corporation.
Whitehouse P.J. (1986). The concept of subcortical dementia and cortical dementia: another look. Ann. Neurol., 19, 1-6.
Zakzanis K.K. (1998). The subcortical dementia of Huntington's disease. Journal of Clinical and Experimental Neuropsychology, 20, 565-578.
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Mw. drs. M.N.W. Witjes-Ané, gz-psychologe-neuropsychologe, lumc afdeling Neurologie, Sectie Neuropsychologie, Postbus 9600, 2300 RC Leiden,
A. Tibben, psycholoog-psycho therapeut, lumc afdeling. Klinische Genetica en afd. Neurologie, postbus 9600, 2300 RC Leiden.
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Witjes-Ané, M.N., Tibben, A. De ziekte van Huntington. NEPR 7, 13–18 (2003). https://doi.org/10.1007/BF03071039
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DOI: https://doi.org/10.1007/BF03071039