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A Controlled Psychiatric Study of Individuals at Risk for Huntington's Disease

Published online by Cambridge University Press:  02 January 2018

Raj S. Shiwach*
Affiliation:
Deal Mental Health Centre, Deal, Kent
C. Gail Norbury
Affiliation:
Churchill Hospital, Oxford
*
Dr Raj S. Shiwach, Terrell State Hospital, P. O. Box 70, 1200 E. Brin, Texas 75160, USA

Abstract

Background

The study tested specific hypotheses that (a) there is an increased incidence of psychiatric disorders in asymptomatic heterozygotes for Huntington's disease (HD) compared with the normal homozygotes, and (b) there is an increased incidence of psychiatric disorders in the adult offspring of Huntington's disease patients compared with their partners.

Method

A controlled study was made of 93 apparently healthy individuals (at 50% risk), who had given DNA samples for the predictive test, and 70 of their partners. Current and past psychopathology was assessed and compared with the DNA predictive test results based on linkage analyses. The results of psychiatric assessments of the two groups were compared.

Results

DNA test results were available for 53 subjects (of 93). Five subjects at risk for HD were omitted from the study. The asymptomatic heterozygotes (n = 20) showed no significant increase in the incidence of any psychiatric episode, depression, schizophrenia or behavioural disorder when compared with the normal homozygotes (n = 33). The whole tested group showed a significantly greater number of psychiatric episodes than their partners (n = 43).

Conclusions

Asymptomatic HD gene carriers do not have a greater incidence of psychiatric disorders than the non-gene carriers born to a HD parent.

Type
Papers
Copyright
Copyright © Royal College of Psychiatrists, 1994 

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References

Bear, D. (1977) Position paper on emotional and behavioural changes in Huntington's disease. In Report Commission for the Control of Huntington's Disease and its Consequences; vol. 3, part 1. Washington, DC: Government Printing Office.Google Scholar
Bolt, J. M. W. (1970) Huntington's chorea in the west of Scotland. British Journal of Psychiatry, 116, 259270.Google Scholar
Brackenridge, C. J. (1973) Inter relations between some clinical factors in Huntington's chorea. In Advances in Neurology (eds Barbeau, A., Chase, T. N. & Poulson, G. W.), pp. 6573. New York: Raven Press.Google Scholar
Brandt, J., Quaid, K., Folstein, S. E., et al (1989) Presymptomatic diagnosis of delayed onset disease with linked DNA markers: the experience in Huntington's disease. Journal of the American Medical Association, 261, 31083114.Google Scholar
Brothers, C. R. D. (1964) Huntington's chorea in Victoria and Tasmania. Journal of Neurological Science, 1, 405450.Google Scholar
Brothers, C. R. D. & Meadows, A. W. (1955) An investigation of Huntington's chorea. Victoria Journal of Medical Science, 1, 405450.Google Scholar
Cameron, D. & Venters, G. A. (1967) Some problems in Huntington's chorea. Scottish Medical Journal, 12, 152156.CrossRefGoogle ScholarPubMed
Caro, A. J. (1977) Huntington's chorea: a clinical problem in East Anglia. University of East Anglia: PhD thesis.Google Scholar
Chandler, J., Reed, T. & Dejong, R. (1960) Huntington's chorea in Michigan. Clinical observations. Neurology, 10, 148153.Google Scholar
Craufurd, D., Dodge, A., Kerzin-Storrar, L., et al (1989) Uptake of presymptomatic predictive testing for Huntington's disease. Lancet, ii, 603605.Google Scholar
Dewhurst, K. (1970) Personality disorder in Huntington's disease. Psychiatria Clinica, 3, 221229.Google Scholar
Dewhurst, K., Oliver, J., Trick, K. L. K., et al (1969) Neuropsychiatric aspects of Huntington's chorea. Confinia Neurologica, 31, 258268.Google Scholar
Dewhurst, K., Oliver, J., & McKnight, A. L. (1970) Sociopsychiatric consequences of Huntington's disease. British Journal of Psychiatry, 116, 255258.CrossRefGoogle ScholarPubMed
Diefendorf, A. R. (1908) Mental symptoms of Huntington's chorea. Neurographs, 1, 128136.Google Scholar
Dynan, N. J. (1914) The physical and mental states in chronic chorea. American Journal of Insanity, 70, 589636.Google Scholar
Farrer, L. A. (1986) Suicide and attempted suicide in Huntington's disease: implications for preclinical testing of persons at risk. American Journal of Medical Genetics, 24, 305311.Google Scholar
Folstein, S. E., Abbott, M., Folstein, M. F., et al (1983a) The association of affective disorder with Huntington's disease in a case series and in families. Psychological Medicine, 13, 537542.CrossRefGoogle Scholar
Folstein, S. E., Franz, M. L., Jensen, B. A., et al (1983b) Conduct disorder and affective disorder among the offspring of patients with HD. Psychological Medicine, 13, 4552.Google Scholar
Folstein, S. E., Jensen, B., Leigh, R. J., et al (1983c) The measurement of abnormal movements: methods developed for Huntington's disease. Neurobehavioral Toxicology and Teratology, 5, 605609.Google Scholar
Gusella, J. F., Wexler, N. S., Conneally, P. M., et al (1983) A polymorphic DNA marker genetically linked to Huntington's disease. Nature, 306, 234238.Google Scholar
Hayden, M. R. (1981) Huntington's Chorea. Berlin: Springer Verlag.Google Scholar
Heathfield, K. W. G. (1967) Huntington's chorea: investigation into prevalence in N.E. Metropolitan Regional Hospital Board area. Brain, 90, 203233.Google Scholar
Huggins, M., Bloch, M., Wiggins, S., et al (1992) Predictive testing for Huntington's disease in Canada: adverse effects and unexpected results in those receiving a decreased risk. American Journal of Medical Genetics, 42, 491498.Google Scholar
James, W. E., Mefferd, R. B. & Kimbell, I. (1969) Early signs of Huntington's chorea. Diseases of the Nervous System, 30, 558559.Google Scholar
Joint Committee of the International Huntington's Association and the World Federation of Neurology (1987) The Presymptomatic Test for Huntington's Chorea. Leicester: The Association to Combat Huntington's Chorea.Google Scholar
Mattsson, B. (1974) Huntington's chorea in Sweden. II. Social and clinical data. Acta Psychiatrica Scandinavica, suppl. 255, 221235.Google Scholar
Mindham, R. H. S., Steele, C., Folstein, M. F., et al (1985) A comparison of the frequency of major affective disorder in Huntington's disease and Alzheimer's disease. Journal of Neurology, Neurosurgery and Psychiatry, 48, 11721174.CrossRefGoogle ScholarPubMed
Minski, L. & Guttmann, E. (1938) Huntington's chorea: a study of thirty-four families. Journal of Mental Science, 84, 2196.CrossRefGoogle Scholar
Oltman, J. E. & Freidman, S. (1961) Comments on Huntington's chorea. Diseases of the Nervous System, 22, 313319.Google Scholar
Penney, J. B., Young, A. B., Shoulson, I., et al (1990) Huntington's disease in Venezuela: seven years of follow up on symptomatic and asymptomatic individuals. Movement Disorders, 5, 9399.Google Scholar
Rosenbaum, D. (1941) Psychosis with Huntington's chorea. Psychiatric Quarterly, 15, 9399.Google Scholar
Sambrook, J., Fritsch, E. F. & Maniatis, T. (1989) Molecular Cloning: A Laboratory Manual. New York: Cold Spring Harbor Press.Google Scholar
Saugstad, L. & Ødegård, O. (1986) Huntington's chorea in Norway. Psychological Medicine, 16, 3948.CrossRefGoogle ScholarPubMed
Schoenfeld, M., Myers, R. H., Cupples, L. A., et al (1984) Increased rate of suicide among patients with Huntington's disease. Journal of Neurology, Neurosurgery and Psychiatry, 47, 12831287.Google Scholar
Spillane, J. & Phillips, R. (1937) Huntington's chorea in South Wales. Quarterly Journal of Medicine, 24, 403423.Google Scholar
Streletzki, F. (1961) Psychosen im Verlauf der Huntingtonscher Chorea unter besonderer Berucksichtung der Wahnbildungen. Archiv fur Psychiatrie und Nerven Krankheiten, 202, 202214.Google Scholar
Watt, D. C. & Seller, A. (1993) A clinico-genetic study of psychiatric disorder in Huntington's chorea. Psychological Medicine, Monograph suppl. 23.Google Scholar
Wiggins, S., Whyte, P., Huggins, M., et al (1992) The psychological consequences of predictive testing for Huntington's disease. New England Journal of Medicine, 327, 14011405.CrossRefGoogle ScholarPubMed
Wilson, R. S. & Garron, D. C. (1979) Cognitive and affective aspects of Huntington's disease. In Advances in Neurology, vol. 23 (eds Chase, T. N., Wexler, N. S. & Barbeau, A.), pp. 193201. New York: Raven Press.Google Scholar
Wing, J. K., Cooper, E. & Sartorius, N. (1974) The Measurement and Classification of Psychiatric Symptoms. Cambridge-Cambridge University Press.Google Scholar
World Health Organization (1992) The ICD-10 Classification of Mental and Behavioural Disorders. Geneva: WHO.Google Scholar
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