Abstract
Consideration is given to some strategies to combat Huntington's disease in the absence of treatment to arrest its progress. Ethical issues for tests such as levodopa loading, to provoke symptoms prematurely in carriers of the gene, are compared with those associated with schemes for delaying the onset of disease. The major drawback of these designs is the uncertainty that prodromal symptoms may be extended unduly and the severity of deferred symptoms worsened. Some attention is also given to the possible use of post-onset plans to reduce illness severity and prolong survival. It is concluded that the characteristics of persons less prone to undesirable consequences of onset-delay should be adequately investigated before attempting to implement such schedules as a practical measure.
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Selected References
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- Brackenridge C. J. Effect of climatic temperature on the age of onset of Huntington's chorea. J Neurol Neurosurg Psychiatry. 1974 Mar;37(3):297–301. doi: 10.1136/jnnp.37.3.297. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Brackenridge C. J. Relation of occupational stress to the age at onset of Huntington's disease. Acta Neurol Scand. 1979;60(5):272–276. doi: 10.1111/j.1600-0404.1979.tb02981.x. [DOI] [PubMed] [Google Scholar]
- Brackenridge C. J. Validation and aplication of an interval factor in estimating age at onset of Huntington's disease. J Med Genet. 1978 Feb;15(1):23–26. doi: 10.1136/jmg.15.1.23. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Cawein M., Turney F. Test for incipient Huntington's chorea. N Engl J Med. 1971 Mar 4;284(9):504–504. doi: 10.1056/NEJM197103042840920. [DOI] [PubMed] [Google Scholar]
- Cederbaum S. Tests for Huntington's chorea. N Engl J Med. 1971 May 6;284(18):1045–1045. doi: 10.1056/NEJM197105062841829. [DOI] [PubMed] [Google Scholar]
- James W. E., Mefferd R. B., Jr, Kimbell I., Jr Early signs of Huntington's chorea. Dis Nerv Syst. 1969 Aug;30(8):556–559. [PubMed] [Google Scholar]
- Klawans H. L., Jr, Goodman R. M., Paulson G. W., Barbeau A. Levodopa in the presymptomatic diagnosis of Huntington's chorea. Lancet. 1972 Jul 1;2(7766):49–49. doi: 10.1016/s0140-6736(72)91314-1. [DOI] [PubMed] [Google Scholar]
- Korenyi C., Whittier J. R., Conchado D. Stress in Huntington's disease (chorea). (Review of the literature and personal observations). Dis Nerv Syst. 1972 May;33(5):339–344. [PubMed] [Google Scholar]
- Oliver J. E., Dewhurst K. E. Six generations of ill-used children in a Huntington's pedigree. Postgrad Med J. 1969 Dec;45(530):757–760. doi: 10.1136/pgmj.45.530.757. [DOI] [PMC free article] [PubMed] [Google Scholar]
- SPENGLER M. Verschlimmerung von Chorea Huntington in der Gravidität. Schweiz Med Wochenschr. 1956 Aug 18;86(33):931–932. [PubMed] [Google Scholar]
- WHITTIER J. R. RESEARCH ON HUNTINGTON'S CHOREA: PROBLEMS OF PRIVILEGE AND CONFIDENTIALITY. J Forensic Sci. 1963 Oct;8(4):568–575. [PubMed] [Google Scholar]
