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The Effects of Neuroleptics on Longevity in Huntington’s Disease

  • Charles N. Still
  • Thomas J. Goldschmidt

Abstract

Huntington’s disease (HD) comes from the observations of George Sumner Huntington, who gained eponymic immortality by his description of chronic progressive hereditary chorea in 1872. Huntington’s paper includes the following major features of the disease:
  1. 1.

    Its hereditary nature: “When either or both the parents have shown manifestation of the disease, ... one or more of the offspring almost invariably suffer from the disease, if they live to adult age. But if by chance these children go through life without it, the thread is broken and the grandchildren and great grandchildren of the original shakers may rest assured that they are free from the disease.... Unstable and whimsical as the disease may be in other respects, in this it is firm, it never skips a generation to again manifest itself in another; once having yielded its claims, it never regains them.”

     
  2. 2.

    Its rarity: “hereditary chorea ... is confined to certain and fortunately a few families.”

     
  3. 3.

    Its abnormal involuntary movements: “It begins as an ordinary chorea might begin, by the irregular and spasmodic action of certain muscles, as of the face, arms, etc. These movements gradually increase, when muscles hitherto unaffected take on the spasmodic action, until every muscle in the body becomes affected (excepting the involuntary ones)....”

     
  4. 4.

    Its progressive nature: “... increasing by degrees, and often occupying years in its development, until the hapless sufferer is but a quivering wreck of his former self.”

     
  5. 5.

    Its delayed age of onset: “I do not know of a single case that has shown any marked signs of chorea before the age of thirty of forty years, while those who pass the fortieth year without symptoms of the disease, are seldom attacked.”

     
  6. 6.

    Its behavioral abnormalities, including suicide: “In nearly all the families ... in which the choreic taint exists, the nervous temperament greatly preponderates, and in my grandfather’s and father’s experience, which conjointly covered a period of 78 years, nervous excitement in a marked degree almost invariably attends upon every disease these people may suffer from, although they may not when in health be over nervous.... The tendency to insanity, and sometimes that form of insanity which leads to suicide is marked.”

     
  7. 7.

    Its progressive dementia: “As the disease progresses the mind becomes more or less impaired, in the many amounting to insanity, while in others mind and body both gradually fail until death relieves them of their sufferings.”

     
  8. 8.

    Its resistance to treatment: “I have never known a recovery or even an amelioration of symptoms in this form of chorea; when once it begins it clings to the bitter end. No treatment seems to be of any avail, and indeed nowadays its end is so well known to the sufferer and his friends, that medical advice is seldom sought.”

     

Keywords

Tardive Dyskinesia Neuroleptic Drug Subacute Sclerosing Panencephalitis Choreiform Movement Rocky Mountain Spotted Fever 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. Alzheimer, A., 1911, Concerning the anatomical basis of Huntington’s chorea and choreatic movements in general, Neurol. Centralbl. 30: 891–892.Google Scholar
  2. American Psychiatric Association (APA), 1980, Tardive Dyskinesia,American Psychiatric Association Task Force, Report 18, p. 4.Google Scholar
  3. Baldessarini, R. J., 1980, Drugs and the treatment of psychiatric disorders, in: The Pharmacological Basis of Therapeutics, 6th ed. ( A. G. Gilman, L. S. Goodman, and A. Gilman, eds.), pp. 391–447, Macmillan, New York.Google Scholar
  4. Born, G. V. R., 1979, Possible role for chlorpromazine in protection against myocardial infarction, Lancet 1: 822.PubMedCrossRefGoogle Scholar
  5. Bruyn, G. W., 1968, Huntington’s chorea. Historical, clinical and laboratory synopsis, in: Diseases of the Basal Ganglia, Handbook of Clinical Neurology, Vol. 6 ( P. J. Vinken and G. W. Bruyn, eds.), pp. 298–378, North-Holland Publishing Company, Amsterdam.Google Scholar
  6. Bruyn, G. W., 1973, Clinical variants and differential diagnosis, in: Huntington’s Disease, Advances in Neurology, Vol. 1 ( A. Barbeau, T. N. Chase, and G. W. Paulson, eds.), pp. 51–56, Raven Press, New York.Google Scholar
  7. Bruyn, G. W., Bots, G. Th. A. M., and Dom, R., 1979, Huntington’s chorea: Current neuropathological status, in: Huntington’s Disease, Advances in Neurology, Vol. 23 ( T. N. Chase, N. S. Wexler, and A. Barbeau, eds.), pp. 83–93, Raven Press, New York.Google Scholar
  8. Chandler, J. H., Reed, T. E., and DeJong, R. N., 1960, Huntington’s chorea in Michigan, III. Clinical observations, Neurology 10: 148–153.PubMedGoogle Scholar
  9. Craig, T. J., and Lin, S. P., 1981, Mortality among psychiatric inpatients: Age-adjusted comparison of populations before and after psychotropic drug era, Arch. Gen. Psychiatry 38: 935–938.PubMedCrossRefGoogle Scholar
  10. Crane, G. E., 1973, Tardive dyskinesia and Huntington’s chorea: Drug-induced and hereditary dyskinesias, in: Huntington’s Disease, Advances in Neurology, Vol. 1 ( A. Barbeau, T. N. Chase, and G. W. Paulson, eds.), pp. 115–122, Raven Press, New York.Google Scholar
  11. Davenport, C. B., and Muncey, E. B., 1916, Huntington’s chorea in relation to heredity and eugenics, Am. J. Insanity 73: 195–222.Google Scholar
  12. Delay, J., and Deniker, P., 1968, Drug-induced extrapyramidal syndromes, in: Diseases of the Basal Ganglia, Handbook of Clinical Neurology, Vol. 6 ( P. J. Vinken and G. W. Bruyn, eds.), pp. 248–266, North-Holland Publishing Company, Amsterdam.Google Scholar
  13. Foster, C. S., 1979, Chlorpromazine in prophylaxis of myocardial infarction, Lancet 1:12–49.Google Scholar
  14. Fratta, I., Zak, S. B., Greengard, P., and Sigg, E. B., 1964, Fetal death from nicotinamide-deficient diet and its prevention by chlorpromazine and imipramine, Science 145: 1429–1430.CrossRefGoogle Scholar
  15. Hornykiewicz, O., 1966, Dopamine (3-hydroxytyramine) and brain function, Pharmacol. Rev. 18: 925–964.PubMedGoogle Scholar
  16. Hornykiewicz, 0., 1979, Pharmacology of Huntington’s disease, in: Huntington’s Disease, Advances in Neurology, Vol. 23 ( T. N. Chase, N. S. Wexler, and A. Barbeau, eds.), pp. 679–686, Raven Press, New York.Google Scholar
  17. Huntington, G., 1872, On chorea, Med. Surg. Reporter 26: 317–321.Google Scholar
  18. Jones, M. B., 1979, Years of life lost due to Huntington disease, Am. J. Hum. Genet. 31:711–717. Martin, J. B., 1984, Huntington’s disease: New approaches to an old problem, Neurology 34: 1059 1072.Google Scholar
  19. McHugh, P. R., and Folstein, M. F., 1975, Psychiatric syndromes of Huntington’s chorea: A clinical and phemomenologic study, in: Psychiatric Aspects of Neurologic Disease ( D. F. Benson and D. Blumer, eds.), pp. 267–286, Grune & Stratton, New York.Google Scholar
  20. Oepen, H., 1963, Paroxysmal disorders in Huntington’s chorea, Arch. Psychiat. Nervenkr. 204: 245–261.PubMedCrossRefGoogle Scholar
  21. Quadfasel, F. A., 1973, Why Huntington’s disease now instead of Huntington’s chorea, Psychiatr. Forum 4: 61–62.Google Scholar
  22. Reed, T. E., Chandler, J. H., Hughes, E. M., and Davidson, R. T., 1958, Huntington’s chorea in Michigan, I. Demography and genetics, Am. J. Hum. Genet. 10: 201–225.PubMedGoogle Scholar
  23. Rockstein, M., Chesky, J. A., and Sussman, M. D., 1977, Comparative biology and evoluation of aging, in: Handbook of the Biology of Aging ( C. E. Finch and L. Hayflick, eds.), pp. 3–34, Van Nostrand Reinhold Co., New York.Google Scholar
  24. Roizin, L., Kaufman, M. A., Willson, N., Stellar, S., and Liu, J. C., 1976, Neuropathologic observations in Huntington’s chorea, in: Progress in Neuropathology, Vol. 3 ( H. M. Zimmerman, ed.), pp. 447–488, Grune & Stratton, New York.Google Scholar
  25. Roos, R. A. C., and Bots, G. Th. A. M., 1983, Nuclear membrane indentations in Huntington’s chorea, J. Neurol. Sci., 61: 37–47.PubMedCrossRefGoogle Scholar
  26. Shoulson, I., 1981, Huntington disease: Functional capacities in patients treated with neuroleptic and antidepressant drugs, Neurology 31: 1333–1335.PubMedGoogle Scholar
  27. Shoulson, I., 1984, Huntington’s disease: A decade of progress, Neurol. Clin. 2: 515–526.PubMedGoogle Scholar
  28. Spokes, E. G. S., 1979, Dopamine in Huntington’s disease: A study of postmortem brain tissue, in: Huntington’s Disease, Advances in Neurology, Vol. 23 ( T. N. Chase, N. S. Wexler, and A. Barbeau, eds.), pp. 481–493, Raven Press, New York.Google Scholar
  29. Still, C. N., 1984, Involuntary movement disorders, Neurol. Clin. 2: 71–89.PubMedGoogle Scholar
  30. Still, C. N., and Young, S. R., 1974, Age of onset in Huntington’s disease: a reappraisal of the Wendt hypothesis, Am. J. Hum. Genet. 26: 84A.Google Scholar
  31. Streletzki, F., 1961, Psychoses in the course of Huntington’s chorea with special reference to the formation of delusions, Arch. Psychiat, Nrvenkr. 202: 202–214.Google Scholar
  32. Tellez-Nagel, I., Johnson, A. B., and Terry, R. D., 1974, Studies on brain biopsies of patients with Huntington’s chorea, J. Neuropathol. Exp. Neurol. 33: 308–332.PubMedCrossRefGoogle Scholar
  33. Wendt, G. G., Landzettel, I., and Solth, L., 1959, The age of onset of illness in Huntington’s chorea, Acta Genet. (Basel) 9: 18–32.Google Scholar
  34. Wendt, G. G., Landzettel, I., and Solth, K., 1960, Length of illness and life expectancy in Huntington’s chorea, Arch. Psychiat. Nervenkr. 201: 298–312.PubMedCrossRefGoogle Scholar
  35. Whittier, J. R., 1967, Clinical aspects of Huntington’s disease, in: Progress in Neurogenetics ( A. Barbeau and J. R. Brunette, eds.), pp. 632–644, Excerpta Medica Foundation, Amsterdam.Google Scholar

Copyright information

© Plenum Publishing Corporation 1986

Authors and Affiliations

  • Charles N. Still
    • 1
  • Thomas J. Goldschmidt
    • 1
  1. 1.Department of Neuropsychiatry and Behavioral ScienceUniversity of South Carolina School of MedicineColumbiaUSA

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