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Management

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Huntington’s Chorea

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Abstract

At the present time there is still no cure for Huntington’s chorea, nor is there any therapy which significantly alters the natural progression of this disease. Nevertheless there are numerous aspects of this disorder which may be improved by appropriate medical intervention. In the absence of a cure, amelioration of the patient’s symptoms becomes a desired and realistic objective. Although the precise pathogenetic mechanisms in Huntington’s chorea are yet to be determined, recent advances in neuropharmacology have facilitated a more rational approach to the pharmacotherapy of the disease and this will be reviewed here. There is a wide range of other therapeutic modalities that may be beneficially used in the management of Huntington’s chorea (Fig. 9.1) and a brief résumé of these services will also be presented.

No treatment seems to be of any avail and indeed nowadays its end is so well known… that medical advice is seldom sought. It seems at least to be one of the incurables. (George Huntington 1872) Huntington’s chorea Means there’s no help known, In the science of medicine For me And all of you, Choreanites, like me Because all of my good nurses And all of my medicine men And all of my good attenders All look at me and say By your words and by your looks Or maybe by your whispers There’s just not no hope Nor not no treatments known To cure me of my dizzy Called Chorea. (Woody Guthrie, November 1954 Brooklyn State Hospital, New York State. By courtesy of Marjorie Guthrie)

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References

  • Agid Y (1979) Communication to Eighth International Workshop on Huntington’s Chorea, Oxford, England

    Google Scholar 

  • Albano C, Cocito L (1979) Huntington’s chorea and bromocryptine (letter). Arch Neurol 36: 322

    PubMed  CAS  Google Scholar 

  • Anden NE, Dalen P, Johansson B (1973) Baclofen and lithium in Huntington’s chorea. Lancet II: 93

    Article  PubMed  CAS  Google Scholar 

  • Aquilonius SM, Eckernas SA (1977) Choline therapy in Huntington’s chorea. Neurology 27: 887–889

    PubMed  CAS  Google Scholar 

  • Bachman DS, Butler IJ, McKhann GM (1977) Long–term treatment of juvenile Huntington’s chorea with dipropylacetic acid. Neurology (Minneap) 27: 193–197

    CAS  Google Scholar 

  • Barbeau A (1973) Biochemistry of Huntington’s chorea. In: Barbeau A, Chase TN, Paulson GW (eds) Huntington’s chorea, 1872 Huntington’s 1972. Raven Press, New York, pp 525–531

    Google Scholar 

  • Barette J, Marsden CD (1979) Attitudes of families to some aspects of Huntington’s chorea. Psychol Med 9: 327–336

    Article  PubMed  CAS  Google Scholar 

  • Barr AN, Heinze W, Mendoza JE, Perlik S (1978) Long–term treatment of Huntington’s disease with L–glutamate and pyridoxine. Neurology (Minneap) 28: 1280–1282

    CAS  Google Scholar 

  • Binswanger C (1974) Physical therapy in Huntington’s chorea. In: Huntington’s disease handbook for health professionals. CCHD, New York, pp 17–18

    Google Scholar 

  • Borgelt PH, Linde TF (1974) Huntington’s disease. In: Huntington’s disease handbook for health professionals. CCHD, New York, pp 15–16

    Google Scholar 

  • Brown WT, Sanberg PR, McGeer PL (1979) Corticosteroids and chorea. Arch Neurol 7: 452–453

    Google Scholar 

  • Calne DB, Teychenne PF, Cleveria LE, Eastman R, Greenacre JK, Petrie A (1974) Bromocryptine in Parkinsonism. Br Med J IV: 442–444

    Article  PubMed  CAS  Google Scholar 

  • Carlsson, A (1976) Some aspects of dopamine in the basal ganglia. In: Yahr MD (ed) The basal ganglia. Raven Press, New York, pp 181–189

    Google Scholar 

  • Chase TN (1976) Rational approaches to the pharmacotherapy of chorea. In: Yahr MD (ed) The basal ganglia. Raven Press, New York, pp 337–347

    Google Scholar 

  • Chiu, E (1979) Notes on the management of Huntington’s disease. Aust Fam Physician 8: 197–200 Corrodi H, Fuxe K, Hokfelt T, Lidbrink P, Ungerstedt U (1973) Effect of ergot drugs on central catecholamine neurons: evidence for a stimulation of central dopamine neurons. J Pharm Pharmacol 25: 409–413

    Google Scholar 

  • Corsini GU, Onali P, Masala C, Cianchetti C, Mangoni A, Gessa G (1978) Apomorphine hydrochloride– induced improvement in Huntington’s chorea: stimulation of dopamine receptor. Arch Neurol 35: 27–30

    PubMed  CAS  Google Scholar 

  • Dalby MA (1969) Effect of tetrabenzine on extrapyramidal movement disorders. Br Med J ii: 422–423 Davies DD (1966) Abnormal response to anaesthesia in a case of Huntington’s chorea. Br J Anaesth 38: 490–491

    Google Scholar 

  • Enna SJ, Maggi A (1979) Biochemical pharmacology of gabaergic agonists. Life Sci 24: 1727–1738

    Article  PubMed  CAS  Google Scholar 

  • Fahn S (1972) Treatment of choreic movements with perphenazine. Dis Nerv Syst 33: 653–658

    PubMed  CAS  Google Scholar 

  • Fahn S (1973) Treatment of choreic movements with perphenazine. In: Barbeau A, Chase TN, Paulson GW (eds) Huntington’s chorea, 1872–1972. Raven Press, New York, pp 755–764

    Google Scholar 

  • Farina J, Rauscher LA (1977) Anaesthesia and Huntington’s chorea: a report of two cases. Br J Anaesth 11: 1167–1168

    Article  Google Scholar 

  • Frattola L, Albiazzati MG, Spano PF, Trabucchi M (1977) Treatment of Huntington’s chorea with bromocryptine. Acta Neurol Scand 56: 37–45

    Article  PubMed  CAS  Google Scholar 

  • Growden JH, Cohen EL, Wurtman RJ (1977) Huntington’s disease: clinical and chemical effects of choline administration. Am Neurol 1: 418–422

    Article  Google Scholar 

  • Gualandi W, Bonfanti G (1968) Un caso di apnea prolungata in corea di Huntington. Acta Anaesth (Padua) 19: 235–238

    Google Scholar 

  • Horn, AS, Snyder SH (1972) Chlorpromazine and dopamine conformational similarities that correlate with the antischizophrenic activity of phenothiazine drugs. Proc Natl Acad Sci USA 68: 2325–2328

    Article  Google Scholar 

  • Jenner P, Marsden CD (1979) The substituted benzamides: a novel class of dopamine antagonists. Life Sci 25: 479–486

    Article  PubMed  CAS  Google Scholar 

  • Kartzinel R, Perlow MD, Carter AC, Chase TN, Calne DB, Shoulson I (1976) Metabolic studies with bromocryptine in patients with idiopathic Parkinsonism and Huntington’s chorea. Trans Am Neurol Assoc 101: 53–56

    PubMed  CAS  Google Scholar 

  • Kelly R (1977) Testimony to Commission for Control of Huntington’s Disease and its Consequences, vol IV/2, US Government Printing Office, Washington, DC, pp 77–78

    Google Scholar 

  • Kingston D (1979) Tetrabenzine for involuntary movement disorders. Med J Aust 1: 628–630

    PubMed  CAS  Google Scholar 

  • Klawans HL, Paulson GW, Ringel SP, Barbeau A (1972) The use of L–dopa in the detection of presymptomatic Huntington’s chorea. N Engl J Med 286: 1332–1334

    Article  PubMed  Google Scholar 

  • Lamont AM (1979) Brief report: anaesthesia and Huntington’s chorea. Anaesth Intens Care 7: 189–190

    CAS  Google Scholar 

  • Lazarte JA, Petersen MC, Baars CW, Pearson JS (1955) Huntington’s chorea: results of treatment with reserpine. Proc Mayo Clin 30: 358–365

    CAS  Google Scholar 

  • Loeb C, Roccatagliata G, Albano C, Besio G (1979) Bromocryptine and dopaminergic function in Huntington’s disease. Neurology (Minneap) 29: 730–734

    CAS  Google Scholar 

  • Miller E (1976) The social work component in community–based action on behalf of victims of Huntington’s disease. Soc Work Health Care 76, 2 (1): 25–32

    Article  CAS  Google Scholar 

  • Perry TL, Hansen S, Klosker M (1973) Huntington’s chorea: Deficiency of γ–aminobutyric acid in brain. N Engl J Med 288: 337–342

    Article  PubMed  CAS  Google Scholar 

  • Perry TL, Wright JM, Hansen S, MacLeod P (1979) Isoniazid therapy of Huntington’s chorea. Neurology 29: 370–377

    PubMed  CAS  Google Scholar 

  • Perry TL, Wright JM, Hansen S, Allen BM, Baird PA, MacLeod PM (1980) Failure of aminooxyacetic acid therapy in Huntington’s disease. Neurology (Minneap) 30: 772–775

    CAS  Google Scholar 

  • Ringel SP, Guthrie M, Klawans HL (1973) Current treatment of Huntington’s chorea. In: Barbeau A, Chase TN, Paulson GW (eds) Huntington’s chorea, 1872–1972. Raven Press, New York, pp 797–801

    Google Scholar 

  • Schou M (1979) Lithium in the treatment of other psychiatric and non–psychiatric disorders. Arch Gen Psychiatry 36: 856–S58

    PubMed  CAS  Google Scholar 

  • Shoulson I (1977) Clinical care of the patient and family with Huntington’s disease. In: Commission for the Control of Huntington’s Disease and its Consequences, vol II. US Government Printing Office, Washington, DC, pp 421–451

    Google Scholar 

  • Shoulson I (1979) Huntington’s disease: overview of experimental therapeutics. In: Chase TN, Wexler NS, Barbeau A (eds) Advances in neurology, vol 23. Raven Press, New York, pp 751–759

    Google Scholar 

  • Shoulson I, Chase TN, Roberts E, Van Balgooy JN (1975) Huntington’s disease: treatment with imidazole–4– acetic acid. N Engl J Med 293: 504–505

    PubMed  CAS  Google Scholar 

  • Shoulson I, Kartzinel R, Chase TN (1976) Huntington’s disease: treatment with dipropylacetic acid and gamma–aminobutyric acid. Neurology (Minneap) 26: 61–63

    CAS  Google Scholar 

  • Shoulson I, Goldblatt D, Charlton M, Joynt RJ (1978) Huntington’s disease: treatment with muscimol, a GABA–mimetic drug. Ann Neurol 4: 279–284

    Article  PubMed  CAS  Google Scholar 

  • Spiegel EA, Wycis HT (1950) Pallido–thalamotomy in chorea. Arch Neurol Psychiatr 64: 295–296

    CAS  Google Scholar 

  • Stern R, Eldridge R (1975) Attitudes of patients and their relatives to Huntington’s disease. J Med Genet 12: 217–223

    Article  PubMed  CAS  Google Scholar 

  • Still CN (1979) Nutritional therapy in Huntington’s chorea: concepts based on the model of pellagra. Psychiatr Forum, 74–78

    Google Scholar 

  • Stipe J, White D, Van Arsdale E (1979) Huntington’s disease. Am J Nurs 79 (8): 1428–1432

    PubMed  CAS  Google Scholar 

  • Stokes HB (1975) Trifluoperazine for the symptomatic treatment of chorea. Dis Nerv Syst 36: 102–105

    PubMed  CAS  Google Scholar 

  • Taglia JU, McGlamery M, Sambandham RR (1978) Tetrabenzine in the treatment of Huntington’s chorea 150 and other hyperkinetic movement disorders. J Clin Psychiatr 39: 81–87

    Google Scholar 

  • Tarsy D, Leopold N, Sax DS (1974) Physostigmine in choreiform movement disorders. Neurology (Minneap) 24: 28–33

    CAS  Google Scholar 

  • Tolosa ES, Sparber SB (1974) Apomorphine in Huntington’s chorea: clinical observations and theoretical considerations. Life Sci 15: 1371–1380

    Article  PubMed  CAS  Google Scholar 

  • Whittier JR, Korenyi C (1968) Effect of oral fluphenazine on Huntington’s chorea. Int J Neuropsychiatr 4: 1–3

    CAS  Google Scholar 

  • Zanglein JP, Buldauf E, Hussaini M (1978) A new treatment of Huntington’s chorea: report of three cases. Sem Hop Paris 54: 871–874

    Google Scholar 

Download references

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Authors and Affiliations

  1. Division of Clinical Genetics, Children’s Hospital Medical Center, 300 Longwood Avenue, 02115, Boston, MA., USA

    Michael R. Hayden MB, ChB, PhD, DCH(SA)

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  1. Michael R. Hayden MB, ChB, PhD, DCH(SA)
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© 1981 Springer-Verlag Berlin Heidelberg

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Hayden, M.R. (1981). Management. In: Huntington’s Chorea. Springer, London. https://doi.org/10.1007/978-1-4471-1308-9_9

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  • DOI: https://doi.org/10.1007/978-1-4471-1308-9_9

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-1310-2

  • Online ISBN: 978-1-4471-1308-9

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