Hostname: page-component-745bb68f8f-f46jp Total loading time: 0 Render date: 2025-01-29T23:32:56.524Z Has data issue: false hasContentIssue false
  • English
  • Français

Systemic Therapy of Dystonia

Published online by Cambridge University Press:  05 January 2016

Stanley Fahn
Stanley Fahn*
Affiliation:
Department of Neurology, Columbia University College of Physicians & Surgeons and The Neurological Institute of New York, Presbyterian Hospital, New York
*
Neurological Institute, 710 West 168th Street, New York, N.Y. U.S.A. 10032
Rights & Permissions [Opens in a new window]

Abstract:

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

A number of pharmacologic agents have been found to be effective for the dystonias. Anticholinergic drugs have been shown to be the most effective in terms of percentage of subjects who receive moderate to marked benefit. About 50% of children and 40% of adults obtain such improvement. Peripheral adverse effects are usually overcome by pyridostigmine. It may be necessary to utilize pilocarpine eyedrops for blurred vision. Central adverse effects, such as forgetfulness, can be reduced only by a reduction in dosage of the anticholinergic. In comparing trihexyphenidyl and ethopropazine, we found that children tend to have better tolerance of the former and adults tend to have better tolerance of the latter. The antidopaminergics are the group of drugs that were found to be the next most effective agents in terms of percentage of patients who respond. However, these drugs, particularly the dopamine receptor blockers, have the capacity to induce tardive dyskinesia and tardive dystonia. Tardive syndromes are difficult to treat and can persist indefinitely. Other agents that have shown usefulness in controlling dystonia are levodopa, baclofen, carbamazepine, and the benzodiazepines, either alone or in combination with each other and with the anticholinergics. Stereotactic thalamotomy is particularly useful in contralateral hemidystonia. The risk of adverse effects is less than with bilateral thalamotomy, which may need to be employed when generalized dystonia is severely disabling and not responsive to pharmacotherapy.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 14 , Issue S3 , August 1987 , pp. 528 - 532
Copyright
Copyright © Canadian Neurological Sciences Federation 1987

References

REFERENCES

1.Fahn, S, Marsden, CD, Calne, DB.Classification and investigation of dystonia. In: Marsden, CD, Fahn, S, eds: Movement Disorders 2. London, Butterworth Scientific 1987: 332–358,Google Scholar
2.Herz, E.Dystonia, I.. Historical review: Analysis of dystonic symp-toms and physiologic mechanisms involved. Arch Neurol Psychiat 1944;51: 305–318.CrossRefGoogle Scholar
3.Eldridge, R.The torsion dystonias: Literature review and genetic and clinical studies. Neurology 1970; 20: No. 11 Part 2: 1–78.CrossRefGoogle ScholarPubMed
4.Burke, RE, Fahn, S, Jankovic, J.et al. Tardive dystonia: Late-onset and persistent dystonia caused by antipsychotic drugs. Neurology 1982; 32: 1335–1346.CrossRefGoogle ScholarPubMed
5.Fahn, S.Treatment of dystonia with high-dosage anticholinergic medication. Neurology 1979; 29: 605.Google Scholar
6.Fahn, S.High dosage anticholinergic therapy in dystonia. Neurol-ogy 1983; 33: 1255–1261.Google ScholarPubMed
7.Burke, RE, Fahn, S, Marsden, CD.Torsion dystonia: A double-blind, prospective trial of high-dosage trihexyphenidyl. Neurology 1986; 36: 160–164.CrossRefGoogle ScholarPubMed
8.Duvoisin, RC.Meige syndrome: Relief on high-dose anticholinergic therapy. Clinical Neuropharmacology 1983; 6: 63–66.CrossRefGoogle ScholarPubMed
9.Lang, AE.High dose anticholinergic therapy in adult dystonia. Canadian J Neurol Sci 1986; 13: 42–46.CrossRefGoogle ScholarPubMed
10.Marsden, CD, Marion, M-H, Quinn, N.The treatment of severe dystonia in children and adults. J Neurol Neurosurg Psychiatry 1984; 47: 1166–1173.CrossRefGoogle ScholarPubMed
11.Greene, PE, Shale, H, Fahn, S.Analysis of a large series of patients with torsion dystonia treated with high dosages of anticholinergic and other drugs. Advances in Neurology. In press.Google Scholar
12.Lang, AE.Dopamine agonists in the treatment of dystonia. Clinical Neuropharmacology 1985; 8: 38–57.CrossRefGoogle ScholarPubMed
13.Segawa, M, Hosaka, A, Miyagawa, F, et al. Hereditary progressive dystonia with marked diurnal fluctuation. Adv Neurol 1976; 14: 215–233.Google ScholarPubMed
14.Deonna, T.DOPA-sensitive progressive dystonia of childhood with fluctuations of symptoms: Segawa’s syndrome and possible variants. Neuropediatrics 1986; 17: 81–85.CrossRefGoogle ScholarPubMed
15.Rondot, P, Ziegler, M.Dystonia — L-dopa responsive or juvenile parkinsonism? J Neural Transm Suppl 1983; 19: 273–281.Google ScholarPubMed
16.Nygaard, TG, Marsden, CD, Duvoisin, RC.Dopa-responsive dystonia. Advances in Neurology. In press.Google Scholar
17.Burns, CLC.The treatment of torsion spasm in children with trihexyphenidyl (Artane). The Medical Press 1959;241: 148–149.Google Scholar
18.Corner, BD.Dystonia musculorum deformans in siblings treated with Artane (trihexyphenidyl). Proc Royal Soc Med 1952; 45: 51–452.Google ScholarPubMed
19.Newman, RP, Lewitt, PA, Shults, C, et al. Dystonia: treatment with bromocriptine. Clinical Neuropharmacology 1985; 8: 328–333.CrossRefGoogle ScholarPubMed
20.Nutt, JG, Hammerstad, JP, Carter, JH, et al. Lisuride treatment of focal dystonias. Neurology 1985; 35: 1242–1243.CrossRefGoogle ScholarPubMed
21.Quinn, NP, Lang, AE, Sheehy, MP, et al. Lisuride in dystonia. Neurology 1985; 35: 766–769.CrossRefGoogle ScholarPubMed
22.Stahl, SM, Berger, PA.Bromocriptine, physostigmine, and neuro- transmitter mechanisms in the dystonias. Neurology 1982; 32: 889–892.CrossRefGoogle Scholar
23.Jankovic, J.Treatment of hyperkinetic movement disorders with tetrabenazine: A double-blind crossover study. Ann Neurol 1982; 11: 1–47.CrossRefGoogle ScholarPubMed
24.Jankovic, J, Ford, J.Blepharospasm and orofacial-cervical dystonia: clinical and pharmacological findings in 100 patients. Ann Neurol 1983; 13: 402–411.CrossRefGoogle ScholarPubMed
25.Lang, AE, Marsden, CD.Alphamethylparatyrosine and tetrabena-zine in movement disorders. Clinical Neuropharmacology 1982; 5: 375–387.CrossRefGoogle Scholar
26.Lang, AE.Dopamine agonists and antagonists in the treatment of idiopathic dystonia. Advances in Neurology 1987. In press.Google Scholar
27.Fahn, S, Hening, WA, Bressman, S, et al. Long-term usefulness of baclofen in the treatment of essential blepharospasm. Advances in Ophthalmic Plastic Reconstructive Surgery 1985; 4: 219–226.Google Scholar
28.Gollomp, SM, Fahn, S, Burke, RE, et al. Therapeutic trials in Meige syndrome. Advances in Neurology 1983; 37: 207–213.Google ScholarPubMed
29.Ziegler, DK.Prolonged relief of dy stonic movements with diazepam. Neurology 1981; 31: 1457–1458.CrossRefGoogle Scholar
30.Girotti, F, Scigliano, G, Nardocci, N, et al. Idiopathic dystonia: neuropharmacologic study. J Neurol 1982; 227: 239–247.CrossRefGoogle Scholar
31.Geller, M, Kaplan, B, Cristoff, N.Treatment of dystonia symptoms with carbamazepine. Advances in Neurology 1976; 14: 403–410.Google ScholarPubMed
32.Isgreen, WP, Fahn, S, Barrett, RE, et al. Carbamazepine in torsion dystonia. Advances in Neurology 1976; 14: 411–416.Google Scholar
33.Sheehy, MP, Marsden, CD.Writer’s cramp — a focal dystonia.Brain 1982; 105: 461–480.CrossRefGoogle ScholarPubMed
34.Fahn, S.The tardive dyskinesias. In: Matthews, WB, Glaser, GH, eds: Recent Advances in Clinical Neurology. Churchill Livingstone, Edinburgh, 1984; 4: 229–260.Google Scholar