Opinion statement
Therapy for most people with dystonia is symptomatic, directed at lessening the intensity of the dystonic contractions. For a small minority of patients (eg, those with dopa-responsive dystonia [DRD], Wilson’s disease, or psychogenic dystonia), specific therapy directed at one of the many causes of dystonia is available. Before initiating treatment, clinicians need to decide if a patient has a form of dystonia amenable to such therapy. The most sensitive and least costly method to diagnose DRD is a therapeutic trial of levodopa. It is, therefore, recommended to treat all those with dystonia beginning in childhood or adolescence with low-dose levodopa. For patients with generalized or segmental signs who do not respond to levodopa, other oral medications, including anticholinergics, baclofen, and benzodiazepines, may provide mild to moderate relief; these medications are often given in combinations. For those with focal dystonia, most having adult-onset disease, botulinum toxin A injections often effectively control contractions. The injections produce transient weakness and need to be repeated, generally every 3 to 5 months. There is growing renewed interest in surgical treatment. Peripheral denervating procedures may be helpful for patients with torticollis who do not obtain adequate benefit with botulinum toxin A. The central procedures of pallidotomy and pallidal stimulation are under study; their place in the treatment of the many dystonia subtypes (eg, limb vs axial, generalized vs focal, primary vs secondary) still needs to be established. There are very few studies evaluating physical and psychological therapies or the impact of diet or lifestyle in dystonia. Most clinicians consider physical therapy, including massage, a potential adjunct to medical therapy, and psychological support and stress reduction may help individuals cope with this chronic and frequently disabling condition.
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References and Recommended Reading
Fahn S, Marsden CD, Calne DB: Classification and investigation of dystonia. In Movement Disorders, edn 2. Edited by Marsden CD, Fahn S. London: Butterworth; 1987:332–358. An overview of the clinical approach to patients with dystonia.
Greene P, Kang UJ, Fahn S: Spread of symptoms in idiopathic torsion dystonia. Mov Disord 1995, 10:143–152.
Ozelius LJ, Hewett JW, Page C, et al.: The early-onset torsion dystonia gene (DYT1) encodes an ATP-binding protein. Nat Genet 1997, 17:40–48. Describes the current understanding of the gene underlying most cases of childhood-onset idiopathic dystonia.
Nutt JG, Muenter MD, Aronson A, et al.: Epidemiology of focal and generalized dystonia in Rochester, Minnesota. Mov Disord 1988, 3:188–194.
Nygaard TG, Takahashi H, Heiman GA, et al.: Longterm treatment response and fluorodopa positron emission tomographic scanning of Parkinsonism in a family with dopa-responsive dystonia. Ann Neurol 1992, 32:603–608.
Jarman PR, Bandmann O, Marsden CD, Wood NW: GTP cyclohydrolase I mutations in patients with dystonia responsive to anticholinergic drugs. J Neurol Neurosurg Psychiatry 1997, 63:304–308.
Ichinose H, Ohye T, Takahiashi E, et al.: Hereditary progressive dystonia with marked diurnal fluctuation caused by mutations in the GTP cyclohydrolase gene. Nat Genet 1994, 8:236–242.
Ludecke B, Knappskog PM, Clayton PT, et al.: Recessively inherited L-DOPA-responsive parkinsonism in infancy caused by a point mutation (L205P) in the tyrosine hydroxylase gene. Hum Mol Genet 1996, 5:1023–1028.
Hyland K, Fryburg JS, Wilson WG, et al.: Oral phenylalanine loading in dopa-responsive dystonia: a possible diagnostic test. Neurology 1997, 48:1290–1297.
Bhatia K, Marsden CD: The behavioral and motor consequences of focal lesions of the basal ganglia in man. Brain 1994, 117:859–876.
Vitek JL, Chockkan V, Zhang JY, et al.: Neuronal activity in the basal ganglia in patients with generalized dystonia and hemiballism. Ann Neurol 1999, 46:22–35.
Burke RE, Fahn S, Marsden CD: Torsion dystonia: a double-blind, prospective trial of high-dosage trihexyphenidyl. Neurology 1986, 36:160–164. Established anticholinergic therapy as the most successful approach to treating childhood-onset idiopathic dystonia.
Greene P, Shale H, Fahn S: Analysis of open-label trials in torsion dystonia using high dosages of anticholinergics and other drugs. Mov Disord 1988, 3:46–60.
Greene P, Fahn S: Baclofen in the treatment of idiopathic dystonia in children. Mov Disord 1992, 7:48–52.
Jankovic J, Brin MF: Therapeutic uses of botulinum toxin. N Engl J Med 1991, 324:1186–1194. A review of the use of botulinum toxin to treat adult-onset focal dystonia. Botulinum toxin remains the most useful treatment for patients with focal dystonia.
Ford B, Greene PE, Louis ED, et al.: Use of intrathecal baclofen in patients with dystonia. Arch Neurol 1996, 11:63–69.
Tasker RR, Doorly T, Yamashiro K: Thalamotomy in generalized dystonia. Adv Neurol 1988, 50:615–631.
Bertrand CM, Molina-Negro P: Selective peripheral denervation in 111 cases of spasmodic torticollis: rationale and results. Adv Neurol 1988, 50:637–643.
Ondo WG, Desaloms JM, Jankovic J, Grossman RG: Pallidotomy for generalized dystonia. Mov Disord 1998, 13:693–698.
Coubes P, Echenne B, Vayssiere N, et al.: Treatment of early-onset generalized dystonia by chronic bilateral stimulations of the internal globus pallidus. Neurochirurgie 1999, 45:139–144.
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Bressman, S.B., Greene, P.E. Dystonia. Curr Treat Options Neurol 2, 275–285 (2000). https://doi.org/10.1007/s11940-000-0009-y
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DOI: https://doi.org/10.1007/s11940-000-0009-y