Treatment of Focal Dystonia
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Opinion statement
Dystonia is characterized by repetitive twisting movements or abnormal postures due to involuntary muscle activity. When limited to a single body region it is called focal dystonia. Examples of focal dystonia include cervical dystonia (neck), blepharospasm (eyes), oromandibular dystonia, focal limb dystonia, and spasmodic dysphonia, which are discussed here. Once the diagnosis is established, the therapeutic plan is discussed with the patients. They are informed that there is no cure for dystonia and treatment is symptomatic. The main therapeutic option for treating focal dystonias is botulinum toxin (BoNT). There have been several attempts to characterize the procedure, the type of toxin, dosage, techniques, and combination with physical measures in each of the focal dystonia forms. The general treatment principles are similar. The affected muscles are injected at muscle sites based on evidence and experience using standard dosages based on the type of toxin used. The injections are repeated after 3 to 6 months based on the individual response duration. In the uncommon event of nonresponse with BoNT, the dose and site are reassessed. Oral drug treatment could be considered as an additional option. Once the condition is thought to be medically refractory, the opinion from the deep brain stimulation (DBS) team for the suitability of the patient for DBS is taken. The successful use of DBS in cervical dystonia has led to increased acceptance for trial in other forms of focal dystonias. DBS surgery in focal dystonias other than cervical is, however, still experimental. The patients may be offered the surgery with adequate explanation of the risks and benefits. Patient education and directing the patients towards dystonia support groups and relevant websites that provide scientific information may be useful for long-term compliance and benefit.
Keywords
Focal dystonia Treatment Botulinum toxin Anticholinergics Deep brain stimulationNotes
Disclosure
A. Batla: none. M. Stamelou: none. K.P. Bhatia has received research support from Ipsen and from the Halley Stewart Trust through Dystonia Society UK, and the Wellcome Trust MRC strategic neurodegenerative disease initiative award (Ref. number WT089698), a grant from the Dystonia Coalition, and a grant from Parkinson’s UK (Ref. number G-1009). He has received personal compensation for scientific advisory board for GlaxoSmithKline and Boehringer Ingelheim, and royalties from the publication of Oxford Specialist Handbook of Parkinson’s Disease and Other Movement Disorders (Oxford University Press, 2008). He has also received funding for travel from GlaxoSmithKline, Orion Corporation, Ipsen, and Merz Pharmaceuticals, LLC, and received speaker honoraria from GlaxoSmithKline, Ipsen, Merz Pharmaceuticals, LLC, and Sun Pharmaceutical Industries Ltd.
References and Recommended Reading
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- 1.Fahn S, Bressman S, Marsden CD. Classification of dystonia. Adv Neurol. 1998;78:1–10.PubMedGoogle Scholar
- 2.Jankovic J. Dystonic disorders. In: Tolosa JJ, editor. Parkinson’s disease and movement disorders. 5th ed. Philadelphia: Williams and Wilkins; 2007. p. 321–47.Google Scholar
- 3.Jankovic J. Treatment of dystonia. Lancet Neurol. 2006;5:864–72.PubMedCrossRefGoogle Scholar
- 4.Montal M. Botulinum neurotoxin: a marvel of protein design. Annu Rev Biochem. 2010;79:591–617.PubMedCrossRefGoogle Scholar
- 5.Simpson DM, Blitzer A, Brashear A, Comella C, et al. Assessment: Botulinum neurotoxin for the treatment of movement disorders (an evidence-based review): report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2008;70(19):1699–706.PubMedCrossRefGoogle Scholar
- 6.••Albanese A, Asmus F, Bhatia KP, et al. EFNS guidelines on diagnosis and treatment of primary dystonias. Eur J Neurol. 2011;18(1):5–18PubMedCrossRefGoogle Scholar
- 7.Albanese A, Barnes MP, Bhatia KP, et al. A systematic review on the diagnosis and treatment of primary (idiopathic) dystonia and dystonia plus syndromes: report of an EFNS/MDS-ES Task Force. Eur J Neurol. 2006;13(5):433–44.PubMedCrossRefGoogle Scholar
- 8.Costa J, Espírito-Santo C, Borges A, et al. Botulinum toxin type A therapy for cervical dystonia. Cochrane Database Syst Rev. 2005;1:CD003633.PubMedGoogle Scholar
- 9.Costa J, Espírito-Santo C, Borges A, et al. Botulinum toxin type A versus anticholinergics for cervical dystonia. Cochrane Database Syst Rev. 2005;1:CD004312.PubMedGoogle Scholar
- 10.Costa J, Espírito-Santo C, Borges A, et al. Botulinum toxin type B for cervical dystonia. Cochrane Database Syst Rev. 2005;1:CD004315.PubMedGoogle Scholar
- 11.Costa J, Borges A, Espírito-Santo C, Ferreira J, et al. Botulinum toxin type A versus botulinum toxin type B for cervical dystonia. Cochrane Database Syst Rev. 2005;1:CD004314.PubMedGoogle Scholar
- 12.Lew MF, Adornato BT, Duane DD, et al. Botulinum toxin type B: a double-blind, placebo-controlled, safety and efficacy study in cervical dystonia. Neurology. 1997;49(3):701–7.PubMedCrossRefGoogle Scholar
- 13.Brashear A, Lew MF, Dykstra DD, et al. Safety and efficacy of NeuroBloc (Botulinum toxin type B) in type A-responsive cervical dystonia. Neurology. 1999;53(7):1439–46.PubMedCrossRefGoogle Scholar
- 14.Brin MF, Lew MF, Adler CH, et al. Safety and efficacy of NeuroBloc (Botulinum toxin type B) in type A-resistant cervical dystonia. Neurology. 1999;53(7):1431–8.PubMedCrossRefGoogle Scholar
- 15.Comella CL, Jankovic J, Shannon KM, Dystonia Study Group, et al. Comparison of botulinum toxin serotypes A and B for the treatment of cervical dystonia. Neurology. 2005;65(9):1423–9.PubMedCrossRefGoogle Scholar
- 16.Pappert EJ, Germanson T, Myobloc/Neurobloc European Cervical Dystonia Study Group. Botulinum toxin type B vs. type A in toxin-naïve patients with cervical dystonia: Randomized, double-blind, non inferiority trial. Mov Disord. 2008;23(4):510–7.PubMedCrossRefGoogle Scholar
- 17.Poewe W, Schelosky L, Kleedorfer B, et al. Treatment of spasmodic torticollis with local injections of botulinum toxin. One-year follow-up in 37 patients. J Neurol. 1992;239(1):21–5.PubMedCrossRefGoogle Scholar
- 18.Truong D, Duane DD, Jankovic J, et al. Efficacy and safety of botulinum type A toxin (Dysport) in cervical dystonia: results of the first US randomized, double-blind, placebo-controlled study. Mov Disord. 2005;20(7):783–91.PubMedCrossRefGoogle Scholar
- 19.Benecke R, Jost WH, Kanovsky P, et al. A new botulinum toxin type A free of complexing proteins for treatment of cervical dystonia. Neurology. 2005;64(11):1949–51.PubMedCrossRefGoogle Scholar
- 20.Brans JW, Lindeboom R, Snoek JW, et al. Botulinum toxin versus trihexyphenidyl in cervical dystonia: a prospective, randomized, double-blind controlled trial. Neurology. 1996;46(4):1066–72.PubMedCrossRefGoogle Scholar
- 21.Greene P, Kang U, Fahn S, et al. Double-blind, placebo-controlled trial of botulinum toxin injections for the treatment of spasmodic torticollis. Neurology. 1990;40:1213–8.PubMedCrossRefGoogle Scholar
- 22.Marchetti A, Magar R, Findley L, et al. Retrospective evaluation of the dose of Dysport and BOTOX in the management of cervical dystonia and blepharospasm: the REAL DOSE study. Mov Disord. 2005;20(8):937–44.PubMedCrossRefGoogle Scholar
- 23.Odergren T, Hjaltason H, Kaakkola S, et al. A double blind, randomised, parallel group study to investigate the dose equivalence of Dysport and Botox in the treatment of cervical dystonia. J Neurol Neurosurg Psychiatry. 1998;64(1):6–12.PubMedCrossRefGoogle Scholar
- 24.Edwards M, Quinn N, Bhatia K. Dystonia. In: Edwards M, Quinn N, Bhatia K, editors. Oxford specialist handbook: Parkinson’s disease and other movement disorders. Oxford: Oxford University Press; 2008. p. 202–7.CrossRefGoogle Scholar
- 25.Allergan. BOTOX® Prescribing Information [online]. Available at: http://www.allergan.com/assets/pdf/botox_pi.pdf
- 26.•Burbaud P, Ducerf C, Cugy E, et al. Botulinum toxin treatment in neurological practice: how much does it really cost? A prospective cost-effectiveness study. J Neurol. 2011;258(9):1670–5.PubMedCrossRefGoogle Scholar
- 27.Comella CL. The treatment of cervical dystonia with botulinum toxins. J Neural Transm. 2008;115(4):579–83.PubMedCrossRefGoogle Scholar
- 28.Jankovic J, Leder S, Warner D, et al. Cervical dystonia: clinical findings and associated movement disorders. Neurology. 1991;41(7):1088–91.PubMedCrossRefGoogle Scholar
- 29.Jankovic J, Vuong KD, Ahsan J. Comparison of efficacy and immunogenicity of original versus current botulinum toxin in cervical dystonia. Neurology. 2003;60(7):1186–8.PubMedCrossRefGoogle Scholar
- 30.••Lawrence I, Moy R. An evaluation of neutralizing antibody induction during treatment of glabellar lines with a new US formulation of botulinum neurotoxin type A. Aesthet Surg J. 2009;29(6):S66–71.PubMedCrossRefGoogle Scholar
- 31.••Skogseid IM, Ramm-Pettersen J, Volkmann J, et al. Good long-term efficacy of pallidal stimulation in cervical dystonia: a prospective, observer-blinded study. Eur J Neurol. 2011. doi: 10.1111/j.1468-1331.2011.03591.x.This is a recent study assessing long-term efficacy of DBS in CD in a blinded form.
- 32.Kiss ZH, Doig-Beyaert K, Eliasziw M, et al. The Canadian multicentre study of deep brain stimulation for cervical dystonia. Functional and Stereotactic Section of the Canadian Neurosurgical Society; Canadian Movement Disorders Group. Brain. 2007;130(11):2879–86.PubMedCrossRefGoogle Scholar
- 33.Pretto TE, Dalvi A, Kang UJ, et al. A prospective blinded evaluation of deep brain stimulation for the treatment of secondary dystonia and primary torticollis syndromes. J Neurosurg. 2008;109(3):405–9.PubMedCrossRefGoogle Scholar
- 34.Hung SW, Hamani C, Lozano AM, et al. Long-term outcome of bilateral pallidal deep brain stimulation for primary cervical dystonia. Neurology. 2007;68(6):457–9.PubMedCrossRefGoogle Scholar
- 35.••Cacciola F, Farah JO, Eldridge PR, et al. Bilateral deep brain stimulation for cervical dystonia: long-term outcome in a series of 10 patients. Neurosurgery. 2010;67(4):957–63.PubMedCrossRefGoogle Scholar
- 36.••Tagliati M, Krack P, Volkmann J, et al. Long-term management of DBS in dystonia: response to stimulation, adverse events, battery changes, and special considerations. Mov Disord. 2011;26(1):S54–62.PubMedCrossRefGoogle Scholar
- 37.••Jeong SG, Lee MK, Kang JY, et al. Pallidal deep brain stimulation in primary cervical dystonia with phasic type: clinical outcome and postoperative course. J Korean Neurosurg Soc. 2009;46(4):346–50.PubMedCrossRefGoogle Scholar
- 38.Yianni J, Green AL, McIntosh E, et al. The costs and benefits of deep brain stimulation surgery for patients with dystonia: an initial exploration. Neuromodulation. 2005;8(3):155–61.PubMedCrossRefGoogle Scholar
- 39.Bertrand CM. Selective peripheral denervation for spasmodic torticollis: surgical technique, results, and observations in 260 cases. Surg Neurol. 1993;40(2):96–103.PubMedCrossRefGoogle Scholar
- 40.Chen X, Ma A, Liang J, et al. Selective denervation and resection of cervical muscles in the treatment of spasmodic torticollis: long-term follow-up results in 207 cases. Stereotact Funct Neurosurg. 2000;75(2–3):96–102.PubMedCrossRefGoogle Scholar
- 41.Münchau A, Palmer JD, Dressler D, et al. Prospective study of selective peripheral denervation for botulinum-toxin resistant patients with cervical dystonia. Brain. 2001;124(4):769–83.PubMedCrossRefGoogle Scholar
- 42.National Institute for Clinical Excellence. Interventional Procedure Consultation Document Selective peripheral denervation for cervical dystonia www.nice.org.uk/nicemedia/pdf/ip/IPG080guidance.pdf.
- 43.Burke RE, Fahn S. Double-blind evaluation of trihexyphenidyl in dystonia. Adv Neurol. 1983;37:189–92.PubMedGoogle Scholar
- 44.Jankovic J. Treatment of hyperkinetic movement disorders with tetrabenazine: a double-blind crossover study. Ann Neurol. 1982;11:41–7.PubMedCrossRefGoogle Scholar
- 45.Gilbert GJ. The medical treatment of spasmodic torticollis. Arch Neurol. 1972;27:503–6.PubMedCrossRefGoogle Scholar
- 46.West HH. Treatment of spasmodic torticollis with amantadine: a double blind study. Neurology. 1977;27:198–9.PubMedCrossRefGoogle Scholar
- 47.Shaw KM, Hunter KR, Stren GM. Medical treatment of spasmodic Torticollis. Lancet. 1972;1(7765):1399.PubMedCrossRefGoogle Scholar
- 48.Ansari KA, Webster D, Manning N. Spasmodic torticollis and L-Dopa: results of therapeutic trial in six patients. Neurology. 1972;22:672–4.CrossRefGoogle Scholar
- 49.Myerson A, Loman J. Amphetamine sulfate in treatment of spasmodic torticollis. Arch Neurol Psychiatry. 1942;48:823–8.Google Scholar
- 50.Jantunen J, Kaste M, Iivanainen M, et al. Bromocriptine treatment of spasmodic torticollis: a double blind crossover study. Arch Neurol. 1979;36:449–50.CrossRefGoogle Scholar
- 51.Truong D, Comella C, Fernandez HH, Dysport Benign Essential Blepharospasm Study Group, et al. Efficacy and safety of purified botulinum toxin type A (Dysport) for the treatment of benign essential blepharospasm: a randomized, placebo-controlled, phase II trial. Parkinsonism Relat Disord. 2008;14(5):407–14.PubMedCrossRefGoogle Scholar
- 52.••Jankovic J, Comella C, Hanschmann A, et al. Efficacy and safety of incobotulinumtoxinA (NT 201, Xeomin) in the treatment of blepharospasm-a randomized trial. Mov Disord. 2011;26(8):1521–8.PubMedCrossRefGoogle Scholar
- 53.••Wabbels B, Reichel G, Fulford-Smith A, et al. Double-blind, randomised, parallel group pilot study comparing two botulinum toxin type A products for the treatment of blepharospasm. J Neural Transm. 2011;118(2):233–9.PubMedCrossRefGoogle Scholar
- 54.Jankovic J, Orman J. Botulinum A toxin for cranial cervical dystonia: a double-blind, placebo-controlled study. Neurology. 1987;37:616–23.PubMedCrossRefGoogle Scholar
- 55.Nussgens Z, Roggenkamper P. Comparison of two botulinum-toxin preparations in the treatment of essential blepharospasm. Graefes Arch Clin Exp Ophthalmol. 1997;235:197–9.PubMedCrossRefGoogle Scholar
- 56.Sampaio C, Ferreira JJ, Simoes F, et al. DYSBOT: a single-blind, randomized parallel study to determine whether any differences can be detected in the efficacy and tolerability of two formulations of botulinum toxin type A—Dysport and Botox—assuming a ratio of 4:1. Mov Disord. 1997;12:1013–8.PubMedCrossRefGoogle Scholar
- 57.Roggenkamper P, Jost WH, Bihari K, et al. Efficacy and safety of a new botulinum toxin type A free of complexing proteins in the treatment of blepharospasm. J Neural Transm. 2006;113:303–12.PubMedCrossRefGoogle Scholar
- 58.•Yomtoob DE, Dewan MA, Lee MS, et al. Comparison of pain scores with 30-gauge and 32-gauge needles for periocular botulinum toxin type A injections. Ophthalmic Plast Reconstr Surg. 2009;25(5):376–7.CrossRefGoogle Scholar
- 59.•Boyle MH, McGwin Jr G, Flanagan CE, et al. High versus low concentration botulinum toxin A for benign essential blepharospasm: does dilution make a difference? Ophthalmic Plast Reconstr Surg. 2009;25(2):81–4.CrossRefGoogle Scholar
- 60.•Reese R, Gruber D, Schoenecker T, et al. Long-term clinical outcome in meige syndrome treated with internal pallidum deep brain stimulation. Mov Disord. 2011;26(4):691–8.PubMedCrossRefGoogle Scholar
- 61.Brin MF. Oromandibular dystonia: treatment of 96 patients with botulinum toxin type A. In: Jankovic J, Hallett M, editors. Therapy with Botulinum Toxin. New York: Marcel Dekker; 1994. p. 429–35.Google Scholar
- 62.Blitzer A, Brin MF, Greene PE, et al. Botulinum toxin injection for the treatment of oromandibular dystonia. Ann Otol Rhinol Laryngol. 1989;98:93–7.PubMedGoogle Scholar
- 63.Hermanowicz N, Truong DD. Treatment of oromandibular dystonia with botulinum toxin. Laryngoscope. 1991;101:1216–8.PubMedCrossRefGoogle Scholar
- 64.Van den Bergh P, Francart J, Mourin S, et al. Five-year experience in the treatment of focal movement disorders with low-dose Dysport botulinum toxin. Muscle Nerve. 1995;18:720–9.PubMedCrossRefGoogle Scholar
- 65.Jankovic J, Orman J. Botulinum A toxin for cranial-cervical dystonia: a double-blind, placebo-controlled study. Neurology. 1987;37:616–23.PubMedCrossRefGoogle Scholar
- 66.Tan EK, Jankovic J. Botulinum toxin A in patients with oromandibular dystonia: long-term follow-up. Neurology. 1999;53:2102–7.PubMedCrossRefGoogle Scholar
- 67.Bhidayasiri R, Cardoso F, Truong DD. Botulinum toxin in blepharospasm and oromandibular dystonia: comparing different botulinum toxin preparations. Eur J Neurol. 2006;13(1):21–9.PubMedCrossRefGoogle Scholar
- 68.•Hallett M, Benecke R, Blitzer A, et al. Treatment of focal dystonias with botulinum neurotoxin. Toxicon. 2009;54(5):628–33.PubMedCrossRefGoogle Scholar
- 69.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.PubMedCrossRefGoogle Scholar
- 70.Klawans HL, Tanner CM. Cholinergic pharmacology of blepharospasm with oromandibular dystonia (Meige’s syndrome). Adv Neurol. 1988;49:443–50.PubMedGoogle Scholar
- 71.•Ludlow CL. Spasmodic dysphonia: a laryngeal control disorder specific to speech. J Neurosci. 2011;31(3):793–7.PubMedCrossRefGoogle Scholar
- 72.Watts CC, Whurr R, Nye C. Botulinum toxin injections for the treatment of spasmodic dysphonia. Cochrane Database Syst Rev. 2004:CD004327.Google Scholar
- 73.Watts CR, Nye C, Whurr R. Botulinum toxin for treating spasmodic dysphonia (laryngeal dystonia): a systematic Cochrane review. Clin Rehabil. 2006;20(2):112–22.PubMedCrossRefGoogle Scholar
- 74.Boutsen F, Cannito MP, Taylor M, et al. Botox treatment in adductor spasmodic dysphonia: a meta-analysis. J Speech Lang Hear Res. 2002;45(3):469–81.PubMedCrossRefGoogle Scholar
- 75.Whurr R, Nye C, Lorch M. Meta-analysis of botulinum toxin treatment of spasmodic dysphonia: a review of 22 studies. Int J Lang Commun Disord. 1998;33:327–9.PubMedCrossRefGoogle Scholar
- 76.Troung DD, Rontal M, Rolnick M, et al. Double-blind controlled study of botulinum toxin in adductor spasmodic dysphonia. Laryngoscope. 1991;101:630–4.PubMedGoogle Scholar
- 77.Wong DL, Adams SG, Irish JC, et al. Effect of neuromuscular activity on the response to botulinum toxin injections in spasmodic dysphonia. J Otolaryngol. 1995;24:209–16.PubMedGoogle Scholar
- 78.Bielamowicz S, Squire S, Bidus K, et al. Assessment of posterior cricoarytenoid botulinum toxin injections in patients with abductor spasmodic dysphonia. Ann Otol Rhinol Laryngol. 2001;110:406–12.PubMedGoogle Scholar
- 79.Sulica L, Blitzer A, Brin MF, et al. Botulinum toxin management of adductor spasmodic dysphonia after failed recurrent laryngeal nerve section. Ann Otol Rhinol Laryngol. 2003;112(6):499–505.PubMedGoogle Scholar
- 80.Kruisdijk JJ, Koelman JH, Ongerboer de Visser BW, et al. Botulinum toxin for writer’s cramp: a randomised, placebo-controlled trial and 1-year follow-up. J Neurol Neurosurg Psychiatry. 2007;78:264–70.PubMedCrossRefGoogle Scholar
- 81.Yoshimura DM, Aminoff MJ, Olney RK. Botulinum toxin therapy for limb dystonias. Neurology. 1992;42:627–30.PubMedCrossRefGoogle Scholar
- 82.Tsui JK, Bhatt M, Calne S, Calne DB. Botulinum toxin in the treatment of writer’s cramp: a double-blind study. Neurology. 1993;43:183–5.PubMedCrossRefGoogle Scholar
- 83.Cole R, Hallett M, Cohen LG. Double-blind trial of botulinum toxin for treatment of focal hand dystonia. Mov Disord. 1995;10:466–71.PubMedCrossRefGoogle Scholar
- 84.Marion MH, Afors K, Sheehy MP. Problems of treating writer’s cramp with botulinum toxin injections: results from 10 years of experience. Rev Neurol (Paris). 2003;159:923–7.Google Scholar
- 85.Molloy FM, Shill HA, Kaelin-Lang A, et al. Accuracy of muscle localization without EMG: implications for treatment of limb dystonia. Neurology. 2002;58:805–7.PubMedCrossRefGoogle Scholar
- 86.Geenen C, Consky E, Ashby P. Localizing muscles for botulinum toxin treatment of focal hand dystonia. Can J Neurol Sci. 1996;23:194–7.PubMedGoogle Scholar
- 87.Chen R, Karp BI, Goldstein SR, et al. Effect of muscle activity immediately after botulinum toxin injection for writer’s cramp. Mov Disord. 1999;14:307–12.PubMedCrossRefGoogle Scholar
- 88.Fukaya C, Katayama Y, Kano T, et al. Thalamic deep brain stimulation for writer’s cramp. J Neurosurg. 2007;107(5):977–82.PubMedCrossRefGoogle Scholar