Distonias: aspectos terapêuticos

Limongi, João Carlos Papaterra

doi:10.1590/S0004-282X1996000100025

Resumos

Diversas abordagens terapêuticas são utilizadas em pacientes com distonias. Sempre que possível, causas específicas devem ser identificadas e tratadas. As modalidades de tratamento sintomático podem ser agrupadas em três categorias: tratamento farmacológico, cirúrgico e injeções locais de toxina botulínica. Cada uma dessas modalidades apresenta algumas vantagens e limitações. Formas generalizadas, particularmente as de ocorrência na infância, podem se beneficiar com drogas anticolinérgicas ou, em alguns casos, com a levodopa ou outros agentes tais como antagonistas da dopamina, baclofeno e benzodiazepínicos. As formas focais não respondem adequadamente ao tratamento farmacológico sistêmico mas beneficiam-se significativamente com injeções de toxina botulínica nos grupos musculares acometidos. Cerca de 90% dos pacientes com blefarospasmo e 70% daqueles com distonia cervical apresentam resposta satisfatória a esse tipo de terapia. O tratamento cirúrgico tem sido utilizado em algumas formas de distonias generalizadas (lesões estereotáxicas), axiais (rizotomias) ou focais (miectomias e neurectomias) com resultados variáveis.

distonias; blefarospasmo; distonia cervical; toxina botulínica

Several approaches have been employed for the treatment of dystonias. Possible specific causes should be searched for and specific treatment should be instituted. Different types of symptomatic treatment are grouped according to the following categories: pharmacological systemic therapy, surgical therapy and botulinum toxin injections in the affected muscles. Each of these approaches has its advantages and limitations. Generalized dystonias should be treated with anticholinergic agents. In some cases, levodopa or other drugs such as dopamine antagonists, baclofen and benzodiazepines should be preferred. Focal dystonias respond dramatically to local injections of botulinum toxin. Over 90% of patients with blepharospasm and 70% of patients with cervical dystonia present a satisfactory response to this procedure. Surgical approaches have been utilized in some cases of generalized (stereotaxic lesions), axial (rhizotomies) and focal dystonias (myectomies and neurectomies) with variable results.

dystonias; blepharospasm; cervical dystonia; botulinum toxin

Distonias: aspectos terapêuticos

Dystonias: therapeutic aspects

João Carlos Papaterra Limongi

Doutor, Médico Assistente da Divisão de Neurologia do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo

RESUMO

Diversas abordagens terapêuticas são utilizadas em pacientes com distonias. Sempre que possível, causas específicas devem ser identificadas e tratadas. As modalidades de tratamento sintomático podem ser agrupadas em três categorias: tratamento farmacológico, cirúrgico e injeções locais de toxina botulínica. Cada uma dessas modalidades apresenta algumas vantagens e limitações. Formas generalizadas, particularmente as de ocorrência na infância, podem se beneficiar com drogas anticolinérgicas ou, em alguns casos, com a levodopa ou outros agentes tais como antagonistas da dopamina, baclofeno e benzodiazepínicos. As formas focais não respondem adequadamente ao tratamento farmacológico sistêmico mas beneficiam-se significativamente com injeções de toxina botulínica nos grupos musculares acometidos. Cerca de 90% dos pacientes com blefarospasmo e 70% daqueles com distonia cervical apresentam resposta satisfatória a esse tipo de terapia. O tratamento cirúrgico tem sido utilizado em algumas formas de distonias generalizadas (lesões estereotáxicas), axiais (rizotomias) ou focais (miectomias e neurectomias) com resultados variáveis.

Palavras-chave:distonias, blefarospasmo, distonia cervical, toxina botulínica.

ABSTRACT

Several approaches have been employed for the treatment of dystonias. Possible specific causes should be searched for and specific treatment should be instituted. Different types of symptomatic treatment are grouped according to the following categories: pharmacological systemic therapy, surgical therapy and botulinum toxin injections in the affected muscles. Each of these approaches has its advantages and limitations. Generalized dystonias should be treated with anticholinergic agents. In some cases, levodopa or other drugs such as dopamine antagonists, baclofen and benzodiazepines should be preferred. Focal dystonias respond dramatically to local injections of botulinum toxin. Over 90% of patients with blepharospasm and 70% of patients with cervical dystonia present a satisfactory response to this procedure. Surgical approaches have been utilized in some cases of generalized (stereotaxic lesions), axial (rhizotomies) and focal dystonias (myectomies and neurectomies) with variable results.

Key-words: dystonias, blepharospasm, cervical dystonia, botulinum toxin

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Full text available only in PDF format.

Aceite: 20-novembro-1995.

Dr. João Carlos Papaterra Limongi - Rua Atlântica 460 - 01440-000 São Paulo SP - Brasil

1. Alderson K, Holds JB, Anderson RL. Botulinum-induced alteration of nerve-muscle interactions in the human orbicularis oculi following treatment for blepharospasm. Neurology 1991; 41:1800-5.
2. Anderson RL, Patrinely JR. Surgical management of blepharospasm.In Jankovic J, Tolosa E. (eds). Facial dyskinesias.Advances in Neurology. New York: Raven Press, 1988; 49:501-520.
3. Andrade LAF, Brucki SMD. Botulinum toxin A for trismus in cephalic tetanus. Arq Neuropsiquiatr 1994; 52:410-3.
4. Andrew J, Fowler CJ, Harrison MJG. Stereotaxic thalamotomy in 55 cases of dystonia. Brain 1983; 106:981-1000.
5. Barinaga M. Secrets of secretion revealed. Science 1993; 260:487-9.
6. Bertrand C. Peripheral versus central surgical approach for the treatment of spasmodic torticollis. In: Marsden CD, Fahn S, (eds). Movement disorders. London: Butterworth, 1982; 315-318.
7. Bertrand CM, Molina-Negro P. Selective peripheral denervation in 111 cases of spasmodic torticollis: rationale and results. In Fahn S, Marsden CD, Calne DB, (eds) Dystonia 2. Advances in Neurology. New York: Raven Press, 1988; 50:637-643.
8. Blackie JD, Lees AJ. Botulinum toxin treatment in spasmodic torticollis. J Neurol Neurosurg Psychiatry 1990; 53:640-3.
9. Bressman SB, Fahn S, Burke RE. Paroxismal non-kinesigenic dystonia. In Fahn S, Marsden CD, Calne DB, (eds) Dystonia 2. Advances in Neurology . New York: Raven Press, 1988; 50:403-13.
10. Brin MF, Blitzer A, Stewart C, Fahn S. Treatment of spasmodic dysphonia (laryngeal dystonia) with local injections of botulinum toxin: review and technical aspects. In Blitzer A, Brin MF, Sasaki CT, Fahn S, Harris KS (eds). Neurologic disorders of the larynx. New York: Springer Verlag, 1992; 214-28.
11. Brin MF, Fahn S.Moskowitz C, Friedman A, Shale HM, Greene PE et al. Localized injections of botulinum toxin for the treatment of focal dystonia and hemifacial spasm. Mov Disord 1987; 2:237-54.
12. Cohen LG, Hallet M, Warden M, Dambrosia J. Excitability of blink reflexes in patients with blepharospasm after successful treatment with botulinum toxin. Ann Neurol 1987; 22: 172.
13. Comelia CL, Tanner CM, DeFor L, Smith C. Dysphagia after botulinum toxin injections for spasmodic torticollis: clinical and radiological findings. Neurology 1992; 42:1307-10.
14. Cooper IS. Dystonia: surgical approaches to treatment and physiologic implications. In Yahr MD. (ed). The basal ganglia. New York: Raven Press, 1976; 369-383.
15. Dubinsky RM, Grey CS, Vetere-Overfield B, Koller WC. Electromyographic guidance of botulinum toxin treatment in cervical dystonia. Clin Neuropharmacol 1991; 14:262-7.
16. Fahn S. Treatment of dystonia with high dosage anti-cholinergics. Neurology 1979; 29:605.
17. Fahn S, Marsden CD. The treatment of dystonia. In Marsden CD, Fahn S (eds) Movement disorders 2. London: Butterworths, 1987; 359-382.
18. Garner CG, Straube A, Witt TN, Gasser T, Oertel WH. Time course of distant effects of local injections of botulinum toxin. Mov Disord 1993; 8:33-7.
19. Greene P, Fahn S, Diamond B. Development of resistance to botulinum toxin type A in patients with torticollis. Mov Disord 1994; 9:213-7.
20. Greene P, Kang U, Fahn S, Brin M, Moskowitz C, Flaster E. Double-blind, placebo-controlled trial of botulinum toxin injections for the treatment of spasmodic torticollis. Neurology 1990; 40:1213-18.
21. Greene PE, Fahn S. Use of botulinum toxin type F injections to treat torticollis in patients with immunity to botulinum toxin type A. Mov Disord 1993; 8:479-83.
22. Huttner WB. Snappy exotoxins. Nature 1993; 365:104-5.
23. Jabbari B, Paul J, Scherokman B, Gunderson CH, Rosenberg ML, Müller J. Treatment of movement disorders with trihexyphenidyl. Mov Disord 1989; 4:202-12.
24. Jankovic J. Botulinum toxin in the treatment of dystonic tics. Mov Disord 1994; 9:347-9.
25. Jankovic J, Brin MF. Therapeutic uses of botulinum toxin. N Engl J Med 1991; 324:1186-94.
26. Jankovic J, Fahn S. Dystonic syndromes. In Jankovic J, Tolosa E. (eds) Parkinson's disease and movement disorders. Baltimore: Urban-Scharzenberg, 1988; 283-314.
27. Jankovic J, Schwartz KS. Botulinum toxin injections for cervical dystonia. Neurology 1990; 40:277-80.
28. Jankovic J, Schwartz KS. Clinical correlates of response to botulinum toxin injections. Arch Neurol 1990; 48: 1253-6.
29. Jankovic J, Schwartz KS. Botulinum toxin treatment of tremors. Neurology 1991; 41:1185-8.
30. Jankovic J, Schwartz KS. Logitudinal experience with botulinum toxin injections for treatment of blepharospasm and cervcal dystonia. Neurology 1993; 43:834-6.
31. Kao I, Drachman DB, Price DL. Botulinum toxin: mechanism of presynaptic blockade. Science 1976; 193:1256-8.
32. Karp BI, Cole RA, Cohen LG, Grill S, Lou JS, Hallet M. Long-term botulinum toxin treatment of focal hand dystonia. Neurology 1994; 44:70-6.
33. Limongi JCP. Utilização da toxina botulínica tipo A na terapêutica das distonias. Tese de Doutorado, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Abstract in Arq Neuropsiquiatr 1995; 53:531-2.
34. Manning KA, Evinger C, Sibony PA. Eyelid movements before and after botulinum therapy in patients with lid spasms. Ann Neurol 1990; 28:653-60.
35. Marsden CD, Lang AE, Sheehy MP. Pharmacology of cranial dystonia. Neurology 1983; 33:1100-1.
36. Marsden CD, Marion MH, Quinn NP. The treatment of severe dystonia in children and adults. J Neurol Neurosurg Psychiatry 1984;47:1166-73.
37. Marsden CD, Parkes JD, Quinn NP. Fluctuations of disability of Parkinson's disease: clinical aspects. In Marsden CD, Fahn S (eds) Movement disorders. London: Butterworths, 1982; 96-122.
38. McCord CD, Coles WH, Shore JW, Spector R, Putnam JR. The surgical treatment of essential blepharospasm: I. Comparison of facial nerve avulsion and eyebrow-eyelid muscle stripping procedures. Arch Ophthalmol 1984; 102:266-8.
39. Narayan RK, Loubse PG, Jankovic J. Intrathecal baclofen for intractable axial dystonia. Neurology 1991; 41:1141-2.
40. Polo KB, Jabbari B. Botulinum toxin-A improves the rigidity of progressive supranuclear palsy. Ann Neurol 1994; 35:237-9.
41. Quinn NP, Lang AE, Sheehy MP, Marsden CD. Lisuride in dystonia. Neurology 1985; 35:766-9.
42. Rivest J, Lees AJ, Marsden CD. Writer's cramp: treatment with botulinum toxin injections. Mov Disord 1991; 6:55-9.
43. Sanders DB, Massey EW, Buckley EG. Botulinum toxin for blepharospasm: single-fiber EMG studies. Neurology 1986; 36:545-7.
44. Scheiberg IH, Stemlieb I. Wilson's disease. Philadelphia: Saunders, 1984.
45. Schiavo G, Benfenati F, Poulain B. Tetanus and botulinum-B neurotoxins block neurotransmitter release by proteolytic cleavage of synaptobrevin. Nature 1992; 359:832-5.
46. Scott AB. Botulinum toxin injection into extraocular muscles as an alternative to strabismus surgery. Ophthalmology 1980; 87:1044-9.
47. Sethi KD, Hess DC, Huffnagle VH, Adams RJ. Acetazolamide treatment of paroxismal dystonia in central demyelinating disease. Neurology 1992; 42:919-21.
48. Simpson LL. Molecular pharmacology of botulinum toxin and tetanus toxin. Annu Rev Pharmacol Toxicol 1986; 26:427-553.
49. Tasker RR, Doorly T, Yamashiro K. Thalamotomy in generalized dystonia. In Fahn S, Marsden CD, Calne DB, (eds) Dystonia 2. Advances in Neurology. New York: Raven Press, 1988; 50:615-631.
50. Tsui JK, Wong NLM, Wong E, Calne DB. Production of circulating antibodies to botulinum-A toxin in patients receiving repeated injections for dystonia [Abstr]. Ann Neurol 1988; 23:181.
51. Zuber M, Sebald M, Bathien N, de Recondo J, Rondot P. Botulinum antibodies in dystonic patients treated with type A botulinum toxin: frequence and significance. Neurology 1993; 43:1715-8.

Datas de Publicação

Publicação nesta coleção
07 Dez 2010
Data do Fascículo
Mar 1996

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Alderson K, Holds JB, Anderson RL. Botulinum-induced alteration of nerve-muscle interactions in the human orbicularis oculi following treatment for blepharospasm. Neurology 1991; 41:1800-5.

[2] 2. Anderson RL, Patrinely JR. Surgical management of blepharospasm.In Jankovic J, Tolosa E. (eds). Facial dyskinesias.Advances in Neurology. New York: Raven Press, 1988; 49:501-520.

[3] 3. Andrade LAF, Brucki SMD. Botulinum toxin A for trismus in cephalic tetanus. Arq Neuropsiquiatr 1994; 52:410-3.

[4] 4. Andrew J, Fowler CJ, Harrison MJG. Stereotaxic thalamotomy in 55 cases of dystonia. Brain 1983; 106:981-1000.

[5] 5. Barinaga M. Secrets of secretion revealed. Science 1993; 260:487-9.

[6] 6. Bertrand C. Peripheral versus central surgical approach for the treatment of spasmodic torticollis. In: Marsden CD, Fahn S, (eds). Movement disorders. London: Butterworth, 1982; 315-318.

[7] 7. Bertrand CM, Molina-Negro P. Selective peripheral denervation in 111 cases of spasmodic torticollis: rationale and results. In Fahn S, Marsden CD, Calne DB, (eds) Dystonia 2. Advances in Neurology. New York: Raven Press, 1988; 50:637-643.

[8] 8. Blackie JD, Lees AJ. Botulinum toxin treatment in spasmodic torticollis. J Neurol Neurosurg Psychiatry 1990; 53:640-3.

[9] 9. Bressman SB, Fahn S, Burke RE. Paroxismal non-kinesigenic dystonia. In Fahn S, Marsden CD, Calne DB, (eds) Dystonia 2. Advances in Neurology . New York: Raven Press, 1988; 50:403-13.

[10] 10. Brin MF, Blitzer A, Stewart C, Fahn S. Treatment of spasmodic dysphonia (laryngeal dystonia) with local injections of botulinum toxin: review and technical aspects. In Blitzer A, Brin MF, Sasaki CT, Fahn S, Harris KS (eds). Neurologic disorders of the larynx. New York: Springer Verlag, 1992; 214-28.

[11] 11. Brin MF, Fahn S.Moskowitz C, Friedman A, Shale HM, Greene PE et al. Localized injections of botulinum toxin for the treatment of focal dystonia and hemifacial spasm. Mov Disord 1987; 2:237-54.

[12] 12. Cohen LG, Hallet M, Warden M, Dambrosia J. Excitability of blink reflexes in patients with blepharospasm after successful treatment with botulinum toxin. Ann Neurol 1987; 22: 172.

[13] 13. Comelia CL, Tanner CM, DeFor L, Smith C. Dysphagia after botulinum toxin injections for spasmodic torticollis: clinical and radiological findings. Neurology 1992; 42:1307-10.

[14] 14. Cooper IS. Dystonia: surgical approaches to treatment and physiologic implications. In Yahr MD. (ed). The basal ganglia. New York: Raven Press, 1976; 369-383.

[15] 15. Dubinsky RM, Grey CS, Vetere-Overfield B, Koller WC. Electromyographic guidance of botulinum toxin treatment in cervical dystonia. Clin Neuropharmacol 1991; 14:262-7.

[16] 16. Fahn S. Treatment of dystonia with high dosage anti-cholinergics. Neurology 1979; 29:605.

[17] 17. Fahn S, Marsden CD. The treatment of dystonia. In Marsden CD, Fahn S (eds) Movement disorders 2. London: Butterworths, 1987; 359-382.

[18] 18. Garner CG, Straube A, Witt TN, Gasser T, Oertel WH. Time course of distant effects of local injections of botulinum toxin. Mov Disord 1993; 8:33-7.

[19] 19. Greene P, Fahn S, Diamond B. Development of resistance to botulinum toxin type A in patients with torticollis. Mov Disord 1994; 9:213-7.

[20] 20. Greene P, Kang U, Fahn S, Brin M, Moskowitz C, Flaster E. Double-blind, placebo-controlled trial of botulinum toxin injections for the treatment of spasmodic torticollis. Neurology 1990; 40:1213-18.

[21] 21. Greene PE, Fahn S. Use of botulinum toxin type F injections to treat torticollis in patients with immunity to botulinum toxin type A. Mov Disord 1993; 8:479-83.

[22] 22. Huttner WB. Snappy exotoxins. Nature 1993; 365:104-5.

[23] 23. Jabbari B, Paul J, Scherokman B, Gunderson CH, Rosenberg ML, Müller J. Treatment of movement disorders with trihexyphenidyl. Mov Disord 1989; 4:202-12.

[24] 24. Jankovic J. Botulinum toxin in the treatment of dystonic tics. Mov Disord 1994; 9:347-9.

[25] 25. Jankovic J, Brin MF. Therapeutic uses of botulinum toxin. N Engl J Med 1991; 324:1186-94.

[26] 26. Jankovic J, Fahn S. Dystonic syndromes. In Jankovic J, Tolosa E. (eds) Parkinson's disease and movement disorders. Baltimore: Urban-Scharzenberg, 1988; 283-314.

[27] 27. Jankovic J, Schwartz KS. Botulinum toxin injections for cervical dystonia. Neurology 1990; 40:277-80.

[28] 28. Jankovic J, Schwartz KS. Clinical correlates of response to botulinum toxin injections. Arch Neurol 1990; 48: 1253-6.

[29] 29. Jankovic J, Schwartz KS. Botulinum toxin treatment of tremors. Neurology 1991; 41:1185-8.

[30] 30. Jankovic J, Schwartz KS. Logitudinal experience with botulinum toxin injections for treatment of blepharospasm and cervcal dystonia. Neurology 1993; 43:834-6.

[31] 31. Kao I, Drachman DB, Price DL. Botulinum toxin: mechanism of presynaptic blockade. Science 1976; 193:1256-8.

[32] 32. Karp BI, Cole RA, Cohen LG, Grill S, Lou JS, Hallet M. Long-term botulinum toxin treatment of focal hand dystonia. Neurology 1994; 44:70-6.

[33] 33. Limongi JCP. Utilização da toxina botulínica tipo A na terapêutica das distonias. Tese de Doutorado, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Abstract in Arq Neuropsiquiatr 1995; 53:531-2.

[34] 34. Manning KA, Evinger C, Sibony PA. Eyelid movements before and after botulinum therapy in patients with lid spasms. Ann Neurol 1990; 28:653-60.

[35] 35. Marsden CD, Lang AE, Sheehy MP. Pharmacology of cranial dystonia. Neurology 1983; 33:1100-1.

[36] 36. Marsden CD, Marion MH, Quinn NP. The treatment of severe dystonia in children and adults. J Neurol Neurosurg Psychiatry 1984;47:1166-73.

[37] 37. Marsden CD, Parkes JD, Quinn NP. Fluctuations of disability of Parkinson's disease: clinical aspects. In Marsden CD, Fahn S (eds) Movement disorders. London: Butterworths, 1982; 96-122.

[38] 38. McCord CD, Coles WH, Shore JW, Spector R, Putnam JR. The surgical treatment of essential blepharospasm: I. Comparison of facial nerve avulsion and eyebrow-eyelid muscle stripping procedures. Arch Ophthalmol 1984; 102:266-8.

[39] 39. Narayan RK, Loubse PG, Jankovic J. Intrathecal baclofen for intractable axial dystonia. Neurology 1991; 41:1141-2.

[40] 40. Polo KB, Jabbari B. Botulinum toxin-A improves the rigidity of progressive supranuclear palsy. Ann Neurol 1994; 35:237-9.

[41] 41. Quinn NP, Lang AE, Sheehy MP, Marsden CD. Lisuride in dystonia. Neurology 1985; 35:766-9.

[42] 42. Rivest J, Lees AJ, Marsden CD. Writer's cramp: treatment with botulinum toxin injections. Mov Disord 1991; 6:55-9.

[43] 43. Sanders DB, Massey EW, Buckley EG. Botulinum toxin for blepharospasm: single-fiber EMG studies. Neurology 1986; 36:545-7.

[44] 44. Scheiberg IH, Stemlieb I. Wilson's disease. Philadelphia: Saunders, 1984.

[45] 45. Schiavo G, Benfenati F, Poulain B. Tetanus and botulinum-B neurotoxins block neurotransmitter release by proteolytic cleavage of synaptobrevin. Nature 1992; 359:832-5.

[46] 46. Scott AB. Botulinum toxin injection into extraocular muscles as an alternative to strabismus surgery. Ophthalmology 1980; 87:1044-9.

[47] 47. Sethi KD, Hess DC, Huffnagle VH, Adams RJ. Acetazolamide treatment of paroxismal dystonia in central demyelinating disease. Neurology 1992; 42:919-21.

[48] 48. Simpson LL. Molecular pharmacology of botulinum toxin and tetanus toxin. Annu Rev Pharmacol Toxicol 1986; 26:427-553.

[49] 49. Tasker RR, Doorly T, Yamashiro K. Thalamotomy in generalized dystonia. In Fahn S, Marsden CD, Calne DB, (eds) Dystonia 2. Advances in Neurology. New York: Raven Press, 1988; 50:615-631.

[50] 50. Tsui JK, Wong NLM, Wong E, Calne DB. Production of circulating antibodies to botulinum-A toxin in patients receiving repeated injections for dystonia [Abstr]. Ann Neurol 1988; 23:181.

[51] 51. Zuber M, Sebald M, Bathien N, de Recondo J, Rondot P. Botulinum antibodies in dystonic patients treated with type A botulinum toxin: frequence and significance. Neurology 1993; 43:1715-8.