Distonias: aspectos clínicos e terapêuticos em 64 pacientes

Mattos, James Pitágoras de; Rosso, Ana Lúcia Zuma de; Novis, Sérgio

doi:10.1590/S0004-282X1996000100005

Resumos

Os Autores apresentam a experiência em 64 pacientes, com as várias formas clínicas de apresentação de distonias, acompanhados no Setor de Doenças Extrapiramidais do Serviço de Neurologia do Hospital Universitário Clementino Fraga Filho da UFRJ, assim como, revisam a literatura, cotejando os resultados. O acompanhamento desses pacientes durante 5 anos e 6 meses resultou nas seguintes observações: 33 do sexo masculino e 31 do feminino; 48 brancos, 10 pardos e 6 negros; a média do tempo de doença foi 9 anos e 8 meses. Quanto à distribuição do movimento anormal, 30 (46,9%) eram focais; 17 (26,6%), segmentares; 13 (20,3%), generalizadas; 3 (4,7%), hemidistonias; 1 (1,5%), multifocal. Quanto à idade de início, em 11 (17,2%) se apresentou antes dos 12 anos; em 6 (9,4%), entre 13 e 20 anos; e em 47 (73,4%), após os 20 anos. Correspondiam à origem idiopática esporádica, 39 (60,9%); idiopática familiar, 6 (9,4%); sintomática, 19 (29,7%). No que se refere à abordagem terapêutica destes pacientes, destacamos o emprego de anticolinérgicos, de agonistas e antagonistas dopaminérgicos e do baclofen isolado ou associado aos anticolinérgicos para as formas generalizadas. Para as distonias focais, os Autores concluem ser a toxina botulínica do tipo A o agente terapêutico mais eficaz aconselhado atualmente.

distonia; distonia musculorum deformans; toxina botulínica

The experience with 64 patients with dystonia seen at the Extrapyramidal Diseases Sector of the Neurology Department of the Hospital Universitário Clementino Fraga Filho of the UFRJ is presented as well as the pertinent review of the literature. The five-and-a-half-year of follow-up showed that 33 were male and 31 female; 48 were white, 10 mulatto and 6 negro; the mean time of disease was 9 years and 8 months. According to the distribution of the movement disorder, 30 (46.9%) were focal, 17 (26.6%) segmental, 13 (20.3%) generalized, 3 (4.7%) hemidystonia and 1 (1.5%) multifocal. In 11 (17.2%) the age of onset was before 12 years old, in 6 (9.4%) between 13 and 20 years old, and in 47 (73.4%) after 20 years old. According to the etiology, 39 (60.9%) were idiopathic sporadic, 6 (9.4%) were idiopathic familial and 19 (29.7%) were symptomatic. The therapeutical approach used in these patients were anticholinergic drugs, dopaminergic agonists or antagonists and baclofen, alone or associated with anticholinergic drugs for generalized dystonia. The authors conclude that botulinum toxin type A is the most valuable therapeutic agent in the treatment of focal dystonia.

dystonia; dystonia musculorum deformans; botulinum toxin

Distonias: aspectos clínicos e terapêuticos em 64 pacientes

Dystonias: clinical and therapeutic features in 64 patients

James Pitágoras de Mattos^I; Ana Lúcia Zuma de Rosso^II; Sérgio Novis^III

^IServiço de Neurologia do Hospital Universitário Clementino Fraga Filho da Universidade Federal do Rio de Janeiro (UFRJ): Professor Adjunto e Chefe do Setor de Doenças Extrapiramidais

^IIServiço de Neurologia do Hospital Universitário Clementino Fraga Filho da Universidade Federal do Rio de Janeiro (UFRJ): Médica

^IIIServiço de Neurologia do Hospital Universitário Clementino Fraga Filho da Universidade Federal do Rio de Janeiro (UFRJ): Professor Titular e Chefe de Serviço

RESUMO

Os Autores apresentam a experiência em 64 pacientes, com as várias formas clínicas de apresentação de distonias, acompanhados no Setor de Doenças Extrapiramidais do Serviço de Neurologia do Hospital Universitário Clementino Fraga Filho da UFRJ, assim como, revisam a literatura, cotejando os resultados. O acompanhamento desses pacientes durante 5 anos e 6 meses resultou nas seguintes observações: 33 do sexo masculino e 31 do feminino; 48 brancos, 10 pardos e 6 negros; a média do tempo de doença foi 9 anos e 8 meses. Quanto à distribuição do movimento anormal, 30 (46,9%) eram focais; 17 (26,6%), segmentares; 13 (20,3%), generalizadas; 3 (4,7%), hemidistonias; 1 (1,5%), multifocal. Quanto à idade de início, em 11 (17,2%) se apresentou antes dos 12 anos; em 6 (9,4%), entre 13 e 20 anos; e em 47 (73,4%), após os 20 anos. Correspondiam à origem idiopática esporádica, 39 (60,9%); idiopática familiar, 6 (9,4%); sintomática, 19 (29,7%). No que se refere à abordagem terapêutica destes pacientes, destacamos o emprego de anticolinérgicos, de agonistas e antagonistas dopaminérgicos e do baclofen isolado ou associado aos anticolinérgicos para as formas generalizadas. Para as distonias focais, os Autores concluem ser a toxina botulínica do tipo A o agente terapêutico mais eficaz aconselhado atualmente.

Palavras-chave: distonia, distonia musculorum deformans, toxina botulínica

ABSTRACT

The experience with 64 patients with dystonia seen at the Extrapyramidal Diseases Sector of the Neurology Department of the Hospital Universitário Clementino Fraga Filho of the UFRJ is presented as well as the pertinent review of the literature. The five-and-a-half-year of follow-up showed that 33 were male and 31 female; 48 were white, 10 mulatto and 6 negro; the mean time of disease was 9 years and 8 months. According to the distribution of the movement disorder, 30 (46.9%) were focal, 17 (26.6%) segmental, 13 (20.3%) generalized, 3 (4.7%) hemidystonia and 1 (1.5%) multifocal. In 11 (17.2%) the age of onset was before 12 years old, in 6 (9.4%) between 13 and 20 years old, and in 47 (73.4%) after 20 years old. According to the etiology, 39 (60.9%) were idiopathic sporadic, 6 (9.4%) were idiopathic familial and 19 (29.7%) were symptomatic. The therapeutical approach used in these patients were anticholinergic drugs, dopaminergic agonists or antagonists and baclofen, alone or associated with anticholinergic drugs for generalized dystonia. The authors conclude that botulinum toxin type A is the most valuable therapeutic agent in the treatment of focal dystonia.

Key-words: dystonia, dystonia musculorum deformans, botulinum toxin.

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Aceite: 26-outubro-1995.

Estudo realizado no Serviço de Neurologia do Hospital Universitário Clementino Fraga Filho da Universidade Federal do Rio de Janeiro (UFRJ).

Dr. James Pitágoras de Mattos - Av. São Sebastião 165/101 - 22291-070 Rio de Janeiro RJ - Brasil.

1. Adams RD, Victor M. Principles of neurology. Ed 5. New York: McGraw-Hill, 1993:56-73.
2. Andrade LAF, Ferraz HB. Idiopathic dystonia: clinical profile of 76 Brazilian patients. Arq Neuropsiquiatr 1992; 50:426-432.
3. Brin MF. Botox Bulletin. International Conference on botulinum toxin: basic science and clinical therapeutics Munich, Germany, 1995, Vol 1, June 14-16.
4. Fahn S. High dosage anticholinergic therapy in dystonia. Neurology 1983; 33:1255-1261.
5. Fahn S. Clinical variants of idiopathic torsion dystonia. J Neurol Neurosurg Psychiatry, 1989; Special Suppl:96-100.
6. Fahn S. Psychogenic movements disorders. In Marsden CD, Fahn S (eds). Movement disorders 3. London Butterworths, 1994: 358-372.
7. Fahn S, Marsden CD, Calne DB. Classification and investigation of dystonia. In Marsden CD, Fahn S (eds). Movement disorders 2. London: Butterworths, 1987:332-358.
8. Fahn S, Williams DT. Psychogenic dystonia. Adv Neurol 1988; 50:431-455.
9. Ferraz HB, Andrade LAF. Symptomatic dystonia: clinical profile on 46 Brazilian patients. Can J Neurol Sci 1992; 19:504-507.
10. Greene P, Fahn S. Patterns of spread of dystonia in childhood onset dystonia. Neurology 1987; 37(Supp 1):269.
11. Greene P, Fahn S. Use of botulinum toxin type F injections to treat torticollis in patients with immunity tc botulinum toxin type A. Mov Disord 1993; 8:479-483.
12. Greene P, Fahn S, Brin MF, Blitzer A. Botulinum toxin therapy. In Marsden CD, Fahn S (eds). Movement Disorders 3. London: Butterworths, 1994: 477-502.
13. Greene P, Fahn S, Diamond B. Development of resistance to botulinum toxin type A in patients with torticollis. Mov Disord 1994; 9:213-217.
14. Jankovic J, Brin MF. Therapeutic uses of botulinum toxin. N Engl .J Med 1991; 324:1186-1194.
15. Lees AJ. Botulinum toxin: useful in adult onset focal dystonias. BMJ 1992; 305:1169-1170
16. Lorentz IT, Subramaniam SS, Yiannikas C. Treatment of idiopathic spasmodic torticollis with botulinun toxin A: a double-blind study on twenty-three patients. Mov Disord 1991; 6:145-150.
17. Marsden CD, Harrison MJG, Bundey S. Natural history of idiopathic torsion dystonia Adv Neurol 1976 14:177-186.
18. Novis S, Mattos JP, Rosso ALZ. Toxina botulínica no blefaroespasmo, no espasmo hemifacial e na distoni; cervical: resultados em 33 pacientes. Arq Neuropsiquiatr 1995; 53:403-410.
19. Oppenheim H. Uber eine eigenartige Krampfkrankheit des Kindlichen und Jungendlichen Alters (Dysbasia lordotica progressiva. Dystonia musculorum deformans). Neurol Centralbl 1911b; 30:1090-1107.
20. Savino PJ, Maus M. Botulinum toxin therapy. Neurol Clin 1991; 9:205-224.
21. Schneider S, Heinen F, Feitel E, Köster B, Maximov M, Deuschel G. Longterm results of botulinum toxir treatment. Mov Disord 1994; 9:47.
22. Schwalbe W. Eine eigentumliche tonische Krampfform mit hysterischen Symptomen. Thesis (Inaug.Diss) Berlin: G. Schade, 1908.
23. Scott AB. Botulinum toxin injection into extraocular muscles as an alternative to strabismus surgery.Ophthalmology 1980; 87:1044-1049.

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. Adams RD, Victor M. Principles of neurology. Ed 5. New York: McGraw-Hill, 1993:56-73.

[2] 2. Andrade LAF, Ferraz HB. Idiopathic dystonia: clinical profile of 76 Brazilian patients. Arq Neuropsiquiatr 1992; 50:426-432.

[3] 3. Brin MF. Botox Bulletin. International Conference on botulinum toxin: basic science and clinical therapeutics Munich, Germany, 1995, Vol 1, June 14-16.

[4] 4. Fahn S. High dosage anticholinergic therapy in dystonia. Neurology 1983; 33:1255-1261.

[5] 5. Fahn S. Clinical variants of idiopathic torsion dystonia. J Neurol Neurosurg Psychiatry, 1989; Special Suppl:96-100.

[6] 6. Fahn S. Psychogenic movements disorders. In Marsden CD, Fahn S (eds). Movement disorders 3. London Butterworths, 1994: 358-372.

[7] 7. Fahn S, Marsden CD, Calne DB. Classification and investigation of dystonia. In Marsden CD, Fahn S (eds). Movement disorders 2. London: Butterworths, 1987:332-358.

[8] 8. Fahn S, Williams DT. Psychogenic dystonia. Adv Neurol 1988; 50:431-455.

[9] 9. Ferraz HB, Andrade LAF. Symptomatic dystonia: clinical profile on 46 Brazilian patients. Can J Neurol Sci 1992; 19:504-507.

[10] 10. Greene P, Fahn S. Patterns of spread of dystonia in childhood onset dystonia. Neurology 1987; 37(Supp 1):269.

[11] 11. Greene P, Fahn S. Use of botulinum toxin type F injections to treat torticollis in patients with immunity tc botulinum toxin type A. Mov Disord 1993; 8:479-483.

[12] 12. Greene P, Fahn S, Brin MF, Blitzer A. Botulinum toxin therapy. In Marsden CD, Fahn S (eds). Movement Disorders 3. London: Butterworths, 1994: 477-502.

[13] 13. Greene P, Fahn S, Diamond B. Development of resistance to botulinum toxin type A in patients with torticollis. Mov Disord 1994; 9:213-217.

[14] 14. Jankovic J, Brin MF. Therapeutic uses of botulinum toxin. N Engl .J Med 1991; 324:1186-1194.

[15] 15. Lees AJ. Botulinum toxin: useful in adult onset focal dystonias. BMJ 1992; 305:1169-1170

[16] 16. Lorentz IT, Subramaniam SS, Yiannikas C. Treatment of idiopathic spasmodic torticollis with botulinun toxin A: a double-blind study on twenty-three patients. Mov Disord 1991; 6:145-150.

[17] 17. Marsden CD, Harrison MJG, Bundey S. Natural history of idiopathic torsion dystonia Adv Neurol 1976 14:177-186.

[18] 18. Novis S, Mattos JP, Rosso ALZ. Toxina botulínica no blefaroespasmo, no espasmo hemifacial e na distoni; cervical: resultados em 33 pacientes. Arq Neuropsiquiatr 1995; 53:403-410.

[19] 19. Oppenheim H. Uber eine eigenartige Krampfkrankheit des Kindlichen und Jungendlichen Alters (Dysbasia lordotica progressiva. Dystonia musculorum deformans). Neurol Centralbl 1911b; 30:1090-1107.

[20] 20. Savino PJ, Maus M. Botulinum toxin therapy. Neurol Clin 1991; 9:205-224.

[21] 21. Schneider S, Heinen F, Feitel E, Köster B, Maximov M, Deuschel G. Longterm results of botulinum toxir treatment. Mov Disord 1994; 9:47.

[22] 22. Schwalbe W. Eine eigentumliche tonische Krampfform mit hysterischen Symptomen. Thesis (Inaug.Diss) Berlin: G. Schade, 1908.

[23] 23. Scott AB. Botulinum toxin injection into extraocular muscles as an alternative to strabismus surgery.Ophthalmology 1980; 87:1044-1049.