Sindrome de irritabilidade muscular aumentada: tratamento com nifedipina. Registro de um caso

Mion, Carmen Chaib; Tsanaclis, Ana Maria; Lusvarghi, Edgard; Brotto, Mario Wilson; Levy, José Antonio

doi:10.1590/S0004-282X1984000100014

Resumos

Em 1980 Alberca e col. descreveram caso de paciente que apresentava irritabilidade muscular aumentada, com contrações ondulantes do músculo, mioedema e respostas musculares à estimulação mecânica semelhantes à miotonia, sugerindo um disfunção ao nível de miofibrilas. Os autores descrevem caso semelhante de paciente com contraturas intensas em membros inferiores, silenciosas eletricamente, principalmente ao iniciar a marcha ou qualquer movimento voluntário, contração idiomuscular intensa com período de relaxamento aumentado, semelhante à reação miotônica, mioedema prolongado e movimentos musculares ondulantes em tronco e membros superiores. A EMG assim como estudos histoquímicos e de microscopia eletrônica dos músculos foram normais. Apesar da fisiopatogênese do aumento da irritabilidade muscular não estar elucidada, assim como a ação de nifedipina, bloqueador de Ca++, no músculo esquelético ser desconhecida, a administração desta droga na dose de 40 mg/dia v.o. determinou melhora acentuada da sintomatologia. Com a introdução de placebo, houve reaparecimento dos sintomas. Os autores inferem que provavelmente, a irritabilidade muscular aumentada deve estar relacionada ao metabolismo celular de Ca++.

In 1980 Alberca et al. described a patient with a syndrome of increased muscle irritability, who presented ondulating muscle rolling moviments and electrically silent cramps, myoedema and muscle reactions to mechanical stimulation similar to myotonic response, suggesting a disfunction at myofibrilar level. We saw a similar case, of a male patient, 21 years of age, who complained of cramps of severe intensity for the past four years. These cramps were painful in the upper and lower limbs and impaired his locomotion; they were electrically silent. At percussion the patient showed severe idiomuscular contraction, with a period of increased relaxation, similar to a myotonic reaction and also, prolonged myoedema and rolling muscle contractions. Electromyography was normal, as were histochemical and electron microscopy studies. We carried out a therapeutic trial with niphedipine (a calcium antagonist), on the assumption that the patient showed a disturbance of the myofibrilar function - even though physiopathogenesis of the hyperirritability muscle syndrome was not yet clearly defined - and with a basis on the importance of the intracytoplasmatic level of Ca++ free in the muscle contraction mechanism, not only as the initiating factor of the contactile process, but also as a quantitative controller of the mechanic tension development through regulation of the amount of ATP metabolized during muscle activity. Administration of the drug in a dose of 40 mg daily, per os, brought a remission of the symptons after two weeks, and the patient could walk normally again. On the introduction of a placebo, on two different opportunities, there occurred a recrudescence of the symptons after about one week's time. It is important to note that the patient did not show improvement in the symptomatology on utilization of benzodyazepinics or diphenil-hydantoine.

Sindrome de irritabilidade muscular aumentada: tratamento com nifedipina. Registro de um caso

Increased muscle irritability syndrome: treatment by niphedipine. Report of one case

Carmen Chaib Mion^I; Ana Maria Tsanaclis^I; Edgard Lusvarghi^I; Mario Wilson Brotto^I; José Antonio Levy^II

^IMédico-assistente. Centro de Investigações em Neurologia da Clínica Neurológica da Faculdade de Medicina da Universidade de São Paulo

^IIProfessor-adjunto. Centro de Investigações em Neurologia da Clínica Neurológica da Faculdade de Medicina da Universidade de São Paulo

RESUMO

Em 1980 Alberca e col. descreveram caso de paciente que apresentava irritabilidade muscular aumentada, com contrações ondulantes do músculo, mioedema e respostas musculares à estimulação mecânica semelhantes à miotonia, sugerindo um disfunção ao nível de miofibrilas. Os autores descrevem caso semelhante de paciente com contraturas intensas em membros inferiores, silenciosas eletricamente, principalmente ao iniciar a marcha ou qualquer movimento voluntário, contração idiomuscular intensa com período de relaxamento aumentado, semelhante à reação miotônica, mioedema prolongado e movimentos musculares ondulantes em tronco e membros superiores. A EMG assim como estudos histoquímicos e de microscopia eletrônica dos músculos foram normais. Apesar da fisiopatogênese do aumento da irritabilidade muscular não estar elucidada, assim como a ação de nifedipina, bloqueador de Ca⁺⁺, no músculo esquelético ser desconhecida, a administração desta droga na dose de 40 mg/dia v.o. determinou melhora acentuada da sintomatologia. Com a introdução de placebo, houve reaparecimento dos sintomas. Os autores inferem que provavelmente, a irritabilidade muscular aumentada deve estar relacionada ao metabolismo celular de Ca⁺⁺.

SUMMARY

In 1980 Alberca et al. described a patient with a syndrome of increased muscle irritability, who presented ondulating muscle rolling moviments and electrically silent cramps, myoedema and muscle reactions to mechanical stimulation similar to myotonic response, suggesting a disfunction at myofibrilar level. We saw a similar case, of a male patient, 21 years of age, who complained of cramps of severe intensity for the past four years. These cramps were painful in the upper and lower limbs and impaired his locomotion; they were electrically silent. At percussion the patient showed severe idiomuscular contraction, with a period of increased relaxation, similar to a myotonic reaction and also, prolonged myoedema and rolling muscle contractions. Electromyography was normal, as were histochemical and electron microscopy studies. We carried out a therapeutic trial with niphedipine (a calcium antagonist), on the assumption that the patient showed a disturbance of the myofibrilar function - even though physiopathogenesis of the hyperirritability muscle syndrome was not yet clearly defined - and with a basis on the importance of the intracytoplasmatic level of Ca⁺⁺ free in the muscle contraction mechanism, not only as the initiating factor of the contactile process, but also as a quantitative controller of the mechanic tension development through regulation of the amount of ATP metabolized during muscle activity. Administration of the drug in a dose of 40 mg daily, per os, brought a remission of the symptons after two weeks, and the patient could walk normally again. On the introduction of a placebo, on two different opportunities, there occurred a recrudescence of the symptons after about one week's time. It is important to note that the patient did not show improvement in the symptomatology on utilization of benzodyazepinics or diphenil-hydantoine.

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1. ALBERGA, R.; RAFEL, E.; CASTILDA, J.M. & GIL-PERALTA, A. - Increased mechanical muscle irritability syndrome. Acta neurol. scand. 62:250, 1980.
2. BRAUNWALD, E. - Mechanism of action of calcium chanel blocking agents. New Engl. J. Med. 307:1618, 1982.
3. BRODY, I.A. - Muscle contracture induced by exercise. A syndrome attributable to decreased relaxing factor. New. Engl. J. Med. 281:187, 1969.
4. DUBOWITZ, V. & BROOKE, M.H. - Muscle biopsy: a modern approach. Saunders. London, 1973, pg. 74.
5. DUSTIN, H., ed. - Hypertension. American Heart Association, vol. 5 (suppl. 2), 1983.
6. FLECKENSTEIN, A. - Specific pharmacology of calcium in myocardium cardiac pacemakers and vascular smooth muscle. Ann. Rev. Pharmacol. Toxicol. 17:149, 1977.
7. GRUENER, R.; McARDLE, B. & RYMAN, B. - Contracture of phosphorilase deficient muscle. J. Neurol. Neurosur. Psychiat. 31:268, 1968.
8. LAYZER, R.B. & ROWLAND, L.P. - Cramps. New Engl. J. Med. 285:31, 1971.
9. MUNSAT, T.L. - A standardized forearm ischemic exercise test. Neurology (Minneapolis) 27:1171, 1970.
10. NAUSS, K.M. & DAVIES, R.E. - Changes in phosphate compounds during the development and maintenance of rigor mortis. J. biol. Chem. 24:2818, 1966.
11. SAUNDERS, D.B. - Myotonia congenita with painful muscle contractions. Arch. Neurol. (Chicago) 33:580, 1976.
12. SCHMITT, J. - Cramps musculaires. Ehcyclop. Médico-Chirurgicale: Neurologie 17186 Aw, 5:01. Paris, 1979.
13. TORBERGSEN, T. - A family with dominant hereditary myotonia, muscular hypertrophy and increased muscular irritability. Acta neurol. scand. 51:225, 1975.
14. WILSON, J. & WALTON, J.N. - Some muscular manifestations of hypothyroidism. J. Neurol. Neurosurg. Psychiat. 22:320, 1959.

Datas de Publicação

Publicação nesta coleção
14 Ago 2012
Data do Fascículo
Mar 1984

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

[1] 1. ALBERGA, R.; RAFEL, E.; CASTILDA, J.M. & GIL-PERALTA, A. - Increased mechanical muscle irritability syndrome. Acta neurol. scand. 62:250, 1980.

[2] 2. BRAUNWALD, E. - Mechanism of action of calcium chanel blocking agents. New Engl. J. Med. 307:1618, 1982.

[3] 3. BRODY, I.A. - Muscle contracture induced by exercise. A syndrome attributable to decreased relaxing factor. New. Engl. J. Med. 281:187, 1969.

[4] 4. DUBOWITZ, V. & BROOKE, M.H. - Muscle biopsy: a modern approach. Saunders. London, 1973, pg. 74.

[5] 5. DUSTIN, H., ed. - Hypertension. American Heart Association, vol. 5 (suppl. 2), 1983.

[6] 6. FLECKENSTEIN, A. - Specific pharmacology of calcium in myocardium cardiac pacemakers and vascular smooth muscle. Ann. Rev. Pharmacol. Toxicol. 17:149, 1977.

[7] 7. GRUENER, R.; McARDLE, B. & RYMAN, B. - Contracture of phosphorilase deficient muscle. J. Neurol. Neurosur. Psychiat. 31:268, 1968.

[8] 8. LAYZER, R.B. & ROWLAND, L.P. - Cramps. New Engl. J. Med. 285:31, 1971.

[9] 9. MUNSAT, T.L. - A standardized forearm ischemic exercise test. Neurology (Minneapolis) 27:1171, 1970.

[10] 10. NAUSS, K.M. & DAVIES, R.E. - Changes in phosphate compounds during the development and maintenance of rigor mortis. J. biol. Chem. 24:2818, 1966.

[11] 11. SAUNDERS, D.B. - Myotonia congenita with painful muscle contractions. Arch. Neurol. (Chicago) 33:580, 1976.

[12] 12. SCHMITT, J. - Cramps musculaires. Ehcyclop. Médico-Chirurgicale: Neurologie 17186 Aw, 5:01. Paris, 1979.

[13] 13. TORBERGSEN, T. - A family with dominant hereditary myotonia, muscular hypertrophy and increased muscular irritability. Acta neurol. scand. 51:225, 1975.

[14] 14. WILSON, J. & WALTON, J.N. - Some muscular manifestations of hypothyroidism. J. Neurol. Neurosurg. Psychiat. 22:320, 1959.