Spinal muscular atrophy type II (intermediary) and III (Kugelberg-Welander): evolution of 50 patients with physiotherapy and hydrotherapy in a swimming pool

Cunha, Márcia C. B.; Oliveira, Acary S. B.; Labronici, Rita Helena D. D.; Gabbai, Alberto Alain

doi:10.1590/S0004-282X1996000300007

Abstracts

We added hydrotherapy to 50 patients with spinal muscular atrophy (SMA) who were being treated with individual conventional physiotherapy. Hydrotherapy was performed at an approximate temperature of 30 degrees Celsius, twice a week, for thirty minutes in children and for forty-five minutes in adults during a 2-year period. The outcome derived from this combined modality of treatment was rated according to physiotherapeutic evaluations, the MMT (Manual Muscular Test), and the Barthel Ladder. Patients were reevaluated at 2-month intervals. After two years of ongoing treatment, we were able to observe that the deformities in hip, knee and foot were progressive in all SMA Type II patients, and in some Type III. Muscle strength stabilized in most SMA Type III patients, and improved in some. MMT was not done in SMA Type II. In all patients we were able to detect an improvement in the Barthel Ladder scale. This study suggests that a measurable improvement in the quality of daily living may be obtained in patients with SMA Types II and III subjected to conventional physiotherapy when associated with hydrotherapy.

physiotherapy; hydrotherapy; spinal muscular atrophy

A hidroterapia foi realizada em SO pacientes com atrofia muscular espinhal, os quais foram também tratados com fisioterapia individual convencional. O tratamento hidroterápico foi realizado em piscina aquecida numa temperatura de aproximadamente 30° Celsius, duas vezes por semana, durante 30 minutos em crianças e 45 minutos em adultos num período de dois anos. Os benefícios deste tipo de tratamento foram avaliados de acordo com a evolução clínica, o MMT(Teste de Força Muscular) e a Escala de Barthel. Os pacientes foram reavaliados a cada dois meses. Após dois anos de tratamento nós observamos que as deformidades nos quadris, joelhos e pés foram progressivas em todos os pacientes do Tipo II e em alguns do Tipo III. Houve estabilização da força muscular na maioria dos pacientes com SMA Tipo III, e melhora da força em alguns; nos pacientes com SMA Tipo II este teste não pode ser realizado. Em todos os pacientes pudemos verificar melhora em realção à Escala de Barthel. Este estudo sugere que houve melhora destes pacientes com SMA Tipos II e III em relação as atividades de vida diária quando a fisioterapia convencional foi associada a hidroterapia.

fisioterapia; hidroterapia; atrofia muscular espinhal

Spinal muscular atrophy type II (intermediary) and III (Kugelberg-Welander): evolution of 50 patients with physiotherapy and hydrotherapy in a swimming pool

Atrofia muscular espinhal tipo II (intermediária) e III (Kugelberg-Welander): evolução de 50 pacientes com fisioterapia e hidroterapia em piscina

Márcia C. B. Cunha^I; Acary S. B. Oliveira^II; Rita Helena D. D. Labronici^III; Alberto Alain Gabbai^IV

^IFisioterapeuta, Mestre em Neurociências Universidade Federal de São Paulo - Escola Paulista de Medicina (UNIFESP-EPM)

^IIDoutor em Neurologia,UNIFESP-EPM

^IIIMestranda em Neurociências UNIFESP-EPM

^IVLivre Docente em Neurologia UNIFESP-EPM

ABSTRACT

We added hydrotherapy to 50 patients with spinal muscular atrophy (SMA) who were being treated with individual conventional physiotherapy. Hydrotherapy was performed at an approximate temperature of 30 degrees Celsius, twice a week, for thirty minutes in children and for forty-five minutes in adults during a 2-year period. The outcome derived from this combined modality of treatment was rated according to physiotherapeutic evaluations, the MMT (Manual Muscular Test), and the Barthel Ladder. Patients were reevaluated at 2-month intervals. After two years of ongoing treatment, we were able to observe that the deformities in hip, knee and foot were progressive in all SMA Type II patients, and in some Type III. Muscle strength stabilized in most SMA Type III patients, and improved in some. MMT was not done in SMA Type II. In all patients we were able to detect an improvement in the Barthel Ladder scale. This study suggests that a measurable improvement in the quality of daily living may be obtained in patients with SMA Types II and III subjected to conventional physiotherapy when associated with hydrotherapy.

Key words: physiotherapy, hydrotherapy, spinal muscular atrophy.

RESUMO

A hidroterapia foi realizada em SO pacientes com atrofia muscular espinhal, os quais foram também tratados com fisioterapia individual convencional. O tratamento hidroterápico foi realizado em piscina aquecida numa temperatura de aproximadamente 30° Celsius, duas vezes por semana, durante 30 minutos em crianças e 45 minutos em adultos num período de dois anos. Os benefícios deste tipo de tratamento foram avaliados de acordo com a evolução clínica, o MMT(Teste de Força Muscular) e a Escala de Barthel. Os pacientes foram reavaliados a cada dois meses. Após dois anos de tratamento nós observamos que as deformidades nos quadris, joelhos e pés foram progressivas em todos os pacientes do Tipo II e em alguns do Tipo III. Houve estabilização da força muscular na maioria dos pacientes com SMA Tipo III, e melhora da força em alguns; nos pacientes com SMA Tipo II este teste não pode ser realizado. Em todos os pacientes pudemos verificar melhora em realção à Escala de Barthel. Este estudo sugere que houve melhora destes pacientes com SMA Tipos II e III em relação as atividades de vida diária quando a fisioterapia convencional foi associada a hidroterapia.

Palavras-chaves: fisioterapia, hidroterapia, atrofia muscular espinhal.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Aceite: 6-março-1996.

Fisiot. Márcia Cristina Bauer Cunha - Disciplina de Neurologia, Escola Paulista de Medicina, UNIFESP-Rua Botucatu 762 - 04023-900 São Paulo SP - Brasil.

1. Benady SG. Spinal muscular atrophy in childhood: review of SO cases. Develop Med Chlid Neurol 1978;20:746-757.
2. Bob, Anderson. Alongue-se. São Paulo: Summus, 1994.
3. Brzustowick LM, Lehner T, Castila LH et al. Genetic mapping of chronic childhood-onset spinal muscular atrophy to chromosome Sq 11.2-13.3. Nature 1990,344:540-541.
4. Bussi L, Mangosto V. La disability motoria progressiva nelle atrofie muscolari spinuli. Min Med 1986;77:155-169.
5. Byres RK, Banker BQ. Infantile muscular atrophy. Arch Neurol 1961 ;5:140-164.
6. Dubowitz V. Recent advances in neuromuscular disorders in chidhood. Acta Paediutr Jpn 1991,33:198-205.
7. Engel AG, Banker BQ. Myology. New York: Mcgraw-Hill, 1994.
8. Fanburg BL, Sicilian L. Clinics in chest medicine: respiratory dysfinction in neuromuscular disease. London: W.B.Saunders, 1994.
9. Gloria D, Binder H, Koch B. Spinal muscular atrophy: experience in diagnosis and rehabilitation management of 60 patients. Arch Phys Med Rehabil 1984;65:549-553.
10. Goland A. Basic hydrotherapy. Physiotherapy 1981,67:33-54.
11. Hoffmann J. Ueber cronische spinale Muskelatrophie im Kindesalter auf familiare Basis. Dtsch Z Nervenheilkd 1983;3:427-430.
12. Hurley R, Turner C. Neurology and aquatic theraphy clinical management. Physiotherapy 1991; 11:26-29.
13. Klauck J. Swimming in school. Ed3. Schorndorf: Hoffmann, 1982.
14. Kleyn PW, Gilliam TC. Progress toward cloning of the gene responsible for childhood spinal muscular atrophy. Semin Neurol 1993;13:276-282.
15. Lovelace RE, Schotlund DL, DeNapoli RA. The Kugelberg-Welander syndrome. Am Neurol Assoc 1966,2:126-132.
16. Macmillun J. The water in rehabilitation. Physiotheraphy 1978;45:35-38.
17. McGinnis GE, Seward ML, Dejong G. Osberg S. Program evaluation of physical medicine and rehabilitation departments using self-report "Barthel". Arch Phys Med Rehabil 1986;67:123-125.
18. Melki J, Abdelhak S, Sheth P et al. Gene for chronic proximal spinal muscular atrophies maps to chromossome 5q. Nature 1990;344:767-768.
19. Pearn J. Classification of spinal muscular atrophies. Lancet 1980;1:919-923.
20. PrioraU, Quaglia P, Vialda M, Gianchino-Amista MT, Domenegheti G, Sardi R. Atrofie muscolare spinale infantile. Min Ped 1987;39:709-714.
21. Riddick MF, Winter RB, Lutter LD. Spinal deformities in patients with spinal muscle atrophy: a review of 36 patients. J Bone Joint Surg 1981;57A: 179-186.
22. Russman BS, Melchreit R, Drennan JC. Spinal muscular atrophy: the natural course of disease. Muscle & Nerve 1983;6:179-181.
23. Schmidt HM. Halliwick Method by MacMillan. Motorik 1981 ;4:103-111.
24. Schwentker ET, Gibson DA. Orthopaedic aspects of spinal muscular atrophy. J Bone Joint Surg 1976,58A:32-36.
25. Shapiro F, Specht AL. The diagnosis and orthopedic treatment of inherited muscular diseases of childhood. Bone Joint Surg 1993;75:439-454.
26. Skiner R, Thomson AM. Duffields: exercises in water. Ed3. São Paulo: Manole, 1985.
27. Sullivan MS, Cullen KE, Schmitz TJ. Fisioterapia: tratamento, procedimentos e avaliação. São Paulo: Manole, 1983.
28. Werdnig G. Die fruhinfantile progressive spinale Amyotrofie. Arch Psychiatr 1894;26:706-710.
29. Zulneraitis EL, Halperín JJ, Grunnet ML, Russman BS, Peress N. Muscle biopsy and the clinical course of infantile spinal muscular atrophy. J Child Neurol 1991,6:324-328.

Publication Dates

Publication in this collection
10 Nov 2010
Date of issue
Sept 1996

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

[1] 1. Benady SG. Spinal muscular atrophy in childhood: review of SO cases. Develop Med Chlid Neurol 1978;20:746-757.

[2] 2. Bob, Anderson. Alongue-se. São Paulo: Summus, 1994.

[3] 3. Brzustowick LM, Lehner T, Castila LH et al. Genetic mapping of chronic childhood-onset spinal muscular atrophy to chromosome Sq 11.2-13.3. Nature 1990,344:540-541.

[4] 4. Bussi L, Mangosto V. La disability motoria progressiva nelle atrofie muscolari spinuli. Min Med 1986;77:155-169.

[5] 5. Byres RK, Banker BQ. Infantile muscular atrophy. Arch Neurol 1961 ;5:140-164.

[6] 6. Dubowitz V. Recent advances in neuromuscular disorders in chidhood. Acta Paediutr Jpn 1991,33:198-205.

[7] 7. Engel AG, Banker BQ. Myology. New York: Mcgraw-Hill, 1994.

[8] 8. Fanburg BL, Sicilian L. Clinics in chest medicine: respiratory dysfinction in neuromuscular disease. London: W.B.Saunders, 1994.

[9] 9. Gloria D, Binder H, Koch B. Spinal muscular atrophy: experience in diagnosis and rehabilitation management of 60 patients. Arch Phys Med Rehabil 1984;65:549-553.

[10] 10. Goland A. Basic hydrotherapy. Physiotherapy 1981,67:33-54.

[11] 11. Hoffmann J. Ueber cronische spinale Muskelatrophie im Kindesalter auf familiare Basis. Dtsch Z Nervenheilkd 1983;3:427-430.

[12] 12. Hurley R, Turner C. Neurology and aquatic theraphy clinical management. Physiotherapy 1991; 11:26-29.

[13] 13. Klauck J. Swimming in school. Ed3. Schorndorf: Hoffmann, 1982.

[14] 14. Kleyn PW, Gilliam TC. Progress toward cloning of the gene responsible for childhood spinal muscular atrophy. Semin Neurol 1993;13:276-282.

[15] 15. Lovelace RE, Schotlund DL, DeNapoli RA. The Kugelberg-Welander syndrome. Am Neurol Assoc 1966,2:126-132.

[16] 16. Macmillun J. The water in rehabilitation. Physiotheraphy 1978;45:35-38.

[17] 17. McGinnis GE, Seward ML, Dejong G. Osberg S. Program evaluation of physical medicine and rehabilitation departments using self-report "Barthel". Arch Phys Med Rehabil 1986;67:123-125.

[18] 18. Melki J, Abdelhak S, Sheth P et al. Gene for chronic proximal spinal muscular atrophies maps to chromossome 5q. Nature 1990;344:767-768.

[19] 19. Pearn J. Classification of spinal muscular atrophies. Lancet 1980;1:919-923.

[20] 20. PrioraU, Quaglia P, Vialda M, Gianchino-Amista MT, Domenegheti G, Sardi R. Atrofie muscolare spinale infantile. Min Ped 1987;39:709-714.

[21] 21. Riddick MF, Winter RB, Lutter LD. Spinal deformities in patients with spinal muscle atrophy: a review of 36 patients. J Bone Joint Surg 1981;57A: 179-186.

[22] 22. Russman BS, Melchreit R, Drennan JC. Spinal muscular atrophy: the natural course of disease. Muscle & Nerve 1983;6:179-181.

[23] 23. Schmidt HM. Halliwick Method by MacMillan. Motorik 1981 ;4:103-111.

[24] 24. Schwentker ET, Gibson DA. Orthopaedic aspects of spinal muscular atrophy. J Bone Joint Surg 1976,58A:32-36.

[25] 25. Shapiro F, Specht AL. The diagnosis and orthopedic treatment of inherited muscular diseases of childhood. Bone Joint Surg 1993;75:439-454.

[26] 26. Skiner R, Thomson AM. Duffields: exercises in water. Ed3. São Paulo: Manole, 1985.

[27] 27. Sullivan MS, Cullen KE, Schmitz TJ. Fisioterapia: tratamento, procedimentos e avaliação. São Paulo: Manole, 1983.

[28] 28. Werdnig G. Die fruhinfantile progressive spinale Amyotrofie. Arch Psychiatr 1894;26:706-710.

[29] 29. Zulneraitis EL, Halperín JJ, Grunnet ML, Russman BS, Peress N. Muscle biopsy and the clinical course of infantile spinal muscular atrophy. J Child Neurol 1991,6:324-328.

Brasil

Brasil

Spinal muscular atrophy type II (intermediary) and III (Kugelberg-Welander): evolution of 50 patients with physiotherapy and hydrotherapy in a swimming pool

Atrofia muscular espinhal tipo II (intermediária) e III (Kugelberg-Welander): evolução de 50 pacientes com fisioterapia e hidroterapia em piscina

Abstracts

Publication Dates