Sleep polygraphic parameters in neuromuscular diseases

Pradella, Márcia

doi:10.1590/S0004-282X1994000400004

Abstracts

In a polysomnography study of 32 neuromuscular patients - 22 with a form of muscular dystrophy, 3 with a form of congenital myopathy, 4 with a form of spinal muscular atrophy, 1 with a recurrent form of polymyositis and 1 with osteogenesis imperfecta syndrome - of which 21 were nonambulatory, we observed sleep related respiratory disturbances represented by: drops in oxygen saturation (SaO2), cardiac arrythmia, sleep disruption, apneas, tachypnea, tachycardia and snoring. Nine out of the cohort of 32 patients presented with significant desaturations periods. These patients presented with an associated restrictive syndrome and thoracic deformities, some with tachypnea and/or SaO2 below 90% during wakefulness. In this group, snoring was observed in those patients with a form of muscular dystrophy while tachypnea was observed in patients who presented the highest desaturations levels. Sleep quantification revealed an increase of stage 1 sleep coupled with a decrease or even total absence of REM sleep. This is, we believe, a likely consequence of episodic desaturations that may accompany sleep hypoventilation which is potentialised during REM sleep stage.

polysomnography; neuromuscular diseases; sleep related respiratory disturbances

Estudando 32 pacientes com doenças neuromusculares -22 com forma de distrofia muscular, 3 com forma de miopatia congênita, 4 com forma de atrofia muscular espinal, 1 com forma recorrente de polimiosite e 1 com síndrome de osteogenesis imperfecta - dos quais 21 estavam impossibilitados de deambulação, distúrbios respiratórios relacionados ao sono foram observados sob forma dessaturação da oxi-hemogloblina, arritmia cardíaca, taquipnéia, taquicardia e roncos. Nove desses 32 pacientes apresentaram períodos de dessaturação de oxi-hemoglobina maiores que 4% em relação aos níveis obtidos em vigília e repouso. Esse grupo de pacientes era caracterizado por apresentar síndrome respiratória restritiva associada a deformidade torácica (principalmente escoliose). Alguns apresentaram taquipnéia e/ou saturação da oxi-hemoglobina abaixo de 90% durante o repouso e em vigília. Neste grupo, roncos foram observados principalmente nos pacientes com forma de distrofia muscular, enquanto a taquipnéia foi observada sobretudo nos pacientes com os maiores níveis de dessaturação. A quantificação do sono mostrou aumento na porcentagem do estado 1 do sono associada a diminuição ou mesmo ausência do estado do sono paradoxal. Isso nos leva a crer em um provável mecanismo de proteção consequente à potencialização da hipoventilação observada durante o estado de sono paradoxal, em que os maiores níveis de dessaturação são observados.

polissonografia; doenças neuromusculares; distúrbios respiratórios relacionados ao sono

Sleep polygraphic parameters in neuromuscular diseases

Parâmetros poligráficos do sono em doenças neuromusculares

Márcia Pradella

Sleep Laboratory and Pediatric Neurology Unit of the St Luc Training Hospital, Brussels, Belgium

SUMMARY

In a polysomnography study of 32 neuromuscular patients - 22 with a form of muscular dystrophy, 3 with a form of congenital myopathy, 4 with a form of spinal muscular atrophy, 1 with a recurrent form of polymyositis and 1 with osteogenesis imperfecta syndrome - of which 21 were nonambulatory, we observed sleep related respiratory disturbances represented by: drops in oxygen saturation (SaO2), cardiac arrythmia, sleep disruption, apneas, tachypnea, tachycardia and snoring. Nine out of the cohort of 32 patients presented with significant desaturations periods. These patients presented with an associated restrictive syndrome and thoracic deformities, some with tachypnea and/or SaO2 below 90% during wakefulness. In this group, snoring was observed in those patients with a form of muscular dystrophy while tachypnea was observed in patients who presented the highest desaturations levels. Sleep quantification revealed an increase of stage 1 sleep coupled with a decrease or even total absence of REM sleep. This is, we believe, a likely consequence of episodic desaturations that may accompany sleep hypoventilation which is potentialised during REM sleep stage.

Key words: polysomnography, neuromuscular diseases, sleep related respiratory disturbances.

RESUMO

Estudando 32 pacientes com doenças neuromusculares -22 com forma de distrofia muscular, 3 com forma de miopatia congênita, 4 com forma de atrofia muscular espinal, 1 com forma recorrente de polimiosite e 1 com síndrome de osteogenesis imperfecta - dos quais 21 estavam impossibilitados de deambulação, distúrbios respiratórios relacionados ao sono foram observados sob forma dessaturação da oxi-hemogloblina, arritmia cardíaca, taquipnéia, taquicardia e roncos. Nove desses 32 pacientes apresentaram períodos de dessaturação de oxi-hemoglobina maiores que 4% em relação aos níveis obtidos em vigília e repouso. Esse grupo de pacientes era caracterizado por apresentar síndrome respiratória restritiva associada a deformidade torácica (principalmente escoliose). Alguns apresentaram taquipnéia e/ou saturação da oxi-hemoglobina abaixo de 90% durante o repouso e em vigília. Neste grupo, roncos foram observados principalmente nos pacientes com forma de distrofia muscular, enquanto a taquipnéia foi observada sobretudo nos pacientes com os maiores níveis de dessaturação. A quantificação do sono mostrou aumento na porcentagem do estado 1 do sono associada a diminuição ou mesmo ausência do estado do sono paradoxal. Isso nos leva a crer em um provável mecanismo de proteção consequente à potencialização da hipoventilação observada durante o estado de sono paradoxal, em que os maiores níveis de dessaturação são observados.

Palavras-chave: polissonografia, doenças neuromusculares, distúrbios respiratórios relacionados ao sono.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Acknowledgements - Our deepest appreciation goes to Prof. G Aubert, director of the Sleep laboratory, St Luc's training Hospital, Brussels, Belgium where the bulk of this research was carried out. Sincerest thanks to Prof. G Ferriére of the Pediatric Neurology Unit for academic and technical skills provided.

Aceite: 5-maio-1994.

Dra. Marcia Pradella - Rua Antonio Rodrigues Passos 227, Jd. Cd Pirituba - 02941-040 São Paulo SP - Brasil.

1. Aldrich MS. Neurologic aspects of sleep apnea and related respiratory disturbances. Otolaryngol Clin North Am 1990, 23: 761-769.
2. American Thoracic Society. Indications and standards for cardiopulmonary sleep studies. Am Rev Resp Dis 1989, 139: 559-568.
3. Back JR, Campagnolo DI, Hoeman S. Life satisfaction of individuals with Duchenne muscular dystrophy using long-term mechanical ventilatory support. Am J Phys Med Rehabil 1991, 70: 129-135.
4. Baydur A, Gilgoff I, Prentice W, Carlson M, Fischer A. Decline in respiratory function and experience with long-term assisted ventilation in advanced Duchenne's muscular dystrophy. Chest 1990, 97: 884-889.
5. Bertrand A, Milane J, Jonquet O, Bertrand P. De la poliomyélite antérieure aiguë aux myopathies: prise en charge de l'insuffisance respiratoire chronique par déficit neuromusculaire. Bull Acad Natle Méd 1989, 173: 769-776.
6. Bye PTP, Ellis ER, Donnelly PD, Issa FG, Sullivan CE. Role of sleep in the development of respiratory failure in neuromucular disease. Am Rev Respir Dis 1985, 131: A108.
7. Carrol N, Bain RJ, Smith PEM, Saltissi E, Edwards RHT, Calverley PMA. Domiciliary investigation of sleep-related hypoxaemia in Duchenne muscular dystrophy. Eur Respir J 1991, 4: 434-440.
8. De Troyer A, Borenstein S, Cordier R. Analysis of lung volume restriction in patients with respiratory muscle weakness. Thorax 1980, 35: 603-610.
9. Delguste P, Rodenstein DO. Désaturations nocturnes dans la myopathie de Duchenne de Boulogne. Entretiens de la Fondation Garches 1990, 263-264.
10. Del mas MC, Bérard C. Prise en charge globale de 1'amyotrophic spinale infantile: guide practique pour les équipes médicales. Pédiatrie 1990, 457-464.
11. Douglas NJ. Control of breathing during sleep. Clin Sci 1984, 67: 465-472.
12. Duron B. Respiration et sommeil. Association des Physiologistes 1987, A57-A71.
13. Estournet B, Bataille J, Commare MC, Urtizberea JA, Barois A. La ventilation nasale chez l'enfant atteint de maladie neuro-musculaire. Entretiens de la Fondation Garches 1990, 273.
14. Gastaut H, Duron B, Papy JJ, Tassinari CA, Waltregny J. Etude polygraphique comparative du cycle nycthémérique chez les narcoléptiques, les pickwickiens, les obèses et les insuffisants respiratoires. Rev Neurol 1966, 115:456-462.
15. Guilleminault C. The role of sleep and sleep states on breathing disorders. Schweiz Med Wschr 1988, 118: 1331-1332.
16. Heckmatt JZ. Respiratory care in muscular dystrophy. Br Med J 1987, 295: 1014-1015.
17. Heckmatt JZ, Loh L, Dubowits V. Nocturnal hypoventilation in children with nonprogressive neuromuscular disease. Pediatrics 1989, 83: 250-255.
18. Krieger J, Turlot JC, Mangin P,Kurtz D. Breathing during sleep in normal young and elderly subjects : hypopneas, apneas and correlated factors. Sleep 1983, 6: 108-120.
19. Kryger MH, Steljes DG, Yee WC, Mate E, Smith SA, Mahowald M. Central sleep apnoea in congenital muscular dystrophy. J Neurol Neurosurg Psychiatry 1991, 54: 710-712.
20. Le Balle F, Quera-Salva MA, McCann C, Barbe F, Lattre J, Gajdos P, Troche G. Apnées du sommeil chez les patients atteints de dystrophie musculaire de Duchenne de Boulogne. 1990, 265-266.
21. Lugaresi E, Cirignotta F, Montagna P. Snoring: pathogenic, clinical and therapeutic aspects. In Kryger M, Roth T, Dement WC (eds). Principles and practice of sleep medicine. Philadelphia, W B Saunders Company 1989, 494-500.
22. Manni R, Zucca C, Galimgerti CA, Ottolini A, Cerveri I, Bruschi C, Tartara A. Nocturnal sleep and oxygen balance in Duchenne muscular dystrophy. Eur Arch Psychiatry Clin Neurosci 1991, 240: 255-257.
23. Miller RG, Chalmers AC, Dao H, Filler-Katz A, Holman D, Bost F. The effect of spine fusion on respiratory function in Duchenne muscular dystrophy. Neurology 1991, 41: 38-40.
24. Newson-Davis JM, Goldman M, Loh LC. Diaphragm function and alveolar hypoventilation. Q J Med 1976, 177: 87-100.
25. Pradella M. A study of oxygen saturation and other polygraphic parameters in neuromuscular diseases with particular reference to muscular dystrophies. Master of Biomedical Science (Thesis), Université Catholique de Louvain (Belgium). Lowain, 1993.
26. Raphael JC, Bouvet F, Chevret S. Evaluation des différents moyens de ventilation mécanique à domicile dans la dystrophie musculaire de Duchenne de Boulogne: résultats intermédiares d'une étude prospective multicentrique. Entretiens de la Fondation Garches 1990, 259-260.
27. Rechtschaffen A, Kales A. A manual of standardized terminology, techniques and scoring system for sleep stages of human sugjects. Los Angeles: UCLA, 1968.
28. Smith PEM, Edwards RHT, Calverley PMA. Mechanisms of sleep-disordered breathing in chronic neuromuscular disease: implications for management. Q J Med 1991, 296: 961-973.
29. Tobin MJ. Respiratory muscles in disease. Clin Chest Med 1988, 9: 263-286.
30. Vignos PJ, Spencer GE Jr, Archibald KC. Management of progressive muscular dystrophy of childhood. JAMA 1963, 184: 103-110.
31. Williams RL, Karacan I, Hursch CJ. Electroencephalography of human sleep: clinical applications. New York: John Wiley & Sons, 1974.

Publication Dates

Publication in this collection
19 Jan 2011
Date of issue
Dec 1994

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

[1] 1. Aldrich MS. Neurologic aspects of sleep apnea and related respiratory disturbances. Otolaryngol Clin North Am 1990, 23: 761-769.

[2] 2. American Thoracic Society. Indications and standards for cardiopulmonary sleep studies. Am Rev Resp Dis 1989, 139: 559-568.

[3] 3. Back JR, Campagnolo DI, Hoeman S. Life satisfaction of individuals with Duchenne muscular dystrophy using long-term mechanical ventilatory support. Am J Phys Med Rehabil 1991, 70: 129-135.

[4] 4. Baydur A, Gilgoff I, Prentice W, Carlson M, Fischer A. Decline in respiratory function and experience with long-term assisted ventilation in advanced Duchenne's muscular dystrophy. Chest 1990, 97: 884-889.

[5] 5. Bertrand A, Milane J, Jonquet O, Bertrand P. De la poliomyélite antérieure aiguë aux myopathies: prise en charge de l'insuffisance respiratoire chronique par déficit neuromusculaire. Bull Acad Natle Méd 1989, 173: 769-776.

[6] 6. Bye PTP, Ellis ER, Donnelly PD, Issa FG, Sullivan CE. Role of sleep in the development of respiratory failure in neuromucular disease. Am Rev Respir Dis 1985, 131: A108.

[7] 7. Carrol N, Bain RJ, Smith PEM, Saltissi E, Edwards RHT, Calverley PMA. Domiciliary investigation of sleep-related hypoxaemia in Duchenne muscular dystrophy. Eur Respir J 1991, 4: 434-440.

[8] 8. De Troyer A, Borenstein S, Cordier R. Analysis of lung volume restriction in patients with respiratory muscle weakness. Thorax 1980, 35: 603-610.

[9] 9. Delguste P, Rodenstein DO. Désaturations nocturnes dans la myopathie de Duchenne de Boulogne. Entretiens de la Fondation Garches 1990, 263-264.

[10] 10. Del mas MC, Bérard C. Prise en charge globale de 1'amyotrophic spinale infantile: guide practique pour les équipes médicales. Pédiatrie 1990, 457-464.

[11] 11. Douglas NJ. Control of breathing during sleep. Clin Sci 1984, 67: 465-472.

[12] 12. Duron B. Respiration et sommeil. Association des Physiologistes 1987, A57-A71.

[13] 13. Estournet B, Bataille J, Commare MC, Urtizberea JA, Barois A. La ventilation nasale chez l'enfant atteint de maladie neuro-musculaire. Entretiens de la Fondation Garches 1990, 273.

[14] 14. Gastaut H, Duron B, Papy JJ, Tassinari CA, Waltregny J. Etude polygraphique comparative du cycle nycthémérique chez les narcoléptiques, les pickwickiens, les obèses et les insuffisants respiratoires. Rev Neurol 1966, 115:456-462.

[15] 15. Guilleminault C. The role of sleep and sleep states on breathing disorders. Schweiz Med Wschr 1988, 118: 1331-1332.

[16] 16. Heckmatt JZ. Respiratory care in muscular dystrophy. Br Med J 1987, 295: 1014-1015.

[17] 17. Heckmatt JZ, Loh L, Dubowits V. Nocturnal hypoventilation in children with nonprogressive neuromuscular disease. Pediatrics 1989, 83: 250-255.

[18] 18. Krieger J, Turlot JC, Mangin P,Kurtz D. Breathing during sleep in normal young and elderly subjects : hypopneas, apneas and correlated factors. Sleep 1983, 6: 108-120.

[19] 19. Kryger MH, Steljes DG, Yee WC, Mate E, Smith SA, Mahowald M. Central sleep apnoea in congenital muscular dystrophy. J Neurol Neurosurg Psychiatry 1991, 54: 710-712.

[20] 20. Le Balle F, Quera-Salva MA, McCann C, Barbe F, Lattre J, Gajdos P, Troche G. Apnées du sommeil chez les patients atteints de dystrophie musculaire de Duchenne de Boulogne. 1990, 265-266.

[21] 21. Lugaresi E, Cirignotta F, Montagna P. Snoring: pathogenic, clinical and therapeutic aspects. In Kryger M, Roth T, Dement WC (eds). Principles and practice of sleep medicine. Philadelphia, W B Saunders Company 1989, 494-500.

[22] 22. Manni R, Zucca C, Galimgerti CA, Ottolini A, Cerveri I, Bruschi C, Tartara A. Nocturnal sleep and oxygen balance in Duchenne muscular dystrophy. Eur Arch Psychiatry Clin Neurosci 1991, 240: 255-257.

[23] 23. Miller RG, Chalmers AC, Dao H, Filler-Katz A, Holman D, Bost F. The effect of spine fusion on respiratory function in Duchenne muscular dystrophy. Neurology 1991, 41: 38-40.

[24] 24. Newson-Davis JM, Goldman M, Loh LC. Diaphragm function and alveolar hypoventilation. Q J Med 1976, 177: 87-100.

[25] 25. Pradella M. A study of oxygen saturation and other polygraphic parameters in neuromuscular diseases with particular reference to muscular dystrophies. Master of Biomedical Science (Thesis), Université Catholique de Louvain (Belgium). Lowain, 1993.

[26] 26. Raphael JC, Bouvet F, Chevret S. Evaluation des différents moyens de ventilation mécanique à domicile dans la dystrophie musculaire de Duchenne de Boulogne: résultats intermédiares d'une étude prospective multicentrique. Entretiens de la Fondation Garches 1990, 259-260.

[27] 27. Rechtschaffen A, Kales A. A manual of standardized terminology, techniques and scoring system for sleep stages of human sugjects. Los Angeles: UCLA, 1968.

[28] 28. Smith PEM, Edwards RHT, Calverley PMA. Mechanisms of sleep-disordered breathing in chronic neuromuscular disease: implications for management. Q J Med 1991, 296: 961-973.

[29] 29. Tobin MJ. Respiratory muscles in disease. Clin Chest Med 1988, 9: 263-286.

[30] 30. Vignos PJ, Spencer GE Jr, Archibald KC. Management of progressive muscular dystrophy of childhood. JAMA 1963, 184: 103-110.

[31] 31. Williams RL, Karacan I, Hursch CJ. Electroencephalography of human sleep: clinical applications. New York: John Wiley & Sons, 1974.