Critical and Respiratory Care in Neuromuscular Disorders



Critical care of patients with neuromuscular disease has evolved over the years to include diagnosis, recognition, and management of the natural progression of these disorders as well as the treatments, potential side effects, and comorbid illnesses that are often seen in the intensive care units. In this chapter, we discuss the general critical care approach, the basic changes in pulmonary function, the electrodiagnostic testing, and the pulmonary aspects of the neuromuscular disorders seen in the ICU. We also discuss long-term respiratory care and management of chronic respiratory failure since it constitutes an essential aspect of medical care in patients with progressive neuromuscular diseases.


muscular dystrophy Malignant hyperthermia Acid maltase deficiency Periodic paralysis myasthenia gravis inspiratory force vital capacity pulmonary function tests 


  1. 1.
    Boulton TB, Mulvein JT. Intensive care for neurological patients. Proc R Soc Med. 1970;63:825–31.PubMedGoogle Scholar
  2. 2.
    Curran FJ, Colbert AP. Ventilator management in Duchenne muscular dystrophy and postpoliomyelitis: twelve years’ experience. Arch Phys Med Rehabil. 1989;70:180–5.PubMedGoogle Scholar
  3. 3.
    Lynn DJ, Woda RP, Mendell JR. Respiratory dysfunction in muscular dystrophy and myopathies. Clin Chest Med. 1994;15:661–74.PubMedGoogle Scholar
  4. 4.
    Syabbalo N. Respiratory muscle function in patients with neuromuscular disorders and cardiopulmonary diseases. Int J Clin Pract. 1998;52:319–29.PubMedGoogle Scholar
  5. 5.
    Tobin MJ. Respiratory muscles in disease. Clin Chest Med. 1988;9:263–86.PubMedGoogle Scholar
  6. 6.
    Coccagna G, Martinelli P, Lugarsi E. Alveolar hypoventilation in myotonic dystrophy. Bull Eur Physiopathol Respir. 1982;18:155–6.Google Scholar
  7. 7.
    Estenne M, Gevenois PA, Kinnear W, Soudon P, Heilporn A, De Troyer A. Lung volume restriction in patients with chronic respiratory muscle weakness: the role of microatelectasis. Thorax. 1993;48:698–701.PubMedGoogle Scholar
  8. 8.
    Pride NB. Interaction between chest wall, respiratory muscles, and lung function in disease. Bull Eur Physiopathol Respir. 1983;20:423–8.Google Scholar
  9. 9.
    Estenne M, Heilporn A, Delhez L, Yernault JC, De Troyer A. Chest wall stiffness in patients with chronic respiratory muscle weakness. Am Rev Respir Dis. 1983;128:1002–7.PubMedGoogle Scholar
  10. 10.
    De Troyer A, Deisser P. The effect of intermittent positive pressure breathing on patients with respiratory muscle weakness. Am Rev Respir Dis. 1981;124:132–7.PubMedGoogle Scholar
  11. 11.
    O’Donohue Jr WJ, Baker JP, Bell GM, Murren O, Parker CL, Patterson Jr JL. Respiratory failure in neuromuscular disease. Management in a respiratory intensive care unit. JAMA. 1976;235:733–5.PubMedGoogle Scholar
  12. 12.
    Borel CO, Teitelbaum JS, Hanely DF. Ventilatory drive and carbon dioxide response in ventilatory failure due to myasthenia gravis and Guillain-Barre syndrome. Crit Care Med. 1993;21:1717–26.PubMedGoogle Scholar
  13. 13.
    Borel CO, Tilford C, Nicholas DG, Hanley DF, Traystman RJ. Diaphragmatic performance during recovery from acute ventilatory failure in Guillain-Barre syndrome and myasthenia gravis. Chest. 1991;99:444–51.PubMedGoogle Scholar
  14. 14.
    Vitacca M, Clini E, Facchetti D, Pagani M, Poloni M, Porta R, et al. Breathing pattern and respiratory mechanics in patients with amyotrophic lateral sclerosis. Eur Respir J. 1997;10:1614–21.PubMedGoogle Scholar
  15. 15.
    Perez A, Mulot R, Vardon G, Barois A, Gallego J. Thoracoabdominal pattern of breathing in neuromuscular disorders. Chest. 1996;110:454–61.PubMedGoogle Scholar
  16. 16.
    Vincken W, Elleker G, Cosio MG. Detection of upper airway muscle involvement in neuromuscular disorders using the flow-volume loop. Chest. 1986;90:52–7.PubMedGoogle Scholar
  17. 17.
    Vincken WG, Elleker MG, Cosio MG. Flow-volume loop changes reflecting respiratory muscle weakness in chronic neuromuscular disorders. Am J Med. 1987;83:673–80.PubMedGoogle Scholar
  18. 18.
    van Lunteren E, Strohl KP. The muscles of the upper airways. Clin Chest Med. 1986;7(2):171–88.PubMedGoogle Scholar
  19. 19.
    Weng TR, Schultz GE, Chang CH, Nigro MA. Pulmonary function and ventilatory response to chemical stimuli in familial myopathy. Chest. 1985;88:488–95.PubMedGoogle Scholar
  20. 20.
    Begin P, Mathieu J, Almirall J, Grassino A. Relationship between chronic hypercapnia and inspiratory-muscle weakness in myotonic dystrophy. Am J Respir Crit Care Med. 1997;156:133–9.PubMedGoogle Scholar
  21. 21.
    Griggs RC, Donohoe KM, Utell MJ, Goldblatt D, Moxley 3rd RT. Evaluation of pulmonary function in neuromuscular disease. Arch Neurol. 1981;38:9–12.PubMedGoogle Scholar
  22. 22.
    Heritier F, Rahm F, Pasche P, Fitting JW. Sniff nasal inspiratory pressure. A noninvasive assessment of inspiratory muscle strength. Am J Respir Crit Care Med. 1994;150:1678–83.PubMedGoogle Scholar
  23. 23.
    Warren RH, Alderson SH. A pulmonary monitoring and treatment plan for children with Duchenne-type muscular dystrophies. J Ark Med Soc. 1996;93:333–7.PubMedGoogle Scholar
  24. 24.
    Hughes RA. Management of acute neuromuscular paralysis. J R Coll Physicians Lond. 1998;32:254–9.PubMedGoogle Scholar
  25. 25.
    Bennet DA, Bleck TP. Diagnosis and treatment of neuromuscular causes of acute respiratory failure. Clin Neuropharmacol. 1988;11:303–47.Google Scholar
  26. 26.
    Reider P, Louis M, Jolliet P, Chevrolet JC. The repeated measurement of vital capacity is a poor predictor of the need for mechanical ventilation in myasthenia gravis. Intensive Care Med. 1995;21:663–8.Google Scholar
  27. 27.
    Hall WJ. Respiratory failure as a complication of neuromuscular disease. Adv Neurol. 1977;17:317–24.PubMedGoogle Scholar
  28. 28.
    Ropper AH. Neurological intensive care. Ann Neurol. 1992;32:564–9.PubMedGoogle Scholar
  29. 29.
    Thomas CE, Mayer SA, Gungor Y, Swarup R, Webster EA, Chang I, et al. Myasthenic crisis: clinical features, mortality, complications, and risk factors for prolonged intubation. Neurology. 1997;48:1253–60.PubMedGoogle Scholar
  30. 30.
    Mayer SA. Intensive care of the myasthenic patients. Neurology. 1997;48:S70–5.Google Scholar
  31. 31.
    Drachman D. Myasthenia gravis. N Engl J Med. 1994;330:1797–810.PubMedGoogle Scholar
  32. 32.
    Ropper AH. Guillain-Barre syndrome. In: Ropper AH, Kennedy SK, editors. Neurological and neurosurgical intensive care. Baltimore: Aspen Publisher; 1988. p. 253–69.Google Scholar
  33. 33.
    Tejeda M, Boix JH, Alvarez F, Balanza R, Morales M. Comparison of pressure support ventilation and assist-control ventilation in the treatment of respiratory failure. Chest. 1997;111:1322–5.PubMedGoogle Scholar
  34. 34.
    Higgs BD, Bevan JC. Use of mandatory minute volume ventilation in the perioperative management of a patient with myasthenia. Br J Anaesth. 1979;51:1181–4.PubMedGoogle Scholar
  35. 35.
    Esteban A, Frutos F, Tobin MJ, Alia I, Solsona JF, Valverdu I, et al. For the Spanish lung failure collaborative group. A comparison of the four method methods of weaning patients from mechanical ventilation. N Engl J Med. 1995;332:345–50.PubMedGoogle Scholar
  36. 36.
    Shneerson J. Pathophysiology of the respiratory consequences of childhood neuromuscular disorders. Pediatr Pulmonol Suppl. 1997;16:134–6.PubMedGoogle Scholar
  37. 37.
    Birnkrant DJ, Pope JF, Eiben RM. Management of respiratory complications of neuromuscular diseases in the pediatric intensive care unit. J Child Neurol. 1999;14:130–43.Google Scholar
  38. 38.
    Howard RS, Wiles CM, Hirsch NP, Spencer GT. Respiratory involvement in primary neuromuscular disorders; assessment and management. Q J Med. 1993;86:175–89.PubMedGoogle Scholar
  39. 39.
    Polkey MI, Lyall RA, Moxham J, Leigh PN. Respiratory aspects of neurological disease. J Neurol Neurosurg Psychiatry. 1999;66:5–15.PubMedGoogle Scholar
  40. 40.
    Barden C, Lee R, Parchment Y, Dempsey RM. An unusual neurologic problem: Werdnig-Hoffman disease. Crit Care Nurse. 1990;10:60–7.PubMedGoogle Scholar
  41. 41.
    Moss AH, Oppenheimer EA, Casey P, Cazzolli PA, Roos RP, Stocking CB, et al. Patients with amyotrophic lateral sclerosis receiving long-term mechanical ventilation. Advanced care planning and outcomes. Chest. 1996;110:249–55.PubMedGoogle Scholar
  42. 42.
    Braun RS, Sufit RL, Giovannoni R, O’Connor M, Peters H. Intermittent negative pressure ventilation in the treatment of respiratory failure in progressive neuromuscular disease. Neurology. 1987;37:1874–5.PubMedGoogle Scholar
  43. 43.
    Pfeiffer G, Winkler G, Neunzig P, Wolf W, Thayssen G, Kunze K. Long term management of acute respiratory failure in metabolic myopathy. Intensive Care Med. 1996;22:1406–9.PubMedGoogle Scholar
  44. 44.
    Bye PT, Ellis ER, Issa FG, Donnelly PM, Sullivan CE. Respiratory failure and sleep in neuromuscular disease. Thorax. 1990;45:241–7.PubMedGoogle Scholar
  45. 45.
    Sivak ED, Gipson WT, Hanson MR. Long term management of respiratory failure in amyotrophic lateral sclerosis. Ann Neurol. 1982;12:18–23.PubMedGoogle Scholar
  46. 46.
    Escarrabill J, Estopa R, Farrero E, Monasterio C, Manresa F. Long-term mechanical ventilation in amyotrophic lateral sclerosis. Respir Med. 1998;92:438–41.PubMedGoogle Scholar
  47. 47.
    Moss AH, Casey P, Stocking CB, Roos RP, Brooks BR, Siegler M. Home ventilation for amyotrophic lateral sclerosis patients. Neurology. 1993;43:438–43.PubMedGoogle Scholar
  48. 48.
    Goldblatt D, Greeniaw J. Starting and stopping the ventilator for patients with amyotrophic lateral sclerosis. Neurol Clin. 1989;7:789–806.PubMedGoogle Scholar
  49. 49.
    Martinez M, Mitchell A. Home planning and discharge for the ventilator-dependent patient: a case study. Crit Care Nurse. 1989;9:79–82.PubMedGoogle Scholar
  50. 50.
    Davis RH. Home ventilation of a child with motor and sensory neuropathy. BMJ. 1996;313:153–4.Google Scholar
  51. 51.
    Gigloff I, Prentice W, Baydur A. Patient and family participation in the management of respiratory failure in Duchenne’s muscular dystrophy. Chest. 1989;95:519–24.Google Scholar
  52. 52.
    Goldberg AI. Mechanical ventilation and respiratory care in the home in the 1990’s: some personal observations. Respir Care. 1990;35:247–59.PubMedGoogle Scholar
  53. 53.
    Goldstein RS, Molotiu N, Skrastins R, Long S, Contreras M. Assisting ventilation in respiratory failure by negative pressure ventilation and rocking bed. Chest. 1987;92:470–4.PubMedGoogle Scholar
  54. 54.
    Hill NS. Clinical application of body ventilator. Chest. 1986;69:369–72.Google Scholar
  55. 55.
    Yang G-FW, Alba A, Lee M, Khan A. Pneumobelt use among high quadriplegic population. Arch Phys Med Rehabil. 1989;70:707–11.PubMedGoogle Scholar
  56. 56.
    Bach JR, Alba AS. Total ventilatory support by the intermittent abdominal pressure ventilator. Chest. 1991;99:630–6.PubMedGoogle Scholar
  57. 57.
    Mohr CH, Hill NS. Long-term follow-up of nocturnal ventilatory assistance in patients with respiratory failure due to Duchenne-type muscular dystrophy. Chest. 1990;97:91–6.PubMedGoogle Scholar
  58. 58.
    Heckmatt J, Loh L, Dubowitz V. Night time nasal ventilation in neuromuscular disease. Lancet. 1990;335:379–582.Google Scholar
  59. 59.
    Carter RE, Donovan UH, Halstead L, Wilkinson MA. Comparative study of electrophrenic nerve stimulation and mechanical ventilatory support in traumatic spinal cord injury. Paraplegia. 1987;25:86–91.PubMedGoogle Scholar
  60. 60.
    Glenn WWL, Holcomb WG, Shaw RK, Hogan JF, Holschuh KR. Long-term ventilatory support by diaphragm pacing in quadriplegia. Ann Surg. 1976;183:566–77.PubMedGoogle Scholar
  61. 61.
    Onders RP, Khansarinia S, Weiser T, Chin C, Hungness E, Soper N, et al. Multicenter analysis of diaphragm pacing in tetraplegics with cardiac pacemakers: positive implications for ventilator weaning in intensive care units. Surgery. 2010;148:893–7.PubMedGoogle Scholar
  62. 62.
    McCool FD, Tzelepis GE. Inspiratory muscle training in the patient with neuromuscular disease. Phys Ther. 1995;75:1006–14.PubMedGoogle Scholar
  63. 63.
    Mason KJ. Neuromuscular scoliosis: a case of the pediatric patient in the adult ICU. Crit Care Nurs Q. 1998;21:64–80.PubMedGoogle Scholar
  64. 64.
    Nardi J, Prigent H, Adata A, Bohic M, Lebargy F, Quera-Salva MA, et al. Nocturnal oximetry and transcutaneous carbon dioxide in home-ventilated neuromuscular patients. Respir Care. 2012;57(9):1425–30.PubMedGoogle Scholar
  65. 65.
    Traux BT. Autonomic disturbances in the Guillain-Barre syndrome. Semin Neurol. 1984;4:462–8.Google Scholar
  66. 66.
    Greenland P, Griggs RC. Arrhythmic complications in the Guillain-Barre syndrome. Arch Intern Med. 1980;140:1053–5.PubMedGoogle Scholar
  67. 67.
    Narayan D, Huang MT, Mathew PK. Bradycardia and asystole requiring permanent pacemaker in Guillain-Barre syndrome. Am Heart J. 1984;108:426–8.PubMedGoogle Scholar
  68. 68.
    Gibson TC. The heart in myasthenia gravis. Am Heart J. 1975;90:389–96.PubMedGoogle Scholar
  69. 69.
    Kornfeld P, Horowitz SH, Genkins G, Papatestas A. Myasthenia gravis unmasked by antiarrhythmic agents. Mt Sinai J Med. 1976;43:10–4.PubMedGoogle Scholar
  70. 70.
    James TN. Observation on the cardiovascular involvement, including the cardiac conduction system, in progressive muscular dystrophy. Am Heart J. 1962;63:48.PubMedGoogle Scholar
  71. 71.
    Lewis WR, Yadlapalli S. Management of cardiac complications in neuromuscular disease. Phys Med Rehabil Clin N Am. 1998;9:145–66.PubMedGoogle Scholar
  72. 72.
    Skyring A, McKusick VA, et al. Clinical, genetic and electrocardiographic studies in childhood muscular dystrophy. Am J Med Sci. 1961;242:54.Google Scholar
  73. 73.
    Chenard AA, Becane HM, Tertrain F, de Kermadec JM, Weiss YA. Ventricular arrhythmia in Duchenne muscular dystrophy: prevalence, significance and prognosis. Neuromuscul Disord. 993;3:201–6.PubMedGoogle Scholar
  74. 74.
    Perloff JK. Cardiac conduction in Duchenne’s muscular dystrophy: a prospective study of 20 patients. J Am Coll Cardiol. 1984;3:1263–8.PubMedGoogle Scholar
  75. 75.
    Mann O, DeLeon Jr AC, Perloff JK, Simanis J, Horrigan FD. Duchenne’s muscular dystrophy: the electrocardiogram in female relatives. Am J Med Sci. 1968;255:376–81.PubMedGoogle Scholar
  76. 76.
    Miller G, D’Orsogna L, Oshea JP. Autonomic function and the sinus tachycardia of Duchenne’s muscular dystrophy. Brain Dev. 1989;11:247–50.PubMedGoogle Scholar
  77. 77.
    Pailloncy M, Citron B, Hersch B, Heiligenstein D, Ponsonnaille J, Gras H. Electrocardiograms of women carriers of Duchenne-type muscular dystrophy: a study of a family with a case of complete atrioventricular heart block. Ann Cardiol Angeiol (Paris). 1982;31:47–50.Google Scholar
  78. 78.
    Perloff JK, Delon AC, O’Doherty D. The cardiomyopathy of progressive muscular dystrophy. Circulation. 1996;33:625–48.Google Scholar
  79. 79.
    Riggs T. Cardiomyopathy and pulmonary emboli in terminal Duchenne’s muscular dystrophy. Am Heart J. 1990;119:690–3.PubMedGoogle Scholar
  80. 80.
    Sanyal SK, Johnson WW, Dische MR, Pitner SE, Beard C. Dystrophic degeneration of papillary muscles and ventricular myocardium. A basis for mitral valve prolapse in Duchenne’s muscular dystrophy. Circulation. 1980;62:430–8.PubMedGoogle Scholar
  81. 81.
    Lazzeroni E, Favaro L, Botti G. Dilated cardiomyopathy with regional myocardial hypoperfusion in Becker’s muscular dystrophy. Int J Cardiol. 1989;22:126–9.PubMedGoogle Scholar
  82. 82.
    Vrints C, Mercelis R, Vanagt E, Snoeck J, Martin JJ. Cardiac manifestations of Becker-type muscular dystrophy. Acta Cardiol. 1983;38:479–86.PubMedGoogle Scholar
  83. 83.
    Quinlivan RM, Lewis P, Marsden P, Dundas R, Robb SA, Baker E, et al. Cardiac function, metabolism and perfusion in Duchenne and Becker muscular dystrophy. Neuromuscul Disord. 1996;6:237–46.PubMedGoogle Scholar
  84. 84.
    Politano L, Nigro V, Nigro G, Petretta VR, Passamano L, Papparella S, et al. Development of cardiomyopathy in female carriers of Duchenne’s and Becker muscular dystrophies. JAMA. 1996;275:1335–8.PubMedGoogle Scholar
  85. 85.
    Bloomfield DA, Sinclair-Smith BC, et al. Persistent atrial standstill. Am J Med. 1965;39:335.PubMedGoogle Scholar
  86. 86.
    Hopkins LC, Jackson JA, Elsas LJ. Emery-Dreifuss humeroperoneal muscular dystrophy: an X-linked myopathy with unusual contracture and bradycardia. Ann Neurol. 1981;10:230–7.PubMedGoogle Scholar
  87. 87.
    Oswald AH, Goldblatt J, Horak AR, Beighton P. Lethal cardiac conduction defect in Emery-Dreifuss muscular dystrophy. S Afr Med J. 1987;729:567–70.Google Scholar
  88. 88.
    DeBacker M, Bergmann P, Perissino A, Gottignies P, Kahn RJ. Respiratory failure and cardiac disturbances in myotonic dystrophy. Eur J Intensive Care Med. 1976;2:63–7.Google Scholar
  89. 89.
    Perloff JK, Stevenson WG, Robert NK, Cabeen W, Weiss J. Cardiac involvement in myotonic dystrophy (Steinert’s disease): a prospective study of 25 patients. Am J Cardiol. 1984;54:1074–81.PubMedGoogle Scholar
  90. 90.
    Morgenlander JC, Nohria V, Saba Z. EKG abnormalities in pediatric patients with myotonic dystrophy. Pediatr Neurol. 1993;9:124–6.PubMedGoogle Scholar
  91. 91.
    Olofsson BO, Forsberg H, Andersson S, Bjerle P, Henriksson A, Wedin I. Electrocardiographic findings in myotonic dystrophy. Br Heart J. 1988;59:47–52.PubMedGoogle Scholar
  92. 92.
    Nguyen HH, Wolfe 3rd JT, Homes Jr DR, Edward WD. Pathology of the cardiac conduction system in myotonic dystrophy: a study of 12 cases. J Am Coll Cardiol. 1988;11:662–71.PubMedGoogle Scholar
  93. 93.
    Petkovich NJ, Dunn M, Reed W. Myotonia dystrophica with AV dissociation and Stokes-Adams attacks. Am Heart J. 1964;63:391.Google Scholar
  94. 94.
    Hiromasa S, Ikeda T, Kubota K, Hattori N, Coto H, Maldonado C, et al. Ventricular tachycardia and sudden death in myotonic dystrophy. Am Heart J. 1988;115:914–5.PubMedGoogle Scholar
  95. 95.
    Moorman JR, Coleman RE, Packer DL, Kisslo JA, Bell J, Hettleman BD, et al. Cardiac involvement in myotonic muscular dystrophy. Medicine. 1985;64:371–87.PubMedGoogle Scholar
  96. 96.
    Child JS, Perloff JK, Bach PM, Wolfe AD, Perlman S, Kark RA. Cardiac involvement in Friedreich’s ataxia. J Am Coll Cardiol. 1986;7:1370–8.PubMedGoogle Scholar
  97. 97.
    Harding AE, Hewer RL. The heart disease of Friedreich’s ataxia: a clinical and electrocardiographic study of 115 patients, with an analysis of serial electrocardiographic changes in 35 cases. Q J Med. 1983;52:489–502.PubMedGoogle Scholar
  98. 98.
    Pasternac A, Wagniart P, Olivenstein R, Petitclerc R, Krol R, Anderman E, et al. Increased plasma catecholamines in patients with Friedreich’s ataxia. Can J Neurol Sci. 1982;9:195–203.PubMedGoogle Scholar
  99. 99.
    Isner JM, Hawley RJ, Weintraub AB, Engel WK. Cardiac findings in Charcot-Marie-Tooth disease. Arch Intern Med. 1979;139:1161–5.PubMedGoogle Scholar
  100. 100.
    Verhiest W, Brucher JM, Goddeeris P, Lauweryns J, De Geest H. Familial centronuclear myopathy associated with cardiomyopathy. Br Heart J. 1976;38:504–9.PubMedGoogle Scholar
  101. 101.
    Bolton CF. Electrophysiologic studies of critically ill patients. Muscle Nerve. 1987;10:129–35.PubMedGoogle Scholar
  102. 102.
    Grand’Maison F. Methods of testing neuromuscular transmission in the intensive care unit. Can J Neurol Sci. 1998;25:S36–9.PubMedGoogle Scholar
  103. 103.
    Zaidat OO, Kaminski HJ, Berenson F, Katirji B. Neuromuscular transmission defect caused by carbamazepine. Muscle Nerve. 1999;22:1293–6.PubMedGoogle Scholar
  104. 104.
    Bolton CF. Assessment of respiratory function in the intensive care unit. Can J Neurol Sci. 1994;21:S28–34.PubMedGoogle Scholar
  105. 105.
    Berrouschot J, Baumann I, Kalischewski P, Sterker M, Schneider D. Therapy of myasthenic crisis. Crit Care Med. 1997;25:1228–35.PubMedGoogle Scholar
  106. 106.
    van der Meche FGA, Schmitz PIM, Dutch Guillain-Barre Study Group. A randomized trial comparing intravenous immunoglobulin and plasma exchange in Guillain-Barre syndrome. N Eng J Med. 1992;325:1123–9.Google Scholar
  107. 107.
    Dalakas MC. Intravenous immune globulin therapy for neurologic diseases. Ann Intern Med. 1997;126:721–30.PubMedGoogle Scholar
  108. 108.
    Brannagan 3rd TH, Nagle KJ, Lange DJ, Rowland LP. Complications of intravenous immune globulin treatment in neurologic diseases. Neurology. 1996;47:674–7.PubMedGoogle Scholar
  109. 109.
    Berlot G, Tomasini A, Silvesteri L, Gullo A. Plasmapheresis in the critically ill patient. Kidney Int Suppl. 1998;66:S178–81.PubMedGoogle Scholar
  110. 110.
    Rodnitzky RL, Goeken JA. Complications of plasma exchange in neurological patients. Arch Neurol. 1982;39:350–4.PubMedGoogle Scholar
  111. 111.
    Braun NMT, Arora NS, Rochester DF. Respiratory muscle and pulmonary function in polymyositis and other proximal myopathies. Thorax. 1983;38:488–92.Google Scholar
  112. 112.
    Gottdiener JS, Sherber HS, Hawley RJ, Engel WK. Cardiac manifestations in polymyositis. Am J Cardiol. 1978;41:1141–9.PubMedGoogle Scholar
  113. 113.
    Amato AA, Barohn RJ. Idiopathic inflammatory myopathies. Neurol Clin. 1997;15:615–48.PubMedGoogle Scholar
  114. 114.
    Dalakas MC. Immunopathogenesis of inflammatory myopathies. Ann Neurol. 1995;37:S74–86.PubMedGoogle Scholar
  115. 115.
    Manzur AY, Kuntzer T, Pick M, Swan A. Glucocorticoid corticosteroids for Duchene muscular dystrophy. Cochrane Database Syst Rev. 2008;(1):CD003725.Google Scholar
  116. 116.
    Malik V, Rodino-Klapac LR, Mendell JR. Emerging drugs for Duchene muscular dystrophy. Expert Opin Emerg Drugs. 2012;17:261–77.PubMedGoogle Scholar
  117. 117.
    Ohkoshi N, Yoshizawa T, Mizusawa H, Shoji S, Toyama M, Iida K, et al. Malignant hyperthermia in a patient with Becker muscular dystrophy: dystrophin analysis and caffeine contracture study. Neuromuscul Disord. 1995;5:53–8.PubMedGoogle Scholar
  118. 118.
    Bushby KM. Making sense of the limb-girdle muscular dystrophies. Brain. 1999;122:1403–20.PubMedGoogle Scholar
  119. 119.
    Duggan DJ, Gorospe JR, Fanin M, Hoffman EP, Angelini C. Mutations in the sarcoglycan genes in patients with myopathy. N Engl J Med. 1997;336:618–24.PubMedGoogle Scholar
  120. 120.
    Newsom Davis J. The respiratory system in muscular dystrophy. Br Med Bull. 1992;36:135–8.Google Scholar
  121. 121.
    Hoshio A, Kotake H, Saitoh M, Ogino K, Fujimoto Y, Hasegawa J, et al. Cardiac involvement in a patient with limb-girdle muscular dystrophy. Heart Lung. 1987;16:439–41.PubMedGoogle Scholar
  122. 122.
    Kawashima S, Ueno M, Kondo T, Yamamoto J, Iwasaki T. Marked cardiac involvement of limb-girdle muscular dystrophy. Am J Med Sci. 1990;299:411–4.PubMedGoogle Scholar
  123. 123.
    Hansotia P, Frens D. Hypersomnolence associated with alveolar hypoventilation in myotonic dystrophy. Neurology. 1981;31:1336–7.PubMedGoogle Scholar
  124. 124.
    Ricker K. Myotonic dystrophy and proximal myotonic myopathy. J Neurol. 1999;246:334–8.PubMedGoogle Scholar
  125. 125.
    Moore JK, Moore AP. Postoperative complications of dystrophia myotonica. Anesthesia. 1987;42:529–33.Google Scholar
  126. 126.
    Pash MP, Balaton J, Eagle C. Anesthetic management of a patient with severe muscular dystrophy, lumbar lordosis and a difficult airway. Can J Anaesth. 1996;43:959–63.PubMedGoogle Scholar
  127. 127.
    Rowland LP, Fetell M, Olarte M, Hays A, Singh N, Wanat FE. Emery-Dreifuss muscular dystrophy. Ann Neurol. 1979;5:111–7.PubMedGoogle Scholar
  128. 128.
    Takamoto K, Hirose K, Uono M, Nonaka I. A genetic variant of Emery-Dreifuss disease. Muscular dystrophy with humeropelvic distribution, early joint contracture and permanent atrial paralysis. Arch Neurol. 1984;41:1292–3.PubMedGoogle Scholar
  129. 129.
    Miller RG, Layzer RB, Mellenthin MA, Golabi M, Francoz RA, Mall JC. Emery-Dreifuss muscular dystrophy with autosomal transmission. Neurology. 1985;35:1230–3.PubMedGoogle Scholar
  130. 130.
    Hopkins LC, Jackson JA, Elsas CJ. Cardiac involvement in Emery Dreifuss muscular dystrophy: a case series. Heart. 1999;82:105–8.Google Scholar
  131. 131.
    Goto I, Nagasaka S, Nagara H, Kuroiwa Y. Rigid spine syndrome. J Neurol Neurosurg Psychiatry. 1979;42:276–9.PubMedGoogle Scholar
  132. 132.
    Morita H, Kondo K, Hoshino K, Maruyama K, Yanagisawa N. Rigid spine syndrome with respiratory failure. J Neurol Neurosurg Psychiatry. 1990;53:782–4.PubMedGoogle Scholar
  133. 133.
    Laforte P, de Toma C, Eymard B, Becane HM, Jeanpierre M, Fardeau M, et al. Cardiac involvement in genetically confirmed facioscapulohumeral dystrophy. Neurology. 1998;51:1454–6.Google Scholar
  134. 134.
    Denborough M. Malignant hyperthermia. Lancet. 1998;352:1131–6.PubMedGoogle Scholar
  135. 135.
    Hirshey Dirksen SJ, Green Larach M, Rosenberg H, Brandom BW, Parness J, Lang RS, et al. Future directions in malignant hyperthermia research and patient care. Anesth Analg. 2011;113:1108–19.PubMedGoogle Scholar
  136. 136.
    Rosenow EC, Engel AG. Acid maltase deficiency in adults presenting as respiratory failure. Am J Med. 1978;64:485–91.PubMedGoogle Scholar
  137. 137.
    Ptacek LJ. Channelopathies: Ion channel disorders of muscle as a paradigm for paroxysmal disorders of the nervous system. Neuromuscul Disord. 1997;7:250–5.PubMedGoogle Scholar
  138. 138.
    Adamiec L, Blachoswka B, Drac H. Assessment of respiratory system changes in a patient with familial periodic paralysis of atypical course. Anaesth Resusc Intensive Ther. 1974;2:379–84.PubMedGoogle Scholar
  139. 139.
    Lisak RP, Lebeau J, Tucker SH, Rowland LP. Hyperkalemic periodic paralysis and cardiac arrhythmias. Neurology. 1972;20:386.Google Scholar
  140. 140.
    Levitt LP, Rose LI, Dawson DM. Hypokalemic periodic paralysis with arrhythmias. N Engl J Med. 1972;286:253–4.PubMedGoogle Scholar
  141. 141.
    Ruff RL. Why do ICU patients become paralyzed? Ann Neurol. 1998;43:154–5.PubMedGoogle Scholar
  142. 142.
    Maher J, Rutledge F, Remtulla H, Parkes A, Bernardi L, Bolton CF. Neuromuscular disorders associated with failure to wean from the ventilator. Intensive Care Med. 1995;21:737–43.PubMedGoogle Scholar
  143. 143.
    Pascuzzi RM. Drugs and toxins associated with myopathies. Curr Opin Rheumatol. 1998;10:511–20.PubMedGoogle Scholar
  144. 144.
    Slater MS, Mullins RJ. Rhabdomyolysis and myoglobinuric renal failure in trauma and surgical patients: a review. J Am Coll Surg. 1998;186:693–716.PubMedGoogle Scholar
  145. 145.
    Hanna JP, Ramundo ML. Rhabdomyolysis and hypoxia associated with prolonged propofol infusion in children. Neurology. 1998;50:301–3.PubMedGoogle Scholar
  146. 146.
    Bolton CF, Young GB, Zochodone DW. The neurological complications of sepsis. Ann Neurol. 1993;33:94–100.PubMedGoogle Scholar
  147. 147.
    Lacomis D. Neuromuscular disorders in critically ill patients: review and update. J Clin Neuromuscul Dis. 2011;12:197–218.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Department of NeurologyMedical College of Wisconsin and Froedtert HospitalMilwaukeeUSA
  2. 2.Division of Pulmonary/Critical Care and Sleep Medicine, Department of MedicineUniversity Hospital Case Medical Center, Case Western Reserve University School of MedicineClevelandUSA
  3. 3.Department of NeurologyBaylor College of MedicineHoustonUSA

Personalised recommendations