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DOI: 10.1055/s-0029-1222449
Hypoventilation in Neuromuscular Disease
Publikationsverlauf
Publikationsdatum:
18. Mai 2009 (online)
ABSTRACT
Hypoventilation in neuromuscular disease is attributed to both respiratory muscle weakness and reduced chemoreceptor sensitivity essential in ventilatory drive. Acute or chronic respiratory failure is seen in a spectrum of neuromuscular disease; whereas some are treatable others are progressive and devastating. Sleep is associated with a reduction in ventilation and hence worsening hypoventilation. Problems with sleep may be an early indicator of further muscle weakness and should prompt the clinician for further investigation, though usefulness of pulmonary function testing, arterial blood gas analysis, and other measures may not be universally predictive. The timing of respiratory failure is variable, but knowledge of the clinical aspects, pathogenesis, and treatment of respiratory failure and hypoventilation may be helpful in evaluating the patient with neuromuscular disease. For those with progressive and terminal disease, additional factors such as end of life care, especially ventilation and cough, may be useful for the patient, caregivers, and treating medical personnel.
KEYWORDS
Hypoventilation - sleep - neuromuscular disease
REFERENCES
-
1 Criner G J, Kelsen S G.
Effects of neuromuscular diseases on ventilation . In: Fishman AP, Elias JA, Fishman JA, Grippi MA, Kaiser LR, Senior RM Fishman's Pulmonary Diseases and Disorders. 3rd ed. New York; McGraw-Hill 1998: 1561-1585 - 2 Bach J R, Alba A S. Management of chronic alveolar hypoventilation by nasal ventilation. Chest. 1990; 97 52-57
- 3 Simon P M, Dempsey J A, Landry D M, Skatrud J B. Effect of sleep on respiratory muscle activity during mechanical ventilation. Am Rev Respir Dis. 1993; 147 32-37
- 4 Gould G A, Gugger M, Molloy J, Tsara V MSC, Shapiro C M, Douglas N J. Breathing pattern and eye movement density during REM sleep in humans. Am Rev Respir Dis. 1988; 138 874-877
- 5 Millman R P, Knight H, Kline L R, Shore E T, Chung D C, Pack A I. Changes in compartmental ventilation in association with rapid eye movements during REM sleep. J Appl Physiol. 1988; 65 1196-1202
- 6 Schäfer T, Schläfke M E. Respiratory changes associated with rapid eye movements in normo- and hypercapnia during sleep. J Appl Physiol. 1988; 85 2213-2219
- 7 Duron B, Marlot D. Intercostal and diaphragmatic electrical activity during wakefulness and sleep in normal unrestrained adult cats. Sleep. 1980; 3 269-280
-
8 Drachman D B.
Myasthenia gravis and other disease of the neuromuscular junction . In: Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL Harrison's Principles of Internal Medicine. New York; McGraw-Hill 2005: 2518-2523 - 9 Drachman D B. Myasthenia gravis. N Engl J Med. 1994; 330 1797-1810
- 10 Oh S J, Kim D E, Kuruoglu R, Bradley R J, Dwyer D. Diagnostic sensitivity of the laboratory tests in myasthenia gravis. Muscle Nerve. 1992; 15 720-724
- 11 Chan K H, Lachance D H, Harper C M, Lennon V A. Frequency of seronegativity in adult-acquired generalized myasthenia gravis. Muscle Nerve. 2007; 36 651-658
- 12 Meriggioli M N, Sanders D B. Myasthenia gravis: diagnosis. Semin Neurol. 2004; 24 31-39
-
13 Bartt R, Shannon K M.
Autoimmune and inflammatory disorders . In: Goeetz CG Textbook of Clinical Neurology. Philadelphia; Saunders 2003: 1118-1120 - 14 Czaplinski A, Steck A J, Fuhr P. Ice pack test for myasthenia gravis: a simple, noninvasive and safe diagnostic method. J Neurol. 2003; 250 883-884
- 15 Seneviratne J, Mandrekar J, Wijdicks E F, Rabinstein A A. Noninvasive ventilation in myasthenic crisis. Arch Neurol. 2008; 65 54-58
- 16 Seneviratne J, Mandrekar J, Wijdicks E F, Rabinstein A A. Predictors of extubation failure in myasthenic crisis. Arch Neurol. 2008; 65 929-933
- 17 Nicolle M W, Rask S, Koopman W J, George C F, Adams J, Wiebe S. Sleep apnea in patients with myasthenia gravis. Neurology. 2006; 67 140-142
- 18 Putman M T, Wise R A. Myasthenia gravis and upper airway obstruction. Chest. 1996; 109 400-404
- 19 Hadden R D, Cornblath D R, Hughes R A et al.. Electrophysiological classification of Guillain-Barré syndrome: clinical associations and outcome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Ann Neurol. 1998; 44 780-788
- 20 Markoula S, Giannopoulos S, Sarmas I, Tzavidi S, Kyritsis A P, Lagos G. Guillain-Barré syndrome in northwest Greece. Acta Neurol Scand. 2007; 115 167-173
- 21 Bogliun G, Beghi E. Guillain-Barrè Syndrome Registry Study Group . Validity of hospital discharge diagnoses for public health surveillance of the Guillain-Barrè syndrome. Neurol Sci. 2002; 23 113-117
- 22 Winer J B. Guillain-Barré syndrome. BMJ. 2008; 337 a671
-
23 Hauser S L, Asbury A K.
Guillain-Barré syndrome and other immune-mediated neuropathies . In: Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL Harrison's Principles of Internal Medicine. New York; Mc-Graw-Hill 2005: 2513-2518 - 24 Yuki N, Taki T, Inagaki F et al.. A bacterium lipopolysaccharide that elicits Guillain-Barré syndrome has a GM1 ganglioside-like structure. J Exp Med. 1993; 178 1771-1775
- 25 Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I. Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies. Neurology. 1993; 43 1911-1917
- 26 Criteria for diagnosis of Guillain-Barré syndrome. Ann Neurol. 1978; 3 565-566
- 27 Appropriate number of plasma exchanges in Guillain-Barré syndrome. The French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome. Ann Neurol. 1997; 41 298-306
- 28 Rees J H, Thompson R D, Smeeton N C, Hughes R A. Epidemiological study of Guillain-Barré syndrome in southeast England. J Neurol Neurosurg Psychiatry. 1998; 64 74-77
- 29 Lawn N D, Fletcher D D, Henderson R D, Wolter T D, Wijdicks E F. Anticipating mechanical ventilation in Guillain-Barré syndrome. Arch Neurol. 2001; 58 893-898
- 30 Chevrolet J C, Deléamont P. Repeated vital capacity measurements as predictive parameters for mechanical ventilation need and weaning success in the Guillain-Barré syndrome. Am Rev Respir Dis. 1991; 144 814-818
- 31 Sharshar T, Chevret S, Bourdain F, Raphaël J C. French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome . Early predictors of mechanical ventilation in Guillain-Barré syndrome. Crit Care Med. 2003; 31 278-283
- 32 Wijdicks E F, Roy T K. BiPAP in early Guillain-Barré syndrome may fail. Can J Neurol Sci. 2006; 33 105-106
- 33 Pearse R M, Draper A, Grounds R M. Non-invasive ventilation to avoid tracheal intubation in a patient with Guillain-Barré syndrome. Br J Anaesth. 2003; 91 913-916
- 34 Atkinson W, Hamborsky J, McIntyre L, Wolfe S. Public Health Foundation, Centers for Disease Control and Prevention .Poliomyelitis. Epidemiology and prevention of vaccine-preventable diseases. 5th ed. Washington, DC; 2007 http://Available at: www.cdc.gov/vaccines/pubs/pinkbook/downloads/polio.pdf
- 35 Bruno R L. Post-polio sequelae: research and treatment in the second decade. , [editorial] Orthopedics. 1991; 14 1169-1170
-
36 Jubelt B, Drucker J.
Poliomyelitis and the post-polio syndrome . In: Younger D Motor Disorders. Philadelphia; Lippincott Williams and Wilkins 1999: 381-395 - 37 Klingman J, Chui H, Corgiat M, Perry J. Functional recovery: a major risk factor for the development of postpoliomyelitis muscular atrophy. Arch Neurol. 1988; 45 645-647
- 38 Dean E, Ross J, Road J D, Courtenay L, Madill K J. Pulmonary function in individuals with a history of poliomyelitis. Chest. 1991; 100 118-123
- 39 Shneerson J M, Simonds A K. Noninvasive ventilation for chest wall and neuromuscular disorders. Eur Respir J. 2002; 20 480-487
- 40 Midgren B. Lung function and clinical outcome in postpolio patients: a prospective cohort study during 11 years. Eur Respir J. 1997; 10 146-149
-
41 Guilleminault C, Motta J.
Sleep apnea syndrome as a long-term sequela of poliomyelitis . In: Guilleminault C Sleep Apnea Syndromes. New York; Editorial. Kroc Foundation; Alan R. Liss 1978: 309-315 - 42 Hsu A A, Staats B A. “Postpolio” sequelae and sleep-related disordered breathing. Mayo Clin Proc. 1998; 73 216-224
- 43 Ulfberg J, Jönsson R, Ekeroth G. Sleep apnea syndrome among poliomyelitis survivors. Neurology. 1997; 49 1189-1190
- 44 Steljes D G, Kryger M H, Kirk B W, Millar T W. Sleep in postpolio syndrome. Chest. 1990; 98 133-140
- 45 Perrin C, D'Ambrosio C, White A, Hill N S. Sleep in restrictive and neuromuscular respiratory disorders. Semin Respir Crit Care Med. 2005; 26 117-130
- 46 Dahan V, Kimoff R J, Petrof B J, Benedetti A, Diorio D, Trojan D A. Sleep-disordered breathing in fatigued postpoliomyelitis clinic patients. Arch Phys Med Rehabil. 2006; 87 1352-1356
- 47 Sivak E D, Shefner J M, Sexton J. Neuromuscular disease and hypoventilation. Curr Opin Pulm Med. 1999; 5 355-362
- 48 Dean A C, Graham B A, Dalakas M, Sato S. Sleep apnea in patients with postpolio syndrome. Ann Neurol. 1998; 43 661-664
- 49 Markström A, Sundell K, Lysdahl M, Andersson G, Schedin U, Klang B. Quality-of-life evaluation of patients with neuromuscular and skeletal diseases treated with noninvasive and invasive home mechanical ventilation. Chest. 2002; 122 1695-1700
- 50 Pinto A C, Evangelista T, Carvalho M, Alves M A, Sales Luís M L. Respiratory assistance with a non-invasive ventilator (BiPAP) in MND/ALS patients: survival rates in a controlled trial. J Neurol Sci. 1995; 129(Suppl) 19-26
- 51 McDonald E R, Hillel A, Wiedenfeld S A. Evaluation of the psychological status of ventilatory-supported patients with ALS/MND. Palliat Med. 1996; 10 35-41
- 52 O'Neill J H, Murray N M, Newsom-Davis J. The Lambert-Eaton myasthenic syndrome: a review of 50 cases. Brain. 1988; 111(Pt 3) 577-596
-
53 Ropper A, Brown A.
Myasthenia gravis and related disorders of the neuromuscular junction . In: Ropper AH, Brown RH Adams and Victor Principles of Neurology. New York; McGraw-Hill 2005: 1250-1264 - 54 Lennon V A, Kryzer T J, Griesmann G E et al.. Calcium-channel antibodies in the Lambert-Eaton syndrome and other paraneoplastic syndromes. N Engl J Med. 1995; 332 1467-1474
- 55 Bhatt J R, Pascuzzi R M. Neuromuscular disorders in clinical practice: case studies. Neurol Clin. 2006; 24 233-265
- 56 Anderson H J, Churchill-Davidson H C, Richardson A G. Bronchial neoplasm after administration of succinylcholine. Lancet. 1953; 2 1291-1293
- 57 Laroche C M, Mier A K, Spiro S G, Newsom-Davis J, Moxham J, Green M. Respiratory muscle weakness in the Lambert-Eaton myasthenic syndrome. Thorax. 1989; 44 913-918
- 58 Sabater L, Titulaer M, Saiz A, Verschuuren J, Güre A O, Graus F. SOX1 antibodies are markers of Lambert-Eaton myasthenic syndrome. Neurology. 2008; 70 924-928
- 59 Titulaer M J, Wirtz P W, Willems L N, van Kralingen K W, Smitt P A, Verschuuren J J. Screening for small-cell lung cancer: a follow-up study of patients with Lambert-Eaton myasthenic syndrome. J Clin Oncol. 2008; 26 4276-4281
-
60 Brown R H, Mendell J R.
Muscular dystrophies and other muscle disorders . In: Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL Harrison's Principles of Internal Medicine. New York; Mc-Graw-Hill 2005: 2527-2540 - 61 Smith P EM, Calverley P M, Edwards R H, Evans G A, Campbell E J. Practical problems in the respiratory care of patients with muscular dystrophy. N Engl J Med. 1987; 316 1197-1205
- 62 Nagai T. Prognostic evaluation of congestive heart failure in patients with Duchenne muscular dystrophy: retrospective study using non-invasive cardiac function tests. Jpn Circ J. 1989; 53 406-415
- 63 Suresh S, Wales P, Dakin C, Harris M A, Cooper D M. Sleep-related breathing disorder in Duchenne muscular dystrophy: disease spectrum in the pediatric population. J Paediatr Child Health. 2005; 41 500-503
- 64 Farkas G A, McCormick K M, Gosselin L E. Episodic hypoxia exacerbates respiratory muscle dysfunction in DMD(mdx) mice. Muscle Nerve. 2007; 36 708-710
- 65 Hukins C A, Hillman D R. Daytime predictors of sleep hypoventilation in Duchenne muscular dystrophy. Am J Respir Crit Care Med. 2000; 161 166-170
- 66 Toussaint M, Steens M, Soudon P. Lung function accurately predicts hypercapnia in patients with Duchenne muscular dystrophy. Chest. 2007; 131 368-375
- 67 Kirk V G, Flemons W W, Adams C, Rimmer K P, Montgomery M D. Sleep-disordered breathing in Duchenne muscular dystrophy: a preliminary study of the role of portable monitoring. Pediatr Pulmonol. 2000; 29 135-140
- 68 Raphael J C, Chevret S, Chastang C, Bouvet F. French Multicentre Cooperative Group on Home Mechanical Ventilation Assistance in Duchenne de Boulogne Muscular Dystrophy . Randomised trial of preventive nasal ventilation in Duchenne muscular dystrophy. Lancet. 1994; 343 1600-1604
- 69 Simonds A K, Muntoni F, Heather S, Fielding S. Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy. Thorax. 1998; 53 949-952
- 70 Vianello A, Bevilacqua M, Salvador V, Cardaioli C, Vincenti E. Long-term nasal intermittent positive pressure ventilation in advanced Duchenne's muscular dystrophy. Chest. 1994; 105 445-448
-
71 Brown R H.
Amyotrophic lateral sclerosis and other motor neuron diseases . In: Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL Harrison's Principles of Internal Medicine. New York; Mc-Graw-Hill 2005: 2424-2428 - 72 Fallat R J, Jewitt B, Bass M, Kamm B, Norris Jr F H. Spirometry in amyotrophic lateral sclerosis. Arch Neurol. 1979; 36 74-80
- 73 Nakano K K, Bass H, Tyler H R, Carmel R J. Amyotrophic lateral sclerosis: a study of pulmonary function. Dis Nerv Syst. 1976; 37 32-35
- 74 Williams D B, Windebank A J. Motor neuron disease (amyotrophic lateral sclerosis). Mayo Clin Proc. 1991; 66 54-82
-
75 Miller A.
Pulmonary function and respiratory failure in neuromuscular diseases with reference to amyotrophic lateral sclerosis . In: Caroscio JT Amyotrophic Lateral Sclerosis: A Guide to Patient Care. Stuttgart; Editorial. Thieme Medical Publishers 1986: 61-77 - 76 Shimizu T, Hayashi H, Kato S, Hayashi M, Tanabe H, Oda M. Circulatory collapse and sudden death in respirator-dependent amyotrophic lateral sclerosis. J Neurol Sci. 1994; 124 45-55
- 77 Kimura K, Tachibana N, Kimura J, Shibasaki H. Sleep-disordered breathing at an early stage of amyotrophic lateral sclerosis. J Neurol Sci. 1999; 164 37-43
- 78 Ferguson K A, Strong M J, Ahmad D, George C F. Sleep-disordered breathing in amyotrophic lateral sclerosis. Chest. 1996; 110 664-669
- 79 Atalaia A, De Carvalho M, Evangelista T, Pinto A. Sleep characteristics of amyotrophic lateral sclerosis in patients with preserved diaphragmatic function. Amyotroph Lateral Scler. 2007; 8 101-105
- 80 Arnulf I, Similowski T, Salachas F et al.. Sleep disorders and diaphragmatic function in patients with amyotrophic lateral sclerosis. Am J Respir Crit Care Med. 2000; 161(3 Pt 1) 849-856
- 81 Aboussouan L S, Lewis R A. Sleep, respiration and ALS. J Neurol Sci. 1999; 164 1-2
- 82 Barthlen G M, Lange D J. Unexpectedly severe sleep and respiratory pathology in patients with amyotrophic lateral sclerosis. Eur J Neurol. 2000; 7 299-302
- 83 Lo Coco D, Marchese S, Corrao S et al.. Development of chronic hypoventilation in amyotrophic lateral sclerosis patients. Respir Med. 2006; 100 1028-1036
- 84 LCD for respiratory assist devices. http://Available at www.cmshhs.gov Accessed December 31, 2008
- 85 Jackson C E, Rosenfeld J, Moore D H et al.. A preliminary evaluation of a prospective study of pulmonary function studies and symptoms of hypoventilation in ALS/MND patients. J Neurol Sci. 2001; 191 75-78
- 86 Aboussouan L S, Khan S U, Meeker D P, Stelmach K, Mitsumoto H. Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis. Ann Intern Med. 1997; 127 450-453
- 87 Kleopa K A, Sherman M, Neal B, Romano G J, Heiman-Patterson T. BiPAP improves survival and rate of pulmonary function decline in patients with ALS. J Neurol Sci. 1999; 164 82-88
- 88 Bourke S C, Bullock R E, Williams T L, Shaw P J, Gibson G J. Noninvasive ventilation in ALS: indications and effect on quality of life. Neurology. 2003; 61 171-177
- 89 Pinto A C, Evangelista T, Carvalho M, Alves M A, Sales Luís M L. Respiratory assistance with a non-invasive ventilator (BiPAP) in MND/ALS patients: survival rates in a controlled trial. J Neurol Sci. 1995; 129(Suppl) 19-26
- 90 McDonald E R, Hillel A, Wiedenfeld S A. Evaluation of the psychological status of ventilatory-supported patients with ALS/MND. Palliative Medicine. 1996; 10 35-41
- 91 Haas C F, Loik P S, Gay S E. Airway clearance applications in the elderly and in patients with neurologic or neuromuscular compromise. Respir Care. 2007; 52 1362-1381
- 92 McCoo F D, Rosen M J. Nonpharmacological airway clearance therapies: ACCP evidence-based clinical practice guidelines. Chest. 2006; 129(suppl I) 28S-32S
- 93 Koessler W, Wanke T, Winkler G et al.. 2 Years' experience with inspiratory muscle training in patients with neuromuscular disorders. Chest. 2001; 120 765-769
- 94 Gozal D, Thiriet P. Respiratory muscle training in neuromuscular disease: long-term effects on strength and load perception. Med Sci Sports Exerc. 1999; 31 1522-1527
- 95 Gosselink R, Kovacs L, Ketelaer P, Carton H, Decramer M. Respiratory muscle weakness and respiratory muscle training in severely disabled multiple sclerosis patients. Arch Phys Med Rehabil. 2000; 81 747-751
- 96 Smeltzer S C, Lavietes M H, Cook S D. Expiratory training in multiple sclerosis. Arch Phys Med Rehabil. 1996; 77 909-912
- 97 Klefbeck B, Hamrah Nedjad J. Effect of inspiratory muscle training in patients with multiple sclerosis. Arch Phys Med Rehabil. 2003; 84 994-999
- 98 Fregonezi G A, Resqueti V R, Güell R, Pradas J, Casan P. Effects of 8-week, interval-based inspiratory muscle training and breathing retraining in patients with generalized myasthenia gravis. Chest. 2005; 128 1524-1530
- 99 Kang S W, Bach J R. Maximum insufflation capacity: vital capacity and cough flows in neuromuscular disease. Am J Phys Med Rehabil. 2000; 79 222-227
- 100 Bach J R. Mechanical insufflation-exsufflation: comparison of peak expiratory flows with manually assisted and unassisted coughing techniques. Chest. 1993; 104 1553-1562
- 101 Winck J C, Gonçalves M R, Lourenço C, Viana P, Almeida J, Bach J R. Effects of mechanical insufflation-exsufflation on respiratory parameters for patients with chronic airway secretion encumbrance. Chest. 2004; 126 774-780
- 102 Sancho J, Servera E, Díaz J, Marín J. Efficacy of mechanical insufflation-exsufflation in medically stable patients with amyotrophic lateral sclerosis. Chest. 2004; 125 1400-1405
- 103 Lange D J, Lechtzin N, Davey C HFCWO Study Group et al. High-frequency chest wall oscillation in ALS: an exploratory randomized, controlled trial. Neurology. 2006; 67 991-997
- 104 Reardon C C, Christiansen D, Barnett E D, Cabral H J. Intrapulmonary percussive ventilation vs incentive spirometry for children with neuromuscular disease. Arch Pediatr Adolesc Med. 2005; 159 526-531
- 105 Finder J D, Birnkrant D, Carl J American Thoracic Society et al. Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med. 2004; 170 456-465
- 106 Peretti-Watel P, Bendiane M K, Galinier A et al.. District nurses' attitudes toward patient consent: the case of mechanical ventilation on amyotrophic lateral sclerosis patients: results from a French national survey. J Crit Care. 2008; 23 332-338
- 107 Trail M, Nelson N D, Van J N, Appel S H, Lai E C. A study comparing patients with amyotrophic lateral sclerosis and their caregivers on measures of quality of life, depression, and their attitudes toward treatment options. J Neurol Sci. 2003; 209 79-85
Shirley F JonesM.D.
Department of Internal Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine
Scott and White Memorial Hospital/Texas A&M Health Science Center, 2401 South 31st. St., Temple, TX 76508
eMail: shjones@swmail.sw.org