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Klinische Neurophysiologie 2015; 46(04): 163-172
DOI: 10.1055/s-0041-106978
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© Georg Thieme Verlag KG Stuttgart · New York

Beatmung und Sekretmanagement bei amyotropher Lateralsklerose

Ventilatory Support and Management of Secretions in Amyotrophic Lateral Sclerosis
M. Boentert
Klinik für Schlafmedizin und Neuromuskuläre Erkrankungen, Universitätsklinikum Münster
,
P. Young
Klinik für Schlafmedizin und Neuromuskuläre Erkrankungen, Universitätsklinikum Münster
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Publikationsverlauf

Publikationsdatum:
04. November 2015 (online)

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Zusammenfassung

Die Symptome und Folgen der muskulären Ateminsuffizienz tragen bei der amyotrophen Lateralsklerose maßgeblich zur Morbidität und Mortalität der Erkrankung bei und beeinträchtigen nachhaltig die Lebensqualität. Während initial Symptome und Zeichen der schlafbezogenen Atmungsstörung im Vordergrund stehen, kommt es im Verlauf zu chronischem hyperkapnischem Atemversagen und zu einer klinisch hoch relevanten Hustenschwäche mit Sekretverhalt, fehlendem Schutz vor Aspiration und erhöhtem Infektrisiko. Die frühzeitige Erkennung und adäquate Therapie atmungsassoziierter Symptome ist für die Lebensqualität und die Prognose der betroffenen Patienten entscheidend. Dieser Übersichtsartikel fasst den gegenwärtigen Stand der diagnostischen und therapeutischen Möglichkeiten zusammen und gibt praxisrelevante Empfehlungen zum Management der in- und exspiratorischen Atemmuskelschwäche.

Abstract

In most patients with ALS, progressive respiratory muscle weakness is a major aspect of morbidity and mortality. It inevitably evolves and substantially reduces quality of life. Initially, respiratory muscle weakness presents as sleep-disordered breathing but progresses to chronic hypercapnic respiratory failure. Diaphragmatic dysfunction also causes significant weakness of cough resulting in mucus retention, increased risk of aspiration, and recurrent pulmonary infections. Early detection and adequate treatment of respiratory muscle weakness is of utmost importance for both quality of life and overall prognosis in ALS patients. This review article summarizes current diagnostic and therapeutic options and gives practical recommendations for the management of inspiratory and expiratory muscle weakness in ALS.

 

  • Literatur

  • 1 Chio A, Logroscino G, Traynor BJ et al. Global epidemiology of amyotrophic lateral sclerosis: a systematic review of the published literature. Neuroepidemiology 2013; 41: 118-130
    CrossrefPubMedGoogle Scholar
  • 2 Mehta P, Antao V, Kaye W et al. Prevalence of amyotrophic lateral sclerosis – United States, 2010-2011. MMWR Surveill Summ 2014; 63 (Suppl. 07) 1-14
    PubMedGoogle Scholar
  • 3 Tsai CP, Wang KC, Hwang CS et al. Incidence, prevalence, and medical expenditures of classical amyotrophic lateral sclerosis in Taiwan, 1999–2008. Journal of the Formosan Medical Association 2015; 114: 612-619
    CrossrefPubMedGoogle Scholar
  • 4 Shoesmith CL, Findlater K, Rowe A et al. Prognosis of amyotrophic lateral sclerosis with respiratory onset. Journal of neurology, neurosurgery, and psychiatry 2007; 78: 629-631
    CrossrefPubMedGoogle Scholar
  • 5 Gil J, Funalot B, Verschueren A et al. Causes of death amongst French patients with amyotrophic lateral sclerosis: a prospective study. European journal of neurology: the official journal of the European Federation of Neurological Societies 2008; 15: 1245-1251
    CrossrefPubMedGoogle Scholar
  • 6 Czaplinski A, Yen AA, Simpson EP et al. Slower disease progression and prolonged survival in contemporary patients with amyotrophic lateral sclerosis: is the natural history of amyotrophic lateral sclerosis changing?. Archives of neurology 2006; 63: 1139-1143
    CrossrefPubMedGoogle Scholar
  • 7 O’toole O, Traynor BJ, Brennan P et al. Epidemiology and clinical features of amyotrophic lateral sclerosis in Ireland between 1995 and 2004. Journal of neurology, neurosurgery, and psychiatry 2008; 79: 30-32
    CrossrefPubMedGoogle Scholar
  • 8 Zoccolella S, Beghi E, Palagano G et al. ALS multidisciplinary clinic and survival. Results from a population-based study in Southern Italy. Journal of neurology 2007; 254: 1107-1112
    CrossrefPubMedGoogle Scholar
  • 9 Engel GL. The need for a new medical model: a challenge for biomedicine. Science 1977; 196: 129-136
    CrossrefPubMedGoogle Scholar
  • 10 Perrin C, Unterborn JN, Ambrosio CD et al. Pulmonary complications of chronic neuromuscular diseases and their management. Muscle & nerve 2004; 29: 5-27
    CrossrefPubMedGoogle Scholar
  • 11 Czaplinski A, Yen AA, Appel SH. Forced vital capacity (FVC) as an indicator of survival and disease progression in an ALS clinic population. Journal of neurology, neurosurgery, and psychiatry 2006; 77: 390-392
    PubMedGoogle Scholar
  • 12 Ragette R, Mellies U, Schwake C et al. Patterns and predictors of sleep disordered breathing in primary myopathies. Thorax 2002; 57: 724-728
    CrossrefPubMedGoogle Scholar
  • 13 Toussaint M, Steens M, Soudon P. Lung function accurately predicts hypercapnia in patients with Duchenne muscular dystrophy. Chest 2007; 131: 368-375
    CrossrefPubMedGoogle Scholar
  • 14 Evans JA, Whitelaw WA. The assessment of maximal respiratory mouth pressures in adults. Respiratory care 2009; 54: 1348-1359
    PubMedGoogle Scholar
  • 15 Kabitz HJ, Walterspacher S, Mellies U et al. Messung der Atemmuskelfunktion. Empfehlungen der Deutschen Atemwegsliga e.V. in der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin (DGP). München, Orlando: Dustri-Verlag Dr. Karl Feistle; 2014
    Google Scholar
  • 16 Jackson CE, Rosenfeld J, Moore DH et al. A preliminary evaluation of a prospective study of pulmonary function studies and symptoms of hypoventilation in ALS/MND patients. Journal of the neurological sciences 2001; 191: 75-78
    CrossrefPubMedGoogle Scholar
  • 17 Kang SW, Bach JR. Maximum insufflation capacity: vital capacity and cough flows in neuromuscular disease. American journal of physical medicine & rehabilitation/Association of Academic Physiatrists 2000; 79: 222-227
    CrossrefPubMedGoogle Scholar
  • 18 Preston DC, Shapiro BE. Electromyography and Neuromuscular Disorders: Clinical-Electrophysiologic Correlations. 3rd. ed. Elsevier; 2014
    Google Scholar
  • 19 Pinto S, De Carvalho M. Symmetry of phrenic nerve motor response in amyotrophic lateral sclerosis. Muscle & nerve 2010; 42: 822-825
    CrossrefPubMedGoogle Scholar
  • 20 Pinto S, Geraldes R, Vaz N et al. Changes of the phrenic nerve motor response in amyotrophic lateral sclerosis: longitudinal study. Clinical neurophysiology 2009; 120: 2082-2085
    CrossrefPubMedGoogle Scholar
  • 21 Pinto S, Pinto A, De Carvalho M. Phrenic nerve studies predict survival in amyotrophic lateral sclerosis. Clinical neurophysiology 2012; 123: 2454-2459
    CrossrefPubMedGoogle Scholar
  • 22 Pinto S, Turkman A, Pinto A et al. Predicting respiratory insufficiency in amyotrophic lateral sclerosis: the role of phrenic nerve studies. Clinical neurophysiology 2009; 120: 941-946
    CrossrefPubMedGoogle Scholar
  • 23 Saadeh PB, Crisafulli CF, Sosner J et al. Needle electromyography of the diaphragm: a new technique. Muscle & nerve 1993; 16: 15-20
    CrossrefPubMedGoogle Scholar
  • 24 De Carvalho M, Pinto S, Swash M. Association of paraspinal and diaphragm denervation in ALS. Amyotrophic lateral sclerosis 2010; 11: 63-66
    PubMedGoogle Scholar
  • 25 Hukins CA, Hillman DR. Daytime predictors of sleep hypoventilation in Duchenne muscular dystrophy. American journal of respiratory and critical care medicine 2000; 161: 166-170
    CrossrefPubMedGoogle Scholar
  • 26 Windisch W, Brambring J, Budweiser S et al. [Non-invasive and invasive mechanical ventilation for treatment of chronic respiratory failure. S2-Guidelines published by the German Medical Association of Pneumology and Ventilatory Support]. Pneumologie 2010; 64: 207-240
    Artikel in Thieme ConnectPubMedGoogle Scholar
  • 27 Vrijsen B, Buyse B, Belge C et al. Upper airway obstruction during noninvasive ventilation induced by the use of an oronasal mask. JCSM 2014; 10: 1033-1035
    PubMedGoogle Scholar
  • 28 Miller RG, Jackson CE, Kasarskis EJ et al. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2009; 73: 1218-1226
    CrossrefPubMedGoogle Scholar
  • 29 Bourke SC, Tomlinson M, Williams TL et al. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. The Lancet. Neurology 2006; 5: 140-147
    PubMedGoogle Scholar
  • 30 Lyall RA, Donaldson N, Fleming T et al. A prospective study of quality of life in ALS patients treated with noninvasive ventilation. Neurology 2001; 57: 153-156
    CrossrefPubMedGoogle Scholar
  • 31 Butz M, Wollinsky KH, Wiedemuth-Catrinescu U et al. Longitudinal effects of noninvasive positive-pressure ventilation in patients with amyotrophic lateral sclerosis. American journal of physical medicine & rehabilitation 2003; 82: 597-604
    CrossrefPubMedGoogle Scholar
  • 32 Boentert M, Brenscheidt I, Glatz C et al. Effects of non-invasive ventilation on objective sleep and nocturnal respiration in patients with amyotrophic lateral sclerosis. Journal of neurology 2015; 262: 2073-2082
    CrossrefPubMedGoogle Scholar
  • 33 Vrijsen B, Buyse B, Belge C et al. Noninvasive ventilation improves sleep in amyotrophic lateral sclerosis: a prospective polysomnographic study. JCSM 2015; 11: 559-566
    PubMedGoogle Scholar
  • 34 Aboussouan LS, Khan SU, Meeker DP et al. Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis. Annals of internal medicine 1997; 127: 450-453
    CrossrefPubMedGoogle Scholar
  • 35 Kleopa KA, Sherman M, Neal B et al. Bipap improves survival and rate of pulmonary function decline in patients with ALS. Journal of the neurological sciences 1999; 164: 82-88
    CrossrefPubMedGoogle Scholar
  • 36 Mustfa N, Walsh E, Bryant V et al. The effect of noninvasive ventilation on ALS patients and their caregivers. Neurology 2006; 66: 1211-1217
    CrossrefPubMedGoogle Scholar
  • 37 Pinto AC, Evangelista T, Carvalho M et al. Respiratory assistance with a non-invasive ventilator (Bipap) in MND/ALS patients: survival rates in a controlled trial. Journal of the neurological sciences 1995; 129 (Suppl): 19-26
    CrossrefPubMedGoogle Scholar
  • 38 Bensimon G, Lacomblez L, Meininger V. A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group. The New England journal of medicine 1994; 330: 585-591
    CrossrefPubMedGoogle Scholar
  • 39 Lacomblez L, Bensimon G, Leigh PN et al. A confirmatory dose-ranging study of riluzole in ALS. ALS/Riluzole Study Group-II. Neurology 1996; 47: S242-250
    CrossrefPubMedGoogle Scholar
  • 40 http://www.fda.gov/MedicalDevices/ProductsandMedicalProcedures/DeviceApprovalsandClearances/Recently-ApprovedDevices/ucm278684.htm
    PubMed
  • 41 Onders RP, Elmo M, Kaplan C et al. Final analysis of the pilot trial of diaphragm pacing in amyotrophic lateral sclerosis with long-term follow-up: diaphragm pacing positively affects diaphragm respiration. American journal of surgery 2014; 207: 393-397; discussion 397
    CrossrefPubMedGoogle Scholar
  • 42 DiPALS Writing Committee, DiPALS Study Group Collaborators. Safety and efficacy of diaphragm pacing in patients with respiratory insufficiency due to amyotrophic lateral sclerosis (DiPALS): a multicentre, open-label, randomised controlled trial. The Lancet Neurology 2015; 14: 883-892
    CrossrefPubMedGoogle Scholar
  • 43 Carratu P, Spicuzza L, Cassano A et al. Early treatment with noninvasive positive pressure ventilation prolongs survival in Amyotrophic Lateral Sclerosis patients with nocturnal respiratory insufficiency. Orphanet journal of rare diseases 2009; 4: 10
    CrossrefPubMedGoogle Scholar
  • 44 Windisch W, Walterspacher S, Siemon K et al. Guidelines for non-invasive and invasive mechanical ventilation for treatment of chronic respiratory failure. Published by the German Society for Pneumology (DGP). Pneumologie 2010; 64: 640-652
    Artikel in Thieme ConnectPubMedGoogle Scholar
  • 45 Pryor JA. Physiotherapy for airway clearance in adults. The European respiratory journal 1999; 14: 1418-1424
    CrossrefPubMedGoogle Scholar
  • 46 Bach JR, Ishikawa Y, Kim H. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest 1997; 112: 1024-1028
    CrossrefPubMedGoogle Scholar
  • 47 Tzeng AC, Bach JR. Prevention of pulmonary morbidity for patients with neuromuscular disease. Chest 2000; 118: 1390-1396
    CrossrefPubMedGoogle Scholar
  • 48 Rafiq MK, Bradburn M, Proctor AR et al. A preliminary randomized trial of the mechanical insufflator-exsufflator versus breath-stacking technique in patients with amyotrophic lateral sclerosis. Amyotrophic lateral sclerosis & frontotemporal degeneration 2015; DOI: 10.3109/21678421.2015.1051992.
    PubMedGoogle Scholar
  • 49 Chatburn RL. High-frequency assisted airway clearance. Respiratory care 2007; 52: 1224-1235; discussion 1235–1227
    PubMedGoogle Scholar