{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T10:23:13Z","timestamp":1790677393916,"version":"4.1.0"},"reference-count":42,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2018,11,14]],"date-time":"2018-11-14T00:00:00Z","timestamp":1542153600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurol"],"published-print":{"date-parts":[[2019,1]]},"DOI":"10.1007\/s00415-018-9112-4","type":"journal-article","created":{"date-parts":[[2018,11,14]],"date-time":"2018-11-14T11:20:41Z","timestamp":1542194441000},"page":"133-147","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Safety and efficacy of short- and long-term inspiratory muscle training in late-onset Pompe disease (LOPD): a pilot study"],"prefix":"10.1007","volume":"266","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8407-3642","authenticated-orcid":false,"given":"Stephan","family":"Wenninger","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eva","family":"Greckl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haris","family":"Baba\u010di\u0107","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristina","family":"Stahl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benedikt","family":"Schoser","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2018,11,14]]},"reference":[{"issue":"3","key":"9112_CR1","doi-asserted-by":"publisher","first-page":"249","DOI":"10.1001\/jama.281.3.249","volume":"281","author":"PJ Meikle","year":"1999","unstructured":"Meikle PJ et al (1999) Prevalence of lysosomal storage disorders. JAMA 281(3):249\u2013254","journal-title":"JAMA"},{"issue":"1\u20132","key":"9112_CR2","doi-asserted-by":"publisher","first-page":"151","DOI":"10.1007\/s004399900075","volume":"105","author":"BJ Poorthuis","year":"1999","unstructured":"Poorthuis BJ et al (1999) The frequency of lysosomal storage diseases in The Netherlands. Hum Genet 105(1\u20132):151\u2013156","journal-title":"Hum Genet"},{"issue":"2","key":"9112_CR3","first-page":"91","volume":"32","author":"M Filosto","year":"2013","unstructured":"Filosto M et al (2013) Non-muscle involvement in late-onset glycogenosis II. Acta Myol 32(2):91\u201394","journal-title":"Acta Myol"},{"issue":"9646","key":"9112_CR4","doi-asserted-by":"publisher","first-page":"1342","DOI":"10.1016\/S0140-6736(08)61555-X","volume":"372","author":"AT Ploeg van der","year":"2008","unstructured":"van der Ploeg AT, Reuser AJ (2008) Pompe\u2019s disease. Lancet 372(9646):1342\u20131353","journal-title":"Lancet"},{"issue":"1","key":"9112_CR5","doi-asserted-by":"publisher","first-page":"80","DOI":"10.1002\/ajmg.c.31322","volume":"160C","author":"A Schuller","year":"2012","unstructured":"Schuller A et al (2012) Toward deconstructing the phenotype of late-onset Pompe disease. Am J Med Gen Part C Semin Med Genet 160C(1):80\u201388","journal-title":"Am J Med Gen Part C Semin Med Genet"},{"issue":"2","key":"9112_CR6","doi-asserted-by":"publisher","first-page":"369","DOI":"10.1111\/ene.12582","volume":"22","author":"M Boentert","year":"2015","unstructured":"Boentert M et al (2015) Sleep-related symptoms and sleep-disordered breathing in adult Pompe disease. Eur J Neurol 22(2):369\u2013376 (e27)","journal-title":"Eur J Neurol"},{"issue":"4","key":"9112_CR7","doi-asserted-by":"publisher","first-page":"477","DOI":"10.1016\/j.rmed.2008.12.009","volume":"103","author":"U Mellies","year":"2009","unstructured":"Mellies U, Lofaso F (2009) Pompe disease: a neuromuscular disease with respiratory muscle involvement. Respir Med 103(4):477\u2013484","journal-title":"Respir Med"},{"key":"9112_CR8","doi-asserted-by":"crossref","unstructured":"Wenninger S, Schoser B (2015) Behandelbare neuromuskul\u00e4re Erkrankungen als wichtige Differentialdiagnose der chronisch-progredienten Dyspnoe im h\u00f6heren Erwachsenenalter. Der Pneumologe, pp\u00a01\u20134","DOI":"10.1007\/s10405-015-0922-0"},{"issue":"8","key":"9112_CR9","doi-asserted-by":"publisher","first-page":"875","DOI":"10.1007\/s00415-005-0922-9","volume":"252","author":"LP Winkel","year":"2005","unstructured":"Winkel LP et al (2005) The natural course of non-classic Pompe\u2019s disease; a review of 225 published cases. J Neurol 252(8):875\u2013884","journal-title":"J Neurol"},{"key":"9112_CR10","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1186\/1750-1172-6-34","volume":"6","author":"D Gungor","year":"2011","unstructured":"Gungor D et al (2011) Survival and associated factors in 268 adults with Pompe disease prior to treatment with enzyme replacement therapy. Orphanet J Rare Dis 6:34","journal-title":"Orphanet J Rare Dis"},{"issue":"6","key":"9112_CR11","doi-asserted-by":"publisher","first-page":"1545","DOI":"10.1183\/09031936.00169011","volume":"39","author":"H Prigent","year":"2012","unstructured":"Prigent H et al (2012) Supine volume drop and diaphragmatic function in adults with Pompe disease. Eur Respir J 39(6):1545\u20131546","journal-title":"Eur Respir J"},{"issue":"15","key":"9112_CR12","doi-asserted-by":"publisher","first-page":"1396","DOI":"10.1056\/NEJMoa0909859","volume":"362","author":"AT Ploeg van der","year":"2010","unstructured":"van der Ploeg AT et al (2010) A randomized study of alglucosidase alfa in late-onset Pompe\u2019s disease. N Engl J Med 362(15):1396\u20131406","journal-title":"N Engl J Med"},{"issue":"1\u20132","key":"9112_CR13","doi-asserted-by":"publisher","first-page":"129","DOI":"10.1016\/j.ymgme.2011.06.012","volume":"104","author":"NA Beek van der","year":"2011","unstructured":"van der Beek NA et al (2011) Rate of progression and predictive factors for pulmonary outcome in children and adults with Pompe disease. Mol Genet Metab 104(1\u20132):129\u2013136","journal-title":"Mol Genet Metab"},{"issue":"4","key":"9112_CR14","doi-asserted-by":"publisher","first-page":"621","DOI":"10.1007\/s00415-016-8219-8","volume":"264","author":"B Schoser","year":"2016","unstructured":"Schoser B et al (2016) Survival and long-term outcomes in late-onset Pompe disease following alglucosidase alfa treatment: a systematic review and meta-analysis. J Neurol 264(4):621\u2013630. https:\/\/doi.org\/10.1007\/s00415-016-8219-8","journal-title":"J Neurol"},{"issue":"4","key":"9112_CR15","doi-asserted-by":"publisher","first-page":"495","DOI":"10.1002\/ana.20019","volume":"55","author":"LP Winkel","year":"2004","unstructured":"Winkel LP et al (2004) Enzyme replacement therapy in late-onset Pompe\u2019s disease: a three-year follow-up. Ann Neurol 55(4):495\u2013502","journal-title":"Ann Neurol"},{"issue":"2","key":"9112_CR16","doi-asserted-by":"publisher","first-page":"159","DOI":"10.1007\/s10545-007-0519-9","volume":"30","author":"DD Koeberl","year":"2007","unstructured":"Koeberl DD, Kishnani PS, Chen YT (2007) Glycogen storage disease types I and II: treatment updates. J Inherit Metab Dis 30(2):159\u2013164","journal-title":"J Inherit Metab Dis"},{"issue":"2","key":"9112_CR17","doi-asserted-by":"publisher","first-page":"99","DOI":"10.1212\/01.wnl.0000251268.41188.04","volume":"68","author":"PS Kishnani","year":"2007","unstructured":"Kishnani PS et al (2007) Recombinant human acid [alpha]-glucosidase: major clinical benefits in infantile-onset Pompe disease. Neurology 68(2):99\u2013109","journal-title":"Neurology"},{"issue":"4","key":"9112_CR18","doi-asserted-by":"publisher","first-page":"951","DOI":"10.1007\/s00415-012-6636-x","volume":"260","author":"A Toscano","year":"2013","unstructured":"Toscano A, Schoser B (2013) Enzyme replacement therapy in late-onset Pompe disease: a systematic literature review. J Neurol 260(4):951\u2013959","journal-title":"J Neurol"},{"key":"9112_CR19","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1186\/1750-1172-7-73","volume":"7","author":"JM Vries de","year":"2012","unstructured":"de Vries JM et al (2012) Effect of enzyme therapy and prognostic factors in 69 adults with Pompe disease: an open-label single-center study. Orphanet J Rare Dis 7:73","journal-title":"Orphanet J Rare Dis"},{"issue":"5","key":"9112_CR20","doi-asserted-by":"publisher","first-page":"537","DOI":"10.1007\/s00408-013-9489-x","volume":"191","author":"A Vianello","year":"2013","unstructured":"Vianello A et al (2013) Enzyme replacement therapy improves respiratory outcomes in patients with late-onset type II glycogenosis and high ventilator dependency. Lung 191(5):537\u2013544","journal-title":"Lung"},{"issue":"1","key":"9112_CR21","doi-asserted-by":"publisher","first-page":"52","DOI":"10.1186\/s13023-017-0598-0","volume":"12","author":"B Schoser","year":"2017","unstructured":"Schoser B et al (2017) Maximum inspiratory pressure as a clinically meaningful trial endpoint for neuromuscular diseases: a comprehensive review of the literature. Orphanet J Rare Dis 12(1):52","journal-title":"Orphanet J Rare Dis"},{"issue":"5","key":"9112_CR22","doi-asserted-by":"publisher","first-page":"529","DOI":"10.1097\/WCO.0000000000000470","volume":"30","author":"M Boentert","year":"2017","unstructured":"Boentert M, Wenninger S, Sansone VA (2017) Respiratory involvement in neuromuscular disorders. Curr Opin Neurol 30(5):529\u2013537","journal-title":"Curr Opin Neurol"},{"key":"9112_CR23","doi-asserted-by":"publisher","first-page":"184","DOI":"10.1186\/1471-2466-14-184","volume":"14","author":"CR Borge","year":"2014","unstructured":"Borge CR et al (2014) Effects of controlled breathing exercises and respiratory muscle training in people with chronic obstructive pulmonary disease: results from evaluating the quality of evidence in systematic reviews. BMC Pulm Med 14:184","journal-title":"BMC Pulm Med"},{"issue":"11","key":"9112_CR24","doi-asserted-by":"publisher","first-page":"1522","DOI":"10.1097\/00005768-199911000-00005","volume":"31","author":"D Gozal","year":"1999","unstructured":"Gozal D, Thiriet P (1999) Respiratory muscle training in neuromuscular disease: long-term effects on strength and load perception. Med Sci Sports Exerc 31(11):1522\u20131527","journal-title":"Med Sci Sports Exerc"},{"issue":"3","key":"9112_CR25","doi-asserted-by":"publisher","first-page":"249","DOI":"10.1016\/0960-8966(94)90026-4","volume":"4","author":"D Vilozni","year":"1994","unstructured":"Vilozni D et al (1994) Computerized respiratory muscle training in children with Duchenne muscular dystrophy. Neuromuscul Disord 4(3):249\u2013255","journal-title":"Neuromuscul Disord"},{"issue":"5","key":"9112_CR26","doi-asserted-by":"publisher","first-page":"736","DOI":"10.1136\/adc.64.5.736","volume":"64","author":"E Rodillo","year":"1989","unstructured":"Rodillo E et al (1989) Respiratory muscle training in Duchenne muscular dystrophy. Arch Dis Child 64(5):736\u2013738","journal-title":"Arch Dis Child"},{"issue":"6","key":"9112_CR27","doi-asserted-by":"publisher","first-page":"894","DOI":"10.2522\/ptj.20090413","volume":"91","author":"SJ Enright","year":"2011","unstructured":"Enright SJ, Unnithan VB (2011) Effect of inspiratory muscle training intensities on pulmonary function and work capacity in people who are healthy: a randomized controlled trial. Phys Ther 91(6):894\u2013905","journal-title":"Phys Ther"},{"issue":"3","key":"9112_CR28","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1093\/ptj\/86.3.345","volume":"86","author":"SJ Enright","year":"2006","unstructured":"Enright SJ et al (2006) Effect of high-intensity inspiratory muscle training on lung volumes, diaphragm thickness, and exercise capacity in subjects who are healthy. Phys Ther 86(3):345\u2013354","journal-title":"Phys Ther"},{"issue":"4","key":"9112_CR29","doi-asserted-by":"publisher","first-page":"508","DOI":"10.1152\/jappl.1976.41.4.508","volume":"41","author":"DE Leith","year":"1976","unstructured":"Leith DE, Bradley M (1976) Ventilatory muscle strength and endurance training. J Appl Physiol 41(4):508\u2013516","journal-title":"J Appl Physiol"},{"issue":"12","key":"9112_CR30","doi-asserted-by":"publisher","first-page":"1390","DOI":"10.1164\/rccm.200508-1211ST","volume":"173","author":"L Nici","year":"2006","unstructured":"Nici L et al (2006) American Thoracic Society\/European Respiratory Society statement on pulmonary rehabilitation. Am J Respir Crit Care Med 173(12):1390\u20131413","journal-title":"Am J Respir Crit Care Med"},{"issue":"6","key":"9112_CR31","doi-asserted-by":"publisher","first-page":"573","DOI":"10.1177\/0269215513512215","volume":"28","author":"GK Aslan","year":"2014","unstructured":"Aslan GK et al (2014) Effects of respiratory muscle training on pulmonary functions in patients with slowly progressive neuromuscular disease: a randomized controlled trial. Clin Rehabil 28(6):573\u2013581","journal-title":"Clin Rehabil"},{"issue":"3","key":"9112_CR32","doi-asserted-by":"crossref","first-page":"255","DOI":"10.3233\/PRM-140294","volume":"7","author":"HN Jones","year":"2014","unstructured":"Jones HN et al (2014) Effects of respiratory muscle training (RMT) in children with infantile-onset Pompe disease and respiratory muscle weakness. J Pediatr Rehabil Med 7(3):255\u2013265","journal-title":"J Pediatr Rehabil Med"},{"issue":"5","key":"9112_CR33","doi-asserted-by":"publisher","first-page":"377","DOI":"10.1249\/00005768-198205000-00012","volume":"14","author":"GA Borg","year":"1982","unstructured":"Borg GA (1982) Psychophysical bases of perceived exertion. Med Sci Sports Exerc 14(5):377\u2013381","journal-title":"Med Sci Sports Exerc"},{"issue":"5","key":"9112_CR34","doi-asserted-by":"publisher","first-page":"759","DOI":"10.1111\/j.1440-1843.2007.01117.x","volume":"12","author":"AN Aggarwal","year":"2007","unstructured":"Aggarwal AN, Agarwal R (2007) The new ATS\/ERS guidelines for assessing the spirometric severity of restrictive lung disease differ from previous standards. Respirology 12(5):759\u2013762","journal-title":"Respirology"},{"issue":"3","key":"9112_CR35","doi-asserted-by":"publisher","first-page":"147","DOI":"10.1055\/s-0034-1391345","volume":"69","author":"C Cri\u00e9e","year":"2015","unstructured":"Cri\u00e9e C et al (2015) Leitlinie zur Spirometrie Leitlinie der Deutschen Atemwegsliga, der Deutschen Gesellschaft f\u00fcr Pneumologie und Beatmungsmedizin und der Deutschen Gesellschaft f\u00fcr Arbeitsmedizin und Umweltmedizin zur Spirometrie. Pneumologie 69(3):147\u2013164","journal-title":"Pneumologie"},{"issue":"6","key":"9112_CR36","doi-asserted-by":"publisher","first-page":"503","DOI":"10.1016\/j.nmd.2017.03.009","volume":"27","author":"A Human","year":"2017","unstructured":"Human A et al (2017) Inspiratory muscle training for children and adolescents with neuromuscular diseases: a systematic review. Neuromuscul Disord 27(6):503\u2013517. https:\/\/doi.org\/10.1016\/j.nmd.2017.03.009","journal-title":"Neuromuscul Disord"},{"issue":"4","key":"9112_CR37","first-page":"240","volume":"102","author":"SF Hsiao","year":"2003","unstructured":"Hsiao SF et al (2003) Comparison of effectiveness of pressure threshold and targeted resistance devices for inspiratory muscle training in patients with chronic obstructive pulmonary disease. J Formos Med Assoc 102(4):240\u2013245","journal-title":"J Formos Med Assoc"},{"issue":"3","key":"9112_CR38","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1097\/01823246-200920030-00002","volume":"20","author":"MJ Shoemaker","year":"2009","unstructured":"Shoemaker MJ, Donker S, Lapoe A (2009) Inspiratory muscle training in patients with chronic obstructive pulmonary disease: the state of the evidence. Cardiopulm Phys Ther J 20(3):5\u201315","journal-title":"Cardiopulm Phys Ther J"},{"issue":"2","key":"9112_CR39","doi-asserted-by":"publisher","first-page":"120","DOI":"10.1016\/j.ymgme.2015.09.003","volume":"117","author":"HN Jones","year":"2016","unstructured":"Jones HN et al (2016) Respiratory muscle training (RMT) in late-onset Pompe disease (LOPD): effects of training and detraining. Mol Genet Metab 117(2):120\u2013128","journal-title":"Mol Genet Metab"},{"issue":"4","key":"9112_CR40","doi-asserted-by":"publisher","first-page":"555","DOI":"10.1007\/s00408-016-9881-4","volume":"194","author":"GK Aslan","year":"2016","unstructured":"Aslan GK et al (2016) Inspiratory muscle training in late-onset Pompe disease: the effects on pulmonary function tests, quality of life, and sleep quality. Lung 194(4):555\u2013561","journal-title":"Lung"},{"key":"9112_CR41","doi-asserted-by":"publisher","first-page":"67","DOI":"10.1016\/j.ymgmr.2015.09.007","volume":"5","author":"M Jevnikar","year":"2015","unstructured":"Jevnikar M et al (2015) Respiratory muscle training with enzyme replacement therapy improves muscle strength in late\u2014onset Pompe disease. Mol Genet Metab Rep 5:67\u201371","journal-title":"Mol Genet Metab Rep"},{"issue":"5","key":"9112_CR42","doi-asserted-by":"publisher","first-page":"837","DOI":"10.1007\/s10545-012-9451-8","volume":"35","author":"C Regnery","year":"2012","unstructured":"Regnery C et al (2012) 36 months observational clinical study of 38 adult Pompe disease patients under alglucosidase alfa enzyme replacement therapy. J Inherit Metab Dis 35(5):837\u2013845","journal-title":"J Inherit Metab Dis"}],"container-title":["Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s00415-018-9112-4\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-018-9112-4.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-018-9112-4.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,11,15]],"date-time":"2020-11-15T06:21:43Z","timestamp":1605421303000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s00415-018-9112-4"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,11,14]]},"references-count":42,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2019,1]]}},"alternative-id":["9112"],"URL":"https:\/\/doi.org\/10.1007\/s00415-018-9112-4","relation":{},"ISSN":["0340-5354","1432-1459"],"issn-type":[{"value":"0340-5354","type":"print"},{"value":"1432-1459","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,11,14]]},"assertion":[{"value":"4 September 2018","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"29 October 2018","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 November 2018","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"14 November 2018","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with ethical standards"}},{"value":"The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. On behalf of all authors, the corresponding author states that there is no conflict of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}},{"value":"All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and\/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The study was approved by the Ethics committee of the Ludwig-Maximilians-University Munich, Germany (vote no. 689\/16).","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical standards"}}]}}