{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T12:36:41Z","timestamp":1791549401744,"version":"4.3.4"},"reference-count":31,"publisher":"Springer Science and Business Media LLC","issue":"8","license":[{"start":{"date-parts":[[2020,3,11]],"date-time":"2020-03-11T00:00:00Z","timestamp":1583884800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2020,3,11]],"date-time":"2020-03-11T00:00:00Z","timestamp":1583884800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Neurol Sci"],"published-print":{"date-parts":[[2020,8]]},"DOI":"10.1007\/s10072-020-04316-6","type":"journal-article","created":{"date-parts":[[2020,3,11]],"date-time":"2020-03-11T06:02:40Z","timestamp":1583906560000},"page":"2175-2184","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":32,"title":["Ultrasound assessment of diaphragm function in patients with late-onset Pompe disease"],"prefix":"10.1007","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0379-9869","authenticated-orcid":false,"given":"Paolo","family":"Ruggeri","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucia","family":"Lo Monaco","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olimpia","family":"Musumeci","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Graziana","family":"Tavilla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michele","family":"Gaeta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gaetano","family":"Caramori","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Toscano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,3,11]]},"reference":[{"issue":"4","key":"4316_CR1","doi-asserted-by":"publisher","first-page":"968","DOI":"10.1007\/s00415-015-7664-0","volume":"262","author":"F Montagnese","year":"2015","unstructured":"Montagnese F, Barca E, Musumeci O, Mondello S, Migliorato A, Ciranni A, Rodolico C, De Filippi P, Danesino C, Toscano A (2015) Clinical and molecular aspects of 30 patients with late-onset Pompe disease (LOPD): unusual features and response to treatment. J Neurol 262(4):968\u2013978. https:\/\/doi.org\/10.1007\/s00415-015-7664-0","journal-title":"J Neurol"},{"issue":"13","key":"4316_CR2","doi-asserted-by":"publisher","first-page":"284","DOI":"10.21037\/atm.2019.07.24","volume":"7","author":"A Toscano","year":"2019","unstructured":"Toscano A, Rodolico C, Musumeci O (2019) Multisystem late onset Pompe disease (LOPD): an update on clinical aspects. Annals of translational medicine 7(13):284. https:\/\/doi.org\/10.21037\/atm.2019.07.24","journal-title":"Annals of translational medicine"},{"issue":"9646","key":"4316_CR3","doi-asserted-by":"publisher","first-page":"1342","DOI":"10.1016\/S0140-6736(08)61555-X","volume":"372","author":"AT van der Ploeg","year":"2008","unstructured":"van der Ploeg AT, Reuser AJ (2008) Pompe's disease. Lancet 372(9646):1342\u20131353. https:\/\/doi.org\/10.1016\/S0140-6736(08)61555-X","journal-title":"Lancet"},{"issue":"4","key":"4316_CR4","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. https:\/\/doi.org\/10.1007\/s00415-012-6636-x","journal-title":"J Neurol"},{"issue":"4","key":"4316_CR5","doi-asserted-by":"publisher","first-page":"621","DOI":"10.1007\/s00415-016-8219-8","volume":"264","author":"B Schoser","year":"2017","unstructured":"Schoser B, Stewart A, Kanters S, Hamed A, Jansen J, Chan K, Karamouzian M, Toscano A (2017) 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":"15","key":"4316_CR6","doi-asserted-by":"publisher","first-page":"1396","DOI":"10.1056\/NEJMoa0909859","volume":"362","author":"AT van der Ploeg","year":"2010","unstructured":"van der Ploeg AT, Clemens PR, Corzo D, Escolar DM, Florence J, Groeneveld GJ, Herson S, Kishnani PS, Laforet P, Lake SL, Lange DJ, Leshner RT, Mayhew JE, Morgan C, Nozaki K, Park DJ, Pestronk A, Rosenbloom B, Skrinar A, van Capelle CI, van der Beek NA, Wasserstein M, Zivkovic SA (2010) A randomized study of alglucosidase alfa in late-onset Pompe's disease. N Engl J Med 362(15):1396\u20131406. https:\/\/doi.org\/10.1056\/NEJMoa0909859","journal-title":"N Engl J Med"},{"issue":"5","key":"4316_CR7","doi-asserted-by":"publisher","first-page":"952","DOI":"10.1007\/s00415-011-6293-5","volume":"259","author":"C Angelini","year":"2012","unstructured":"Angelini C, Semplicini C, Ravaglia S, Bembi B, Servidei S, Pegoraro E, Moggio M, Filosto M, Sette E, Crescimanno G, Tonin P, Parini R, Morandi L, Marrosu G, Greco G, Musumeci O, Di Iorio G, Siciliano G, Donati MA, Carubbi F, Ermani M, Mongini T, Toscano A, Italian GG (2012) Observational clinical study in juvenile-adult glycogenosis type 2 patients undergoing enzyme replacement therapy for up to 4 years. J Neurol 259(5):952\u2013958. https:\/\/doi.org\/10.1007\/s00415-011-6293-5","journal-title":"J Neurol"},{"key":"4316_CR8","doi-asserted-by":"publisher","unstructured":"Ronzitti G, Collaud F, Laforet P, Mingozzi F (2019) Progress and challenges of gene therapy for Pompe disease. Ann Transl Med 7(13):287. https:\/\/doi.org\/10.21037\/atm.2019.04.67","DOI":"10.21037\/atm.2019.04.67"},{"issue":"3","key":"4316_CR9","doi-asserted-by":"publisher","first-page":"290","DOI":"10.1016\/j.ymgme.2013.06.023","volume":"110","author":"M Gaeta","year":"2013","unstructured":"Gaeta M, Barca E, Ruggeri P, Minutoli F, Rodolico C, Mazziotti S, Milardi D, Musumeci O, Toscano A (2013) Late-onset Pompe disease (LOPD): correlations between respiratory muscles CT and MRI features and pulmonary function. Mol Genet Metab 110(3):290\u2013296. https:\/\/doi.org\/10.1016\/j.ymgme.2013.06.023","journal-title":"Mol Genet Metab"},{"issue":"9","key":"4316_CR10","doi-asserted-by":"publisher","first-page":"497","DOI":"10.1093\/qjmed\/94.9.497","volume":"94","author":"N Mustfa","year":"2001","unstructured":"Mustfa N, Moxham J (2001) Respiratory muscle assessment in motor neurone disease. QJM 94(9):497\u2013502. https:\/\/doi.org\/10.1093\/qjmed\/94.9.497","journal-title":"QJM"},{"issue":"3","key":"4316_CR11","doi-asserted-by":"publisher","first-page":"319","DOI":"10.1002\/mus.23671","volume":"47","author":"A Sarwal","year":"2013","unstructured":"Sarwal A, Walker FO, Cartwright MS (2013) Neuromuscular ultrasound for evaluation of the diaphragm. Muscle Nerve 47(3):319\u2013329. https:\/\/doi.org\/10.1002\/mus.23671","journal-title":"Muscle Nerve"},{"issue":"1","key":"4316_CR12","doi-asserted-by":"publisher","first-page":"892","DOI":"10.1016\/j.clinph.2015.03.024","volume":"127","author":"S Pinto","year":"2016","unstructured":"Pinto S, Alves P, Pimentel B, Swash M, de Carvalho M (2016) Ultrasound for assessment of diaphragm in ALS. Clin Neurophysiol 127(1):892\u2013897. https:\/\/doi.org\/10.1016\/j.clinph.2015.03.024","journal-title":"Clin Neurophysiol"},{"issue":"5","key":"4316_CR13","doi-asserted-by":"publisher","first-page":"932","DOI":"10.1111\/resp.12759","volume":"21","author":"R Fantini","year":"2016","unstructured":"Fantini R, Mandrioli J, Zona S, Antenora F, Iattoni A, Monelli M, Fini N, Tonelli R, Clini E, Marchioni A (2016) Ultrasound assessment of diaphragmatic function in patients with amyotrophic lateral sclerosis. Respirology 21(5):932\u2013938. https:\/\/doi.org\/10.1111\/resp.12759","journal-title":"Respirology"},{"issue":"5","key":"4316_CR14","doi-asserted-by":"publisher","first-page":"472","DOI":"10.1136\/thx.52.5.472","volume":"52","author":"PF De Bruin","year":"1997","unstructured":"De Bruin PF, Ueki J, Bush A, Khan Y, Watson A, Pride NB (1997) Diaphragm thickness and inspiratory strength in patients with Duchenne muscular dystrophy. Thorax 52(5):472\u2013475. https:\/\/doi.org\/10.1136\/thx.52.5.472","journal-title":"Thorax"},{"issue":"6","key":"4316_CR15","doi-asserted-by":"publisher","first-page":"768","DOI":"10.1111\/ene.13285","volume":"24","author":"AT van der Ploeg","year":"2017","unstructured":"van der Ploeg AT, Kruijshaar ME, Toscano A, Laforet P, Angelini C, Lachmann RH, Pascual Pascual SI, Roberts M, Rosler K, Stulnig T, van Doorn PA, Van den Bergh PYK, Vissing J, Schoser B, European Pompe C (2017) European consensus for starting and stopping enzyme replacement therapy in adult patients with Pompe disease: a 10-year experience. Eur J Neurol 24(6):768\u2013e731. https:\/\/doi.org\/10.1111\/ene.13285","journal-title":"Eur J Neurol"},{"issue":"6","key":"4316_CR16","doi-asserted-by":"publisher","first-page":"831","DOI":"10.1002\/mus.23340","volume":"45","author":"C Angelini","year":"2012","unstructured":"Angelini C, Semplicini C, Ravaglia S, Moggio M, Comi GP, Musumeci O, Pegoraro E, Tonin P, Filosto M, Servidei S, Morandi L, Crescimanno G, Marrosu G, Siciliano G, Mongini T, Toscano A, Italian Group on G (2012) New motor outcome function measures in evaluation of late-onset Pompe disease before and after enzyme replacement therapy. Muscle Nerve 45(6):831\u2013834. https:\/\/doi.org\/10.1002\/mus.23340","journal-title":"Muscle Nerve"},{"issue":"1","key":"4316_CR17","doi-asserted-by":"publisher","first-page":"60","DOI":"10.1378\/chest.113.1.60","volume":"113","author":"MB Horowitz","year":"1998","unstructured":"Horowitz MB, Mahler DA (1998) Dyspnea ratings for prescription of cross-modal exercise in patients with COPD. Chest 113(1):60\u201364. https:\/\/doi.org\/10.1378\/chest.113.1.60","journal-title":"Chest"},{"issue":"2","key":"4316_CR18","doi-asserted-by":"publisher","first-page":"319","DOI":"10.1183\/09031936.05.00034805","volume":"26","author":"MR Miller","year":"2005","unstructured":"Miller MR, Hankinson J, Brusasco V, Burgos F, Casaburi R, Coates A, Crapo R, Enright P, van der Grinten CP, Gustafsson P, Jensen R, Johnson DC, MacIntyre N, McKay R, Navajas D, Pedersen OF, Pellegrino R, Viegi G, Wanger J, Force AET (2005) Standardisation of spirometry. Eur Respir J 26(2):319\u2013338. https:\/\/doi.org\/10.1183\/09031936.05.00034805","journal-title":"Eur Respir J"},{"issue":"6","key":"4316_CR19","doi-asserted-by":"publisher","first-page":"1324","DOI":"10.1183\/09031936.00080312","volume":"40","author":"PH Quanjer","year":"2012","unstructured":"Quanjer PH, Stanojevic S, Cole TJ, Baur X, Hall GL, Culver BH, Enright PL, Hankinson JL, Ip MS, Zheng J, Stocks J, Initiative ERSGLF (2012) Multi-ethnic reference values for spirometry for the 3-95-yr age range: the global lung function 2012 equations. Eur Respir J 40(6):1324\u20131343. https:\/\/doi.org\/10.1183\/09031936.00080312","journal-title":"Eur Respir J"},{"key":"4316_CR20","doi-asserted-by":"publisher","unstructured":"Laveneziana P, Albuquerque A, Aliverti A, Babb T, Barreiro E, Dres M, Dube BP, Fauroux B, Gea J, Guenette JA, Hudson AL, Kabitz HJ, Laghi F, Langer D, Luo YM, Neder JA, O'Donnell D, Polkey MI, Rabinovich RA, Rossi A, Series F, Similowski T, Spengler CM, Vogiatzis I, Verges S (2019) ERS statement on respiratory muscle testing at rest and during exercise. Eur Respir J 53(6). https:\/\/doi.org\/10.1183\/13993003.01214-2018","DOI":"10.1183\/13993003.01214-2018"},{"issue":"1","key":"4316_CR21","doi-asserted-by":"publisher","first-page":"123","DOI":"10.1053\/apmr.2001.18053","volume":"82","author":"C Fromageot","year":"2001","unstructured":"Fromageot C, Lofaso F, Annane D, Falaize L, Lejaille M, Clair B, Gajdos P, Raphael JC (2001) Supine fall in lung volumes in the assessment of diaphragmatic weakness in neuromuscular disorders. Arch Phys Med Rehabil 82(1):123\u2013128. https:\/\/doi.org\/10.1053\/apmr.2001.18053","journal-title":"Arch Phys Med Rehabil"},{"issue":"7","key":"4316_CR22","doi-asserted-by":"publisher","first-page":"535","DOI":"10.1136\/thx.39.7.535","volume":"39","author":"SH Wilson","year":"1984","unstructured":"Wilson SH, Cooke NT, Edwards RH, Spiro SG (1984) Predicted normal values for maximal respiratory pressures in caucasian adults and children. Thorax 39(7):535\u2013538. https:\/\/doi.org\/10.1136\/thx.39.7.535","journal-title":"Thorax"},{"issue":"6","key":"4316_CR23","doi-asserted-by":"publisher","first-page":"884","DOI":"10.1002\/mus.23702","volume":"47","author":"AJ Boon","year":"2013","unstructured":"Boon AJ, Harper CJ, Ghahfarokhi LS, Strommen JA, Watson JC, Sorenson EJ (2013) Two-dimensional ultrasound imaging of the diaphragm: quantitative values in normal subjects. Muscle Nerve 47(6):884\u2013889. https:\/\/doi.org\/10.1002\/mus.23702","journal-title":"Muscle Nerve"},{"issue":"1","key":"4316_CR24","doi-asserted-by":"publisher","first-page":"44","DOI":"10.1016\/j.ultrasmedbio.2010.10.004","volume":"37","author":"A Testa","year":"2011","unstructured":"Testa A, Soldati G, Giannuzzi R, Berardi S, Portale G, Gentiloni Silveri N (2011) Ultrasound M-mode assessment of diaphragmatic kinetics by anterior transverse scanning in healthy subjects. Ultrasound Med Biol 37(1):44\u201352. https:\/\/doi.org\/10.1016\/j.ultrasmedbio.2010.10.004","journal-title":"Ultrasound Med Biol"},{"issue":"1","key":"4316_CR25","doi-asserted-by":"publisher","first-page":"1","DOI":"10.3233\/JND-170276","volume":"5","author":"A Fayssoil","year":"2018","unstructured":"Fayssoil A, Behin A, Ogna A, Mompoint D, Amthor H, Clair B, Laforet P, Mansart A, Prigent H, Orlikowski D, Stojkovic T, Vinit S, Carlier R, Eymard B, Lofaso F, Annane D (2018) Diaphragm: pathophysiology and ultrasound imaging in neuromuscular disorders. Journal of neuromuscular diseases 5(1):1\u201310. https:\/\/doi.org\/10.3233\/JND-170276","journal-title":"Journal of neuromuscular diseases"},{"issue":"4","key":"4316_CR26","doi-asserted-by":"publisher","first-page":"522","DOI":"10.1002\/mus.24902","volume":"53","author":"Y Noda","year":"2016","unstructured":"Noda Y, Sekiguchi K, Kohara N, Kanda F, Toda T (2016) Ultrasonographic diaphragm thickness correlates with compound muscle action potential amplitude and forced vital capacity. Muscle Nerve 53(4):522\u2013527. https:\/\/doi.org\/10.1002\/mus.24902","journal-title":"Muscle Nerve"},{"issue":"6","key":"4316_CR27","doi-asserted-by":"publisher","first-page":"679","DOI":"10.1002\/mus.26717","volume":"60","author":"C Henke","year":"2019","unstructured":"Henke C, Spiesshoefer J, Kabitz HJ, Herkenrath S, Randerath W, Brix T, Gorlich D, Young P, Boentert M (2019) Respiratory muscle weakness in facioscapulohumeral muscular dystrophy. Muscle Nerve 60(6):679\u2013686. https:\/\/doi.org\/10.1002\/mus.26717","journal-title":"Muscle Nerve"},{"issue":"11","key":"4316_CR28","doi-asserted-by":"publisher","first-page":"852","DOI":"10.1016\/j.nmd.2015.09.003","volume":"25","author":"M Gaeta","year":"2015","unstructured":"Gaeta M, Musumeci O, Mondello S, Ruggeri P, Montagnese F, Cucinotta M, Vinci S, Milardi D, Toscano A (2015) Clinical and pathophysiological clues of respiratory dysfunction in late-onset Pompe disease: new insights from a comparative study by MRI and respiratory function assessment. Neuromuscular disorders : NMD 25(11):852\u2013858. https:\/\/doi.org\/10.1016\/j.nmd.2015.09.003","journal-title":"Neuromuscular disorders : NMD"},{"key":"4316_CR29","doi-asserted-by":"publisher","unstructured":"Harlaar L, Ciet P, van der Ploeg AT, Brusse E, van der Beek N, Wielopolski PA, de Bruijne M, Tiddens H, van Doorn PA (2018) Imaging of respiratory muscles in neuromuscular disease: a review.\u00a0Neuromuscul Disord 28(3):246\u2013256. https:\/\/doi.org\/10.1016\/j.nmd.2017.11.010","DOI":"10.1016\/j.nmd.2017.11.010"},{"issue":"8","key":"4316_CR30","doi-asserted-by":"publisher","first-page":"618","DOI":"10.1016\/j.nmd.2019.06.011","volume":"29","author":"J Spiesshoefer","year":"2019","unstructured":"Spiesshoefer J, Henke C, Kabitz HJ, Brix T, Gorlich D, Herkenrath S, Randerath W, Young P, Boentert M (2019) The nature of respiratory muscle weakness in patients with late-onset Pompe disease. Neuromuscular disorders : NMD 29(8):618\u2013627. https:\/\/doi.org\/10.1016\/j.nmd.2019.06.011","journal-title":"Neuromuscular disorders : NMD"},{"issue":"5","key":"4316_CR31","doi-asserted-by":"publisher","first-page":"796","DOI":"10.1007\/s00134-012-2547-7","volume":"38","author":"E Vivier","year":"2012","unstructured":"Vivier E, Mekontso Dessap A, Dimassi S, Vargas F, Lyazidi A, Thille AW, Brochard L (2012) Diaphragm ultrasonography to estimate the work of breathing during non-invasive ventilation. Intensive Care Med 38(5):796\u2013803. https:\/\/doi.org\/10.1007\/s00134-012-2547-7","journal-title":"Intensive Care Med"}],"container-title":["Neurological Sciences"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10072-020-04316-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s10072-020-04316-6\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10072-020-04316-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,3,11]],"date-time":"2021-03-11T00:14:15Z","timestamp":1615421655000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s10072-020-04316-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,3,11]]},"references-count":31,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2020,8]]}},"alternative-id":["4316"],"URL":"https:\/\/doi.org\/10.1007\/s10072-020-04316-6","relation":{},"ISSN":["1590-1874","1590-3478"],"issn-type":[{"value":"1590-1874","type":"print"},{"value":"1590-3478","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,3,11]]},"assertion":[{"value":"7 January 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 February 2020","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 March 2020","order":3,"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 they have no conflict of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"Approved by the local Research Ethics Committee.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}}]}}