{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T14:03:15Z","timestamp":1788357795176,"version":"build-2803163510"},"reference-count":49,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2023,11,2]],"date-time":"2023-11-02T00:00:00Z","timestamp":1698883200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2023,11,2]],"date-time":"2023-11-02T00:00:00Z","timestamp":1698883200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100022302","name":"Societat Catalana de Pneumologia","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100022302","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100007509","name":"Sociedad Espa\u00f1ola de Neumolog\u00eda y Cirug\u00eda Tor\u00e1cica","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007509","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Raras","award":["ACCI ER21P1AC705"],"award-info":[{"award-number":["ACCI ER21P1AC705"]}]},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Raras","award":["ACCI ER21P1AC705"],"award-info":[{"award-number":["ACCI ER21P1AC705"]}]},{"DOI":"10.13039\/501100023561","name":"Ministerio de Universidades","doi-asserted-by":"publisher","award":["FPU20\/06692"],"award-info":[{"award-number":["FPU20\/06692"]}],"id":[{"id":"10.13039\/501100023561","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100011104","name":"Universitat Aut\u00f2noma de Barcelona","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100011104","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurol"],"published-print":{"date-parts":[[2024,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) is an hereditary autosomal recessive disease. Recent studies propose including chronic cough (CC) as a symptom of CANVAS. For 10 patients with CANVAS as genetically confirmed by biallelic expansion of the AAGG repeat motif (AAGGGexp) in intron 2 of replication factor C subunit 1 (<jats:italic>RFC1<\/jats:italic>), our aim was, as a multidisciplinary team, to describe clinical and functional characteristics and possible causes of CC following European Respiratory Society (ERS) recommendations, and to evaluate CC impact on quality of life (QoL) using self-administered questionnaires (Cough Severity Diary, Leicester Cough Questionnaire, Discrete Emotions Questionnaire, and EQ-5D-5L). In all 10 patients, the CC was a dry cough that developed several years prior to the neurological symptoms (mean 14.2\u00a0years); 7 patients had symptoms compatible with gastroesophageal reflux (GER), 5 with pathological GER diagnosed by 24-h esophageal pH testing, and 6 patients had impaired esophageal motility diagnosed by high-resolution esophageal manometry, most frequently ineffective peristalsis. Although further studies are required for confirmation, we conclude that CC may be a characteristic prodrome of CANVAS and may be related to GER and esophageal disorders. Furthermore, CC affects patients\u2019 QoL, especially in the psychosocial sphere.<\/jats:p>","DOI":"10.1007\/s00415-023-12001-9","type":"journal-article","created":{"date-parts":[[2023,11,2]],"date-time":"2023-11-02T15:02:41Z","timestamp":1698937361000},"page":"1204-1212","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Clinical and functional characteristics, possible causes, and impact of chronic cough in patients with cerebellar ataxia, neuropathy, and bilateral vestibular areflexia syndrome (CANVAS)"],"prefix":"10.1007","volume":"271","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5034-3296","authenticated-orcid":false,"given":"Esther","family":"Palones","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Curto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vicente","family":"Plaza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lidia","family":"Gonzalez-Quereda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alba","family":"Segarra-Casas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luis","family":"Querol","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federico","family":"Bertoletti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mar\u00eda Jos\u00e9","family":"Rodriguez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P\u00eda","family":"Gallano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Astrid","family":"Crespo-Lessmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,11,2]]},"reference":[{"issue":"4","key":"12001_CR1","doi-asserted-by":"publisher","first-page":"649","DOI":"10.1038\/s41588-019-0372-4","volume":"51","author":"A Cortese","year":"2019","unstructured":"Cortese A, Simone R, Sullivan R, Vandrovcova J, Tariq H, Yau WY, Humphrey J, Jaunmuktane Z, Sivakumar P, Polke J, Ilyas M, Tribollet E, Tomaselli PJ, Devigili G, Callegari I, Versino M, Salpietro V, Efthymiou S, Kaski D, Wood NW, Andrade NS, Buglo E, Rebelo A, Rossor AM, Bronstein A, Fratta P, Marques WJ, Z\u00fcchner S, Reilly MM, Houlden H (2019) Biallelic expansion of an intronic repeat in RFC1 is a common cause of late-onset ataxia. Nat Genet 51(4):649\u2013658","journal-title":"Nat Genet"},{"issue":"11","key":"12001_CR2","doi-asserted-by":"publisher","first-page":"3770","DOI":"10.1093\/brain\/awac280","volume":"145","author":"M Benkirane","year":"2022","unstructured":"Benkirane M, Da Cunha D, Marelli C, Larrieu L, Renaud M, Varilh J, Pointaux M, Baux D, Ardouin O, Vangoethem C, Taulan M, Daumas Duport B, Bergougnoux A, Corbill\u00e9 AG, Coss\u00e9e M, Juntas Morales R, Tuffery-Giraud S, Koenig M, Isidor B, Vincent MC (2022) RFC1 nonsense and frameshift variants cause CANVAS: clues for an unsolved pathophysiology. Brain 145(11):3770\u20133775","journal-title":"Brain"},{"issue":"5","key":"12001_CR3","doi-asserted-by":"publisher","first-page":"e543","DOI":"10.1212\/WNL.0000000000201486","volume":"100","author":"R Ronco","year":"2023","unstructured":"Ronco R, Perini C, Curr\u00f2 R, Dominik N, Facchini S, Gennari A, Simone R, Stuart S, Nagy S, Vegezzi E, Quartesan I, El-Saddig A, Lavin T, Tucci A, Szymura A, Novis De Farias LE, Gary A, Delfeld M, Kandikatla P, Niu N, Tawde S, Shaw J, Polke J, Reilly MM, Wood NW, Crespan E, Gomez C, Chen JYH, Schmahmann JD, Gosal D, Houlden H, Das S, Cortese A (2023) Truncating variants in RFC1 in cerebellar ataxia, neuropathy, and vestibular areflexia syndrome. Neurology 100(5):e543\u2013e554","journal-title":"Neurology"},{"issue":"2","key":"12001_CR4","doi-asserted-by":"publisher","first-page":"236","DOI":"10.1111\/cge.14249","volume":"103","author":"A Arteche-L\u00f3pez","year":"2023","unstructured":"Arteche-L\u00f3pez A, Avila-Fernandez A, Damian A, Soengas-Gonda E, de la Fuente RP, G\u00f3mez PR, Merlo JG, Burgos LH, Fern\u00e1ndez CC, Rosales JML, Mart\u00ednez JFG, Quesada-Espinosa JF, Corton M, Guerrero-Molina MP (2023) New cerebellar ataxia, neuropathy, vestibular areflexia syndrome cases are caused by the presence of a nonsense variant in compound heterozygosity with the pathogenic repeat expansion in the RFC1 gene. Clin Genet 103(2):236\u2013241","journal-title":"Clin Genet"},{"issue":"22","key":"12001_CR5","doi-asserted-by":"publisher","first-page":"1903","DOI":"10.1212\/WNL.0b013e31821d746e","volume":"76","author":"DJ Szmulewicz","year":"2011","unstructured":"Szmulewicz DJ, Waterston JA, Halmagyi GM, Mossman S, Chancellor AM, McLean CA, Storey E (2011) Sensory neuropathy as part of the cerebellar ataxia neuropathy vestibular areflexia syndrome. Neurology 76(22):1903\u20131910","journal-title":"Neurology"},{"issue":"1","key":"12001_CR6","doi-asserted-by":"publisher","first-page":"61","DOI":"10.1212\/CPJ.0000000000000215","volume":"6","author":"DJ Szmulewicz","year":"2016","unstructured":"Szmulewicz DJ, Roberts L, McLean CA, MacDougall HG, Halmagyi GM, Storey E (2016) Proposed diagnostic criteria for cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS). Neurol Clin Pract 6(1):61\u201368","journal-title":"Neurol Clin Pract"},{"issue":"1","key":"12001_CR7","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1136\/practneurol-2020-002822","volume":"22","author":"A Cortese","year":"2022","unstructured":"Cortese A, Curro\u2019 R, Vegezzi E, Yau WY, Houlden H, Reilly MM (2022) Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS): genetic and clinical aspects. Pract Neurol 22(1):14\u201318","journal-title":"Pract Neurol"},{"issue":"6","key":"12001_CR8","doi-asserted-by":"publisher","first-page":"2121","DOI":"10.1093\/brain\/awab449","volume":"145","author":"V Huin","year":"2022","unstructured":"Huin V, Coarelli G, Guemy C, Boluda S, Debs R, Mochel F, Stojkovic T, Grabli D, Maisonobe T, Gaymard B, Lenglet T, Tard C, Davion JB, Sablonni\u00e8re B, Monin ML, Ewenczyk C, Viala K, Charles P, Le Ber I, Reilly MM, Houlden H, Cortese A, Seilhean D, Brice A, Durr A (2022) Motor neuron pathology in CANVAS due to RFC1 expansions. Brain 145(6):2121\u20132132","journal-title":"Brain"},{"issue":"2","key":"12001_CR9","doi-asserted-by":"publisher","first-page":"480","DOI":"10.1093\/brain\/awz418","volume":"143","author":"A Cortese","year":"2020","unstructured":"Cortese A, Tozza S, Yau WY, Rossi S, Beecroft SJ, Jaunmuktane Z, Dyer Z, Ravenscroft G, Lamont PJ, Mossman S, Chancellor A, Maisonobe T, Pereon Y, Cauquil C, Colnaghi S, Mallucci G, Curro R, Tomaselli PJ, Thomas-Black G, Sullivan R, Efthymiou S, Rossor AM, Laur\u00e1 M, Pipis M, Horga A, Polke J, Kaski D, Horvath R, Chinnery PF, Marques W, Tassorelli C, Devigili G, Leonardis L, Wood NW, Bronstein A, Giunti P, Z\u00fcchner S, Stojkovic T, Laing N, Roxburgh RH, Houlden H, Reilly MM (2020) Cerebellar ataxia, neuropathy, vestibular areflexia syndrome due to RFC1 repeat expansion. Brain 143(2):480\u2013490","journal-title":"Brain"},{"issue":"5","key":"12001_CR10","doi-asserted-by":"publisher","first-page":"687","DOI":"10.1007\/s12311-020-01192-w","volume":"20","author":"M Dupr\u00e9","year":"2021","unstructured":"Dupr\u00e9 M, Hermann R, Froment Tilikete C (2021) Update on cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS). Cerebellum 20(5):687\u2013700","journal-title":"Cerebellum"},{"issue":"5","key":"12001_CR11","doi-asserted-by":"publisher","first-page":"1542","DOI":"10.1093\/brain\/awab072","volume":"144","author":"R Curr\u00f2","year":"2021","unstructured":"Curr\u00f2 R, Salvalaggio A, Tozza S, Gemelli C, Dominik N, Galassi Deforie V, Magrinelli F, Castellani F, Vegezzi E, Businaro P, Callegari I, Pichiecchio A, Cosentino G, Alfonsi E, Marchioni E, Colnaghi S, Gana S, Valente EM, Tassorelli C, Efthymiou S, Facchini S, Carr A, Laura M, Rossor AM, Manji H, Lunn MP, Pegoraro E, Santoro L, Grandis M, Bellone E, Beauchamp NJ, Hadjivassiliou M, Kaski D, Bronstein AM, Houlden H, Reilly MM, Mandich P, Schenone A, Manganelli F, Briani C, Cortese A (2021) RFC1 expansions are a common cause of idiopathic sensory neuropathy. Brain 144(5):1542\u20131550","journal-title":"Brain"},{"issue":"8","key":"12001_CR12","doi-asserted-by":"publisher","first-page":"2046","DOI":"10.3390\/biomedicines10082046","volume":"10","author":"L Magy","year":"2022","unstructured":"Magy L, Chazelas P, Richard L, Deschamps N, Frachet S, Vallat JM, Magdelaine C, Favreau F, Bessaguet F, Lia AS, Duchesne M (2022) Early diagnosis in cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) by focusing on major clinical clues: beyond ataxia and vestibular impairment. Biomedicines 10(8):2046","journal-title":"Biomedicines"},{"issue":"6","key":"12001_CR13","doi-asserted-by":"publisher","first-page":"1454","DOI":"10.1007\/s00415-018-8872-1","volume":"265","author":"J Infante","year":"2018","unstructured":"Infante J, Garc\u00eda A, Serrano-C\u00e1rdenas KM, Gonz\u00e1lez-Aguado R, Gazulla J, de Lucas EM, Berciano J (2018) Cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) with chronic cough and preserved muscle stretch reflexes: evidence for selective sparing of afferent Ia fibres. J Neurol 265(6):1454\u20131462","journal-title":"J Neurol"},{"key":"12001_CR14","unstructured":"Cortese A, Reilly MM, Houlden H (2020)\u00a0RFC1\u00a0CANVAS\/spectrum disorder. In: Adam MP, Everman DB, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, Gripp KW, Amemiya A, editors. GeneReviews\u00ae [Internet]. University of Washington, Seattle; 1993\u20132023"},{"issue":"4","key":"12001_CR15","doi-asserted-by":"publisher","first-page":"939","DOI":"10.1007\/s13760-021-01721-2","volume":"122","author":"A \u00c7akar","year":"2022","unstructured":"\u00c7akar A, \u015eahin E, Tezel S, Candayan A, Samanc\u0131 B, Battalo\u011flu E, Ba\u015fak AN, Bilgi\u00e7 B, Hana\u011fas\u0131 H, Durmu\u015f H, Parman Y (2022) Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (canvas): an important cause of late-onset ataxia with unique clinical features. Acta Neurol Belg 122(4):939\u2013945","journal-title":"Acta Neurol Belg"},{"issue":"4","key":"12001_CR16","doi-asserted-by":"publisher","first-page":"583","DOI":"10.1136\/jnnp.65.4.583","volume":"65","author":"J Kimber","year":"1998","unstructured":"Kimber J, Mitchell D, Mathias CJ (1998) Chronic cough in the Holmes-Adie syndrome: association in five cases with autonomic dysfunction. J Neurol Neurosurg Psychiatry 65(4):583\u2013586","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"Pt 12","key":"12001_CR17","doi-asserted-by":"publisher","first-page":"2797","DOI":"10.1093\/brain\/awh653","volume":"128","author":"PJ Spring","year":"2005","unstructured":"Spring PJ, Kok C, Nicholson GA, Ing AJ, Spies JM, Bassett ML, Cameron J, Kerlin P, Bowler S, Tuck R, Pollard JD (2005) Autosomal dominant hereditary sensory neuropathy with chronic cough and gastro-oesophageal reflux: clinical features in two families linked to chromosome 3p22-p24. Brain 128(Pt 12):2797\u20132810","journal-title":"Brain"},{"issue":"5\u20136","key":"12001_CR18","doi-asserted-by":"publisher","first-page":"465","DOI":"10.3233\/VES-140536","volume":"24","author":"DJ Szmulewicz","year":"2014","unstructured":"Szmulewicz DJ, McLean CA, MacDougall HG, Roberts L, Storey E, Halmagyi GM (2014) CANVAS an update: clinical presentation, investigation and management. J Vestib Res 24(5\u20136):465\u2013474","journal-title":"J Vestib Res"},{"issue":"11","key":"12001_CR19","doi-asserted-by":"publisher","first-page":"579","DOI":"10.1016\/j.arbres.2015.03.019","volume":"51","author":"A Pacheco","year":"2015","unstructured":"Pacheco A, de Diego A, Domingo C, Lamas A, Gutierrez R, Naberan K, Garrigues V, L\u00f3pez Vime R (2015) Chronic cough. Arch Bronconeumol 51(11):579\u2013589","journal-title":"Arch Bronconeumol"},{"issue":"3","key":"12001_CR20","doi-asserted-by":"publisher","first-page":"975","DOI":"10.1152\/physrev.00039.2015","volume":"96","author":"SB Mazzone","year":"2016","unstructured":"Mazzone SB, Undem BJ (2016) Vagal afferent innervation of the airways in health and disease. Physiol Rev 96(3):975\u20131024","journal-title":"Physiol Rev"},{"issue":"Pt 2","key":"12001_CR21","doi-asserted-by":"publisher","first-page":"543","DOI":"10.1113\/jphysiol.2003.057885","volume":"557","author":"BJ Canning","year":"2004","unstructured":"Canning BJ, Mazzone SB, Meeker SN, Mori N, Reynolds SM, Undem BJ (2004) Identification of the tracheal and laryngeal afferent neurones mediating cough in anaesthetized guinea-pigs. J Physiol 557(Pt 2):543\u2013558","journal-title":"J Physiol"},{"issue":"1","key":"12001_CR22","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1038\/s41572-022-00370-w","volume":"8","author":"KF Chung","year":"2022","unstructured":"Chung KF, McGarvey L, Song WJ, Chang AB, Lai K, Canning BJ, Birring SS, Smith JA, Mazzone SB (2022) Cough hypersensitivity and chronic cough. Nat Rev Dis Prim 8(1):45","journal-title":"Nat Rev Dis Prim"},{"issue":"1","key":"12001_CR23","doi-asserted-by":"publisher","first-page":"1901136","DOI":"10.1183\/13993003.01136-2019","volume":"55","author":"AH Morice","year":"2020","unstructured":"Morice AH, Millqvist E, Bieksiene K, Birring SS, Dicpinigaitis P, Domingo Ribas C, Hilton Boon M, Kantar A, Lai K, McGarvey L, Rigau D, Satia I, Smith J, Song WJ, Tonia T, van den Berg JWK, van Manen MJG, Zacharasiewicz A (2020) ERS guidelines on the diagnosis and treatment of chronic cough in adults and children. Eur Respir J 55(1):1901136","journal-title":"Eur Respir J"},{"issue":"Pt 1","key":"12001_CR24","doi-asserted-by":"publisher","first-page":"239","DOI":"10.1111\/j.1469-7793.1999.00239.x","volume":"521","author":"MA McAlexander","year":"1999","unstructured":"McAlexander MA, Myers AC, Undem BJ (1999) Adaptation of guinea-pig vagal airway afferent neurones to mechanical stimulation. J Physiol 521(Pt 1):239\u2013247","journal-title":"J Physiol"},{"key":"12001_CR25","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2023.120565","volume":"446","author":"D S\u00e1nchez-Tejerina","year":"2023","unstructured":"S\u00e1nchez-Tejerina D, Alvarez PF, La\u00ednez E, Martinez VG, Santa-Cruz DI, Verdaguer L, Gratac\u00f2s M, Seoane JL, Raguer N, Hern\u00e1ndez-Vara J, Llaurad\u00f3 A, Sotoca J, Salvado M, Arumi EG, Tizzano EF, Juntas R (2023) RFC1 repeat expansions and cerebellar ataxia, neuropathy and vestibular areflexia syndrome: experience and perspectives from a neuromuscular disorders unit. J Neurol Sci 446:120565","journal-title":"J Neurol Sci"},{"issue":"4","key":"12001_CR26","doi-asserted-by":"publisher","first-page":"911","DOI":"10.1016\/j.chest.2022.11.014","volume":"163","author":"L Guilleminault","year":"2023","unstructured":"Guilleminault L, Chazelas P, Melloni B, Magdelaine C, Villeneuve T, Brouqui\u00e8res D, Lia AS, Magy L (2023) Repeat expansions of RFC1 in refractory chronic cough: a missing piece of the puzzle? Chest 163(4):911\u2013915","journal-title":"Chest"},{"issue":"4","key":"12001_CR27","doi-asserted-by":"publisher","first-page":"199","DOI":"10.1177\/1753465810372526","volume":"4","author":"M Vernon","year":"2010","unstructured":"Vernon M, Kline Leidy N, Nacson A, Nelsen L (2010) Measuring cough severity: development and pilot testing of a new seven-item cough severity patient-reported outcome measure. Ther Adv Respir Dis 4(4):199\u2013208","journal-title":"Ther Adv Respir Dis"},{"issue":"4","key":"12001_CR28","doi-asserted-by":"publisher","first-page":"339","DOI":"10.1136\/thorax.58.4.339","volume":"58","author":"SS Birring","year":"2003","unstructured":"Birring SS, Prudon B, Carr AJ, Singh SJ, Morgan MD, Pavord ID (2003) Development of a symptom specific health status measure for patients with chronic cough: Leicester Cough Questionnaire (LCQ). Thorax 58(4):339\u2013343","journal-title":"Thorax"},{"issue":"2","key":"12001_CR29","doi-asserted-by":"publisher","first-page":"128","DOI":"10.1177\/1479972316632005","volume":"13","author":"G Mu\u00f1oz","year":"2016","unstructured":"Mu\u00f1oz G, Bux\u00f3 M, de Gracia J, Olveira C, Martinez-Garcia MA, Giron R, Polverino E, Alvarez A, Birring SS, Vendrell M (2016) Validation of a Spanish version of the Leicester Cough Questionnaire in non-cystic fibrosis bronchiectasis. Chron Respir Dis 13(2):128\u2013136","journal-title":"Chron Respir Dis"},{"issue":"8","key":"12001_CR30","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0159915","volume":"11","author":"C Harmon-Jones","year":"2016","unstructured":"Harmon-Jones C, Bastian B, Harmon-Jones E (2016) The discrete emotions questionnaire: a new tool for measuring state self-reported emotions. PLoS ONE 11(8):e0159915","journal-title":"PLoS ONE"},{"issue":"10","key":"12001_CR31","doi-asserted-by":"publisher","first-page":"1727","DOI":"10.1007\/s11136-011-9903-x","volume":"20","author":"M Herdman","year":"2011","unstructured":"Herdman M, Gudex C, Lloyd A, Janssen M, Kind P, Parkin D, Bonsel G, Badia X (2011) Development and preliminary testing of the new five-level version of EQ-5D (EQ-5D-5L). Qual Life Res 20(10):1727\u20131736","journal-title":"Qual Life Res"},{"key":"#cr-split#-12001_CR32.1","unstructured":"Ramos-Go\u00f1i JM, Pinto Prades JL, Oppe M, Cabas\u00e9s Hita JM, Rivero-Arias O (2010) Estimaci\u00f3n del conjunto de valores para los estados de salud del EQ-5D-5L basados en las preferencias de la poblaci\u00f3n espa\u00f1ola. Plan de Calidad para el Sistema Nacional de Salud del Ministerio de Sanidad, Pol\u00edtica Social e Igualdad. Servicio de Evaluaci\u00f3n del Servicio Canario de la Salud"},{"key":"#cr-split#-12001_CR32.2","unstructured":"2014. Informes de Evaluaci\u00f3n de Tecnolog\u00edas Sanitarias: SESCS N\u00ba 2010\/05"},{"key":"12001_CR33","unstructured":"EuroQol Research Foundation (2019) EQ-5D-5L UserGuide. https:\/\/euroqol.org\/publications\/user-guides. Accessed 11 Aug 2022"},{"key":"12001_CR34","doi-asserted-by":"publisher","first-page":"153","DOI":"10.1183\/09031936.05.00034505","volume":"1","author":"MR Miller","year":"2005","unstructured":"Miller MR, Crapo R, Hankinson J, Brusasco V, Burgos F, Casaburi R, Coates A, 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 (2005) ATS\/ERS Task Force. General considerations for lung function testing. Eur Respir J 1:153\u2013161","journal-title":"Eur Respir J"},{"key":"12001_CR35","doi-asserted-by":"publisher","first-page":"912","DOI":"10.1164\/rccm.200406-710ST","volume":"171","author":"American Thoracic Society, European Respiratory Society","year":"2005","unstructured":"American Thoracic Society, European Respiratory Society (2005) ATS\/ERS recommendations for standardized procedures for the online and offline measurement of exhaled lower respiratory nitric oxide and nasal nitric oxide, 2005. Am J Respir Crit Care Med 171:912\u2013930","journal-title":"Am J Respir Crit Care Med"},{"issue":"3","key":"12001_CR36","doi-asserted-by":"publisher","first-page":"176","DOI":"10.1016\/S1579-2129(07)60044-5","volume":"43","author":"AM Fortuna","year":"2007","unstructured":"Fortuna AM, Feixas T, Casan P (2007) Measurement of fraction of exhaled nitric oxide with the portable NIOX-MINO monitor in healthy adults. Arch Bronconeumol 43(3):176\u2013179","journal-title":"Arch Bronconeumol"},{"key":"12001_CR37","doi-asserted-by":"crossref","unstructured":"(1988) Report on skin test standardization. The Committee on Skin Test Standardization of The Netherlands Society of Allergology. Clin Allergy 18(3):305\u2013310","DOI":"10.1111\/j.1365-2222.1988.tb02873.x"},{"issue":"7","key":"12001_CR38","doi-asserted-by":"publisher","first-page":"1351","DOI":"10.1136\/gutjnl-2017-314722","volume":"67","author":"CP Gyawali","year":"2018","unstructured":"Gyawali CP, Kahrilas PJ, Savarino E, Zerbib F, Mion F, Smout AJPM, Vaezi M, Sifrim D, Fox MR, Vela MF, Tutuian R, Tack J, Bredenoord AJ, Pandolfino J, Roman S (2018) Modern diagnosis of GERD: the Lyon Consensus. Gut 67(7):1351\u20131362","journal-title":"Gut"},{"key":"12001_CR39","doi-asserted-by":"crossref","unstructured":"Yadlapati R, Kahrilas PJ, Fox MR, Bredenoord AJ, Prakash Gyawali C, Roman S, Babaei A, Mittal RK, Rommel N, Savarino E, Sifrim D, Smout A, Vaezi MF, Zerbib F, Akiyama J, Bhatia S, Bor S, Carlson DA, Chen JW, Cisternas D, Cock C, Coss-Adame E, de Bortoli N, Defilippi C, Fass R, Ghoshal UC, Gonlachanvit S, Hani A, Hebbard GS, Wook Jung K, Katz P, Katzka DA, Khan A, Kohn GP, Lazarescu A, Lengliner J, Mittal SK, Omari T, Park MI, Penagini R, Pohl D, Richter JE, Serra J, Sweis R, Tack J, Tatum RP, Tutuian R, Vela MF, Wong RK, Wu JC, Xiao Y, Pandolfino JE (2021) Esophageal motility disorders on high-resolution manometry: Chicago classification version 4.0\u00a9. Neurogastroenterol Motil 33(1):e14058.","DOI":"10.1111\/nmo.14058"},{"issue":"3","key":"12001_CR40","doi-asserted-by":"publisher","first-page":"1119","DOI":"10.1007\/s00415-020-10183-0","volume":"268","author":"N Dominik","year":"2021","unstructured":"Dominik N, Galassi Deforie V, Cortese A, Houlden H (2021) CANVAS: a late onset ataxia due to biallelic intronic AAGGG expansions. J Neurol 268(3):1119\u20131126","journal-title":"J Neurol"},{"issue":"15","key":"12001_CR41","doi-asserted-by":"publisher","first-page":"1657","DOI":"10.1001\/archinte.158.15.1657","volume":"158","author":"CL French","year":"1998","unstructured":"French CL, Irwin RS, Curley FJ, Krikorian CJ (1998) Impact of chronic cough on quality of life. Arch Intern Med 158(15):1657\u20131661","journal-title":"Arch Intern Med"},{"issue":"4","key":"12001_CR42","doi-asserted-by":"publisher","first-page":"183","DOI":"10.1097\/SPC.0000000000000623","volume":"16","author":"\u00d6I Emilsson","year":"2022","unstructured":"Emilsson \u00d6I (2022) The burden and impact of chronic cough in severe disease. Curr Opin Support Palliat Care 16(4):183\u2013187","journal-title":"Curr Opin Support Palliat Care"},{"issue":"2","key":"12001_CR43","doi-asserted-by":"publisher","DOI":"10.5415\/apallergy.2021.11.e22","volume":"11","author":"HK Won","year":"2021","unstructured":"Won HK, Song WJ (2021) Impact and disease burden of chronic cough. Asia Pac Allergy 11(2):e22","journal-title":"Asia Pac Allergy"},{"issue":"1","key":"12001_CR44","doi-asserted-by":"publisher","first-page":"372","DOI":"10.1186\/s12890-022-02171-z","volume":"22","author":"N Ueda","year":"2022","unstructured":"Ueda N, Yakushiji A, Schelfhout J, Tokita S, Kubo T (2022) Impact of refractory and unexplained chronic cough on disease burden: a qualitative study. BMC Pulm Med 22(1):372","journal-title":"BMC Pulm Med"},{"key":"12001_CR45","doi-asserted-by":"publisher","first-page":"964","DOI":"10.4168\/aair.2020.12.6.964","volume":"12","author":"HK Won","year":"2020","unstructured":"Won HK, Lee JH, An J, Sohn KH, Kang MG, Kang SY, Morice AH, Cho SH, Song WJ (2020) Impact of chronic cough on health-related quality of life in the Korean adult general population: the Korean National Health and Nutrition Examination Survey 2010\u20132016. Allergy Asthma Immunol Res 12:964\u2013979","journal-title":"Allergy Asthma Immunol Res"},{"key":"12001_CR46","doi-asserted-by":"publisher","DOI":"10.1136\/bmjresp-2020-000764","volume":"8","author":"T Kubo","year":"2021","unstructured":"Kubo T, Tobe K, Okuyama K, Kikuchi M, Chen Y, Schelfhout J, Abe M, Tokita S (2021) Disease burden and quality of life of patients with chronic cough in Japan: a population-based cross-sectional survey. BMJ Open Respir Res 8:e000764","journal-title":"BMJ Open Respir Res"},{"issue":"3","key":"12001_CR47","doi-asserted-by":"publisher","first-page":"401","DOI":"10.1007\/s00408-015-9701-2","volume":"193","author":"SA Chamberlain","year":"2015","unstructured":"Chamberlain SA, Garrod R, Douiri A, Masefield S, Powell P, B\u00fccher C, Pandyan A, Morice AH, Birring SS (2015) The impact of chronic cough: a cross-sectional European survey. Lung 193(3):401\u2013408","journal-title":"Lung"},{"key":"12001_CR48","unstructured":"Orphanet (2023) The portal for rare diseases and orphan drugs: cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome. https:\/\/www.orpha.net\/consor\/cgi-bin\/OC_Exp.php?lng=EN&Expert=504476. Accessed 7 Sept 2023"}],"container-title":["Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-023-12001-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00415-023-12001-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-023-12001-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,2,26]],"date-time":"2024-02-26T09:05:12Z","timestamp":1708938312000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00415-023-12001-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11,2]]},"references-count":49,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2024,3]]}},"alternative-id":["12001"],"URL":"https:\/\/doi.org\/10.1007\/s00415-023-12001-9","relation":{},"ISSN":["0340-5354","1432-1459"],"issn-type":[{"value":"0340-5354","type":"print"},{"value":"1432-1459","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,11,2]]},"assertion":[{"value":"7 August 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"9 September 2023","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 September 2023","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 November 2023","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"EP has received conference travel and attendance expenses from Gebro Pharma, Chiesi, FAES Farma, Rovi, GlaxoSmithKline, AstraZeneca, and Sanofi, and funds\/grants for research projects from state agencies, non-profit foundations, and Alpha Bioresearch. ECS reports non-financial support from ALK, AstraZeneca, Novartis, and Menarini, personal fees from Boehringer-Ingelheim and TEVA, and personal fees\/non-financial support from Chiesi outside the submitted work. VP in the last three years received honoraria for speaking at sponsored meetings from Astrazeneca, Boehringer-Ingelheim, Chiesi, Gebro, GSK, Luminova-Medwell and Sanofi. Received help assistance to meeting travel from Astrazeneca and Chiesi. Act as a consultant for Astrazeneca, Chiesi, GSK and Menarini. LGQ has received conference travel and attendance expenses and fees for talks at meetings sponsored by PTC Therapeutics, and has received funds\/grants for research projects from state agencies. PG, LQ, FB, MR and ASC declare that they have no conflicts of interest. AC has received fees in the last 3\u00a0years for talks at meetings sponsored by AstraZeneca, Zamb\u00f3n, Boehringer Ingelheim, Chiesi, GlaxoSmithKline, MSD, Novartis, Orion Pharma, and Sanofi, conference travel and attendance expenses from Gebro, GlaxoSmithKline, and Novartis, and funds\/grants for research projects from several state agencies, non-profit foundations, AstraZeneca, and GlaxoSmithKline.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}