{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T23:57:52Z","timestamp":1789516672512,"version":"build-2803163510"},"reference-count":32,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2024,2,22]],"date-time":"2024-02-22T00:00:00Z","timestamp":1708560000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,2,22]],"date-time":"2024-02-22T00:00:00Z","timestamp":1708560000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/100018007","name":"Ulla-Carin Lindquists stiftelse f\u00f6r ALS-forskning","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100018007","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004359","name":"Vetenskapsr\u00e5det","doi-asserted-by":"publisher","award":["Swedish Research Grant DNR 2019-01088"],"award-info":[{"award-number":["Swedish Research Grant DNR 2019-01088"]}],"id":[{"id":"10.13039\/501100004359","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003509","name":"\u00d6rebro University","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100003509","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurol"],"published-print":{"date-parts":[[2024,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Motor neuron diseases (MND), with amyotrophic lateral sclerosis constituting most cases, are rare conditions of unknown etiology. There have been reports of an increase in incidence during the latter half of the twentieth century in various Western countries, including Sweden. This study provides updated data on the incidence of MND in Sweden during the last 20\u00a0years.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Data was obtained from the Swedish National Patient Register on individuals diagnosed with MND from 2002 to 2021 and analysed in relation to group level data for the entire Swedish population. Incidence rates were calculated and presented in relation to year, age, sex, and region.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>In the early 2000s, there was a crude incidence rate of 3.5\u20133.7 per 100,000 person-years, which then increased to 4.0\u20134.6 from 2008 onward. Age standardization to the starting year (2002) partially mitigated this increase. The incidence rate was greater among men compared to women and was highest within the age range of 70 to 84\u00a0years. There were indications of a higher incidence rate in the northernmost parts of the country, although the difference was not statistically significant.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>The incidence rate of MND in Sweden now seems to have surpassed 4 cases per 100,000 person-years. This is higher when compared to both other European countries and previous Swedish studies. It remains to be determined if this increase reflects an actual increasing incidence of MND in Sweden or is due to other factors such as better registry coverage.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s00415-024-12219-1","type":"journal-article","created":{"date-parts":[[2024,2,22]],"date-time":"2024-02-22T12:18:20Z","timestamp":1708604300000},"page":"2730-2735","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Increased incidence of motor neuron disease in Sweden: a population-based study during 2002\u20132021"],"prefix":"10.1007","volume":"271","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-3855-5937","authenticated-orcid":false,"given":"Sofia","family":"Imrell","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fang","family":"Fang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Ingre","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Sennf\u00e4lt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,2,22]]},"reference":[{"issue":"3\u20134","key":"12219_CR1","doi-asserted-by":"publisher","first-page":"133","DOI":"10.1080\/21678421.2018.1550518","volume":"20","author":"E Finegan","year":"2019","unstructured":"Finegan E, Chipika RH, Shing SLH et al (2019) Primary lateral sclerosis: a distinct entity or part of the ALS spectrum? Amyotroph Lateral Scler Frontotemporal Degener 20(3\u20134):133\u2013145","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"issue":"4","key":"12219_CR2","doi-asserted-by":"publisher","first-page":"761","DOI":"10.1016\/j.ncl.2015.07.005","volume":"33","author":"T Liewluck","year":"2015","unstructured":"Liewluck T, Saperstein DS (2015) Progressive muscular atrophy. Neurol Clin 33(4):761\u2013773","journal-title":"Neurol Clin"},{"issue":"10","key":"12219_CR3","doi-asserted-by":"publisher","first-page":"1918","DOI":"10.1111\/ene.14393","volume":"27","author":"P Masrori","year":"2020","unstructured":"Masrori P, Van Damme P (2020) Amyotrophic lateral sclerosis: a clinical review. Eur J Neurol 27(10):1918\u20131929","journal-title":"Eur J Neurol"},{"issue":"5","key":"12219_CR4","doi-asserted-by":"publisher","first-page":"813","DOI":"10.1212\/01.WNL.0000049472.47709.3B","volume":"60","author":"MA del Aguila","year":"2003","unstructured":"del Aguila MA, Longstreth WT Jr, McGuire V et al (2003) Prognosis in amyotrophic lateral sclerosis: a population-based study. Neurology 60(5):813\u2013819","journal-title":"Neurology"},{"issue":"5","key":"12219_CR5","doi-asserted-by":"publisher","first-page":"342","DOI":"10.1159\/000516752","volume":"55","author":"CA Brown","year":"2021","unstructured":"Brown CA, Lally C, Kupelian V et al (2021) Estimated prevalence and incidence of amyotrophic lateral sclerosis and SOD1 and C9orf72 genetic variants. Neuroepidemiology 55(5):342\u2013353","journal-title":"Neuroepidemiology"},{"issue":"12","key":"12219_CR6","doi-asserted-by":"publisher","first-page":"1083","DOI":"10.1016\/S1474-4422(18)30404-6","volume":"17","author":"GBDMND Collaborators","year":"2018","unstructured":"GBDMND Collaborators (2018) Global, regional, and national burden of motor neuron diseases 1990\u20132016: a systematic analysis for the Global Burden of Disease Study 2016. Lancet Neurol. 17(12):1083\u201397","journal-title":"Lancet Neurol."},{"issue":"4","key":"12219_CR7","doi-asserted-by":"publisher","first-page":"238","DOI":"10.1159\/000531238","volume":"57","author":"M Hanhisuanto","year":"2023","unstructured":"Hanhisuanto M, Solje E, Jokela M et al (2023) Amyotrophic lateral sclerosis in Southwestern and Eastern Finland. Neuroepidemiology 57(4):238\u2013245","journal-title":"Neuroepidemiology"},{"issue":"2","key":"12219_CR8","doi-asserted-by":"publisher","first-page":"227","DOI":"10.1002\/acn3.51501","volume":"9","author":"M Johansen","year":"2022","unstructured":"Johansen M, Svenstrup K, Joensen P et al (2022) High incidence of amyotrophic lateral sclerosis in the Faroe Islands 2010\u20132020. Ann Clin Transl Neurol 9(2):227\u2013231","journal-title":"Ann Clin Transl Neurol"},{"issue":"3\u20134","key":"12219_CR9","doi-asserted-by":"publisher","first-page":"284","DOI":"10.1080\/21678421.2017.1281961","volume":"18","author":"M Tesauro","year":"2017","unstructured":"Tesauro M, Consonni M, Filippini T et al (2017) Incidence of amyotrophic lateral sclerosis in the province of Novara, Italy, and possible role of environmental pollution. Amyotroph Lateral Scler Frontotemporal Degener 18(3\u20134):284\u2013290","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"issue":"3","key":"12219_CR10","doi-asserted-by":"publisher","first-page":"323","DOI":"10.1002\/mus.27939","volume":"68","author":"G Borghero","year":"2023","unstructured":"Borghero G, Sechi MM, Vasta R et al (2023) Spatial clustering of amyotrophic lateral sclerosis in Sardinia, Italy: the contribution of age, sex, and genetic factors. Muscle Nerve 68(3):323\u2013328","journal-title":"Muscle Nerve"},{"issue":"5\u20136","key":"12219_CR11","doi-asserted-by":"publisher","first-page":"363","DOI":"10.1080\/21678421.2021.2016837","volume":"23","author":"JM Burchardt","year":"2022","unstructured":"Burchardt JM, Mei XW, Ranger T et al (2022) Analysis of incidence of motor neuron disease in England 1998\u20132019: use of three linked datasets. Amyotroph Lateral Scler Frontotemporal Degener 23(5\u20136):363\u2013371","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"issue":"1\u20132","key":"12219_CR12","doi-asserted-by":"publisher","first-page":"90","DOI":"10.1080\/21678421.2018.1511732","volume":"20","author":"F Palese","year":"2019","unstructured":"Palese F, Sartori A, Verriello L et al (2019) Epidemiology of amyotrophic lateral sclerosis in Friuli-Venezia Giulia, North-Eastern Italy, 2002\u20132014: a retrospective population-based study. Amyotroph Lateral Scler Frontotemporal Degener 20(1\u20132):90\u201399","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"issue":"4","key":"12219_CR13","doi-asserted-by":"publisher","first-page":"749","DOI":"10.1007\/s00415-017-8413-3","volume":"264","author":"A Rosenbohm","year":"2017","unstructured":"Rosenbohm A, Peter RS, Erhardt S et al (2017) Epidemiology of amyotrophic lateral sclerosis in Southern Germany. J Neurol 264(4):749\u2013757","journal-title":"J Neurol"},{"issue":"11","key":"12219_CR14","doi-asserted-by":"publisher","first-page":"1181","DOI":"10.1007\/s10654-022-00904-2","volume":"37","author":"F Boumediene","year":"2022","unstructured":"Boumediene F, Marin B, Luna J et al (2022) Spatio-temporal clustering of amyotrophic lateral sclerosis in France: a population-based study. Eur J Epidemiol 37(11):1181\u20131193","journal-title":"Eur J Epidemiol"},{"issue":"7\u20138","key":"12219_CR15","doi-asserted-by":"publisher","first-page":"522","DOI":"10.1080\/21678421.2018.1513534","volume":"19","author":"E Benjaminsen","year":"2018","unstructured":"Benjaminsen E, Alstadhaug KB, Gulsvik M et al (2018) Amyotrophic lateral sclerosis in Nordland county, Norway, 2000\u20132015: prevalence, incidence, and clinical features. Amyotroph Lateral Scler Frontotemporal Degener 19(7\u20138):522\u2013527","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"issue":"5\u20136","key":"12219_CR16","doi-asserted-by":"publisher","first-page":"401","DOI":"10.1080\/21678421.2021.1891249","volume":"22","author":"I Jerico","year":"2021","unstructured":"Jerico I, Elizalde-Beiras I, Pagola I et al (2021) Clinical features and incidence trends of amyotrophic lateral sclerosis in Navarre, Spain, 2007\u20132018: a population-based study. Amyotroph Lateral Scler Frontotemporal Degener 22(5\u20136):401\u2013409","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"issue":"7\u20138","key":"12219_CR17","doi-asserted-by":"publisher","first-page":"585","DOI":"10.1080\/21678421.2022.2041670","volume":"23","author":"G Borghero","year":"2022","unstructured":"Borghero G, Pierri V, Vasta R et al (2022) Incidence of amyotrophic lateral sclerosis in Sardinia, Italy: age-sex interaction and spatial-temporal variability. Amyotroph Lateral Scler Frontotemporal Degener 23(7\u20138):585\u2013591","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"issue":"5","key":"12219_CR18","doi-asserted-by":"publisher","first-page":"771","DOI":"10.1097\/WCO.0000000000000730","volume":"32","author":"E Longinetti","year":"2019","unstructured":"Longinetti E, Fang F (2019) Epidemiology of amyotrophic lateral sclerosis: an update of recent literature. Curr Opin Neurol 32(5):771\u2013776","journal-title":"Curr Opin Neurol"},{"issue":"9","key":"12219_CR19","doi-asserted-by":"publisher","first-page":"1262","DOI":"10.1212\/WNL.55.9.1262","volume":"55","author":"YM Seljeseth","year":"2000","unstructured":"Seljeseth YM, Vollset SE, Tysnes OB (2000) Increasing mortality from amyotrophic lateral sclerosis in Norway? Neurology 55(9):1262\u20131266","journal-title":"Neurology"},{"issue":"4","key":"12219_CR20","doi-asserted-by":"publisher","first-page":"232","DOI":"10.1034\/j.1600-0404.2001.00361.x","volume":"104","author":"P Maasilta","year":"2001","unstructured":"Maasilta P, Jokelainen M, Loytonen M et al (2001) Mortality from amyotrophic lateral sclerosis in Finland, 1986\u20131995. Acta Neurol Scand 104(4):232\u2013235","journal-title":"Acta Neurol Scand"},{"issue":"4","key":"12219_CR21","doi-asserted-by":"publisher","first-page":"817","DOI":"10.1007\/s00415-019-09190-7","volume":"266","author":"DJ Leighton","year":"2019","unstructured":"Leighton DJ, Newton J, Stephenson LJ et al (2019) Changing epidemiology of motor neurone disease in Scotland. J Neurol 266(4):817\u2013825","journal-title":"J Neurol"},{"issue":"23","key":"12219_CR22","doi-asserted-by":"publisher","first-page":"1889","DOI":"10.1212\/01.wnl.0000336653.65605.ac","volume":"71","author":"M Murphy","year":"2008","unstructured":"Murphy M, Quinn S, Young J et al (2008) Increasing incidence of ALS in Canterbury, New Zealand: a 22-year study. Neurology 71(23):1889\u20131895","journal-title":"Neurology"},{"issue":"4","key":"12219_CR23","doi-asserted-by":"publisher","first-page":"515","DOI":"10.1001\/archneurol.2009.13","volume":"66","author":"F Fang","year":"2009","unstructured":"Fang F, Valdimarsdottir U, Bellocco R et al (2009) Amyotrophic lateral sclerosis in Sweden, 1991\u20132005. Arch Neurol 66(4):515\u2013519","journal-title":"Arch Neurol"},{"key":"12219_CR24","doi-asserted-by":"publisher","first-page":"450","DOI":"10.1186\/1471-2458-11-450","volume":"11","author":"JF Ludvigsson","year":"2011","unstructured":"Ludvigsson JF, Andersson E, Ekbom A et al (2011) External review and validation of the Swedish national inpatient register. BMC Public Health 11:450","journal-title":"BMC Public Health"},{"issue":"7\u20138","key":"12219_CR25","doi-asserted-by":"publisher","first-page":"528","DOI":"10.1080\/21678421.2018.1497065","volume":"19","author":"E Longinetti","year":"2018","unstructured":"Longinetti E, Regodon Wallin A, Samuelsson K et al (2018) The Swedish motor neuron disease quality registry. Amyotroph Lateral Scler Frontotemporal Degener 19(7\u20138):528\u2013537","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"issue":"2","key":"12219_CR26","doi-asserted-by":"publisher","first-page":"118","DOI":"10.1159\/000351153","volume":"41","author":"A Chio","year":"2013","unstructured":"Chio A, Logroscino G, Traynor BJ et al (2013) Global epidemiology of amyotrophic lateral sclerosis: a systematic review of the published literature. Neuroepidemiology 41(2):118\u2013130","journal-title":"Neuroepidemiology"},{"issue":"3","key":"12219_CR27","doi-asserted-by":"publisher","DOI":"10.1136\/bmjopen-2020-045343","volume":"11","author":"R Moynihan","year":"2021","unstructured":"Moynihan R, Sanders S, Michaleff ZA et al (2021) Impact of COVID-19 pandemic on utilisation of healthcare services: a systematic review. BMJ Open 11(3):e045343","journal-title":"BMJ Open"},{"key":"12219_CR28","doi-asserted-by":"publisher","first-page":"217","DOI":"10.1016\/j.jns.2019.02.030","volume":"399","author":"S Mathis","year":"2019","unstructured":"Mathis S, Goizet C, Soulages A et al (2019) Genetics of amyotrophic lateral sclerosis: a review. J Neurol Sci 399:217\u2013226","journal-title":"J Neurol Sci"},{"issue":"4","key":"12219_CR29","doi-asserted-by":"publisher","first-page":"271","DOI":"10.1159\/000525091","volume":"56","author":"CG Olsen","year":"2022","unstructured":"Olsen CG, Busk OL, Aanjesen TN et al (2022) Genetic epidemiology of amyotrophic lateral sclerosis in Norway: a 2-year population-based study. Neuroepidemiology 56(4):271\u2013282","journal-title":"Neuroepidemiology"},{"issue":"4","key":"12219_CR30","doi-asserted-by":"publisher","first-page":"323","DOI":"10.1016\/S1474-4422(12)70043-1","volume":"11","author":"E Majounie","year":"2012","unstructured":"Majounie E, Renton AE, Mok K et al (2012) Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study. Lancet Neurol 11(4):323\u2013330","journal-title":"Lancet Neurol"},{"key":"12219_CR31","first-page":"181","volume":"7","author":"C Ingre","year":"2015","unstructured":"Ingre C, Roos PM, Piehl F et al (2015) Risk factors for amyotrophic lateral sclerosis. Clin Epidemiol 7:181\u2013193","journal-title":"Clin Epidemiol"},{"issue":"1\u20132","key":"12219_CR32","first-page":"45","volume":"24","author":"S Sennfalt","year":"2022","unstructured":"Sennfalt S, Klappe U, Thams S et al (2022) The path to diagnosis in ALS: delay, referrals, alternate diagnoses, and clinical progression. Amyotroph Lateral Scler Frontotemporal Degener. 24(1\u20132):45\u201353","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener."}],"container-title":["Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-024-12219-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00415-024-12219-1\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00415-024-12219-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,4,27]],"date-time":"2024-04-27T07:16:24Z","timestamp":1714202184000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00415-024-12219-1"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,2,22]]},"references-count":32,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2024,5]]}},"alternative-id":["12219"],"URL":"https:\/\/doi.org\/10.1007\/s00415-024-12219-1","relation":{},"ISSN":["0340-5354","1432-1459"],"issn-type":[{"value":"0340-5354","type":"print"},{"value":"1432-1459","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,2,22]]},"assertion":[{"value":"1 December 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 January 2024","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 January 2024","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 February 2024","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Caroline Ingre has consulted for Cytokinetics, Pfizer, BioArctic, Novartis and Tikomed and was a DMC member for Appelis Pharmaceutical. She is also a board member of Tobii Dynavox.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"Ethical approval for the study was obtained from the regional ethical review board in Stockholm (reg. no. 2017\/1895-31 and 2022-04337-02. The data used in the study from the SNPR did not include personal identification numbers, thus precluding direct identification of the individual.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}}]}}