{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T15:48:38Z","timestamp":1791388118300,"version":"4.3.3"},"reference-count":36,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2024,9,10]],"date-time":"2024-09-10T00:00:00Z","timestamp":1725926400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2024,9,10]],"date-time":"2024-09-10T00:00:00Z","timestamp":1725926400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2024,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction\/Aims<\/jats:title>\n                    <jats:p>Multifocal motor neuropathy (MMN) is a rare disease for which epidemiological and clinical data are limited. We conducted a nationwide survey to determine disease prevalence, incidence, clinical profile, and current treatment status in Japan.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A nationwide survey was conducted in 2021 using an established epidemiological method. Questionnaires were sent to all neurology and pediatric neurology departments in Japan. An initial questionnaire was administered to determine the number of patients with and incidence of MMN. A second questionnaire was administered to collect detailed clinical information. The European Federation of Neurological Societies\/Peripheral Nerve Society 2010 guidelines were used as diagnostic criteria.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The estimated number of patients with MMN was 507. The estimated prevalence was 0.40 per 100,000 individuals. Detailed clinical profiles were available for 120 patients. The male\u2010to\u2010female ratio was 2.3:1 and the median onset age was 42\u2009years. The median disease duration at diagnosis was 25\u2009months. Most patients presented with upper limb\u2010dominant muscle weakness. Motor nerve conduction blocks were found in 62% of patients and positive anti\u2010GM1 IgM antibody results in 54%. A total of 117 (98%) patients received immunoglobulin therapy, and 91% of them showed improvement. At the time of the last visit (median, 82\u2009months from treatment initiation), 89 (74%) patients were receiving maintenance immunoglobulin therapy. A slight progression of neurological deficits was observed during follow\u2010up.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>Most patients with MMN in Japan received induction and maintenance immunoglobulin therapies, which appear to suppress long\u2010term disease progression.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mus.28251","type":"journal-article","created":{"date-parts":[[2024,9,10]],"date-time":"2024-09-10T05:34:35Z","timestamp":1725946475000},"page":"1027-1033","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Multifocal motor neuropathy in Japan: A nationwide survey on prevalence, clinical profiles, and treatment"],"prefix":"10.1002","volume":"70","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4232-2772","authenticated-orcid":false,"given":"Yuya","family":"Aotsuka","sequence":"first","affiliation":[{"name":"Department of Neurology, Graduate School of Medicine Chiba University  Chiba Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonoko","family":"Misawa","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medicine Chiba University  Chiba Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomoki","family":"Suichi","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medicine Chiba University  Chiba Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4061-0412","authenticated-orcid":false,"given":"Kazumoto","family":"Shibuya","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medicine Chiba University  Chiba Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keigo","family":"Nakamura","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medicine Chiba University  Chiba Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroki","family":"Kano","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medicine Chiba University  Chiba Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryo","family":"Otani","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medicine Chiba University  Chiba Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie","family":"Morooka","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medicine Chiba University  Chiba Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Moeko","family":"Ogushi","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medicine Chiba University  Chiba Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4529-9045","authenticated-orcid":false,"given":"Kengo","family":"Nagashima","sequence":"additional","affiliation":[{"name":"Biostatistics Unit, Clinical and Translational Research Center Keio University Hospital  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasunori","family":"Sato","sequence":"additional","affiliation":[{"name":"Biostatistics Unit, Clinical and Translational Research Center Keio University Hospital  Tokyo Japan"},{"name":"Department of Preventive Medicine and Public Health Keio University of Medicine  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nagato","family":"Kuriyama","sequence":"additional","affiliation":[{"name":"Department of Social Health Medicine Shizuoka Graduate University of Public Health  Shizuoka Japan"},{"name":"Department of Epidemiology for Community Health and Medicine Kyoto Prefectural University of Medicine  Kyoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Kuwabara","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medicine Chiba University  Chiba Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,9,10]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp\u20102019\u2010321532"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/14651858.cd004429.pub3"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.5692\/clinicalneurol.52.920"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1085\u20109489.2006.00058.x"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1529\u20108027.2010.00290.x"},{"key":"e_1_2_12_7_1","unstructured":"Population Estimates.Jinkou suikei 2021 nen 3 gatsu hou (in Japanese).2021Accessed July 30 2024.https:\/\/www.stat.go.jp\/data\/jinsui\/pdf\/202103.pdf"},{"key":"e_1_2_12_8_1","unstructured":"NakamuraY the Study Group on Epidemiologic Research for Intractable Diseases.A Manual of a Nationwide Epidemiological Survey for Estimating the Number of Patients and Assessing Clinico\u2010Epidemiological Characteristics of Patients with Intractable Diseases (3rd edition).2017.http:\/\/www.jichi.ac.jp\/dph\/nanbyou\/manual_2017.pdf"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1212\/wnl.0000000000209130"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1212\/wnl.0000000000008062"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/hepr.13343"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.2188\/jea.10.29"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1212\/wnl.0b013e3181f0738e"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1212\/wnl.0000000000003504"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415\u2010018\u20109071\u20109"},{"key":"e_1_2_12_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.27698"},{"key":"e_1_2_12_17_1","doi-asserted-by":"publisher","DOI":"10.1212\/wnl.0000000000010538"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.1177\/1756285616629869"},{"key":"e_1_2_12_19_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000277697.55288.d0"},{"key":"e_1_2_12_20_1","doi-asserted-by":"publisher","DOI":"10.1212\/wnl.47.4.951"},{"key":"e_1_2_12_21_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.60.6.599"},{"key":"e_1_2_12_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jocn.2011.12.030"},{"key":"e_1_2_12_23_1","first-page":"1448","article-title":"Multifocal motor neuropathy\u2014unusual cause of hypoglossal palsy","volume":"99","author":"Axelsson G","year":"2002","journal-title":"Lakartidningen"},{"key":"e_1_2_12_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/(sici)1097\u20104598(199703)20:3<347::aid\u2010mus12>3.0.co;2\u2010y"},{"key":"e_1_2_12_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.13380"},{"key":"e_1_2_12_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.51357"},{"key":"e_1_2_12_27_1","doi-asserted-by":"publisher","DOI":"10.1212\/wnl.0000000000008697"},{"key":"e_1_2_12_28_1","unstructured":"OECD.Stat.Health Status. Accessed January 28 2024.https:\/\/stats.oecd.org\/index.aspx?DataSetCode=HEALTH_STAT"},{"key":"e_1_2_12_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejrad.2020.108816"},{"key":"e_1_2_12_30_1","doi-asserted-by":"publisher","DOI":"10.3389\/fnins.2021.649071"},{"key":"e_1_2_12_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ultrasmedbio.2013.03.031"},{"key":"e_1_2_12_32_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2007.121756"},{"key":"e_1_2_12_33_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awf193"},{"key":"e_1_2_12_34_1","doi-asserted-by":"publisher","DOI":"10.1111\/jns.12311"},{"key":"e_1_2_12_35_1","unstructured":"ClinicalTrials.gov.A Clinical Trial to Investigate the Safety and Tolerability Efficacy Pharmacokinetics Pharmacodynamics and Immunogenicity of 2 Dose Regimens of ARGX\u2010117 in Adults with Multifocal Motor Neuropathy. Accessed October 2 2023.https:\/\/clinicaltrials.gov\/study\/NCT05225675?cond=Multifocal%20Motor%20Neuropathy&rank=4"},{"key":"e_1_2_12_36_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13311\u2010022\u201001222\u2010x"},{"key":"e_1_2_12_37_1","unstructured":"UMIN Clinical Trials Registry.JANIMA. Accessed June 7 2023.https:\/\/center6.umin.ac.jp\/cgi-open-bin\/ctr_e\/ctr_view.cgi?recptno=R000052437"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.28251","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mus.28251","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.28251","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T15:06:50Z","timestamp":1772982410000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.28251"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9,10]]},"references-count":36,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2024,11]]}},"alternative-id":["10.1002\/mus.28251"],"URL":"https:\/\/doi.org\/10.1002\/mus.28251","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,9,10]]},"assertion":[{"value":"2023-10-22","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-08-27","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-09-10","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}