{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T13:58:15Z","timestamp":1787061495360,"version":"build-2736575974"},"reference-count":23,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2025,11,29]],"date-time":"2025-11-29T00:00:00Z","timestamp":1764374400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,11,29]],"date-time":"2025-11-29T00:00:00Z","timestamp":1764374400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100017026","name":"Spastic Paraplegia Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100017026","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000971","name":"ALS Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000971","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100012351","name":"Mitsubishi Tanabe Pharma Corporation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100012351","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2026,2]]},"abstract":"ABSTRACT<\/jats:title>\n \n Introduction\/Aims<\/jats:title>\n \n There is a lack of information about startle reflex (\n SR<\/jats:styled-content>\n ) in primary lateral sclerosis (\n PLS<\/jats:styled-content>\n ). This study examined the presence and prevalence of\n SR<\/jats:styled-content>\n in\n PLS<\/jats:styled-content>\n and compared findings with amyotrophic lateral sclerosis (\n ALS<\/jats:styled-content>\n ).\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n 46 PLS and 54\n ALS<\/jats:styled-content>\n participants were assessed through structured interviews in this cross\u2010sectional study. Fisher's exact test was used to compare reported\n SR<\/jats:styled-content>\n prevalence. Multivariable linear regression was utilized to study associations between disease group and\n SR<\/jats:styled-content>\n frequency in response to sudden stimuli.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n SR differed markedly between the two groups, with a higher prevalence in PLS (93.5%) than ALS (20.4%;\n p<\/jats:italic>\n < 0.001). Among\n ALS<\/jats:styled-content>\n patients,\n SR<\/jats:styled-content>\n was present in all upper motor neuron (\n UMN<\/jats:styled-content>\n )\u2013predominant cases, which accounted for 54.5% of the\n SR<\/jats:styled-content>\n \u2010positive\n ALS<\/jats:styled-content>\n group, but only 10.4% of probable\/definite\n ALS<\/jats:styled-content>\n cases. In\n SR<\/jats:styled-content>\n \u2010positive patients, response frequency to sudden stimuli exceeded 60% in both\n ALS<\/jats:styled-content>\n and\n PLS<\/jats:styled-content>\n , most often triggered by auditory stimuli. Younger age, shorter disease duration, and\n PLS<\/jats:styled-content>\n diagnosis were associated with more frequent\n SR<\/jats:styled-content>\n .\n <\/jats:p>\n <\/jats:sec>\n \n Discussion<\/jats:title>\n \n SR<\/jats:styled-content>\n is significantly more common in\n PLS<\/jats:styled-content>\n than in\n ALS<\/jats:styled-content>\n . Notably,\n UMN<\/jats:styled-content>\n \u2010predominant\n ALS<\/jats:styled-content>\n , although limited in number, showed a higher prevalence of\n SR<\/jats:styled-content>\n (6 out of 6, 100%), indicating that predominant\n UMN<\/jats:styled-content>\n involvement may be a key determinant of\n SR<\/jats:styled-content>\n across both conditions. These hypothesis\u2010generating findings suggest that\n SR<\/jats:styled-content>\n may serve as a novel clinical marker in\n PLS<\/jats:styled-content>\n and\n UMN<\/jats:styled-content>\n \u2010predominant\n ALS<\/jats:styled-content>\n , warranting further validation through prospective studies.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/mus.70071","type":"journal-article","created":{"date-parts":[[2025,11,29]],"date-time":"2025-11-29T10:14:28Z","timestamp":1764411268000},"page":"304-308","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Startle Reflex in Primary Lateral Sclerosis (\n PLS<\/scp>\n ): A Comparison With Amyotrophic Lateral Sclerosis (\n ALS<\/scp>\n )"],"prefix":"10.1002","volume":"73","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-8051-7052","authenticated-orcid":false,"given":"Grace E.","family":"Jang","sequence":"first","affiliation":[{"name":"Department of Neurology Columbia University Irving Medical Center New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0537-9622","authenticated-orcid":false,"given":"Ikjae","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Neurology Columbia University Irving Medical Center New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jinsy A.","family":"Andrews","sequence":"additional","affiliation":[{"name":"Department of Neurology NYU Grossman School of Medicine New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ying Kuen (Ken)","family":"Cheung","sequence":"additional","affiliation":[{"name":"Department of Biostatistics, Mailman School of Public Health Columbia University New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mersad","family":"Redzepagic","sequence":"additional","affiliation":[{"name":"Department of Neurology Columbia University Irving Medical Center New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroshi","family":"Mitsumoto","sequence":"additional","affiliation":[{"name":"Department of Neurology Columbia University Irving Medical Center New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,11,29]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0301-0082(98)00098-7"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(06)70470-7"},{"issue":"7","key":"e_1_2_12_4_1","first-page":"484","article-title":"The Clinical Spectrum of Primary Lateral Sclerosis","volume":"68","author":"Barohn R. J.","year":"2007","journal-title":"Neurology"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000200962.94777.71"},{"key":"e_1_2_12_6_1","article-title":"Primary Lateral Sclerosis: An Overview","volume":"13","author":"Vacchiano V.","year":"2022","journal-title":"Frontiers in Neurology"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ncl.2015.07.007"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.NXG.0000464294.88607.dd"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1080\/21678421.2022"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2019-322541"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1080\/146608200300079536"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26765"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26764"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-510X(99)00210-5"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000058900.02672.D2"},{"key":"e_1_2_12_16_1","doi-asserted-by":"publisher","DOI":"10.3109\/17482960903171136"},{"key":"e_1_2_12_17_1","first-page":"357","article-title":"Age\u2010Related Changes in Neural Excitability, Inhibitory Tone, and Synaptic Dynamics in Sensory Cortices","volume":"176","author":"Hua T.","year":"2010","journal-title":"Neuroscience"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.1080\/21678421.2020.1837174"},{"key":"e_1_2_12_19_1","article-title":"Imaging Data Reveal Divergent Longitudinal Trajectories in PLS, ALS and Poliomyelitis Survivors","volume":"32","author":"Tahedl M.","year":"2021","journal-title":"NeuroImage: Clinical"},{"key":"e_1_2_12_20_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0000000000001295"},{"key":"e_1_2_12_21_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11682\u2010020\u201000421\u20104"},{"key":"e_1_2_12_22_1","doi-asserted-by":"publisher","DOI":"10.1080\/21678421.2021.2023188"},{"key":"e_1_2_12_23_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-024-12261-z"},{"key":"e_1_2_12_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.23496"}],"container-title":["Muscle & Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.70071","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mus.70071","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.70071","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,7]],"date-time":"2026-02-07T08:21:42Z","timestamp":1770452502000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.70071"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,29]]},"references-count":23,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2026,2]]}},"alternative-id":["10.1002\/mus.70071"],"URL":"https:\/\/doi.org\/10.1002\/mus.70071","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,11,29]]},"assertion":[{"value":"2025-06-27","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-11-11","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-11-29","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}