{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T19:01:51Z","timestamp":1790794911344,"version":"4.1.0"},"reference-count":27,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2018,8,28]],"date-time":"2018-08-28T00:00:00Z","timestamp":1535414400000},"content-version":"vor","delay-in-days":58,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["movementdisorders.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2018,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p><jats:bold>Background:<\/jats:bold> The onset of multiple system atrophy (MSA) before age 40 years is referred to as \u201cyoung\u2010onset MSA.\u201d We identified clinical and pathological characteristics that might help with its early diagnosis and distinction from young\u2010onset Parkinson's disease and late\u2010onset MSA.<\/jats:p><jats:p><jats:bold>Methods:<\/jats:bold> We reviewed the available clinical and pathological features in cases that fulfilled consensus criteria for diagnosis of probable MSA or had autopsy confirmed MSA with an onset before age 40 years and compared the clinical features with 16 autopsy confirmed cases with young\u2010onset Parkinson's disease and a large published series of late\u2010onset MSA from the European MSA Study Group.<\/jats:p><jats:p><jats:bold>Results:<\/jats:bold> We identified 22 patients with young\u2010onset MSA, 8 of whom had available pathology. The mean age of onset was 36.7 years (standard deviation 2.3). Levodopa\u2010induced dyskinesia was more common, whereas myoclonus and pyramidal signs were less common in young\u2010onset Parkinson's disease when compared with young\u2010onset MSA. Dystonia, levodopa responsiveness, levodopa\u2010induced dyskinesia, and pyramidal signs were more common (<jats:italic>P<\/jats:italic>\u2009&lt;\u2009.05) when compared with the data in late\u2010onset MSA. On postmortem analysis, the minimal\u2010change pathological variant was more common in young\u2010onset MSA (n\u2009=\u20092) than late\u2010onset MSA (<jats:italic>P<\/jats:italic>\u2009=\u2009.045), with a mean survival of 11.1\u2009\u00b1\u20093.2 years (range 5.5\u201014.6) in pathologically confirmed cases of young\u2010onset MSA.<\/jats:p><jats:p><jats:bold>Conclusion:<\/jats:bold> This study has identified useful differences that may improve diagnostic accuracy, help us understand the pathological basis, and assist clinicians with the early diagnosis of young\u2010onset MSA. \u00a9 2018 International Parkinson and Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.27450","type":"journal-article","created":{"date-parts":[[2018,8,30]],"date-time":"2018-08-30T01:09:17Z","timestamp":1535591357000},"page":"1099-1107","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Young\u2010onset multiple system atrophy: Clinical and pathological features"],"prefix":"10.1002","volume":"33","author":[{"given":"Amit","family":"Batla","sequence":"first","affiliation":[{"name":"University College London (UCL) Institute of Neurology London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eduardo","family":"De Pablo\u2010Fernandez","sequence":"additional","affiliation":[{"name":"Reta Lila Weston Institute of Neurological Studies UCL Institute of Neurology London UK"},{"name":"Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7800-7419","authenticated-orcid":false,"given":"Roberto","family":"Erro","sequence":"additional","affiliation":[{"name":"University College London (UCL) Institute of Neurology London UK"},{"name":"Center for Neurodegenerative diseases, Department of Medicine, Surgery and Dentistry University of Salerno Salerno Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Reich","sequence":"additional","affiliation":[{"name":"Department of Neurology University Clinic of W\u00fcrzburg W\u00fcrzburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanna","family":"Calandra\u2010Buonaura","sequence":"additional","affiliation":[{"name":"Istituti di Ricovero e Cura a Carattere. Scientifico (IRCCS) Institute of Science and Neurology of Bologna Bologna Italy"},{"name":"Department of Biomedical and Neuromotor Sciences University of Bologna Bologna Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pedro","family":"Barbosa","sequence":"additional","affiliation":[{"name":"Reta Lila Weston Institute of Neurological Studies UCL Institute of Neurology London UK"},{"name":"Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bettina","family":"Balint","sequence":"additional","affiliation":[{"name":"University College London (UCL) Institute of Neurology London UK"},{"name":"Department of Neurology University Hospital Heidelberg Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1532-3526","authenticated-orcid":false,"given":"Helen","family":"Ling","sequence":"additional","affiliation":[{"name":"Reta Lila Weston Institute of Neurological Studies UCL Institute of Neurology London UK"},{"name":"Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saiful","family":"Islam","sequence":"additional","affiliation":[{"name":"UCL Institute of Neurology London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pietro","family":"Cortelli","sequence":"additional","affiliation":[{"name":"Istituti di Ricovero e Cura a Carattere. Scientifico (IRCCS) Institute of Science and Neurology of Bologna Bologna Italy"},{"name":"Department of Biomedical and Neuromotor Sciences University of Bologna Bologna Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens","family":"Volkmann","sequence":"additional","affiliation":[{"name":"Department of Neurology University Clinic of W\u00fcrzburg W\u00fcrzburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niall","family":"Quinn","sequence":"additional","affiliation":[{"name":"National Hospital for Neurology and Neurosurgery London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janice L.","family":"Holton","sequence":"additional","affiliation":[{"name":"Reta Lila Weston Institute of Neurological Studies UCL Institute of Neurology London UK"},{"name":"Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology London UK"},{"name":"Department of Molecular Neuroscience, UCL Institute of Neurology University College London London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas T.","family":"Warner","sequence":"additional","affiliation":[{"name":"Reta Lila Weston Institute of Neurological Studies UCL Institute of Neurology London UK"},{"name":"Queen Square Brain Bank for Neurological Disorders, UCL Institute of Neurology London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8185-286X","authenticated-orcid":false,"given":"Kailash P.","family":"Bhatia","sequence":"additional","affiliation":[{"name":"University College London (UCL) Institute of Neurology London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,8,28]]},"reference":[{"key":"e_1_2_16_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.49.5.1284"},{"key":"e_1_2_16_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2012.04.016"},{"key":"e_1_2_16_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.20534"},{"key":"e_1_2_16_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870120203"},{"key":"e_1_2_16_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(12)70327-7"},{"key":"e_1_2_16_7_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000324625.00404.15"},{"key":"e_1_2_16_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.26220"},{"key":"e_1_2_16_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awn065"},{"key":"e_1_2_16_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870090206"},{"issue":"5","key":"e_1_2_16_11_1","doi-asserted-by":"crossref","first-page":"1070","DOI":"10.1093\/brain\/awf117","article-title":"Progression and prognosis in multiple system atrophy. 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