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We aimed to clarify the clinicopathological features of LO\u2010MSA. Of 102 patients with autopsy\u2010confirmed MSA, 5 were identified as having LO\u2010MSA and 24 as having usual\u2010age\u2010onset MSA (UO\u2010MSA) with a similar disease duration. On the basis of previous reports, we defined UO\u2010MSA as the appearance of initial symptoms between the ages of 55 and 65\u2009years. We compared the clinical pictures of the two groups and assessed their histopathological features using quantitative and semi\u2010quantitative methods. The investigated features included the severity of degeneration in the striatonigral (StrN) and olivopontocerebellar (OPC) systems, the numbers of neurons in the brainstem autonomic and spinal intermediolateral nuclei, and the density of \u03b1\u2010synuclein\u2010immunopositive inclusions in the putamen, inferior olivary nucleus, and ventrolateral medulla (VLM). Most patients with both LO\u2010MSA and UO\u2010MSA exhibited the MSA\u2010olivopontocerebellar atrophy (OPCA) subtype (3\/5 and 18\/24, respectively). The median disease duration for LO\u2010MSA patients was 5.5\u2009years, which was comparable to that for patients in our cohort who had developed symptoms below 75\u2009years of age. Pathologically, degeneration of the StrN and OPC systems in LO\u2010MSA was less severe than that observed in UO\u2010MSA. Quantitative analysis revealed better preservation of neuron numbers in the brainstem autonomic nuclei in LO\u2010MSA than in UO\u2010MSA, with a significantly higher number of serotonergic neurons in the VLM (<jats:italic>p<\/jats:italic>\u2009=\u20090.013). The density of \u03b1\u2010synuclein\u2010positive inclusions in the putamen was significantly lower in LO\u2010MSA than in UO\u2010MSA (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). Neuronal degeneration in LO\u2010MSA may progress more slowly than in UO\u2010MSA. Accordingly, the prognosis of LO\u2010MSA may not necessarily be less favorable than that of MSA generally, especially with appropriate care.<\/jats:p>","DOI":"10.1111\/bpa.70016","type":"journal-article","created":{"date-parts":[[2025,5,19]],"date-time":"2025-05-19T20:44:09Z","timestamp":1747687449000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Late\u2010onset multiple system atrophy: Neuropathological features associated with slow disease progression"],"prefix":"10.1111","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9372-2982","authenticated-orcid":false,"given":"Misato","family":"Ozawa","sequence":"first","affiliation":[{"name":"Department of Pathology Brain Research Institute, Niigata University  Niigata Japan"},{"name":"Division of Neurology, Department of Medicine Jichi Medical University  Shimotsuke Tochigi 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