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Pathological features of neurodegeneration in fragile X\u2013associated tremor\/ataxia syndrome include toxic levels of FMR1<\/jats:italic> mRNA, ubiquitin\u2010positive intranuclear inclusions, white matter disease, iron accumulation, and a proinflammatory state.<\/jats:p><\/jats:sec>Objective<\/jats:title>The objective of this study was to analyze the presence of cerebral microbleeds in the brains of patients with fragile X\u2013associated tremor\/ataxia syndrome and investigate plausible causes for cerebral microbleeds in fragile X\u2013associated tremor\/ataxia syndrome.<\/jats:p><\/jats:sec>Methods<\/jats:title>We collected cerebral and cerebellar tissue from 15 fragile X\u2013associated tremor\/ataxia syndrome cases and 15 control cases carrying FMR1<\/jats:italic> normal alleles. We performed hematoxylin and eosin, Perls and Congo red stains, ubiquitin, and amyloid \u03b2 protein immunostaining. We quantified the number of cerebral microbleeds, amount of iron, presence of amyloid \u03b2 within the capillaries, and number of endothelial cells containing intranuclear inclusions. We evaluated the relationships between pathological findings using correlation analysis.<\/jats:p><\/jats:sec>Results<\/jats:title>We found intranuclear inclusions in the endothelial cells of capillaries and an increased number of cerebral microbleeds in the brains of those with fragile X\u2013associated tremor\/ataxia syndrome, both of which are indicators of cerebrovascular dysfunction. We also found a suggestive association between the amount of capillaries that contain amyloid \u03b2 in the cerebral cortex and the rate of disease progression.<\/jats:p><\/jats:sec>Conclusion<\/jats:title>We propose microangiopathy as a pathologic feature of fragile X\u2013associated tremor\/ataxia syndrome. \u00a9 2021 International Parkinson and Movement Disorder Society<\/jats:p><\/jats:sec>","DOI":"10.1002\/mds.28559","type":"journal-article","created":{"date-parts":[[2021,3,24]],"date-time":"2021-03-24T08:15:34Z","timestamp":1616573734000},"page":"1935-1943","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Cerebral Microbleeds in Fragile X\u2013Associated Tremor\/Ataxia Syndrome"],"prefix":"10.1002","volume":"36","author":[{"given":"Mar\u00eda Jimena","family":"Salcedo\u2010Arellano","sequence":"first","affiliation":[{"name":"Department of Pediatrics University of California Davis School of Medicine 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