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Expansions of more than 200 CGG repeats give rise to fragile X syndrome, the most common inherited form of cognitive impairment. Fragile X\u2010associated tremor\/ataxia syndrome is characterized by cerebellar tremor and ataxia, and the presence of ubiquitin\u2010positive inclusions in neurons and astrocytes. It has been previously suggested that fragile X\u2010associated tremor\/ataxia syndrome is associated with an inflammatory state based on signs of oxidative stress\u2013mediated damage and iron deposition.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>Determine whether the pathology of fragile X\u2010associated tremor\/ataxia syndrome involves microglial activation and an inflammatory state.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Using ionized calcium binding adaptor molecule 1 and cluster differentiation 68 antibodies to label microglia, we examined the number and state of activation of microglial cells in the putamen of 13 fragile X\u2010associated tremor\/ataxia syndrome and 9 control postmortem cases.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Nearly half of fragile X\u2010associated tremor\/ataxia syndrome cases (6 of 13) presented with dystrophic senescent microglial cells. In the remaining fragile X\u2010associated tremor\/ataxia syndrome cases (7 of 13), the number of microglial cells and their activation state were increased compared to controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The presence of senescent microglial cells in half of fragile X\u2010associated tremor\/ataxia syndrome cases suggests that this indicator could be used, together with the presence of intranuclear inclusions and the presence of iron deposits, as a biomarker to aid in the postmortem diagnosis of fragile X\u2010associated tremor\/ataxia syndrome. An increased number and activation indicate that microglial cells play a role in the inflammatory state present in the fragile X\u2010associated tremor\/ataxia syndrome brain. Anti\u2010inflammatory treatment of patients with fragile X\u2010associated tremor\/ataxia syndrome may be indicated to slow neurodegeneration. \u00a9 2018 International Parkinson and Movement Disorder Society<\/jats:p><\/jats:sec>","DOI":"10.1002\/mds.27553","type":"journal-article","created":{"date-parts":[[2018,12,10]],"date-time":"2018-12-10T08:47:07Z","timestamp":1544431627000},"page":"1887-1894","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":27,"title":["Microglial cell activation and senescence are characteristic of the pathology FXTAS"],"prefix":"10.1002","volume":"33","author":[{"given":"Ver\u00f3nica","family":"Mart\u00ednez Cerde\u00f1o","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine UC Davis School of Medicine  Sacramento, California USA"},{"name":"Institute for Pediatric Regenerative Medicine and Shriners Hospitals for Children of Northern California  Sacramento, 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