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Imaging provides diagnostic clues that help identify cases. The objective of this study was to integrate the literature on neuroimaging phenotypes of C<jats:italic>SF1R<\/jats:italic>\u2010related leukoencephalopathy. A systematic review and meta\u2010analysis were performed for neuroimaging findings of <jats:italic>CSF1R<\/jats:italic>\u2010related leukoencephalopathy via PubMed, Web of Science, and Embase on 25 August 2021. The search included cases with confirmed <jats:italic>CSF1R<\/jats:italic> mutations reported under the previous terms hereditary diffuse leukoencephalopathy with spheroids, pigmentary orthochromatic leukodystrophy, and adult\u2010onset leukoencephalopathy with axonal spheroids and pigmented glia. In 78 studies providing neuroimaging data, 195 cases were identified carrying <jats:italic>CSF1R<\/jats:italic> mutations in 14 exons and five introns. Women had a statistically significant earlier age of onset (<jats:italic>p<\/jats:italic> = 0.041, 40 vs 43 years). Mean delay between symptom onset and neuroimaging was 2.3 years. Main magnetic resonance imaging (MRI) findings were frontoparietal white matter lesions, callosal thinning, and foci of restricted diffusion. The hallmark computed tomography (CT) finding was white matter calcifications. Widespread cerebral hypometabolism and hypoperfusion were reported using positron emission tomography and single\u2010photon emission computed tomography. In conclusion, <jats:italic>CSF1R<\/jats:italic>\u2010related leukoencephalopathy is associated with progressive white matter lesions and brain atrophy that can resemble other neurodegenerative\/\u2010inflammatory disorders. However, long\u2010lasting diffusion restriction and parenchymal calcifications are more specific findings that can aid the differential diagnosis. Native brain CT and brain MRI (with and without a contrast agent) are recommended with proposed protocols and pictorial examples are provided.<\/jats:p>","DOI":"10.1111\/joim.13420","type":"journal-article","created":{"date-parts":[[2021,12,7]],"date-time":"2021-12-07T20:07:03Z","timestamp":1638907623000},"page":"269-282","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":37,"title":["Neuroimaging phenotypes of <i>CSF1R<\/i>\u2010related leukoencephalopathy: Systematic review, meta\u2010analysis, and imaging recommendations"],"prefix":"10.1111","volume":"291","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2559-2877","authenticated-orcid":false,"given":"Goda\u2010Camille","family":"Mickeviciute","sequence":"first","affiliation":[{"name":"Department of Physical Medicine and Rehabilitation Vilnius University Hospital Santaros Klinikos  Vilnius 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