{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T16:52:46Z","timestamp":1777395166018,"version":"3.51.4"},"reference-count":38,"publisher":"SAGE Publications","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JAD"],"published-print":{"date-parts":[[2021,3,9]]},"abstract":"<jats:p>Background: Cerebral amyloid angiopathy with related inflammation (CAA-ri) is a rare age-associated disorder characterized by an inflammatory response to amyloid in cerebral blood vessels. CAA-ri is often treated with corticosteroids, but response to treatment is variable. Objective: To assess the relationship between clinical and paraclinical measures and outcomes in patients with CAA-ri treated with high doses of methylprednisolone. Methods: Longitudinal clinical course, and results from serum and cerebrospinal fluid (CSF) testing, electroencephalography, and neuroimaging were reviewed from 11 prospectively-accrued CAA-ri patients diagnosed, treated, and followed at Barnes Jewish Hospital (St. Louis, MO, USA). Magnetic resonance imaging (MRI) changes were quantified using a scoring system validated in cases of amyloid related imaging abnormality (ARIA-E). Clinical outcomes were assessed as change in modified Rankin Scale (\u0394mRS) from baseline to final assessment (median 175 days from treatment with high doses of methylprednisolone; range, 31\u2013513). Results: Worse outcomes following methylprednisolone treatment were associated with requirement for intensive care unit admission (median \u0394mRS, 5 versus 1.5; p\u200a=\u200a0.048), CSF pleocytosis (median \u0394mRS 4.5 versus 1; p\u200a=\u200a0.04), or lower CSF A\u03b240 at presentation (rho\u200a=\u200a\u20130.83; p\u200a=\u200a0.02), and diffusion restriction (median \u0394mRS 4 versus 1.5; p\u200a=\u200a0.03) or higher late ARIA-E scores (rho\u200a=\u200a0.70; p\u200a=\u200a0.02) on MRI, but not preexisting cognitive decline (median \u0394mRS 2 versus 2; p\u200a=\u200a0.66). Conclusion: Clinical and paraclinical measures associated with outcomes may inform clinical counseling and treatment decisions in patients with CAA-ri. Baseline cognitive status was not associated with treatment responsiveness.<\/jats:p>","DOI":"10.3233\/jad-201299","type":"journal-article","created":{"date-parts":[[2021,1,22]],"date-time":"2021-01-22T15:59:56Z","timestamp":1611331196000},"page":"133-142","source":"Crossref","is-referenced-by-count":16,"title":["Clinical and Paraclinical Measures Associated with Outcome in Cerebral Amyloid Angiopathy with Related Inflammation"],"prefix":"10.1177","volume":"80","author":[{"given":"Alan S.","family":"Plotzker","sequence":"first","affiliation":[{"name":"The Charles F. and Joanne Knight Alzheimer Disease Research Center, St. Louis, MO, USA"},{"name":"Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rachel L.","family":"Henson","sequence":"additional","affiliation":[{"name":"The Charles F. and Joanne Knight Alzheimer Disease Research Center, St. Louis, MO, USA"},{"name":"Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anne M.","family":"Fagan","sequence":"additional","affiliation":[{"name":"The Charles F. and Joanne Knight Alzheimer Disease Research Center, St. Louis, MO, USA"},{"name":"Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John C.","family":"Morris","sequence":"additional","affiliation":[{"name":"The Charles F. and Joanne Knight Alzheimer Disease Research Center, St. Louis, MO, USA"},{"name":"Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gregory S.","family":"Day","sequence":"additional","affiliation":[{"name":"Department of Neurology, Mayo Clinic, Jacksonville, FL, 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