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Abstract Details

Predictors of treatment-responsiveness in cerebral amyloid angiopathy with related inflammation
Aging, Dementia, and Behavioral Neurology
Aging and Dementia Posters (7:00 AM-5:00 PM)
071
To explore clinically-accessible associations of treatment-responsiveness, we assessed the relationship between prospectively-measured clinical and paraclinical features and outcomes in patients with cerebral amyloid angiopathy with related inflammation (CAA-ri) treated with high doses of methylprednisolone.
 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.
Longitudinal clinical course, and results from serum and CSF testing, electroencephalography and neuroimaging were reviewed from 11 prospectively-accrued CAA-ri patients diagnosed, treated and followed at a tertiary care center (Washington University School of Medicine; St. Louis, MO). 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 (ΔmRS) from baseline to final assessment (median 175 days from treatment with high doses of methylprednisolone; range, 31-513).
Worse outcomes following methylprednisolone treatment were associated with requirement for intensive care unit admission (median ΔmRS, 5 vs. 1.5; p=0.048), CSF pleocytosis (median ΔmRS 4.5 vs. 1; p=0.04) or lower CSF Aβ40 at presentation (rho=-0.83; p=0.02), and diffusion restriction (median ΔmRS 4 vs 1.5; p=0.03) or higher late ARIA-E scores (rho=0.70; p=0.02) on MR neuroimaging, but not preexisting cognitive decline (median ΔmRS 2 vs 2; p=0.66).
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 suggesting that patients with preexisting dementia may still benefit from treatment of CAA-ri.
Authors/Disclosures
Alan Plotzker, MD (UPP Neurology Department)
PRESENTER 		Dr. Plotzker has nothing to disclose.
Rachel Henson (Washington University in St. Louis) No disclosure on file
No disclosure on file
John C. Morris, MD, FÂ鶹´«Ã½Ó³»­ (Washington University) Dr. Morris has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for CBR International Advisory Board. Dr. Morris has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cure Alzheimers Fund. Dr. Morris has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for LEADS Steering Commitee. The institution of Dr. Morris has received research support from NIH grants. Dr. Morris has received intellectual property interests from a discovery or technology relating to health care. Dr. Morris has received intellectual property interests from a discovery or technology relating to health care.
Gregory S. Day, MD, MSc, FÂ鶹´«Ã½Ó³»­ (Mayo Clinic) Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys Therapeutics. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for DynaMed (EBSCO Health). Dr. Day has or had stock in ANI Pharmaceuticals. The institution of Dr. Day has received research support from National Institutes of Health / NIA. The institution of Dr. Day has received research support from National Institutes of Health / NINDS. The institution of Dr. Day has received research support from Amgen Pharmaceuticals. The institution of Dr. Day has received research support from AVID Radiopharmaceuticals. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Presenter at Annual Meeting (CME) with Â鶹´«Ã½Ó³»­. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Content Development (CME) with PeerView, Inc. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Content Development (CME) with Continuing Â鶹´«Ã½Ó³»­, Inc. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Content Development (CME) with Ionis Pharmaceuticals. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Â鶹´«Ã½Ó³»­al Case Development + Presentation (video) with PeerDirect (P\S\L Group). Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Content Development / Presentation (non-CME) with MJH Life Sciences (NeurologyLive). Dr. Day has a non-compensated relationship as a Clinical Director with Anti-NMDA Receptor Encephalitis Foundation that is relevant to Â鶹´«Ã½Ó³»­ interests or activities.