The Charles F. and Joanne Knight Alzheimer Disease Research Center, St. Louis, MO, USA.
Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA.
J Alzheimers Dis. 2021;80(1):133-142. doi: 10.3233/JAD-201299.
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.
To assess the relationship between clinical and paraclinical measures and outcomes in patients with CAA-ri treated with high doses of methylprednisolone.
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 (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 versus 1.5; p = 0.048), CSF pleocytosis (median ΔmRS 4.5 versus 1; p = 0.04), or lower CSF Aβ40 at presentation (rho = -0.83; p = 0.02), and diffusion restriction (median ΔmRS 4 versus 1.5; p = 0.03) or higher late ARIA-E scores (rho = 0.70; p = 0.02) on MRI, but not preexisting cognitive decline (median ΔmRS 2 versus 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.
与炎症相关的脑淀粉样血管病（CAA-ri）是一种罕见的与年龄相关的疾病，其特征是血管淀粉样蛋白引起的炎症反应。CAA-ri 常采用皮质类固醇治疗，但治疗效果因人而异。
评估 CAA-ri 患者接受大剂量甲基强的松龙治疗后的临床和临床前指标与结局的关系。
对 11 例在巴恩斯犹太医院（美国密苏里州圣路易斯）确诊、治疗和随访的 CAA-ri 患者的纵向临床病程及血清和脑脊液（CSF）检测、脑电图和神经影像学结果进行回顾性分析。采用经淀粉样相关影像异常（ARIA-E）病例验证的评分系统对磁共振成像（MRI）变化进行量化。临床结局评估为从基线到最终评估时改良 Rankin 量表（mRS）的变化（中位数为接受大剂量甲基强的松龙治疗后 175 天；范围 31-513 天）。
接受甲基强的松龙治疗后结局较差与需要入住重症监护病房（mRS 中位数分别为 5 分和 1.5 分；p = 0.048）、CSF 细胞增多症（mRS 中位数分别为 4.5 分和 1 分；p = 0.04）或更低的 CSF Aβ40 水平（rho = -0.83；p = 0.02）、弥散受限（mRS 中位数分别为 4 分和 1.5 分；p = 0.03）或更高的晚期 ARIA-E 评分（rho = 0.70；p = 0.02）相关，但与基线认知下降无关（mRS 中位数分别为 2 分和 2 分；p = 0.66）。
与结局相关的临床和临床前指标可为 CAA-ri 患者的临床咨询和治疗决策提供依据。基线认知状态与治疗反应无关。
