Primary angiitis of the central nervous system associated with cerebral amyloid angiopathy
Primary angiitis of the central nervous system associated with cerebral amyloid angiopathy
复制标题
与脑淀粉样血管病相关的中枢神经系统原发性血管炎
作者：
N. Fountain;D. Eberhard
文章摘要-早期诊断对于有效治疗原发性中枢神经系统血管炎（PACNS）至关重要，但脑血管淀粉样血管病（CAA）的存在可能使病理诊断复杂化，因为非血管炎性炎症反应可伴随CAA。我们报告了两例与CAA相关的PACNS患者，在皮质类固醇/环磷酰胺联合治疗期间症状停止进展。1例患者有明显的嗜酸性血管炎和嗜酸性CSF细胞增多。基于对报告病例的审查，支持这些患者诊断为症状性血管炎的特征包括亚急性进展的精神状态变化和多灶性缺陷、ESR和CSF蛋白升高以及影像学检查显示的多灶性非出血性病变。我们的结论是，合并症（PACNS/CAA）是类似的PACNS，可能发生的频率比预期的巧合。CAA的存在不应改变出现症状和实验室研究结果与PACNS一致的患者的治疗策略。神经病学1996;46：190-197
Article abstract-Early diagnosis is essential for the effective management of primary angiitis of the CNS (PACNS), but the presence of cerebrovascular amyloid angiopathy (CAA) may complicate the pathologic diagnosis since nonvasculitic inflammatory reactions can accompany CAA. We report two patients with PACNS associated with CAA in whom the progression of symptoms ceased during combined corticosteroid/cyclophosphamide therapy. One patient had prominent eosinophilic vasculitis and eosinophilic CSF pleocytosis. Based on review of reported cases, features supporting the diagnosis of symptomatic vasculitis in these patients include subacute progression of mental status changes and multifocal deficits, elevated ESR and CSF protein, and multifocal nonhemorrhagic lesions on imaging studies. We conclude that combined disease (PACNS/CAA) is similar to PACNS and probably occurs more frequently than expected by coincidence. The presence of CAA should not alter the treatment strategy in patients presenting with symptoms and laboratory studies consistent with PACNS. NEUROLOGY 1996;46: 190-197
脑淀粉样血管病和肉芽肿性血管炎：使用阿尔茨海默 A4 肽抗体进行免疫组织化学研究。
影响因子：
3.3
作者：
Gray,F;Vinters,HV;LeNoan,H;Salama,J;Delaporte,P;Poirier,J
通讯作者：
Poirier,J
DOI：
10.1073/pnas.89.21.10075
发表时间：
1992-11-01
影响因子：
11.1
作者：
BUXBAUM, JD;OISHI, M;GREENGARD, P
通讯作者：
GREENGARD, P
DOI：
10.1073/pnas.86.19.7606
发表时间：
1989-10-01
影响因子：
11.1
作者：
GOLDGABER, D;HARRIS, HW;GAJDUSEK, DC
通讯作者：
GAJDUSEK, DC