From the Unità Operativa di Reumatologia (C.S.), Azienda Ospedaliera ASMN, Istituto di Ricovero e Cura a Carattere Scientifico, Reggio Emilia, Italy; and Anatomic Pathology, Department of Laboratory Medicine and Pathology (C.G.), Departments of Radiology (J.M.M.) and Neurology (R.D.B.), and Divisions of Biostatistics (T.C.) and Rheumatology (G.G.H.), Mayo Clinic, Rochester, MN.
Neurology. 2013 Oct 29;81(18):1596-603. doi: 10.1212/WNL.0b013e3182a9f545. Epub 2013 Sep 27.
To analyze the clinical findings, response to therapy, and outcomes of patients with cerebral vascular amyloid-β (Aβ) deposition with and without inflammatory vascular infiltration.
We report 78 consecutive patients with cerebral vascular Aβ deposition examined at Mayo Clinic Rochester over 25 years (1987 through 2011). Specimens reviewed by a neuropathologist showed 40 with vascular Aβ peptide without inflammation (cerebral amyloid angiopathy [CAA]), 28 with granulomatous vasculitis (Aβ-related angiitis or ABRA), and 10 with perivascular CAA-related inflammation. We also matched findings in 118 consecutive patients with primary CNS vasculitis (PCNSV) without Aβ seen over 25 years (1983 through 2007).
Compared to the 40 with CAA, the 28 with ABRA were younger at diagnosis (p = 0.05), had less altered cognition (p = 0.02), fewer neurologic deficits (p = 0.02), and fewer intracranial hemorrhages (<0.001), but increased gadolinium leptomeningeal enhancement (p = 0.01) at presentation, and less mortality and disability at last follow-up (p < 0.001). Compared with PCNSV, the 28 patients with ABRA were older at diagnosis (p < 0.001), had a higher frequency of altered cognition (p = 0.05), seizures/spells (p = 0.006), gadolinium leptomeningeal enhancement (p < 0.001), and intracerebral hemorrhage (p = 0.02), lower frequency of hemiparesis (p = 0.01), visual symptoms (p = 0.04), and MRI evidence of cerebral infarction (p = 0.003), but higher CSF protein levels (p = 0.03). Results of treatment and outcomes in ABRA and PCNSV were similar.
ABRA appears to represent a distinct subset of PCNSV.
分析伴有和不伴有炎症性血管浸润的脑血管淀粉样蛋白-β（Aβ）沉积患者的临床发现、治疗反应和结局。
我们报告了在梅奥诊所罗彻斯特分部 25 年间（1987 年至 2011 年）连续检查的 78 例脑血管 Aβ 沉积患者。由神经病理学家检查的标本显示，40 例为无炎症的血管 Aβ 肽（脑淀粉样血管病[CAA]），28 例为肉芽肿性血管炎（Aβ相关血管炎或 ABRA），10 例为血管周围 CAA 相关炎症。我们还对 25 年间（1983 年至 2007 年）连续观察的 118 例无 Aβ 的原发性中枢神经系统血管炎（PCNSV）患者的发现进行了匹配。
与 40 例 CAA 相比，28 例 ABRA 的诊断年龄更小（p = 0.05），认知改变较少（p = 0.02），神经缺损较少（p = 0.02），颅内出血较少（<0.001），但在初次就诊时出现更多的钆脑膜增强（p = 0.01），最后随访时的死亡率和残疾率较低（p < 0.001）。与 PCNSV 相比，28 例 ABRA 的诊断年龄更大（p < 0.001），认知改变、癫痫发作/发作（p = 0.05）、钆脑膜增强（p < 0.001）、脑出血（p = 0.02）的发生率较高，偏瘫（p = 0.01）、视觉症状（p = 0.04）和脑梗死的 MRI 证据（p = 0.003）的发生率较低，CSF 蛋白水平较高（p = 0.03）。ABRA 和 PCNSV 的治疗结果和结局相似。
ABRA 似乎代表了 PCNSV 的一个不同亚群。
