背景:脑淀粉样血管病相关炎症(CAA-ri)是一种实体,其特征是对脑微血管壁中β-淀粉样蛋白沉积的炎症反应。
方法:我们根据组织病理学研究结果或临床-放射学诊断标准,对一系列诊断为CAA-ri的患者进行了回顾性回顾。
结果:该研究包括7名患者(5名男性),平均年龄为79岁。6例患者发病为急性或亚急性。最常见的症状是认知障碍(n=6),行为改变(n=5),癫痫发作(n=5),局灶性神经体征(n=4),头痛(n=2)。3例患者脑脊液异常(淋巴细胞增多和高蛋白质水平)。最常见的MRI表现是微出血(n=7),T2-FLAIR序列上的皮质下白质高信号(n=7),和软脑膜增强(n=6)。3例患者的病变为双侧病变,最常累及顶枕区(n=5)。对2例患者进行了淀粉样蛋白PET研究,其中一人表现出病理结果。两名患者接受了脑活检,证实诊断。所有患者均接受免疫抑制治疗。在所有病例中观察到最初良好的临床放射学反应,2例患者在停药后出现放射学复发,恢复治疗后随后的改善。
结论:早期诊断CAA-ri至关重要:早期治疗已被证明可以改善预后并降低复发风险。尽管需要进行组织病理学研究来确认诊断,临床放射学标准无需活检即可进行诊断。
BACKGROUND: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is an entity characterised by an inflammatory response to β-amyloid deposition in the walls of cerebral microvessels.
METHODS: We conducted a retrospective review of a series of patients with a diagnosis of CAA-ri according to histopathological study findings or clinical-radiological diagnostic criteria.
RESULTS: The study included 7 patients (5 men) with a mean age of 79 years. Disease onset was acute or subacute in 6 patients. The most frequent symptoms were cognitive impairment (n = 6), behavioural alterations (n = 5), epileptic seizures (n = 5), focal neurological signs (n = 4), and headache (n = 2). Cerebrospinal fluid was abnormal in 3 patients (lymphocytic pleocytosis and high protein levels). The most frequent MRI findings were
microbleeds (n = 7), subcortical white matter hyperintensities on T2-FLAIR sequences (n = 7), and leptomeningeal enhancement (n = 6). Lesions were bilateral in 3 patients and most frequently involved the parieto-occipital region (n = 5). Amyloid PET studies were performed in 2 patients, one of whom showed pathological findings. Two patients underwent brain biopsy, which confirmed diagnosis. All patients received immunosuppressive therapy. An initially favourable clinical-radiological response was observed in all cases, with 2 patients presenting radiological recurrence after treatment withdrawal, with a subsequent improvement after treatment was resumed.
CONCLUSIONS: Early diagnosis of CAA-ri is essential: early treatment has been shown to improve prognosis and reduce the risk of recurrence. Although a histopathological study is needed to confirm diagnosis, clinical-radiological criteria enable diagnosis without biopsy.