脑淀粉样血管病:多种神经痛问题

P. Gilbert
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摘要

一位有偏头痛病史的72岁女性,表现为急性表达性失语症、记忆困难和头痛恶化。对大脑进行了核磁共振成像,显示弥漫性白质T2高信号(图1)。由于患者的临床症状恶化,四天后进行了重复MRI检查,发现多处微出血(图2),以及左颞叶叶出血(图2中)。一项广泛的检查,包括HIV检测、包括蛋白14-3-3和脱髓鞘疾病在内的感染性病因的CSF检查均为阴性。副肿瘤和自身免疫检查也是非诊断性的。由于广泛的白质疾病,进行了脑活检,发现脑淀粉样血管病(CAA)伴局灶性肉芽肿性血管炎。患者接受了静脉注射类固醇治疗,但临床上没有明显改善。确诊两个月后,她的病程一直保持不变,没有好转或恶化。
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Cerebral Amyloid Angiopathy: Multiple neurolgic problems
A 72-Year-old female with a history of migraine headaches presented with an acute onset of expressive aphasia, difficulty with memory and worsening of her headaches. An MRI of the brain was done which revealed diffuse white matter T2 hyperintensities (Figures 1). Due to worsening of the patient’s clinical symptoms a repeat MRI was performed four days later that revealed multiple micro-bleeds (Figure 2), as well as a lobar hemorrhage in left temporal lobe (Figure 2). An extensive workup including HIV testing, CSF examination for infectious etiology including protein 14-3-3 and demylineating disease was negative. Paraneoplastic and autoimmune workup was also non-diagnostic. A brain biopsy was performed due to the extensive white matter disease, which revealed Cerebral Amyloid Angiopathy (CAA) with focal granulomatous angiitis. The patient was treated with intravenous steroids with no significant improvement clinically. Two months after diagnosis, her disease course has remained static, without improvement or deterioration.
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