PDF(Pubmed)
Abstract：
A man in his 60s, known with multiple sclerosis, presented with seizures and paresis of the left arm and leg. Brain imaging showed a white matter lesion, right parietal, which was progressive over the last 6 years and not typical for multiple sclerosis. Brain biopsy showed a B-cell infiltrate with IgA lambda monotypic plasma cell differentiation and amyloid deposits, typed as lambda immunoglobulin light chain (AL). Bone marrow biopsy and PET/CT ruled out a systemic lymphoma. Extended history taking, blood and urine testing (including cardiac biomarkers) identified no evidence of systemic amyloidosis-induced organ dysfunction.Primary cerebral AL amyloidoma is a very rare entity where optimal treatment is difficult to assess. The patient was treated with locally applied volumetric modulated arc radiotherapy, 24 Gy, divided in 12 fractions. Afterwards, the paresis of the left arm partially resolved, and the function of the left leg improved. Seizures did not occur anymore.
摘要：
一个60多岁的男人，已知患有多发性硬化症，出现左臂和腿的癫痫和轻瘫。脑成像显示白质病变,右顶骨,在过去的6年里，这是渐进的，并不典型的多发性硬化症。脑活检显示B细胞浸润，IgAλ单型浆细胞分化和淀粉样蛋白沉积，类型为λ免疫球蛋白轻链(AL)。骨髓活检和PET/CT排除了全身性淋巴瘤。延长历史记录，血液和尿液检测(包括心脏生物标志物)未发现系统性淀粉样变性引起的器官功能障碍的证据.原发性脑AL淀粉样瘤是一种非常罕见的实体，难以评估最佳治疗方法。该患者接受了局部应用体积调强电弧放射治疗，24Gy,分成12个分数。之后,左臂的轻瘫部分解决，左腿的功能得到改善。癫痫发作不再发生。
