Reik L, Korn J H
Ann Neurol. 1981 Nov;10(5):488-90. doi: 10.1002/ana.410100514.
A 54-year-old man with essential mixed cryoglobulinemia developed a diffuse and focal encephalopathy while systemic vasculitis was active and serum cryoglobulin was markedly increased. No other cause for his encephalopathy could be determined and the diagnosis of cryoglobulinemic cerebral vasculitis was made. Treatment with plasma exchange, prednisone, and cyclophosphamide resulted in clearing of the encephalopathy and reduction in serum cryoglobulin. The onset of improvement was temporally related to the initiation of plasma exchange. Plasma exchange may have a role in the treatment of other patients with life-threatening immune complex diseases involving the nervous system.
一名患有原发性混合性冷球蛋白血症的54岁男性，在全身血管炎活动且血清冷球蛋白显著升高时，出现了弥漫性和局灶性脑病。未发现其他导致其脑病的原因，遂诊断为冷球蛋白血症性脑血管炎。采用血浆置换、泼尼松和环磷酰胺治疗后，脑病症状消失，血清冷球蛋白水平降低。病情改善的起始时间与开始血浆置换的时间相关。血浆置换可能对治疗其他患有危及生命的累及神经系统的免疫复合物疾病的患者有作用。
