Uesugi Y, Tanno Y, Tanaka M, Tanaka K, Suzuki T, Inuzuka T, Ishikawa T, Toba K, Koike T, Shibata A
Department of Neurology, Niigata University, Japan.
No To Shinkei. 1993 Aug;45(8):773-6.
A 16-year old woman with LGL leukemia developed peripheral neuropathy. She showed virus-associated hemophagocytic syndrome (VAHS)-like signs including high fever, liver dysfunction, huge splenomegaly, hepatomegaly and pancytopenia. The presence of chronic active EB virus infection was proved by marked high titers for IgG and IgA antibodies to the Epstein-Barr viral capsid and early antigens and low titers of antibody to Epstein-Barr nuclear antigens. She showed dysesthesia and paresthesia of bilateral lower extremities with marked swelling and tenderness, and later developed muscular weakness and atrophy with areflexia of lower extremities. Findings of the central nervous system dysfunction were not observed except for the acceleration of jaw jerk. Pleocytosis and increased protein levels in the cerebrospinal fluid were found. Pulse therapy of methyl-prednisolone and high dose intravenous immunoglobulin therapy (20 g/day for 3 days) were effective for neurological findings. The increased neopterin in the cerebrospinal fluid suggested that peripheral neuropathy was caused by activated macrophages.
一名患有大颗粒淋巴细胞白血病的16岁女性出现了周围神经病变。她表现出病毒相关噬血细胞综合征（VAHS）样症状，包括高热、肝功能障碍、巨大脾肿大、肝肿大和全血细胞减少。通过针对爱泼斯坦 - 巴尔病毒衣壳和早期抗原的IgG和IgA抗体显著高滴度以及针对爱泼斯坦 - 巴尔核抗原的抗体低滴度，证实存在慢性活动性EB病毒感染。她表现出双下肢感觉异常和感觉异常，伴有明显肿胀和压痛，随后出现肌肉无力和萎缩，下肢无反射。除下颌反射亢进外，未观察到中枢神经系统功能障碍的表现。脑脊液中发现淋巴细胞增多和蛋白质水平升高。甲基强的松龙脉冲疗法和高剂量静脉注射免疫球蛋白疗法（20 g/天，共3天）对神经学表现有效。脑脊液中蝶呤增加表明周围神经病变是由活化的巨噬细胞引起的。
