Tanaka H, Yuki N, Ohnishi A, Hirata K
Department of Neurology, Dokkyo University School of Medicine, Tochigi, Japan.
No To Shinkei. 1998 Dec;50(12):1107-11.
We report a 59-year-old man with chronic sensorimotor polyneuropathy mimicking chronic inflammatory demyelinating polyneuropathy. Sural nerve biopsy revealed marked loss of myelinated fibers and myelin ovoid formation with vasculitis in the epineurium. Renal biopsy was performed subsequently because of the laboratory findings suspecting systemic vasculitis of which consisted CRP positivity, ESR elevation, perinuclear antineutrophil cytoplasmic antibodies (pANCA), and creatinine clearance reduction. The biopsy specimen revealed segmental necrotizing glomerulonephritis with frequent formation of a crescent. These findings confirmed the diagnosis of microscopic polyangitis. Oral administration of prednisolone with additional azathioprine following ethylprednisolone pulse therapy was effective, and pANCA was not detected anymore. We conclude that vasculitic neuropathy has to be considered as a possible cause of polyneuropathy.
我们报告了一名59岁男性，患有模仿慢性炎症性脱髓鞘性多发性神经病的慢性感觉运动性多发性神经病。腓肠神经活检显示有髓纤维显著丢失，髓鞘卵圆体形成，并伴有神经外膜血管炎。由于实验室检查结果怀疑存在系统性血管炎，包括CRP阳性、ESR升高、核周抗中性粒细胞胞浆抗体（pANCA）以及肌酐清除率降低，随后进行了肾活检。活检标本显示节段性坏死性肾小球肾炎伴新月体频繁形成。这些发现证实了显微镜下多血管炎的诊断。在甲泼尼龙冲击治疗后口服泼尼松龙并加用硫唑嘌呤有效，且不再检测到pANCA。我们得出结论，血管炎性神经病必须被视为多发性神经病的一个可能病因。
