Department of Neurology, Pusan National University School of Medicine, Busan, Korea.
J Korean Med Sci. 2010 Jul;25(7):1101-4. doi: 10.3346/jkms.2010.25.7.1101. Epub 2010 Jun 16.
We report a patient with Lewis-Sumner syndrome (LSS) who showed an improvement only with plasma exchange (PE). The patient, 32-yr old man, had progressive multifocal motor-sensory deficits with persistent, multiple conduction blocks and marked slowing of NCVs. Nerve pathology supported a diagnosis of demyelinating neuropathy by revealing marked loss of myelinated fibers with inter- and intrafascicular variation. Although the patient was refractory to treatment with corticosteroid and intravenous immunoglobulin, PE produced a dramatic improvement. Our experience strongly proposes that PE should be tried for refractory LSS.
我们报告了一例刘易斯-萨姆纳综合征（LSS）患者，仅接受血浆置换（PE）治疗后病情改善。患者为 32 岁男性，表现为进行性多发性运动感觉神经功能障碍，持续存在多发性传导阻滞，NCV 明显减慢。神经病理学显示有髓纤维明显丢失，伴有束间和束内变异，支持脱髓鞘神经病的诊断。尽管患者对皮质类固醇和静脉注射免疫球蛋白治疗无反应，但 PE 治疗产生了显著改善。我们的经验强烈提示，对于难治性 LSS 应尝试 PE 治疗。
