Division of Pediatric Neurology, Department of Pediatrics, Medical Faculty, Uludag University, Bursa, Turkey.
Pediatr Neurol. 2012 Apr;46(4):260-2. doi: 10.1016/j.pediatrneurol.2012.02.009.
Common variable immunodeficiency comprises a heterogeneous group of primary antibody deficiencies with complex clinical and immunologic phenotypes. Immune dysregulation leads to the generation of multiple autoantibodies against various antigenic targets in patients with common variable immunodeficiency. Chronic inflammatory demyelinating polyneuropathy is a heterogeneous disorder that indicates an autoimmune response against peripheral nerve myelin. We describe a 7-year-old girl with common variable immunodeficiency who developed chronic inflammatory polyneuropathy. A 5-day course of intravenous immunoglobulin (500 mg/kg/day) improved her neurologic disorder. Chronic inflammatory demyelinating polyneuropathy should be added to the broadening spectrum of neurologic complications in common variable immunodeficiency. Early detection and consequent treatment may reverse the neurologic sequelae.
普通变异性免疫缺陷症包括一组具有复杂临床和免疫表型的原发性抗体缺陷症。免疫失调导致普通变异性免疫缺陷症患者产生针对多种抗原靶标的多种自身抗体。慢性炎症性脱髓鞘性多发性神经病是一种异质性疾病，表明对周围神经髓鞘的自身免疫反应。我们描述了一例 7 岁女孩患有普通变异性免疫缺陷症，发展为慢性炎症性多发性神经病。静脉注射免疫球蛋白（500mg/kg/天）5 天疗程改善了她的神经功能障碍。慢性炎症性脱髓鞘性多发性神经病应添加到普通变异性免疫缺陷症的神经并发症扩展谱中。早期发现和随后的治疗可能逆转神经后遗症。
