Sempere Angel P, Tahoces Ml, Palao-Duarte Susana, Garcia-Perez Alfonso
Neurology Department, Hospital General Universitario de Alicante, Alicante, Spain.
J Med Case Rep. 2011 Jul 19;5:319. doi: 10.1186/1752-1947-5-319.
Common variable immunodeficiency encompasses a group of heterogeneous conditions linked by a lack of immunoglobulin production and primary antibody failure. Although primary immunodeficiencies are typically characterized by recurrent infections, autoimmune manifestations have increasingly been recognized. Neurological complications are extremely rare and to the best of our knowledge optic neuritis has not been described previously. We report the case of a patient with common variable immunodeficiency who developed loss of vision secondary to bilateral optic neuritis.
A 26-year-old Caucasian man with a diagnosis of common variable immunodeficiency presented to our facility with loss of vision secondary to bilateral optic neuritis. Results of a thorough study for infectious, neoplastic and autoimmune diseases were negative. Our patient was treated with intravenous methylprednisolone with almost complete improvement and he remained asymptomatic at a 12-month follow-up.
Bilateral optic neuritis should be added to the list of autoimmune disorders related to common variable immunodeficiency. If a patient with common variable immunodeficiency experiences loss of vision, the possibility of bilateral optic neuritis should be considered as rapid initiation of high-dose corticosteroids may improve visual recovery.
常见可变免疫缺陷包括一组因免疫球蛋白产生缺乏和原发性抗体缺陷而相互关联的异质性疾病。虽然原发性免疫缺陷通常以反复感染为特征，但自身免疫表现越来越受到认可。神经并发症极为罕见，据我们所知，此前尚未有视神经炎的相关描述。我们报告一例患有常见可变免疫缺陷的患者，其因双侧视神经炎继发视力丧失。
一名26岁诊断为常见可变免疫缺陷的白种男性因双侧视神经炎继发视力丧失前来我院就诊。针对感染性、肿瘤性和自身免疫性疾病的全面检查结果均为阴性。我们的患者接受了静脉注射甲泼尼龙治疗，视力几乎完全恢复，在12个月的随访中无症状。
双侧视神经炎应被列入与常见可变免疫缺陷相关的自身免疫性疾病清单。如果患有常见可变免疫缺陷的患者出现视力丧失，应考虑双侧视神经炎的可能性，因为迅速开始大剂量皮质类固醇治疗可能会改善视力恢复。
