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常见可变免疫缺陷(CVID)的中枢神经系统影响及模仿:一例报告并文献综述

The Central Nervous System Effects and Mimicry of Common Variable Immunodeficiency (CVID): A Case Report with Literature Review.

作者信息

Farshad Sohail, Figueroa Rodriguez Fernando, Halalau Alexandra, Skender Joseph, Rasmussen Cory, Pevzner Martin

机构信息

Internal Medicine, Beaumont Hospital, Royal Oak 48073, MI, USA.

Rheumatology, Beaumont Hospital, Royal Oak 48073, MI, USA.

出版信息

Case Rep Rheumatol. 2019 Sep 29;2019:7623643. doi: 10.1155/2019/7623643. eCollection 2019.

Abstract

There is a scarceness of information on the central nervous system effects of common variable immunodeficiency (CVID). A 30-year-old woman with a history of recurrent upper respiratory infections, vitiligo, and immune thrombocytopenic purpura presented with right-sided numbness. Magnetic resonance imaging (MRI) of the thoracic spine revealed a signal hyperintensity. MRI of the brain demonstrated FLAIR hyperintensity in the right middle frontal gyrus. Cerebral spinal fluid was unremarkable. Serum immunoglobulins revealed hypogammaglobulinemia. Endobronchial and subsequent mediastinum biopsies were all negative for sarcoidosis and malignancy. No infectious etiology was found. She was treated with glucocorticoids and intravenous immunoglobulin (IVIG) replacement therapy for CVID-associated myelitis. Follow-up MRI showed improvement; however, her numbness persisted despite these treatments, which led to an outside physician adding methotrexate for their suspicion of sarcoidosis. Her symptoms remained stable for two years, but when the methotrexate dose was weaned, her numbness worsened. Upon review, the treatment team refuted the diagnosis of sarcoidosis but continued treatment with prednisone, IVIG, and methotrexate for CVID-associated myelitis, from which her symptoms have stabilized. Here, we discuss CVID-associated neurological complications, its similarities to sarcoidosis, and a literature review with treatment regimens and outcomes.

摘要

关于普通可变免疫缺陷(CVID)对中枢神经系统影响的信息匮乏。一名30岁女性,有反复上呼吸道感染、白癜风和免疫性血小板减少性紫癜病史,出现右侧麻木。胸椎磁共振成像(MRI)显示信号高强化。脑部MRI显示右侧额中回FLAIR高强化。脑脊液检查无异常。血清免疫球蛋白显示低丙种球蛋白血症。支气管内及随后的纵隔活检均未发现结节病和恶性肿瘤。未发现感染病因。她因CVID相关脊髓炎接受了糖皮质激素和静脉注射免疫球蛋白(IVIG)替代治疗。随访MRI显示有所改善;然而,尽管进行了这些治疗,她的麻木仍持续存在,这导致外部医生因怀疑结节病而加用甲氨蝶呤。她的症状稳定了两年,但当甲氨蝶呤剂量逐渐减停时,她的麻木加重。经复查,治疗团队否定了结节病的诊断,但继续使用泼尼松、IVIG和甲氨蝶呤治疗CVID相关脊髓炎,此后她的症状已稳定。在此,我们讨论CVID相关的神经系统并发症、其与结节病的相似之处,并对治疗方案和结果进行文献综述。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/883b/6791247/22683a48ea23/CRIRH2019-7623643.001.jpg

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