关键词: GAD IVIg autoimmune retinopathy stiff-leg syndrome stiff-person syndrome

来  源:   DOI:10.3390/brainsci13101361   PDF(Pubmed)

Abstract:
Antibodies to glutamic acid decarboxylase (GAD) have been predominantly associated with stiff-person syndrome (SPS), which is often accompanied by organ-specific autoimmune diseases, such as late-onset type 1 diabetes. Autoimmune retinal pathology in SPS has recently been suggested to coexist in patients suffering from this disease; however, evidence reporting potential treatment options for the neurological and visual symptoms these patients experience remains scarce. We provide a review of the relevant literature, presenting a rare case of a middle-aged woman with autoimmune retinopathy (AIR) followed by stiff-leg syndrome who responded to intravenous immune globulin treatment (IVIg). Our report adds to previously reported data supporting the efficacy of IVIg in SPS spectrum disorders while also proposing the potential effect of IVIg in treating SPS spectrum patients with coexisting AIR.
摘要:
谷氨酸脱羧酶(GAD)的抗体主要与僵硬的人综合征(SPS)有关,通常伴有器官特异性自身免疫性疾病,如晚发性1型糖尿病。SPS中的自身免疫性视网膜病理学最近被认为在患有这种疾病的患者中共存;然而,报道这些患者所经历的神经和视觉症状的潜在治疗方案的证据仍然很少.我们提供了相关文献的回顾,一例罕见的中年妇女患有自身免疫性视网膜病变(AIR),随后是对静脉免疫球蛋白治疗(IVIg)有反应的僵硬腿综合征。我们的报告增加了先前报道的数据,支持IVIg在SPS频谱障碍中的疗效,同时还提出了IVIg在治疗同时存在AIR的SPS频谱患者中的潜在作用。
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