Autoantibody targeting of brain and intestinal transglutaminase in gluten ataxia
Autoantibody targeting of brain and intestinal transglutaminase in gluten ataxia
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DOI：
10.1212/01.wnl.0000196480.55601.3a
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发表时间：
2006-02-14
期刊：
影响因子：
9.9
通讯作者：
Korponay-Szabó, IR
中科院分区：
文献类型：
--
作者：
Hadjivassiliou, M;Mäki, M;Korponay-Szabó, IR
目的：研究谷蛋白共济失调患者和对照组空肠组织和大脑中是否存在抗2型组织转氨酶（TG 2;谷蛋白敏感性全谱的可靠标志物）的自身抗体沉积。研究方法：作者使用双色免疫荧光法评估了9例谷蛋白共济失调患者和7例其他原因共济失调患者的空肠活检样本中是否存在TG 2相关免疫球蛋白沉积。尸检脑组织从一个病人与谷蛋白共济失调和一个神经系统完整的大脑进行了研究。结果：在所有谷蛋白共济失调患者的空肠组织中均发现空肠TG 2上的伊加沉积，而对照组中无IgA沉积。肠道伊加沉积模式与显性和潜伏性乳糜泻患者以及疱疹样皮炎患者相似。广泛的伊加沉积血管周围被发现在大脑中的患者与谷蛋白共济失调，而不是控制大脑。沉积最明显的是在小脑，脑桥和延髓。结论：抗组织转氨酶伊加抗体存在于谷蛋白共济失调伴或不伴肠病的患者的肠道和大脑中，其方式与乳糜泻、潜伏性乳糜泻和疱疹样皮炎患者相似，但不存在于共济失调对照受试者中。这一发现加强了以下论点：麸质共济失调是免疫介导的，与乳糜泻和疱疹样皮炎属于相同的麸质敏感性谱。
Objective: To investigate the presence of autoantibody deposition against type 2 tissue transglutaminase (TG2; a reliable marker of the whole spectrum of gluten sensitivity) in the jejunal tissue and brain of patients with gluten ataxia and in control subjects. Methods: The authors evaluated jejunal biopsy samples from nine patients with gluten ataxia and seven patients with other causes of ataxia for the presence of TG2-related immunoglobulin deposits using double-color immunofluorescence. Autopsy brain tissue from one patient with gluten ataxia and one neurologically intact brain were also studied. Results: IgA deposition on jejunal TG2 was found in the jejunal tissue of all patients with gluten ataxia and in none of the controls. The intestinal IgA deposition pattern was similar to that seen in patients with overt and latent celiac disease and in those with dermatitis herpetiformis. Widespread IgA deposition around vessels was found in the brain of the patient with gluten ataxia but not the control brain. The deposition was most pronounced in the cerebellum, pons, and medulla. Conclusions: Anti-tissue transglutaminase IgA antibodies are present in the gut and brain of patients with gluten ataxia with or without an enteropathy in a similar fashion to patients with celiac disease, latent celiac disease, and dermatitis herpetiformis but not in ataxia control subjects. This finding strengthens the contention that gluten ataxia is immune mediated and belongs to the same spectrum of gluten sensitivity as celiac disease and dermatitis herpetiformis.