Gluten sensitivity in sporadic and hereditary cerebellar ataxia
Gluten sensitivity in sporadic and hereditary cerebellar ataxia
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DOI：
10.1002/ana.108.abs
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发表时间：
2001-04-01
影响因子：
11.2
通讯作者：
Hallett, M
中科院分区：
文献类型：
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作者：
Bushara, KO;Goebel, SU;Hallett, M
麸质敏感性，伴或不伴经典乳糜泻症状和肠道病理，已被认为是散发性小脑性共济失调的潜在可治疗原因。在这里，我们研究了50例小脑性共济失调患者中异常高的血清免疫球蛋白A （IgA）和IgG抗麦胶蛋白抗体滴度和典型的人淋巴细胞抗原（HLA）基因型的患病率，并对这些患者进行了分子特征的遗传性共济失调检测。在散发性共济失调患者（7/26;27%）和常染色体显性共济失调患者（9/24;37%）中发现谷蛋白敏感性的高患病率，包括已知共济失调基因型的患者，这表明迄今为止尚未认识到遗传性共济失调与谷蛋白敏感性之间的关联。需要进一步的研究来确定麸质敏感性是否有助于遗传性小脑共济失调患者的小脑变性。遗传性共济失调患者（包括已知共济失调基因型的无症状患者）应考虑筛查麸质敏感性和无麸质饮食试验。
Gluten sensitivity, with or without classical celiac disease symptoms and intestinal pathology, has been suggested as a potentially treatable cause of sporadic cerebellar ataxia. Here, we investigated the prevalence of abnormally high serum immunoglobulin A (IgA) and IgG anti-gliadin antibody titers and typical human lymphocyte antigen (HLA) genotypes in 50 patients presenting with cerebellar ataxia who were tested for molecularly characterized hereditary ataxias. A high prevalence of gluten sensitivity was found in patients with sporadic (7/26; 27%) and autosomal dominant (9/24; 37%) ataxias, including patients with known ataxia genotypes indicating a hitherto unrecognized association between hereditary ataxias and gluten sensitivity. Further studies are needed to determine whether gluten sensitivity contributes to cerebellar degeneration in patients with hereditary cerebellar ataxia. Patients with hereditary ataxia (including asymptomatic patients with known ataxia genotype) should be considered for screening for gluten sensitivity and gluten-free diet trials.