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首页> 外文期刊>Archives of Neurology >Cerebellar ataxia with anti-glutamic acid decarboxylase antibodies: study of 14 patients.
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Cerebellar ataxia with anti-glutamic acid decarboxylase antibodies: study of 14 patients.

机译：小脑性共济失调与anti-glutamic酸脱羧酶抗体:研究14例。

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BACKGROUND: Antibodies to glutamic acid decarboxylase (GAD-Ab) are described in patients with insulin-dependent (type 1) diabetes mellitus (IDDM), in stiff-man syndrome, and, recently, in a few patients with cerebellar ataxia. OBJECTIVES: To show a link between GAD-Ab and some patients with cerebellar ataxia and to clarify their clinical and immunologic profiles. METHODS: Serum samples were selected from 9000 samples of 4 laboratories. The selection criterion was an immunohistochemical pattern compatible with GAD-Ab that was confirmed by radioimmunoassay. We identified 22 patients with stiff-man syndrome and 14 with cerebellar ataxia and GAD-Ab. RESULTS: Thirteen of the 14 patients with cerebellar ataxia and GAD-Ab were women, and 11 had late-onset IDDM. Patients did not have clinical or radiologic evidence of brainstem involvement. Ten patients had oligoclonal IgG bands in the cerebrospinal fluid, and intrathecal GAD-Ab synthesis was observed in 5 of the 6 patients studied. The level of GAD-Ab of these patients was similar to those with stiff-man syndrome and significantly higher than those with IDDM or with polyendocrine autoimmunity (P<.001). However, the GAD-Ab levels of 6 of the 9 patients with polyendocrine autoimmunity overlapped with those of patients with cerebellar ataxia. CONCLUSIONS: These results suggest a link between high level of GAD-Ab and some cases of cerebellar ataxia, particularly women with IDDM. If high serum levels of GAD-Ab are detected, the cerebrospinal fluid should be evaluated for the presence of oligoclonal IgG bands and intrathecal synthesis of GAD-Ab to further prove an autoimmune origin of the syndrome.
机译：背景:抗体谷氨酸脱羧酶(GAD-Ab)中描述的病人与胰岛素依赖型糖尿病(1型)(IDDM) stiff-man综合症,最近,在少数患者小脑性共济失调。目的:显示GAD-Ab和之间的联系一些病人和小脑性共济失调澄清他们的临床和免疫概要文件。方法:血清样本选自9000年4实验室样品。标准是一个免疫组织化学模式兼容GAD-Ab证实了放射免疫检定法。stiff-man与小脑性共济失调综合征和14和GAD-Ab。小脑性共济失调和GAD-Ab是女性11日晚IDDM。临床和放射脑干的证据参与。乐队在脑脊液和鞘内观察GAD-Ab合成的5 6患者进行了研究。病人与stiff-man类似综合症,明显高于那些IDDM或polyendocrine自身免疫(P <措施)。然而,GAD-Ab水平的6 9例与polyendocrine自身免疫重叠小脑性共济失调患者。结论:这些结果表明之间的联系高水平的GAD-Ab和小脑的某些情况下共济失调,特别是IDDM的女性。检测到血清水平的GAD-Ab,脑脊液应该评估的寡克隆免疫球蛋白乐队和鞘内的存在合成GAD-Ab进一步证明自身免疫性的综合症。

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