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伴有谷氨酸脱羧酶抗体的免疫治疗反应性边缘叶脑炎

Immunotherapy-responsive limbic encephalitis with antibodies to glutamic acid decarboxylase.

作者信息

Markakis Ioannis, Alexopoulos Harry, Poulopoulou Cornelia, Akrivou Sofia, Papathanasiou Athanasios, Katsiva Vassiliki, Lyrakos Georgios, Gekas Georgios, Dalakas Marinos C

机构信息

Department of Neurology, "St. Panteleimon" General State Hospital, 3 Mantouvalou St., 18454 Nikaia, Greece.

Neuroimmunology Unit, Department of Pathophysiology, Medical School, University of Athens, 75 Mikras Asias St., 11527 Athens Greece.

出版信息

J Neurol Sci. 2014 Aug 15;343(1-2):192-4. doi: 10.1016/j.jns.2014.05.032. Epub 2014 May 27.

DOI:10.1016/j.jns.2014.05.032
PMID:24950904
Abstract

Glutamic acid decarboxylase (GAD) has been recently identified as a target of humoral autoimmunity in a small subgroup of patients with non-paraneoplastic limbic encephalitis (NPLE). We present a patient with NPLE and positive anti-GAD antibodies who showed significant improvement after long-term immunotherapy. A 48-year old female was admitted with a two-year history of anterograde amnesia and seizures. Brain MRI revealed bilateral lesions of medial temporal lobes. Screening for anti-neuronal antibodies showed high anti-GAD titers in both serum and cerebrospinal fluid (CSF) with strong evidence of intrathecal production. The patient received treatment with prednisolone and long-term plasma exchange. During a 12-month follow-up, she exhibited complete seizure remission and an improvement in memory and visuo-spatial skills. Anti-GAD antibodies may serve as a useful marker to identify a subset of NPLE patients that respond to immunoregulatory treatment.

摘要

谷氨酸脱羧酶(GAD)最近在一小部分非副肿瘤性边缘叶脑炎(NPLE)患者中被确定为体液自身免疫的靶点。我们报告了一名患有NPLE且抗GAD抗体呈阳性的患者,该患者在长期免疫治疗后有显著改善。一名48岁女性因顺行性失忆和癫痫发作两年入院。脑部MRI显示双侧颞叶内侧病变。抗神经元抗体筛查显示血清和脑脊液(CSF)中抗GAD滴度均很高,并有鞘内产生的有力证据。该患者接受了泼尼松龙治疗和长期血浆置换。在12个月的随访中,她癫痫完全缓解,记忆力和视觉空间技能有所改善。抗GAD抗体可能是识别对免疫调节治疗有反应的NPLE患者亚群的有用标志物。

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