Sharma Chandra Mohan, Pandey Rajendra Kumar, Kumawat Banshi Lal, Khandelwal Dinesh, Gandhi Pankaj
Department of Neurology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India.
Ann Indian Acad Neurol. 2016 Jan-Mar;19(1):146-9. doi: 10.4103/0972-2327.165462.
Antibodies to GAD-65 have been implicated in the pathogenesis of type 1 diabetes, limbic encephalitis and Stiff person syndrome, however these diseases rarely occur concurrently. We intend to present a rare case of 35 year old female who was recently diagnosed as having type 1 diabetes presented with 1½ month history of recurrent seizures, subacute onset gait ataxia, dysathria, psychiatric disturbance and cognitive decline. No tumor was found on imaging and the classic paraneoplastic panel was negative. Cerebrospinal fluid and blood was positive for GAD-65 antibodies. Patient showed significant improvement with immunomodulatory therapy. Association of GAD-65 antibodies has been found with various disorders including type 1 diabetes, limbic encephalitis, Stiff person syndrome, cerebellar ataxia and palatal myoclonus. This case presents with unique combination of type 1 diabetes, Stiff person syndrome and limbic encephalitis associated with GAD-65 antibodies that is responsive to immunotherapy. It also highlights the emerging concept of autoimmunity in the causation of various disorders and there associations.
谷氨酸脱羧酶65（GAD - 65）抗体与1型糖尿病、边缘叶脑炎及僵人综合征的发病机制有关，然而这些疾病很少同时发生。我们打算报告一例罕见病例，一名35岁女性，近期被诊断为1型糖尿病，伴有1个半月的反复癫痫发作史、亚急性起病的步态共济失调、构音障碍、精神障碍及认知功能减退。影像学检查未发现肿瘤，经典的副肿瘤综合征相关检查结果为阴性。脑脊液和血液中GAD - 65抗体呈阳性。患者经免疫调节治疗后有显著改善。已发现GAD - 65抗体与多种疾病相关，包括1型糖尿病、边缘叶脑炎、僵人综合征、小脑共济失调及腭肌阵挛。该病例呈现出1型糖尿病、僵人综合征及边缘叶脑炎与GAD - 65抗体相关的独特组合，且对免疫治疗有反应。它还凸显了自身免疫在各种疾病病因及关联中的新兴概念。
