Mazzi Gustavo, Roia Dina De, Cruciatti Barbara, Matà Sabrina, Catapano Raffaele
Servizio di Immunoematologia e Medicina Trasfusionale, Azienda Ospedaliera. "Santa Maria degli Angeli" Via Montereale 24, 33170 Pordenone, Italy.
Transfus Apher Sci. 2008 Dec;39(3):229-33. doi: 10.1016/j.transci.2008.09.005. Epub 2008 Oct 26.
Limbic encephalitis (LE) is a neurological syndrome usually presenting in a paraneoplastic form. Recently many cases were reported with no concurring neoplasia, presenting with specific antibodies for voltage-gated potassium channel or for neuronal membrane antigens. Anti-glutamic acid decarboxylase (GAD) antibodies act against GABAergic receptors of the central nervous system. These antibodies were found in coeliac disease serum and in neurologic patients. We are reporting a case of a 21-year-old coeliac woman manifesting complex multiple-daily partial drug-resistant seizures for 7 years. The diagnosis was of a non paraneoplastic limbic encephalitis, unresponsive to high dose cortisone and IGIV infusion. The use of therapeutic plasma exchange (TPE) has resulted in an improvement in symptoms with quite a long disease-free period of time. When the frequency of the procedures was decreased, seizures appeared again and, after suspension of TPE, the clinical status worsened. The role of TPE in the treatment of LE still has to be defined.
边缘叶脑炎（LE）是一种通常以副肿瘤形式出现的神经综合征。最近有许多病例报道，这些病例无并发肿瘤，表现为针对电压门控钾通道或神经元膜抗原的特异性抗体。抗谷氨酸脱羧酶（GAD）抗体作用于中枢神经系统的GABA能受体。这些抗体在乳糜泻血清和神经系统疾病患者中被发现。我们报告一例21岁的乳糜泻女性患者，该患者表现为复杂的每日多次部分性耐药癫痫发作达7年。诊断为非副肿瘤性边缘叶脑炎，对高剂量皮质类固醇和静脉注射免疫球蛋白无反应。治疗性血浆置换（TPE）的使用使症状得到改善，且有相当长的无病期。当治疗频率降低时，癫痫再次出现，在停止TPE后，临床状况恶化。TPE在LE治疗中的作用仍有待确定。
