Kobaidze K, Harrison A, Burklin Y, Patidar V, Riccardi M
1Emory University School of Medicine, Division of Hospital Medicine; 2Division of Neurology Atlanta, USA.
Georgian Med News. 2017 May(266):69-74.
Limbic encephalitis (LE) is an autoimmune or paraneoplastic disease that affects the medial temporal lobes. The patient will usually present with cognitive impairment, psychiatric changes, and seizures. Autoimmune limbic encephalitis (LE) is a challenging diagnosis as it is not always included in the typical paraneoplastic/autoimmune panels. Anti-GAD antibodies are associated with various disease including type I diabetes mellitus, various autoimmune processes, some neoplastic and infectious diseases. Thus, it is not as specific as some of the antibodies causing LE. We are presenting two cases of isolated anti-GAD antibody-associated limbic encephalitis. Both patients were adults who developed status epilepticus and refractory seizures, cognitive impairment and mood instability. Patients' cerebrospinal fluid (CSF) and serum anti- GAD antibodies were elevated and after treatment returned to normal reference range. The diagnosis for both patients was delayed (by over one month following hospitalization), both patients required prolonged hospitalization and rehabilitation after discharge. Patient's condition improved only after immunotherapy, but required several antiepileptic drugs for seizure control. The diagnosis was more difficult in the first patient, who had numerous other medical problems including ESRD and moderately severe microvascular changes on brain imaging. In this particular patient, it was hard to appreciate any signal changes on MRI in the mesial temporal lobes given the underlying white matter disease. We recommend inclusion of anti- GAD antibody in the paraneoplastic/encephalopathy panels in order to decrease missed cases of this important cause of LE as well as to hasten the diagnosis. This is a treatable disease, and timely diagnosis is imperative to improve outcomes.
边缘叶脑炎（LE）是一种影响内侧颞叶的自身免疫性或副肿瘤性疾病。患者通常会出现认知障碍、精神变化和癫痫发作。自身免疫性边缘叶脑炎（LE）的诊断具有挑战性，因为它并不总是包含在典型的副肿瘤性/自身免疫性检测项目中。抗谷氨酸脱羧酶（GAD）抗体与多种疾病相关，包括1型糖尿病、各种自身免疫过程、一些肿瘤和感染性疾病。因此，它不如一些导致LE的抗体那样具有特异性。我们报告两例孤立性抗GAD抗体相关的边缘叶脑炎病例。两名患者均为成年人，出现癫痫持续状态和难治性癫痫发作、认知障碍和情绪不稳定。患者的脑脊液（CSF）和血清抗GAD抗体升高，治疗后恢复到正常参考范围。两名患者的诊断均延迟（住院后超过一个月），两名患者出院后均需要长期住院和康复治疗。患者的病情仅在免疫治疗后有所改善，但需要几种抗癫痫药物来控制癫痫发作。第一名患者的诊断更为困难，他有许多其他医疗问题，包括终末期肾病（ESRD），并且脑部影像学显示有中度严重的微血管变化。在这名特殊患者中，鉴于潜在的白质疾病，很难在MRI上观察到内侧颞叶的任何信号变化。我们建议在副肿瘤性/脑病检测项目中加入抗GAD抗体，以减少这种导致LE的重要病因的漏诊病例，并加快诊断。这是一种可治疗的疾病，及时诊断对于改善预后至关重要。
