Vrillon Agathe, Carle Guilhem, Berzero Giulia, Honnorat Jérôme, Huberfeld Gilles, Psimaras Dimitri, Azuar Carole
APHP- Behavioral Neuropsychiatric Unit, University Hospital La Pitié-Salpêtrière, Paris, France.
APHP- Behavioral Neuropsychiatric Unit, University Hospital La Pitié-Salpêtrière, Paris, France; Pierre and Marie Curie University, Paris 6, INSERM- UMR 1022, FrontLab, Paris, France.
Neurosci Biobehav Rev. 2020 Dec;119:128-137. doi: 10.1016/j.neubiorev.2020.08.015. Epub 2020 Oct 3.
Autoimmune Limbic Encephalitis (LE) is a relatively new category of immune-mediated diseases with a wide range of neuropsychiatric symptoms. LE associated with Glutamic Acid Decarboxylase (GAD) antibodies is difficult to diagnose due to its possible atypical presentation with neuropsychiatric and behavioral features. We performed a systematic review of literature and retrieved 21 cases of anti GAD-associated LE with neuropsychiatric signs. Median age at onset was 27 years with a female predominance (81.0 %) and median diagnostic delay of 6 months. Clinical presentation included typical LE symptoms such as anterograde amnesia (95.2 %) and temporal lobe or tonico-clonic seizures (95.2 %). Psychiatric symptoms were described in 61.9 % of patients, presenting as anxiety, depressive symptoms, apathy and behavioral changes. Extra-limbic symptoms were present in 14.3 % of patients. No neoplasia associated was found. Some patients had poor epileptic, cognitive and psychiatric outcomes requiring prolonged immunosuppressive treatment. The description of the neuropsychiatric spectrum of anti-GAD LE and its specificities aims to improve our understanding of this entity, and may lead to earlier diagnosis as well as better outcome.
自身免疫性边缘叶脑炎（LE）是一类相对较新的免疫介导性疾病，具有广泛的神经精神症状。与谷氨酸脱羧酶（GAD）抗体相关的LE因其可能具有神经精神和行为特征的非典型表现而难以诊断。我们对文献进行了系统回顾，检索到21例具有神经精神症状的抗GAD相关LE病例。发病时的中位年龄为27岁，女性占优势（81.0%），中位诊断延迟为6个月。临床表现包括典型的LE症状，如顺行性遗忘（95.2%）和颞叶或强直阵挛性发作（95.2%）。61.9%的患者出现精神症状，表现为焦虑、抑郁症状、冷漠和行为改变。14.3%的患者出现边缘叶以外的症状。未发现相关肿瘤。一些患者的癫痫、认知和精神预后较差，需要长期免疫抑制治疗。对抗GAD LE的神经精神谱及其特异性的描述旨在增进我们对这一实体的理解，并可能导致早期诊断和更好的预后。
