Neurological Sciences ( IF 3.3 ) Pub Date : 2021-08-24 , DOI: 10.1007/s10072-021-05563-x Pavel Štourač 1 , Jana Bednářová 2 , Pavel Zicháček 3 , Zdena Čermáková 4 , Zbyšek Pavelek 5 , Martin Vališ 5
Autoimmune and limbic encephalitides are still rare diseases characterized by rapid diagnostics and treatment development in recent years. The incidence of anti-N-methyl-D- aspartate receptor [NMDAR] encephalitis is about 1.5 per million person per year, and the incidence of paraneoplastic neurological syndromes [PNS] including limbic encephalitis [LE] is about 1.22 per 100 000 person per year (Vogrig et al. J Neurol 267:26–35, 1; Dalmau et al. Ann Neurol 61:25–36, 2). The diagnostic criteria of anti-NMDAR encephalitis are already well established (Zuliani et al. Neurol Sci 40:2017–2030, 10). We provide immunological and clinical characteristics of anti-NMDAR encephalitis case series emphasizing unusual association with colon tumour in one case and complete recovery in two cases. Then we report two cases of onconeural and cell surface antibody negative limbic encephalitis [LE] associated with tumours, seizures, cognitive and behavioural changes resulting in severe cerebellar syndrome and fatal outcome. The clinical characteristics and results of selected paraclinical examinations as electroencephalography [EEG], magnetic resonance imaging [MRI] and cerebrospinal fluid [CSF] analysis are reviewed.
中文翻译:
自身免疫性和边缘系统脑炎:临床实践中具有一些非典型变量的病例系列
自身免疫性和边缘性脑炎仍然是近年来以快速诊断和治疗发展为特征的罕见疾病。抗N-甲基-D-天冬氨酸受体[NMDAR]脑炎的发病率约为每百万人每年1.5例,包括边缘系统脑炎[LE]在内的副肿瘤性神经综合征[PNS]的发病率约为每10万人每人1.22例年(Vogrig 等人 J Neurol 267:26-35, 1; Dalmau 等人 Ann Neurol 61:25-36, 2)。抗 NMDAR 脑炎的诊断标准已经确立(Zuliani et al. Neurol Sci 40:2017–2030, 10)。我们提供了抗 NMDAR 脑炎病例系列的免疫学和临床特征,强调 1 例病例与结肠肿瘤异常相关,2 例病例完全康复。然后我们报告了两例与肿瘤、癫痫发作、认知和行为改变相关的肿瘤和细胞表面抗体阴性边缘脑炎 [LE],导致严重的小脑综合征和致命的结果。回顾了选定的临床旁检查的临床特征和结果,如脑电图 [EEG]、磁共振成像 [MRI] 和脑脊液 [CSF] 分析。