Pastuszak Żanna, Stępień Adam, Tomczykiewicz Kazimierz, Piusińska-Macoch Renata, Kordowska Joanna, Galbarczyk Dariusz, Świstak Jarosław
Department of Neurosurgery, Mossakowski Medical Research Centre, Polish Academy of Sciences, Warsaw, Poland.
Military Institute of Medicine, Warsaw, Poland.
Cent Eur J Immunol. 2017;42(2):213-217. doi: 10.5114/ceji.2017.69365. Epub 2017 Aug 8.
Usually limbic encephalitis (LE) is a paraneoplastic neurologic syndrome. LE symptoms can precede cancer even by a few years. Almost 50% of LE cases are connected with small cell lung carcinoma. Testis and breast cancers, granulomatous disease, thymoma, and teratomas are also often connected with LE. Other cases have infectious and autoimmunological aetiology. In LE limbic system dysfunction is observed, and it is accompanied by cerebellum and brain stem abnormalities as well as polyneuropathy. Paraneoplastic limbic encephalitis is sometimes a part of larger syndrome in which brain stem and spinal cord are involved in an inflammatory process called paraneoplastic encephalomyelitis. The main LE symptoms are: impairment of cognitive functions with subacute beginning, partial and generalised seizures, mental distress, disturbances of consciousness, and limb paresis. In MRI study hyperintensive lesions in the medial part of the temporal lobes in T2 and FLAIR sequences are present. Sharp and slow waves in electroencephalography in the temporal area are also frequent. In cerebrospinal fluid pleocytosis, elevation of protein level, intensification of immunoglobulin synthesis, and oligoclonal bands can be detected. The majority of patients with paraneoplastic LE have onconeural antibodies in the blood. The presented study is a description of the clinical course of the disease in four patients diagnosed with LE.
通常,边缘叶脑炎(LE)是一种副肿瘤性神经综合征。LE症状甚至可能在癌症出现前数年就已出现。近50%的LE病例与小细胞肺癌有关。睾丸癌、乳腺癌、肉芽肿性疾病、胸腺瘤和畸胎瘤也常与LE有关。其他病例则有感染性和自身免疫性病因。在LE中,可观察到边缘系统功能障碍,并伴有小脑和脑干异常以及多发性神经病。副肿瘤性边缘叶脑炎有时是一种更大综合征的一部分,在该综合征中,脑干和脊髓参与了一种称为副肿瘤性脑脊髓炎的炎症过程。LE的主要症状包括:亚急性起病的认知功能损害、部分性和全身性癫痫发作、精神困扰、意识障碍和肢体轻瘫。在MRI检查中,T2和FLAIR序列显示颞叶内侧部分有高信号病变。脑电图检查中颞区出现尖波和慢波也很常见。在脑脊液中可检测到细胞增多、蛋白水平升高、免疫球蛋白合成增强和寡克隆带。大多数副肿瘤性LE患者血液中存在肿瘤神经抗体。本研究描述了4例诊断为LE的患者的疾病临床过程。