Pessa Maria Elena, Janes Francesco, Gigli Gian Luigi
Clinica Neurologica e di Neuroriabilitazione, Azienda Ospedaliero-Universitaria S. Maria della Misericordia, Udine, Italy
Clinica Neurologica e di Neuroriabilitazione, Azienda Ospedaliero-Universitaria S. Maria della Misericordia, Udine, Italy.
Clin EEG Neurosci. 2016 Jul;47(3):207-10. doi: 10.1177/1550059414547758. Epub 2014 Sep 23.
Limbic encephalitis (LE) is an inflammation of structures of limbic system. It may be an autoimmune disease or secondary to a neoplasia. Onset is subacute within a few weeks and clinical presentation is characterized by behavioral changes, psychiatric symptoms, short-term memory loss, and epileptic seizures. Diagnosis is typically set after a magnetic resonance imaging (MRI) scan, revealing hyperintensity in limbic structures on T2, fluid-attenuated inversion recovery (FLAIR), and diffusion-weighted imaging (DWI) sequences or detection of antineuronal antibodies; EEG aspecific alterations on temporal areas usually match with MRI and laboratory findings. Specific diagnostic criteria are still under debate. We describe a case presenting with EEG alterations before MRI ones.A 36-year-old woman came to our attention for a first generalized tonic-clonic seizure, several episodes of likely epigastric auras and memory loss. Her clinical history was unremarkable. Neurological examination and brain MRI with gadolinium were normal. Electroencephalographic (EEG) recordings showed theta activity and sharp elements in frontotemporal regions. Therapy with levetiracetam 1000 mg/day was started, but she had another generalized seizure and episodes of epigastric auras increased to 10 per day. After 2 months, another cerebral MRI revealed areas of swelling and signal alteration in deep left temporal areas, especially in hippocampal and parahippocampal gyrus. A spectroscopic evaluation revealed decreased N-acetyl aspartate peak and increased choline and myo-inositol peaks in left frontotemporal areas. These findings were consistent with LE. Cerebrospinal fluid (CSF) analysis was normal; viral serology and onconeuronal antibodies on CSF and blood were negative. Patient was treated with high-dosage steroids, with improvement in memory, epileptic seizures and auras. A third MRI revealed no signal alterations.In conclusion, the clinical picture initially did not meet accepted diagnostic criteria for LE. Effective steroid therapy was consequently delayed. With this case report we would emphasize diagnostic relevance of EEG alterations early in suspected LE in order to start immunosuppressive therapy as soon as possible.
边缘叶脑炎(LE)是边缘系统结构的炎症。它可能是一种自身免疫性疾病,或继发于肿瘤。起病在几周内呈亚急性,临床表现以行为改变、精神症状、短期记忆丧失和癫痫发作为特征。诊断通常在磁共振成像(MRI)扫描后确定,显示T2加权像、液体衰减反转恢复(FLAIR)序列和扩散加权成像(DWI)序列上边缘叶结构呈高信号,或检测到抗神经元抗体;脑电图(EEG)颞区的非特异性改变通常与MRI和实验室检查结果相符。具体的诊断标准仍在讨论中。我们描述了一例在MRI改变之前出现EEG改变的病例。一名36岁女性因首次全身性强直阵挛发作、数次可能的上腹部先兆发作和记忆丧失前来就诊。她的临床病史无明显异常。神经系统检查和钆增强脑MRI均正常。脑电图(EEG)记录显示额颞区有θ活动和棘波成分。开始使用左乙拉西坦1000毫克/天进行治疗,但她又发作了一次全身性癫痫,上腹部先兆发作次数增加到每天10次。2个月后,另一次脑MRI显示左侧颞叶深部区域有肿胀和信号改变,尤其是海马和海马旁回。光谱评估显示左侧额颞区N-乙酰天门冬氨酸峰降低,胆碱和肌醇峰升高。这些发现符合边缘叶脑炎。脑脊液(CSF)分析正常;脑脊液和血液中的病毒血清学及肿瘤神经元抗体均为阴性。患者接受了高剂量类固醇治疗,记忆力、癫痫发作和先兆症状均有改善。第三次MRI显示无信号改变。总之,最初的临床表现不符合边缘叶脑炎公认的诊断标准。因此有效的类固醇治疗被延迟。通过本病例报告,我们强调在疑似边缘叶脑炎早期EEG改变的诊断相关性,以便尽早开始免疫抑制治疗。