Department of Neurology, First Affiliated Hospital of Jilin University, Changchun, China.
Front Immunol. 2022 Jul 28;13:955170. doi: 10.3389/fimmu.2022.955170. eCollection 2022.
Paraneoplastic neurological syndromes associated with autoantibodies are rare diseases that cause abnormal manifestations of the nervous system. Early diagnosis of paraneoplastic neurological syndromes paves the way for prompt and efficient therapy.
we reported a 56-year-old man presenting with seizures and rapidly progressive cognitive impairment diagnosed as paraneoplastic limbic encephalitis (PLE) with anti-SRY-like high-mobility group box-1 (SOX-1) and anti-γ-aminobutyric acid B (GABAB) receptor antibodies and finally confirmed by biopsy as small cell lung cancer (SCLC). At the first admission, brain magnetic resonance imaging (MRI) showed no abnormal signal in bilateral hippocampal regions and no abnormal enhancement of enhanced scan. The serum anti-GABAB receptor antibody was 1:100 and was diagnosed as autoimmune encephalitis (AE). The computed tomography (CT) scans of the chest showed no obvious tumor signs for the first time. Although positron emission tomography-computed tomography (PET-CT) revealed hypermetabolism in the para mid-esophageal, the patient and his family declined to undertake a biopsy. The patient improved after receiving immunoglobulin, antiepileptic therapy, and intravenous methylprednisolone (IVMP) pulse treatment. However, after 4 months, the symptoms reappeared. Brain MRI revealed abnormal signals in the hippocampal regions. Reexamination of the cerebral fluid revealed anti-GABAB receptor and anti-SOX-1 antibodies, which contributed to the diagnosis of PLE. SCLC was found in a para mid-esophageal pathological biopsy. Antiepileptic medications and immunoglobulin were used to treat the patient, and the symptoms were under control.
Our findings increase the awareness that patients with limbic encephalitis with cognitive dysfunction and epileptic seizures should be enhanced to detect latent malignancy. Our case also highlights the importance of anti-SOX1 antibodies in the detection of underlying neoplasm, particularly SCLC. Our findings raise awareness of the cognitive impairment seen by patients with limbic encephalitis.
与自身抗体相关的副肿瘤性神经系统综合征是一种罕见疾病,可导致神经系统异常表现。副肿瘤性神经系统综合征的早期诊断为及时有效的治疗铺平了道路。
我们报告了一名 56 岁男性,表现为癫痫发作和进行性认知障碍,诊断为副肿瘤性边缘叶脑炎(PLE),抗 SRY 样高迁移率族 box-1(SOX-1)和抗γ-氨基丁酸 B(GABAB)受体抗体,最终通过活检证实为小细胞肺癌(SCLC)。首次入院时,脑磁共振成像(MRI)显示双侧海马区无异常信号,增强扫描无异常强化。血清抗 GABAB 受体抗体为 1:100,诊断为自身免疫性脑炎(AE)。首次胸部 CT 扫描未见明显肿瘤征象。尽管正电子发射断层扫描-计算机断层扫描(PET-CT)显示食管旁中代谢亢进,但患者及其家属拒绝进行活检。患者接受免疫球蛋白、抗癫痫治疗和静脉注射甲基强的松龙(IVMP)脉冲治疗后有所改善。然而,4 个月后,症状再次出现。脑 MRI 显示海马区异常信号。再次检查脑脊液显示抗 GABAB 受体和抗 SOX-1 抗体,有助于诊断 PLE。食管旁病理活检发现 SCLC。使用抗癫痫药物和免疫球蛋白治疗患者,症状得到控制。
我们的发现提高了认识,即伴有认知功能障碍和癫痫发作的边缘性脑炎患者应加强检测潜在的恶性肿瘤。我们的病例还强调了抗 SOX1 抗体在检测潜在肿瘤中的重要性,特别是 SCLC。我们的发现提高了对边缘性脑炎患者认知障碍的认识。