Department of Neurology, Neuroscience Center, The First Hospital of Jilin University, 71 Xinmin Street, Changchun, 130021, Jilin, China.
Department of Emergency, The First Hospital of Jilin University, 71 Xinmin Street, Changchun, 130021, Jilin, China.
Clin Neurol Neurosurg. 2021 Apr;203:106542. doi: 10.1016/j.clineuro.2021.106542. Epub 2021 Feb 2.
To examine the clinical characteristics, laboratory tests, imaging and electroencephalography presentation, treatment, and prognosis of anti-leucine-rich glioma-inactivated 1 (anti-LGI1) encephalitis and improve the awareness of this disease.
We performed a retrospective analysis of the clinical data of 41 patients who were diagnosed with anti-LGI1 encephalitis. Their clinical characteristics, laboratory tests, and imaging and electroencephalography data were collected, and the treatment results and prognosis were evaluated. The modified Rankin Scale (mRS) was used to evaluate neurological function.
A total of 41 patients were included in the study, the average follow-up time is 33.0 months.The initial symptoms included cognitive impairment (n = 16, 39.0%),faciobrachial dystonic seizures (FBDS) (n = 12, 29.3%), grand mal seizures (n = 5, 12.2%) hallucinations (n = 4, 9.8%), loss of consciousness (n = 2, 4.9%), nausea and vomiting (n = 1, 2.4%),and head discharge-like sensation and radiate one limb (n = 1, 2.4%). There were 20 and 21 patients in the good (mRS ≤ 2) and poor (mRS > 2) prognosis groups, respectively. In the good prognosis group, the initial symptoms included faciobrachial dystonic seizures (FBDS) (n = 6, 30.0%), cognitive impairment (n = 6, 30.0%), hallucinations (n = 4, 20.0%), grand mal seizures (n = 3, 15.0%), loss of consciousness (n = 2, 10.0%), and nausea and vomiting (n = 1, 5.0%). There were 10 patients with hyponatremia. Magnetic resonance imaging (MRI) showed limbic system involvement in 12 patients (60.0%).17 patients (85.0%) recovered, 2 (10.0%) showed significant improvement, and 1 (5.0%) died after a mean follow-up period of 36.9 months. In the poor prognosis group, the initial symptoms included FBDS (n = 6, 28.5%), cognitive impairment (n = 10, 47.6%), grand mal seizures (n = 2, 9.5%), and electric shock-like sensation in the left limbs (n = 1, 4.7%). There were 20 patients with hyponatremia. MRI showed limbic system involvement in 11 patients (52.4%). 11 patients (52.4%) recovered, 8 (38.1%) showed significant improvement, and 2 (9.5%) died after a mean follow-up period of 29.0 months.
The clinical characteristics of anti-LGI1 encephalitis include hyponatremia, FBDS, epileptic seizures, hallucinations, cognitive impairment, and loss of consciousness, while the rarely seen characteristics are nausea, vomiting, and other autonomic dysfunctions and electric shock-like sensation. The appearance of hallucinations often indicates a good prognosis.Hyponatremia and elevated cerebrospinal fluid protein levels can be used as indicators that affect the prognosis of patients.Limbic system involvement has nothing to do with prognosis.Attention should be paid to early diagnosis and timely first-line immunotherapy.
探讨抗亮氨酸丰富胶质瘤失活 1 型（anti-LGI1）脑炎的临床特征、实验室检查、影像学和脑电图表现、治疗及预后，提高对本病的认识。
回顾性分析 41 例诊断为抗-LGI1 脑炎患者的临床资料，收集其临床特征、实验室检查及影像学和脑电图资料，评估治疗效果及预后。采用改良 Rankin 量表（mRS）评估神经功能。
共纳入 41 例患者，平均随访时间为 33.0 个月。首发症状包括认知障碍（n=16，39.0%）、面臂肌张力障碍发作（FBDS）（n=12，29.3%）、全面强直阵挛发作（n=5，12.2%）、幻觉（n=4，9.8%）、意识障碍（n=2，4.9%）、恶心呕吐（n=1，2.4%）和头放电样感觉及放射至单肢（n=1，2.4%）。预后良好（mRS≤2）和预后不良（mRS＞2）组分别为 20 例和 21 例。在预后良好组中，首发症状包括 FBDS（n=6，30.0%）、认知障碍（n=6，30.0%）、幻觉（n=4，20.0%）、全面强直阵挛发作（n=3，15.0%）、意识障碍（n=2，10.0%）和恶心呕吐（n=1，5.0%）。低钠血症 10 例。磁共振成像（MRI）显示边缘系统受累 12 例（60.0%）。17 例（85.0%）患者痊愈，2 例（10.0%）显著改善，1 例（5.0%）死亡，平均随访 36.9 个月。在预后不良组中，首发症状包括 FBDS（n=6，28.5%）、认知障碍（n=10，47.6%）、全面强直阵挛发作（n=2，9.5%）和左上肢电击样感觉（n=1，4.7%）。低钠血症 20 例。MRI 显示边缘系统受累 11 例（52.4%）。11 例（52.4%）患者痊愈，8 例（38.1%）显著改善，2 例（9.5%）死亡，平均随访 29.0 个月。
抗-LGI1 脑炎的临床特征包括低钠血症、FBDS、癫痫发作、幻觉、认知障碍和意识障碍，而少见的特征是恶心、呕吐等自主神经功能障碍和电击样感觉。幻觉的出现常提示预后良好。低钠血症和脑脊液蛋白升高可作为影响患者预后的指标。边缘系统受累与预后无关。应注意早期诊断和及时一线免疫治疗。
