Thakolwiboon Smathorn, Gilligan Michael, Orozco Emma, Britton Jeffrey W, Dubey Divyanshu, Flanagan Eoin P, Lopez-Chiriboga A Sebastian, Smith Kelsey, Valencia-Sanchez Cristina, Zalewski Nicholas L, Zekeridou Anastasia, Pittock Sean J, McKeon Andrew
Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Department of Neurology, Mayo Clinic Health System, La Crosse, Wisconsin, USA.
J Neurol Neurosurg Psychiatry. 2025 Jan 19. doi: 10.1136/jnnp-2024-334957.
Data regarding long-term recovery from autoimmune encephalitis (AE) remain limited.
This retrospective observational study investigated outcomes in 182 patients who met the 2016 criteria for definite AE. Recovery data were available in 172 patients. Follow-up data at ≥24 months post-attack were available for 119. Recovery trajectory, residual symptoms, outcome predictors and causes of post-AE death were assessed.
Of 172 patients, 138 (80%) achieved good recovery (modified Rankin Scale (mRS) ≤2) with a median recovery time of 4 months (95% CI: 2 to 6 months). Recovery varied by associated neural antibody, with the best recovery observed in leucine-rich glioma-inactivated 1 (97% good recovery, median recovery time 0 (0 to 2) months). Paraneoplastic AE (p=0.007), severe attacks (eg, mRS ≥4 at attack, p=0.007) and cerebrospinal fluid pleocytosis (p=0.005) were associated with a lower likelihood of good recovery, while seizure presentation (p=0.026) was associated with better recovery. Despite good recovery, several residual symptoms persisted ≥24 months post-AE, including cognitive deficits (53%), seizures (26%), depression (23%), sleep disorders (25%), brainstem/cerebellar symptoms (13%), other movement disorders (14%) and autonomic symptoms (12%). Predictors of long-term sequelae included disabling cognitive deficit at onset and delayed immunotherapy for post AE-dementia, and medial temporal atrophy as well as escalation to cyclophosphamide therapy for both drug-resistant epilepsy and chronic depression. Of 182 patients, 20 (11%) died; the most common cause of death was progression of AE (6/20 (30%)).
While the majority of patients achieved functional independence after AE, several residual symptoms persisted. Several clinical and paraclinical features were associated with long-term sequelae.
自身免疫性脑炎（AE）长期恢复的数据仍然有限。
这项回顾性观察研究调查了182例符合2016年确诊AE标准的患者的预后情况。172例患者有恢复数据。119例患者有攻击后≥24个月的随访数据。评估了恢复轨迹、残留症状、预后预测因素和AE后死亡原因。
在172例患者中，138例（80%）恢复良好（改良Rankin量表（mRS）≤2），中位恢复时间为4个月（95%CI：2至6个月）。恢复情况因相关神经抗体而异，富含亮氨酸的胶质瘤失活蛋白1恢复情况最佳（97%恢复良好，中位恢复时间0（0至2）个月）。副肿瘤性AE（p=0.007）、严重发作（如发作时mRS≥4，p=0.007）和脑脊液淋巴细胞增多（p=0.005）与恢复良好的可能性较低相关，而癫痫发作表现（p=0.026）与更好的恢复相关。尽管恢复良好，但AE后仍有几种残留症状持续≥24个月，包括认知缺陷（53%）、癫痫发作（26%）、抑郁（23%）、睡眠障碍（25%）、脑干/小脑症状（13%）、其他运动障碍（14%）和自主神经症状（12%）。长期后遗症的预测因素包括发病时致残性认知缺陷、AE后痴呆的延迟免疫治疗、内侧颞叶萎缩以及对耐药性癫痫和慢性抑郁升级为环磷酰胺治疗。在182例患者中，20例（11%）死亡；最常见的死亡原因是AE进展（6/20（30%））。
虽然大多数患者在AE后实现了功能独立，但仍有几种残留症状持续存在。一些临床和辅助检查特征与长期后遗症相关。
