Department of Neurological Surgery, Cleveland Clinic, Cleveland, Ohio, USA.
Case Western Reserve University School of Medicine, Cleveland, Ohio, USA.
Neurosurgery. 2022 Jul 1;91(1):93-102. doi: 10.1227/neu.0000000000001958. Epub 2022 May 13.
Rasmussen encephalitis (RE) is a rare inflammatory disease affecting one hemisphere, causing progressive neurological deficits and intractable seizures.
To report long-term seizure outcomes, reoperations, and functional outcomes in patients with RE who underwent hemispherectomy at our institution.
Retrospective review was performed for all patients with RE who had surgery between 1998 and 2020. We collected seizure history, postoperative outcomes, and functional data. Imaging was independently reviewed in a blinded fashion by 2 neurosurgeons and a neuroradiologist.
We analyzed 30 patients with RE who underwent 35 hemispherectomies (5 reoperations). Using Kaplan-Meier analysis, seizure-freedom rate was 81.5%, 63.6%, and 55.6% at 1, 5, and 10 years after surgery, respectively. Patients with shorter duration of hemiparesis preoperatively were less likely to be seizure-free at follow-up (P = .011) and more likely to undergo reoperation (P = .004). Shorter duration of epilepsy (P = .026) and preoperative bilateral MRI abnormalities (P = .011) were associated with increased risk of reoperation. Complete disconnection of diseased hemisphere on postoperative MRI after the first operation improved seizure-freedom (P = .021) and resulted in fewer reoperations (P = .034), and reoperation resulted in seizure freedom in every case.
Obtaining complete disconnection is critical for favorable seizure outcomes from hemispherectomy, and neurosurgeons should have a low threshold to reoperate in patients with RE with recurrent seizures. Rapid progression of motor deficits and bilateral MRI abnormalities may indicate a subpopulation of patients with RE with increased risk of needing reoperation. Overall, we believe that hemispherectomy is a curative surgery for the majority of patients with RE, with excellent long-term seizure outcome.
拉森姆脑炎（RE）是一种罕见的影响单侧半球的炎症性疾病，导致进行性神经功能缺损和难治性癫痫发作。
报告在我们机构接受半脑切除术的 RE 患者的长期癫痫发作结局、再次手术和功能结局。
对 1998 年至 2020 年期间接受手术的所有 RE 患者进行回顾性研究。我们收集了癫痫发作史、术后结果和功能数据。影像学由 2 名神经外科医生和 1 名神经放射科医生以盲法独立进行评估。
我们分析了 30 例接受 35 次半脑切除术（5 次再次手术）的 RE 患者。使用 Kaplan-Meier 分析，术后 1、5 和 10 年的无癫痫发作率分别为 81.5%、63.6%和 55.6%。术前偏瘫持续时间较短的患者在随访时无癫痫发作的可能性较小（P =.011），再次手术的可能性较大（P =.004）。癫痫发作持续时间较短（P =.026）和术前双侧 MRI 异常（P =.011）与再次手术风险增加相关。首次手术后，术后 MRI 显示病变半球完全断开可改善癫痫发作无发作（P =.021），减少再次手术（P =.034），并且再次手术使每个病例都无癫痫发作。
获得完全断开是半脑切除术获得良好癫痫发作结局的关键，神经外科医生应该对 RE 患者再次出现癫痫发作时，有较低的手术门槛。运动功能缺损的快速进展和双侧 MRI 异常可能表明存在需要再次手术的 RE 亚群患者。总的来说，我们认为，对于大多数 RE 患者，半脑切除术是一种有效的手术，具有极好的长期癫痫发作结局。
