Hammed Ali, Badour Maysaa, Baqla Sameer, Amer Fatema
Tishreen University Hospital, Department of Neurosurgery, Lattakia, Syria.
Pediatric University Hospital, Division of Neurology, Damascus, Syria.
Ann Med Surg (Lond). 2021 Aug 4;68:102606. doi: 10.1016/j.amsu.2021.102606. eCollection 2021 Aug.
Rasmussen encephalitis (RE) is a rare disease of unknown etiology that causes severe chronic unihemispheric inflammatory disease of the central nervous system mainly in children. It leads to intractable seizures, cognitive decline and progressive neurological deficits in the affected hemisphere. We report two cases of RE, as defined by fulfillment of the 2005 Bien criteria. The diagnostic challenge of characterizing this rare disease will be highlighted by the extensive serum, CSF, MR imaging and EEG data in the two patients. In addition, we will review the various forms of therapy attempted in these two patients, namely anti-epileptic drug therapy and immunomodulatory therapy. Hemispherectomy was done for the second patient with favorable outcomes of controlling seizures, but unfortunately, he died because of meningitis. Until the causes of Rasmussen's encephalitis are known, it is difficult to anticipate how treatments will improve. Such a situation creates a therapeutic dilemma; hemispherectomy is not favored because of the inevitable postoperative functional deficits, but a real risk exists that treatments used to delay progression of the disease will defer definitive surgical treatment beyond the time when an optimum post-hemispherectomy outcome could be expected.
拉斯穆森脑炎（RE）是一种病因不明的罕见疾病，主要发生于儿童，可导致严重的中枢神经系统慢性单侧半球炎性疾病。它会导致患侧半球出现难治性癫痫、认知功能下降和进行性神经功能缺损。我们报告两例符合2005年比恩标准定义的RE病例。这两名患者广泛的血清、脑脊液、磁共振成像和脑电图数据将凸显诊断这种罕见疾病所面临的挑战。此外，我们将回顾在这两名患者中尝试的各种治疗方式，即抗癫痫药物治疗和免疫调节治疗。为第二名患者实施了半球切除术，在控制癫痫方面取得了良好效果，但不幸的是，他因脑膜炎死亡。在拉斯穆森脑炎的病因明确之前，很难预测治疗方法将如何改进。这种情况造成了治疗困境；由于不可避免的术后功能缺损，半球切除术并不受青睐，但存在这样一种实际风险，即用于延缓疾病进展的治疗方法会将确定性手术治疗推迟到预期的最佳半球切除术后结果出现的时间之后。
