A case of Rasmussen encephalitis treated with rituximab
A case of Rasmussen encephalitis treated with rituximab
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DOI：
10.1038/nrneurol.2009.98
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发表时间：
2009-08-01
影响因子：
38.1
通讯作者：
Lang, Nicolas
中科院分区：
文献类型：
--
作者：
Thilo, Barbara;Stingele, Robert;Lang, Nicolas
背景一名20岁的妇女因高频率（每1-2分钟）的局灶性运动性癫痫发作而被转诊到我们的重症监护室。7年前，她被诊断为患有经活检证实的右半球Rasmussen脑炎（Re），从那时起，她接受了多种抗癫痫药物以及连续免疫疗法（例如他克莫司、皮质类固醇、吗替麦考酚酯、静脉注射免疫球蛋白和免疫吸附）的治疗。尽管由于Re进展缓慢而避免了大脑半球切除术，但自诊断以来，她没有完全切除超过7周。EEG和MRI诊断。与右半球Re相关的局灶性运动癫痫持续状态，引起持续性癫痫活动和右侧半球单侧萎缩伴水肿。免疫吸附最初用于治疗癫痫发作。一旦停止，我们使用375 mg/m2静脉注射利妥昔单抗-一种单克隆抗CD 20抗体-每周一次，持续4周以稳定病情，导致cd 19（+）B细胞完全耗竭。当CD 19（+）B细胞浓度升高，局灶性癫痫发作再次出现时，再次输注利妥昔单抗。在整个治疗过程中，患者继续接受抗癫痫治疗（左乙拉西坦、奥卡西平、唑尼沙胺和苯巴比妥）。
Background. a 20-year-old woman was referred to our intensive care unit with a high frequency (every 1-2 min) of focal motor seizures. she had been diagnosed as having biopsy-proven Rasmussen encephalitis (Re) of the right hemisphere 7 years previously, since when she had been treated with numerous antiepileptic drugs, as well as with continuous immunotherapies, such as tacrolimus, corticosteroids, mycophenolate mofetil, intravenous immunoglobulin and immunoadsorption. although hemispherectomy had been avoided due to slow progression of Re, she had not been seizure-free for more than 7 weeks since diagnosis.Investigations. EEG and MRI.Diagnosis. Focal motor status epilepticus associated with right hemispheric Re, causing continuous epileptic activity and unilateral atrophy with edema in the right hemisphere.Management. Immunoadsorption was used initially to treat the seizures. Once they had ceased, we used 375 mg/m(2) intravenous rituximab-a monoclonal anti-CD20 antibody-once-weekly for 4 weeks to stabilize the condition, leading to complete depletion of cd19(+) B cells. Rituximab infusions were used again when concentrations of CD19(+) B cells rose and focal seizures re-emerged. the patient remained on antiepileptic therapy (levetiracetam, oxcarbazepine, zonisamide and phenobarbital) throughout treatment.