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Rasmussen脑炎(Rasmussen’s encephalitis,RE)是一种罕见的神经系统慢性疾病,又称为Rasmussen综合征。该病多起源于儿童期,常累及一侧大脑半球,主要以药物难治性癫痫、部分性癫痫持续状态、进展性神经功能缺损(言语、运动及认知功能障碍等)为主要特点。目前神经病理学及免疫学机制尚不明确。影像学检查对其早期诊断有指导意义。该病症状重,致残率高,抗癫痫药物治疗的疗效较差,激素及免疫抑制剂等药物治疗不能从根本上阻止疾病的进展,大脑半球切除术仍是目前唯一治愈该病的方法。
Rasmussen encephalitis (RE) is a rare chronic disease of the nervous system, also known as Rasmussen syndrome. The disease mostly originated in childhood, often involving the side of the cerebral hemispheres, mainly in drug refractory epilepsy, partial status epilepticus, progressive neurological deficits (speech, motor and cognitive dysfunction, etc.) as the main feature. The current neuropathological and immunological mechanisms are not yet clear. Imaging examination for its early diagnosis is instructive. The disease symptoms, high morbidity, poor efficacy of antiepileptic drugs, hormone and immunosuppressive drug therapy can not fundamentally prevent the progress of the disease, the hemispherectomy is currently the only way to cure the disease.