Shuper Avinoam, Michovitz Shalom, Amir Jacob, Kornreich Liora, Boikov Olga, Yaniv Yitzhak, Rorke-Adams Lucy B
Department of Hematology-Oncology, Schneider Children's Medical Center of Israel, Petah Tiqva, Sackler Faculty of Medicine, Tel Aviv Univ., Israel, and Department of Neuropathology, The Children's Hospital of Philadelphia, PA, USA.
Pediatr Neurol. 2006 Oct;35(4):280-3. doi: 10.1016/j.pediatrneurol.2006.04.003.
Idiopathic granulomatous encephalitis is a rare disorder of unknown etiology, undetermined treatment, and often grave prognosis. This article describes a 4-year-old female who presented with a single focal febrile convulsion followed a few weeks later by right-sided hemiparesis. A huge infiltrative cerebral mass tumor was found which proved to be a granuloma on histologic study. Despite a thorough evaluation, including tissue studies and search for an infectious agent, no etiology could be identified, and the final diagnosis was idiopathic granulomatous encephalitis. Recurrent resections and high-dose steroid treatment failed to control the process, and the patient died of disease 6 months after presentation. Evaluation and treatment of idiopathic granulomatous encephalitis should be aggressive, and the possibility of chemotherapy and perhaps even radiotherapy should be considered if there is no response to steroids.
特发性肉芽肿性脑炎是一种病因不明、治疗方法未确定且预后往往严重的罕见疾病。本文描述了一名4岁女性，她最初出现一次局灶性热性惊厥，几周后出现右侧偏瘫。发现一个巨大的浸润性脑肿瘤，组织学检查证实为肉芽肿。尽管进行了全面评估，包括组织学研究和寻找感染源，但仍未确定病因，最终诊断为特发性肉芽肿性脑炎。反复手术切除和大剂量类固醇治疗未能控制病情，患者在出现症状6个月后死于该疾病。对于特发性肉芽肿性脑炎的评估和治疗应积极主动，如果对类固醇治疗无反应，应考虑化疗甚至放疗的可能性。
