Motta Micaela, Alongi Filippo, Bolognesi Angelo, Cozzarini Cesare, Di Muzio Nadia, Fazio Ferruccio
Radiotherapy, Scientific Institute San Raffaele, Via Olgettina 60, Milan, Italy.
Neurologist. 2008 Mar;14(2):120-4. doi: 10.1097/NRL.0b013e31815b97ec.
Sarcoidosis is a chronic disease of unknown etiology characterized by the presence of T lymphocytes, mononuclear phagocytes, and noncaseating epithelioid granulomas in the tissues. Central nervous system involvement occurs in about 5% of the cases. The chronic form of neurosarcoidosis is particularly resistant to medical treatments. No universally accepted therapeutic protocols are currently available. Corticosteroids are the first line of therapy, but other immunosuppressive treatments are frequently added to the patient's regimen, although this strategy is not adequately supported by controlled clinical trials. For patients resistant to or not tolerating multiple alternate immunotherapeutic drugs, some authors suggest central nervous system radiotherapy. We present a case of a patient with neurosarcoidosis involving the hypothalamo-hypophyseal region and causing panhypopituitarism who had a poor response to and experienced severe side effects from conventional immunosuppressive treatments. The patient experienced a good clinical response to cranial irradiation. We review the literature on this subject.
结节病是一种病因不明的慢性疾病,其特征是组织中存在T淋巴细胞、单核吞噬细胞和非干酪样上皮样肉芽肿。约5%的病例会累及中枢神经系统。神经结节病的慢性形式对药物治疗特别耐药。目前尚无普遍接受的治疗方案。皮质类固醇是一线治疗药物,但尽管这一策略缺乏对照临床试验的充分支持,其他免疫抑制治疗也经常被添加到患者的治疗方案中。对于对多种替代免疫治疗药物耐药或不耐受的患者,一些作者建议进行中枢神经系统放疗。我们报告一例累及下丘脑 - 垂体区域并导致全垂体功能减退的神经结节病患者,该患者对传统免疫抑制治疗反应不佳且出现严重副作用。该患者对颅脑照射有良好的临床反应。我们回顾了关于这一主题的文献。