Bruns F, Pruemer B, Haverkamp U, Fischedick A-R
Department of Radiology, Clemens Hospital Muenster, Düesbergweg 124, D-48153 Muenster, Germany.
Br J Radiol. 2004 Sep;77(921):777-9. doi: 10.1259/bjr/27963001.
Up to 70% of patients with systemic sarcoidosis developing neurosarcoidosis do so within the first 2 years of their systemic illness. Central nervous system (CNS) involvement as the only manifestation of sarcoidosis can be seen both at first time of disease and at recurrence in a few isolated cases. A young man showed neurological symptoms caused by isolated CNS sarcoidosis after unsuccessful treatment of primary pulmonary sarcoidosis by steroids. MRI scans of the head showed a distinct structural lesion temporodorsal in the left hemisphere and in the left-sided basal ganglia. The diagnosis was proved by neurosurgical resection. Post-operative systemic treatment with long-term corticosteroids was ineffective. After low-dose whole-brain irradiation of the isolated CNS lesion with 20 Gy, partial resolution of the clinical features and stabilization of disease proved by MRI ensued. In neurosarcoidosis the use of radiation therapy remains an appropriate therapy option with minimal adverse sequelae if primary medical treatment fails.
在系统性结节病患者中,高达70%发展为神经结节病的患者是在其系统性疾病的头2年内出现这种情况的。结节病仅累及中枢神经系统(CNS)作为唯一表现,在疾病初发时以及少数复发病例中均可见到。一名年轻男性在原发性肺结节病经类固醇治疗失败后,出现了由孤立性中枢神经系统结节病引起的神经症状。头部MRI扫描显示左半球颞背侧和左侧基底节有明显的结构性病变。经神经外科切除证实了诊断。术后长期使用皮质类固醇进行全身治疗无效。在用20 Gy对孤立的中枢神经系统病变进行低剂量全脑照射后,临床症状部分缓解,MRI证实疾病稳定。在神经结节病中,如果初始药物治疗失败,放射治疗仍是一种具有最小不良后遗症的合适治疗选择。