Beros Vili, Houra Karlo, Rotim Kresimir, Kovac Damir, Cupić Hrvoje
Department of Neurosurgery, University Hospital Sestre milosrdnice, Zagreb, Croatia.
Coll Antropol. 2008 Jun;32(2):645-7.
Sarcoidosis is a chronic, systemic granulomatous reticulosis of unknown origin, characterized by formation of hard tubercles and noncaseatinggranulomas. Since other infectious diseases such as berylliosis, mycobacterium and fungal infections may present with a noncaseating granulomas, histological diagnosis of sarcoidosis is made using the elimination method. Central nervous system manifestations of sarcoidosis may be present in 5-10% of the cases involving cranial nerves, leptomeninges and third ventricle respectively. Any part of the central nervous system can be affected. Involvement of spinal cord in sarcoidosis is extremely rare and presents with only 0.3-0.4% in patients with systemic sarcoidosis. Intramedullary sarcoidosis is a rare first manifestation of the disease and it can mimic an intramedullary tumor, which is often manifested with symptoms that initiate from spinal cord compression, resulting in paraparesis, sensory disorders and sphincter dysfunction. We present a case of intramedullary sarcoidosis that mimics a tumor of the thoracic spinal cord. Clinical features, neuroradiological, pathohistological findings, laboratory analysis and surgical treatment of such a rare entity are being discussed.
结节病是一种病因不明的慢性全身性肉芽肿性网状内皮病,其特征为形成硬性结节和非干酪样肉芽肿。由于其他传染病如铍中毒、分枝杆菌和真菌感染也可能出现非干酪样肉芽肿,因此结节病的组织学诊断采用排除法。结节病的中枢神经系统表现可能出现在5% - 10%的病例中,分别累及脑神经、软脑膜和第三脑室。中枢神经系统的任何部位都可能受到影响。结节病累及脊髓极为罕见,在全身性结节病患者中仅占0.3% - 0.4%。脊髓内结节病是该疾病罕见的首发表现,可类似髓内肿瘤,常表现为因脊髓受压引发的症状,导致双下肢轻瘫、感觉障碍和括约肌功能障碍。我们报告一例酷似胸段脊髓肿瘤的脊髓内结节病病例。本文将讨论这种罕见疾病的临床特征、神经放射学、病理组织学表现、实验室分析及外科治疗。