Boulaajaj F Z, Rafai M A, El Otmani H, El Moutawakkil B, Hakim K, Fadel H, Slassi I
Service de Neurologie--Explorations Fonctionnelles, CHU Ibn Rochd, Quartier des Hôpitaux, Casablanca, Maroc.
Rev Neurol (Paris). 2007 Nov;163(11):1049-53. doi: 10.1016/s0035-3787(07)74177-2.
The spinal localization is rare for neurosarcoidosis (0.43 percent of cases) but can be the inaugural manifestation of the disease. We report two cases of spinal neurosarcoidosis in a 57-year-old man and a 43-year*old woman with uneventful past medical histories. Both presented progressive myelopathic features.
Magnetic resonance imaging (MRI) of the spine demonstrated intramedullary lesions, dorsal in the first case, and cervical in the second case. Serum angiotensin converting enzyme was elevated. Radiographs of the chest revealed bilateral symmetric hilar mediastinal lymphadenopathy in the first patient, and bronchial biopsy demonstrated non caseating granulomas. In the second patient the diagnosis was made on pathological examination of a minor salivary gland biopsy.
The patients received corticosteroid therapy with good response in the second patient.
The diagnosis of intramedullary sarcoidosis is difficult without a previous diagnosis of systemic sarcoidosis or other apparent symptom(s). Extraneurologic biopsies may be suggestive. We reviewed the literature on the diagnosis and treatment of intramedullary sarcoidosis.
神经结节病的脊髓定位罕见(占病例的0.43%),但可能是该疾病的首发表现。我们报告了两例脊髓神经结节病病例,一例为57岁男性,另一例为43岁女性,既往病史均无异常。两人均表现出进行性脊髓病特征。
脊柱磁共振成像(MRI)显示髓内病变,第一例位于背部,第二例位于颈部。血清血管紧张素转换酶升高。第一位患者的胸部X线片显示双侧对称性肺门纵隔淋巴结肿大,支气管活检显示非干酪样肉芽肿。在第二位患者中,通过小唾液腺活检的病理检查做出诊断。
患者接受了皮质类固醇治疗,第二位患者反应良好。
在没有先前系统性结节病诊断或其他明显症状的情况下,诊断髓内结节病很困难。神经外活检可能具有提示性。我们回顾了关于髓内结节病诊断和治疗的文献。