Kapfhammer Irene, Armbruster Christine, Armbruster Christian
2nd Medical Department, Otto Wagner Spital, Vienna, Austria.
Wien Klin Wochenschr. 2006 Sep;118(17-18):554-7. doi: 10.1007/s00508-006-0662-6.
Neurosarcoidosis is often a diagnostic dilemma, especially in the absence of other organ involvement. We report a 64-year-old patient who had suffered from paraplegia due to an intramedullar process since 1995. The presumptive diagnosis based on computed tomography was spinal cord infarction. Six years later, he complained about increasing paresthesia. Magnetic resonance imaging of the spinal cord showed nodular meningeal enhancement. Computed tomography of the thorax revealed mediastinal and hilar lymphadenopathy. Bronchoscopy under generalized anesthesia was performed. The differential cell count in bronchoalveolar lavage fluid showed 39% lymphocytes and a CD4(+)/CD8(+) ratio of 17.7. Histological examination of biopsy specimens from the hilar lymph nodes revealed non-necrotizing granulomas with epitheloid cells and Langerhans-type giant cells, consistent with the diagnosis of sarcoidosis. As a result of these findings, lumbar puncture was undertaken and a raised protein concentration and pleocytosis were found in the cerebrospinal fluid. The number of lymphocytes (9,250 lymphocytes/l) and a CD4(+)/CD8(+) ratio of 10.78 led to the diagnosis of neurosarcoidosis. Paralysis might have been prevented if the correct diagnosis of neurosarcoidosis had been established earlier in this patient.
神经结节病常常是一个诊断难题,尤其是在没有其他器官受累的情况下。我们报告一名64岁患者,自1995年起因髓内病变导致截瘫。基于计算机断层扫描的初步诊断为脊髓梗死。六年后,他抱怨感觉异常加重。脊髓磁共振成像显示结节状脑膜强化。胸部计算机断层扫描显示纵隔和肺门淋巴结肿大。在全身麻醉下进行了支气管镜检查。支气管肺泡灌洗液的细胞分类计数显示淋巴细胞占39%,CD4(+)/CD8(+)比值为17.7。肺门淋巴结活检标本的组织学检查显示有非坏死性肉芽肿,伴有上皮样细胞和朗汉斯型巨细胞,符合结节病的诊断。基于这些发现,进行了腰椎穿刺,脑脊液中蛋白浓度升高且有细胞增多。淋巴细胞数量(9250个淋巴细胞/升)和CD4(+)/CD8(+)比值为10.78,从而诊断为神经结节病。如果该患者能更早确立神经结节病的正确诊断,瘫痪或许可以避免。