Visée H, Duprez T P, Godfraind C, Sindic C J M
Service de Neurologie, Université catholique de Louvain, Cliniques Universitaires Saint-Luc, Brussels, Belgium.
Acta Neurol Belg. 2009 Jun;109(2):91-9.
Neurosarcoidosis is a diagnostic challenge, especially in the absence of systemic involvement, even when cerebral biopsies show noncaseating granulomas. We report a patient with a pineal germinoma associated with a extensive peri- and intra- tumoural granulomatous reaction, who was first diagnosed as possible neurosarcoïdosis. A second patient was initially considered as suffering from Multiple Sclerosis. Brain biopsy showed typical granulomas and gallium scintigraphy revealed other locations of the disease. Unfortunately, he developed a severe, steroid-induced, epidural lipomatosis at the Th3-Th8 levels and died unexpectedly after surgical decompression. Granulomatous inflammation in a tissue obtained by biopsy from a midline lesion should be always considered for the differential diagnosis of germinoma. Corticosteroid-sparing immunosuppressant drugs should be used early in neurosarcoïdosis.
神经结节病是一项诊断难题,尤其是在没有全身受累的情况下,即便脑活检显示非干酪样肉芽肿也是如此。我们报告了一名患有松果体生殖细胞瘤并伴有广泛肿瘤周围和肿瘤内肉芽肿反应的患者,该患者最初被诊断为可能患有神经结节病。另一名患者最初被认为患有多发性硬化症。脑活检显示典型肉芽肿,镓扫描显示了疾病的其他部位。不幸的是,他在胸3至胸8水平出现了严重的、类固醇诱导的硬膜外脂肪增多症,并在手术减压后意外死亡。对于中线病变活检获得的组织中的肉芽肿性炎症,在生殖细胞瘤的鉴别诊断中应始终予以考虑。在神经结节病中应尽早使用可减少皮质类固醇用量的免疫抑制药物。