Kimura Noriyuki, Kumamoto Toshihide, Hanaoka Takuya, Hasama Yusuke, Nakamura Kenichiro, Okazaki Toshio
Department of Neurology and Neuromuscular Disorders, Oita University, Faculty of Medicine, Idaigaoka 1-1, Hasama, Yufu, Oita 879-5593, Japan.
Clin Neurol Neurosurg. 2009 Apr;111(3):296-9. doi: 10.1016/j.clineuro.2008.10.010. Epub 2008 Dec 5.
Here we report two cases of pathologically confirmed tumor-like demyelinating lesions. In comparison with common primary demyelinating diseases, our cases demonstrated atypical radiologic features, such as a large monofocal lesion with mild brain edema, and open ring-like or focal enhancement on magnetic resonance images, suggesting brain tumors. The clinical manifestations included focal neurologic signs due to the lesions, monophasic episodes without relapse over a long follow-up period, and efficacy of oral corticosteroid therapy. Histological analysis of brain biopsy specimens showed the inflammatory demyelination and preserved axons without tumor cells. The present cases suggest the importance of considering inflammatory demyelinating disease in the different diagnosis of monofocal tumor-like lesion.
在此，我们报告两例经病理证实的肿瘤样脱髓鞘病变病例。与常见的原发性脱髓鞘疾病相比，我们的病例表现出非典型的影像学特征，如伴有轻度脑水肿的大的单发病灶，以及磁共振图像上的开放环状或局灶性强化，提示为脑肿瘤。临床表现包括因病变导致的局灶性神经体征、单相发作且在长期随访中无复发，以及口服糖皮质激素治疗有效。脑活检标本的组织学分析显示为炎性脱髓鞘且轴突保留，无肿瘤细胞。本病例提示在单发病灶肿瘤样病变的鉴别诊断中考虑炎性脱髓鞘疾病的重要性。
