Imoto Hirochika, Nishizaki Takafumi, Nogami Kenichiro, Sakamoto Kenichi, Nomura Sadahiro, Akimura Tatsuo, Matsunaga Tokio, Suzuki Michiyasu
Department of Neurosurgery, Yamaguchi University School of Medicine, Ube, Japan.
Neurol Med Chir (Tokyo). 2002 Sep;42(9):406-9. doi: 10.2176/nmc.42.406.
A 50-year-old man presented with neuro-Behçet's disease (NBD) manifesting as a large neoplasm-like lesion affecting the brainstem, basal ganglia, and white matter of the cerebral hemisphere. He had no history of disease except for psychoneurosis. On admission, neurological examination found left hemiparesis and dysarthria. Magnetic resonance (MR) imaging showed multiple small ring-like enhancement in the basal ganglia, brainstem, and deep white matter. Biopsy of the mass was performed. Histological examination revealed invasion of inflammatory cells in the white matter, especially around the blood vessels. After the brain biopsy, the patient developed oral aphthae, genital ulcers, and skin eruptions, which are indicative of Behçet's disease. MR imaging after three courses of steroid pulse therapy revealed that the edematous lesion had become smaller with minimum midline shift. NBD should be considered in the differential diagnosis of lesions with multiple ring-like enhancement extending from the basal ganglia to the brainstem, because dermatological manifestations are sometimes obscured during periods of remission.
一名50岁男性患有神经白塞病(NBD),表现为累及脑干、基底神经节和大脑半球白质的巨大肿瘤样病变。除精神神经症外,他无疾病史。入院时,神经学检查发现左侧偏瘫和构音障碍。磁共振(MR)成像显示基底神经节、脑干和深部白质有多个小环形强化。对肿块进行了活检。组织学检查显示白质中有炎症细胞浸润,尤其是血管周围。脑活检后,患者出现口腔溃疡、生殖器溃疡和皮肤疹,这提示白塞病。三个疗程的类固醇脉冲治疗后的MR成像显示,水肿性病变变小,中线移位最小。对于从基底神经节延伸至脑干的多个环形强化病变的鉴别诊断,应考虑神经白塞病,因为在缓解期皮肤表现有时会不明显。