Suga M, Sato K, Nishimura M, Oda M
Division of Neurology, Tokyo Metropolitan Neurological Hospital.
Rinsho Shinkeigaku. 1990 Sep;30(9):1005-9.
A case of Neuro-Behçet's disease with the right cerebral artery occlusion on cerebral angiogram was reported. A 63 years old man complained of headache and slight fever without exacerbations of ocular and mucocutaneous lesions, 16 years after he had suffered from recurrent oral aphthous ulcers, genital ulcers, uveitis and erythema nodosum. Laboratory examination demonstrated pleocytosis in the cerebrospinal fluid, a low density area with contrast enhancement in right temporal and parietal lobes in brain CT, a high signal intensity in the same area in T2-weighted image in brain MRI and the occlusion of the right middle cerebral artery on cerebral angiogram. After admission, left homonymous hemianopsia and hemiparesis appeared. With steroid therapy, these symptoms diminished and abnormal findings in brain CT and MRI disappeared, but psychiatric symptoms were exacerbated gradually. Finally he died of agranulocytosis and pneumonia. Neuropathologic findings showed panarteritis of branches of the right middle cerebral artery and infarction of its territories in addition to perivascular infiltrations and foci of demyelination which were prominent in the cerebral basal regions.
报告了一例神经白塞病患者，脑血管造影显示右侧大脑动脉闭塞。一名63岁男性，在反复出现口腔阿弗他溃疡、生殖器溃疡、葡萄膜炎和结节性红斑16年后，出现头痛和低热，眼部和皮肤黏膜病变无加重。实验室检查显示脑脊液中细胞增多，脑部CT显示右侧颞叶和顶叶有低密度区并伴有强化，脑部MRI的T2加权图像中同一区域呈高信号强度，脑血管造影显示右侧大脑中动脉闭塞。入院后，出现左侧同向性偏盲和偏瘫。经类固醇治疗后，这些症状减轻，脑部CT和MRI的异常表现消失，但精神症状逐渐加重。最终，他死于粒细胞缺乏症和肺炎。神经病理学检查结果显示，除了在脑基底区域明显的血管周围浸润和脱髓鞘病灶外，右侧大脑中动脉分支出现全动脉炎及其供血区域梗死。
