Hadfield M G, Aydin F, Lippman H R, Sanders K M
Department of Pathology, Medical College of Virginia, Commonwealth University, Richmond 23298, USA.
Clin Neuropathol. 1997 Mar-Apr;16(2):55-60.
We are reporting a case of Bechçet's disease without vasculitis but with acute neutrophilic inflammation which involved the brain and other organs. The patient exhibited waxing and waning neurological deficits which were unresponsive to treatment. The neuroradiologic findings simulated those of multiple sclerosis. The neuropathological examination revealed an acute, focal, though disseminated encephalitis involving the frontal lobe, internal capsule, basal ganglia, cerebellum, and brainstem. The acute inflammation consisted of a neutrophilic and eosinophilic infiltration of the perivascular spaces and parenchyma without evidence of vasculitis, fibrinoid necrosis, or thrombosis. Cultures and special stains for microbial organisms were negative. Ultrastructural examination revealed no viral structures or other microorganisms. In situ hybridization for Epstein-Barr virus (EBV), herpes simplex virus type I (HSV-I), and cytomegalovirus (CMV) was negative. In this case, the lack of inflammation in the vessel walls points out that the necrotizing lesions in neuro-Bechçet's disease need not be the result of vasculitis and superimposed thrombosis, but may occur as a result of primary, acute neutrophilic inflammation.
我们报告一例无血管炎但伴有急性中性粒细胞炎症的白塞病，该炎症累及大脑和其他器官。患者出现反复出现和缓解的神经功能缺损，对治疗无反应。神经放射学检查结果类似于多发性硬化症。神经病理学检查显示为急性、局灶性、尽管呈播散性的脑炎，累及额叶、内囊、基底节、小脑和脑干。急性炎症表现为血管周围间隙和实质内的中性粒细胞和嗜酸性粒细胞浸润，无血管炎、纤维蛋白样坏死或血栓形成的证据。微生物培养及特殊染色均为阴性。超微结构检查未发现病毒结构或其他微生物。针对爱泼斯坦 - 巴尔病毒（EBV）、单纯疱疹病毒I型（HSV-I）和巨细胞病毒（CMV）的原位杂交均为阴性。在该病例中，血管壁无炎症表明神经白塞病中的坏死性病变不一定是血管炎和叠加血栓形成的结果，而可能是原发性急性中性粒细胞炎症导致的。
