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本文报告一例经尸检证实的神经-白塞氏病,并结合文献对其发生率、病因、病理变化、临床表现、诊断与鉴别诊断、治疗及预后加以复习。 本例病变分布广泛,累及皮肤、眼、口、生殖器、周围血管、肝及中枢神经系统等部位。在中枢神经系统,病变主要波及大脑,脑干和脊髓少见。应当注意,BD在临床上,当其神经症状为首发或主要表现,而其它症状出现较迟或较轻时,可能漏诊;在BD确定的基础上,对合并神经系统损害者,应高度考虑nB的可能性。病理诊断应密切结合临床表现,并除外其它神经系统疾病,特别是脱髓鞘疾病,如多发性硬化、急性播散性脑脊髓炎和视神经脊髓炎。
This article reports an autopsy confirmed neuro-Behcet’s disease, combined with the literature on its incidence, etiology, pathological changes, clinical manifestations, diagnosis and differential diagnosis, treatment and prognosis to be reviewed. The extensive distribution of lesions in this case, involving the skin, eyes, mouth, genitalia, peripheral blood vessels, liver and central nervous system and other parts. In the central nervous system, lesions mainly affect the brain, brain stem and spinal cord rare. It should be noted that BD may be misdiagnosed clinically when its neurological symptoms are the first or major manifestation while other symptoms appear later or less severe; BD should consider nB The possibility of. Pathological diagnosis should be closely combined with clinical manifestations, with the exception of other neurological diseases, especially demyelinating diseases such as multiple sclerosis, acute disseminated encephalomyelitis and optic neuromyelitis.