PDF(Pubmed)
Abstract：
Behçet disease (BD) is a multisystemic relapsing autoimmune vascular disorder. It is clinically characterized by recurrent oral ulcers, genital ulcers, eye, and skin manifestations. Development of neurological symptoms in BD cases is rare and occurs several years after the initial diagnosis. We describe a rare case of a 39-year-old Saudi male who presented with isolated neurological manifestations as the first sign of BD. The patient had recurrent strokes, both ischemic and hemorrhagic, over an 11-month period before developing typical BD features. A thorough investigation excluded other potential etiologies of his neurological disorders. Imaging showed multiple brainstem lesions compatible with parenchymal neuro-BD (NBD). The patient was positive for HLA-B51, a genetic marker linked to BD, but had a negative pathergy test. Treatment with corticosteroids and infliximab resulted in symptom improvement. The diagnosis of NBD requires a comprehensive clinical, imaging, and laboratory assessment to rule out other possible causes. This case demonstrates the need to include NBD in the differential diagnosis of young patients with unexplained neurological manifestations, especially if they are followed by an onset of BD features. Treatment with corticosteroids and biologic agents can achieve favorable outcomes. NBD can present with isolated neurological symptoms, emphasizing the need for a high level of suspicion and a multidisciplinary approach for accurate diagnosis and effective management.
摘要：
Behçet病（BD）是一种多系统复发性自身免疫性血管疾病。它的临床特征是复发性口腔溃疡，生殖器溃疡,眼睛,和皮肤表现。BD病例中神经系统症状的发展很少见，并且在最初诊断后几年发生。我们描述了一名39岁的沙特男性的罕见病例，他表现出孤立的神经系统表现是BD的第一个迹象。病人中风复发,缺血性和出血性，在开发典型BD功能之前的11个月内。彻底的调查排除了他的神经系统疾病的其他潜在病因。影像学显示多个脑干病变与实质神经BD（NBD）相容。该患者的HLA-B51阳性，这是一种与BD相关的遗传标记，但做了一个阴性的体形测试.糖皮质激素和英夫利昔单抗治疗可改善症状。NBD的诊断需要全面的临床,成像,和实验室评估以排除其他可能的原因。该病例表明需要在具有无法解释的神经系统表现的年轻患者的鉴别诊断中包括NBD，特别是如果它们之后是BD特征的发作。用皮质类固醇和生物制剂治疗可以取得良好的结果。NBD可以表现为孤立的神经症状，强调需要高度怀疑和多学科方法来准确诊断和有效管理。
