PDF(Pubmed)
Abstract：
Neuro-Behçet\'s disease (NBD) represents a significant complication of Behçet\'s syndrome, potentially leading to elevated mortality and disability rates. The standard treatment for parenchymal NBD typically entails administering high-dose corticosteroids to prompt rapid-onset effects, coupled with immunosuppressants to prevent subsequent relapses. A 48-year-old male with NBD presented with progressively worsening dysarthria over 9 months. This patient experienced increased intraocular pressure while using glucocorticoids, which worsened his pre-existing glaucoma. The patient had a prior diagnosis of NBD and presented with progressive dysarthria over a period of nine months, leading to a brain magnetic resonance imaging (MRI) scan. The brain MRI revealed multifocal punctate high signal intensities in the left frontoparietal area, insula, and basal ganglia. Instead of the standard steroid pulse therapy, the patient received adalimumab-cyclophosphamide combination as an alternative induction therapy. Subsequent serial brain MRI scans exhibited no emergence of new lesions, and the patient remained devoid of clinical relapses even after 17 months from the commencement of induction treatment. Adalimumab-cyclophosphamide combination could be used as a corticosteroid-free induction strategy for NBD. Further investigations are warranted to establish the most suitable combination regimen.
摘要：
神经-Behçet病(NBD)是Behçet综合征的重要并发症,可能导致死亡率和残疾率上升。实质性NBD的标准治疗通常需要给予高剂量皮质类固醇以促进快速起效。加上免疫抑制剂，以防止随后的复发。一名患有NBD的48岁男性在9个月内表现出逐渐恶化的构音障碍。该患者在使用糖皮质激素时出现眼内压升高，恶化了他先前的青光眼.该患者先前诊断为NBD，并在9个月内出现进行性构音障碍。导致脑部磁共振成像（MRI）扫描。脑部MRI显示左额顶区域多灶性点状高信号强度，脑岛,和基底神经节.而不是标准的类固醇脉冲疗法，患者接受阿达木单抗-环磷酰胺联合治疗作为替代诱导治疗.随后的系列脑部MRI扫描显示没有出现新的病变，即使在开始诱导治疗后17个月，患者仍然没有临床复发。阿达木单抗-环磷酰胺组合可用作NBD的无皮质类固醇诱导策略。需要进一步的研究以建立最合适的联合方案。
