Patel Puja, Steinschneider Mitchell, Boneparth Alexis, Lantos George
Montefiore Medical Center and Jacobi Medical Center, Department of Neurology, Bronx, NY, USA
Albert Einstein College of Medicine and Jacobi Medical Center, Department of Neurology, Bronx, NY, USA.
J Child Neurol. 2014 Sep;29(9):NP86-91. doi: 10.1177/0883073813504137. Epub 2013 Oct 3.
Behçet disease is a systemic vasculitis of unknown etiology that can affect the neurologic system. Neuro-Behçet disease is not well defined in children and adolescents, and the diagnosis is difficult to make in this population as they often present with insufficient symptoms to meet diagnostic criteria. Psychiatric symptoms as the initial manifestation of neuro-Behçet disease has rarely been reported. We describe a 17-year-old boy who presented with acute psychosis and was subsequently diagnosed with neuro-Behçet disease. A rare combination of both cerebral venous thrombosis and parenchymal central nervous system involvement was identified by neuroimaging. Although treatment guidelines for neuro-Behçet disease are limited, the patient made demonstrative clinical and radiographic improvement with a combination of corticosteroids, anticoagulation, and immunosuppressants, including a tumor necrosis factor-α (TNFα) blocking agent.
白塞病是一种病因不明的系统性血管炎,可累及神经系统。儿童和青少年的神经白塞病尚无明确界定,该人群的诊断较为困难,因为他们的症状往往不足以满足诊断标准。以精神症状作为神经白塞病的初始表现鲜有报道。我们描述了一名17岁男孩,他以急性精神病起病,随后被诊断为神经白塞病。神经影像学检查发现了一种罕见的脑静脉血栓形成与实质性中枢神经系统受累并存的情况。尽管神经白塞病的治疗指南有限,但该患者通过联合使用皮质类固醇、抗凝剂和免疫抑制剂(包括肿瘤坏死因子-α 阻滞剂)后,临床和影像学表现有明显改善。