Ballut Omar, Almahi Mayas M, Alghamdi Banan S, Alzahrani Najla K, Alghamdi Maali A
Internal Medicine, King Fahad Hospital, Al-Baha, SAU.
Internal Medicine, Royal College of Physicians of Edinburgh, Edinburgh, GBR.
Cureus. 2024 Feb 21;16(2):e54664. doi: 10.7759/cureus.54664. eCollection 2024 Feb.
Behçet disease (BD) is a recurrent, multisystemic autoimmune vasculitis that affects both small and large vessels. A combination of neurological signs and symptoms in BD is called neuro-Behçet syndrome (NBS). We present the case of a 31-year-old male diagnosed with chronic progressive NBS who presented with multiple relapsing episodes concurrent with infective endocarditis due to intravenous drug abuse, drug-induced hepatitis, acute kidney injury, and septic shock that is not related to BD. Neurological relapsing episodes were treated with steroids azathioprine and colchicine. At the same time, concurrent illnesses were managed appropriately. Infective endocarditis needed valve replacement surgery, and sepsis was treated with selected antibiotics. Fortunately, the patient's brain images and laboratory investigation improved accordingly. On average, patients with parenchymal neuro-Behçet syndrome (P-NBS) have a poor prognosis; within 10 years of diagnosis, 50% of those patients are severely disabled as our patient who became aphasic and quadriplegic.
白塞病(BD)是一种复发性、多系统自身免疫性血管炎,可累及小血管和大血管。BD 中神经症状和体征的组合称为神经白塞综合征(NBS)。我们报告了一例 31 岁男性慢性进行性 NBS 病例,该患者因静脉药物滥用并发感染性心内膜炎、药物性肝炎、急性肾损伤和与 BD 无关的感染性休克,出现多次复发发作。神经复发发作采用类固醇、硫唑嘌呤和秋水仙碱治疗。同时,对并发疾病进行了适当处理。感染性心内膜炎需要进行瓣膜置换手术,败血症采用选定的抗生素治疗。幸运的是,患者的脑部影像和实验室检查结果相应改善。平均而言,实质性神经白塞综合征(P-NBS)患者预后较差;在诊断后的 10 年内,50% 的患者会严重致残,就像我们的患者那样,出现了失语和四肢瘫痪。