College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia; King Abdullah International Medical Research Center, Jeddah, Saudi Arabia; Department of Neurosciences, Ministry of the National Guard-Health Affairs, Jeddah, Saudi Arabia.
Neuroscience Department, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia.
Mult Scler Relat Disord. 2024 May;85:105558. doi: 10.1016/j.msard.2024.105558. Epub 2024 Mar 20.
Neuro-Behçet's disease (NBD) is defined as primary neurological involvement in patients with systemic symptoms of BD. The variety of clinical presentations seen in NBD and the long list of similar conditions make diagnosis challenging. This retrospective study aimed to estimate the prevalence and describe neurological involvement in patients with Behçet's disease who presented to King Abdulaziz Medical Cities in Jeddah and Riyadh, Saudi Arabia.
This was a retrospective, cohort study which utilized a non-probability consecutive sampling technique to include all patients diagnosed with NBD patients. All patients with BD (215) were screened for neurological symptoms. Thirty-five patients were found to be diagnosed with NBD. Outcomes were estimated using the modified Rankin scale (mRS).
In our cohort, one in six patients with BD was diagnosed with NBD. A total of 35 patients were diagnosed with NBD (mean age 27.56 ± 10.36 years; [2.88:1; Male: Female]). The main clinical features of NBD were headaches, weakness, unsteadiness, and dysarthria. The most commonly involved sites on imaging were the brainstem, diencephalon, cerebellum and basal ganglia. Oligocolonal bands were negative in all patients. Maintenance therapy most commonly included oral corticosteroids, azathioprine, and/or infliximab. Most patients received pulse corticosteroids alone when presenting with acute relapse. Half of our cohort was asymptomatic and three in four had favorable outcomes.
NBD is common among patients with BD in our population with most patients having favorable outcomes. Patients might have a wide array of symptoms which might make the diagnosis challenging.
神经贝赫切特病（NBD）定义为系统性贝赫切特病（BD）患者的原发性神经系统受累。NBD 的临床表现多种多样，且与许多类似疾病有相似的症状，这使得诊断具有挑战性。本回顾性研究旨在估计沙特阿拉伯吉达和利雅得阿卜杜勒阿齐兹国王医疗城就诊的贝赫切特病患者中 NBD 的患病率并描述其神经受累情况。
这是一项回顾性队列研究，采用非概率连续抽样技术纳入所有诊断为 NBD 的患者。对所有 BD 患者（215 例）进行神经系统症状筛查。发现 35 例患者被诊断为 NBD。使用改良 Rankin 量表（mRS）评估结局。
在我们的队列中，每 6 例 BD 患者中就有 1 例被诊断为 NBD。共诊断 35 例 NBD 患者（平均年龄 27.56 ± 10.36 岁；[2.88:1；男：女]）。NBD 的主要临床特征是头痛、无力、不稳和构音障碍。影像学上最常受累的部位是脑干、间脑、小脑和基底节。所有患者的寡克隆带均为阴性。维持治疗最常包括口服皮质类固醇、硫唑嘌呤和/或英夫利昔单抗。大多数患者急性复发时单独接受脉冲皮质类固醇治疗。我们队列的一半患者无症状，四分之三的患者结局良好。
在我们的人群中，BD 患者中 NBD 很常见，大多数患者结局良好。患者可能有一系列症状，这可能使诊断具有挑战性。
