Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Osmangazi University, Eskişehir, Turkey.
Department of Internal Medicine, Eskisehir Osmangazi University, Eskisehir, Turkey.
Z Rheumatol. 2024 Feb;83(Suppl 1):200-205. doi: 10.1007/s00393-023-01436-0. Epub 2023 Oct 17.
Neurologic involvement in Behçet disease (BD) is a rare manifestation. Herein, we aimed to evaluate the clinical features and treatment choices of neuro-Behçet (NB) patients.
There were records of 800 BD patients between 1998 and 2021. Fifty-five of the BD patients had NB and the files of these patients were retrospectively evaluated. Patients were grouped into three subgroups: 22 (40%) had non-parenchymal, 25 (45%) had parenchymal, and 8 (15%) had both parenchymal and non-parenchymal (mixed) involvement.
Of the 55 patients, 32 were male. Twenty-six of the NB patients were diagnosed with BD simultaneously. The most common complaint was headache (n = 24, 44%). The most affected site was periventricular white matter (n = 21, 38%). All patients had received corticosteroids. Azathioprine (AZA; n = 39, 71%) was the most common immunosuppressive agent after corticosteroids, followed by cyclophosphamide (n = 16, 29%).
Neurologic involvement is a rare complication of BD but is related to increased mortality and morbidity. Neurologic manifestations may be the initial symptom of BD, thus leading to diagnosis. Both neurology and rheumatology specialists should be aware of this rare condition.
神经贝赫切特病（NB）是一种罕见的贝赫切特病（BD）表现。本研究旨在评估神经贝赫切特病（NB）患者的临床特征和治疗选择。
1998 年至 2021 年，我们有 800 例 BD 患者的记录。其中 55 例 BD 患者有 NB，回顾性评估这些患者的病历。患者分为三组：22 例（40%）为非实质型，25 例（45%）为实质型，8 例（15%）为同时存在实质和非实质（混合）型。
55 例患者中，32 例为男性。26 例 NB 患者同时被诊断为 BD。最常见的主诉是头痛（n=24，44%）。最常受累的部位是脑室周围白质（n=21，38%）。所有患者均接受过皮质类固醇治疗。皮质类固醇治疗后，最常用的免疫抑制剂是硫唑嘌呤（AZA；n=39，71%），其次是环磷酰胺（n=16，29%）。
神经受累是 BD 的罕见并发症，但与死亡率和发病率增加有关。神经表现可能是 BD 的首发症状，从而导致诊断。神经科和风湿病科医生都应该了解这种罕见疾病。
