Chen Yongzhen, Banerjee Sumona, Khasiyev Farid, Kiaei Benjamin, Valasareddy Sanhitha, Kilian Adam, Soudagar Turkey Momina
Neurology, Saint Louis University School of Medicine, Saint Louis, USA.
Rheumatology, Saint Louis University School of Medicine, Saint Louis, USA.
Cureus. 2024 Jul 16;16(7):e64701. doi: 10.7759/cureus.64701. eCollection 2024 Jul.
Behçet's disease is a rare multisystemic vasculitis characterized by oral ulcers, genital ulcers, and skin and ocular lesions. Neuro-Behçet's syndrome is a condition in which individuals with Behçet's disease experience neurological symptoms that cannot be attributed to other neurological diseases. We present a rare case of neuro-Behçet's syndrome with acute internuclear ophthalmoplegia and deteriorating neurological function with a prior history of recurrent oral ulcers with pathergy-like features, acneiform papulopustular rash, retinal hemorrhages, and recurrent epididymitis without genital ulcers. Patient improved with cyclophosphamide. This case underscores the importance of diagnosing and managing neuro-Behçet's syndrome in the absence of genital ulcers.
白塞病是一种罕见的多系统血管炎，其特征为口腔溃疡、生殖器溃疡以及皮肤和眼部病变。神经白塞综合征是指患有白塞病的个体出现无法归因于其他神经系统疾病的神经症状的一种病症。我们报告一例罕见的神经白塞综合征病例，该患者有急性核间性眼肌麻痹且神经功能不断恶化，既往有复发性口腔溃疡伴同形反应样特征、痤疮样丘疹脓疱性皮疹、视网膜出血，以及复发性附睾炎但无生殖器溃疡。患者经环磷酰胺治疗后病情好转。该病例强调了在无生殖器溃疡情况下诊断和管理神经白塞综合征的重要性。
