神经性贝赫切特综合征是贝赫切特病（BD）的一种严重而罕见的表现形式，由于其具有挑战性的临床表现，可能会被误诊。本文介绍了一位20岁的生理性男性患者的病例，患者出现间歇性发热、双侧葡萄膜炎和神经症状，脑部成像显示多个脑干肿块。通过仔细的病史询问，发现有反复出现的口腔和生殖器溃疡，这对于作出正确的诊断非常重要。鉴于还没有经过验证的标准或明确的检测方法来确认神经性贝赫切特病，通常是在排除其他潜在病因后通过排斥法来确诊。在我们的病例中，在对传染性、神经退行性和恶性病因进行广泛检查后，结合患者的病史，确诊为伴有神经损害的贝赫切特病（神经性贝赫切特综合征）。患者接受了高剂量的类固醇治疗，疗效良好。两年后进行的反复脑磁共振成像显示没有新的脑病变。在正确的临床背景下，应将神经性贝赫切特疾病纳入未明确解释的脑干病变的鉴别诊断中。在这些情况下，医务人员应获取与生殖器和口腔溃疡以及眼部问题相关的病史资料，因为这些资料有助于缩小诊断范围。本文讨论了贝赫切特病的这种罕见表现的临床表现和挑战，包括神经性贝赫切特病伴有脑干肿块的文献综述。 版权所有，2023 Ayoade, Hernandez, Montreuil, Drews-Elger, Quiroz and Sternberg.

Neuro-Behçet's syndrome, a severe and rare manifestation of Behçet's disease (BD), can be misdiagnosed due to its challenging clinical presentation. This article presents the case of a 20-year-old cis-gender male with intermittent fever, bilateral uveitis, and neurological symptoms who was found to have multiple brain stem mass lesions on brain imaging. A careful medical history elicited recurrent painful oral and genital ulcerations which were important in making the correct diagnosis. As there are no validated criteria or definite set of tests available to confirm neuro-Behçet's disease, the diagnosis is often established by exclusion after ruling out other potential etiologies. In our case, after an extensive negative workup for infectious, neuro-degenerative and malignant etiologies combined with the patient's medical history, a diagnosis of Behçet's disease with neurological involvement (neuro-Behçet's syndrome) was made. High doses of steroids were given, and the patient had a favorable outcome. Repeated magnetic resonance imaging of the brain 2 years later showed no new brain lesions. Neuro-Behçet's disease should be included as a differential diagnosis of unexplained brain stem lesions in the right clinical context. In these situations, providers should obtain medical histories related to genital and oral ulcers and eye problems as these may help to narrow down the diagnosis. The clinical presentation and challenges of this uncommon presentation of BD including a brief literature review of neuro-Behçet's disease with brain stem mass lesions are discussed in this case study.Copyright © 2023 Ayoade, Hernandez, Montreuil, Drews-Elger, Quiroz and Sternberg.