Abstract：
Background: Systemic lupus erythematosus (SLE) is a common autoimmune disease with various symptoms involving multiple organs. Neuropsychological manifestations are various and generally serious. Leukoencephalopathy is particularly rare but life-threatening in patients with SLE. Results: Here, we describe the case of a young woman who developed a subacute onset intracranial hypertension, papillar edema on fundus examination, diffuse cerebral edema on brain CT scan, and diffuse leukoencephalopathy on brain magnetic resonance imaging (MRI). The immunological workup was positive for antinuclear antibodies, anti-DNA and anti-extractable nuclear antigens (ENA) antibodies. She was ultimately diagnosed with SLE and experienced significant improvement after treatment with high dose of corticosteroids, acetazolamide, and immunosuppressant. We additionally review the previously reported cases of SLE with diffuse cerebral edema and leukoencephalopathy with a focus on the possible pathophysiological mechanisms of such association. Conclusions: We highlight, through this case report and the literature review, the importance of considering SLE in patients with cerebral edema and diffuse leukoencephalopathy and treating it aggressively.
摘要：
背景：系统性红斑狼疮（SLE）是一种常见的自身免疫性疾病，症状多样，累及多个器官。神经心理学表现多种多样，通常很严重。在SLE患者中，白质脑病特别罕见，但危及生命。结果:这里,我们描述了一个年轻女性的病例，她出现了亚急性颅内高压，眼底检查乳头水肿，脑CT扫描弥漫性脑水肿，脑磁共振成像（MRI）和弥漫性白质脑病。免疫检查对抗核抗体呈阳性，抗DNA和抗可提取核抗原（ENA）抗体。她最终被诊断为SLE，并在使用高剂量皮质类固醇治疗后经历了显着改善，乙酰唑胺,和免疫抑制剂.我们还回顾了先前报道的SLE伴有弥漫性脑水肿和白质脑病的病例，重点是这种关联的可能的病理生理机制。结论：我们强调，通过本病例报告和文献综述,在脑水肿和弥漫性白质脑病患者中考虑SLE并积极治疗的重要性。
