Diffuse cerebral edema with leukoencephalopathy revealing systemic lupus erythematosus: A case report and review of literature

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.

Keywords: Leukoencephalopathy; cerebral edema; cerebral venous thrombosis; systemic lupus erythematosus.