Concurrent Intracranial Hypertension and Bilateral Optic Neuritis in a Patient With Systemic Lupus Erythematosus: A Case Report

Abstract

Systemic lupus erythematosus (SLE) involves dysregulation of the immune system, consequently affecting multiple organ systems, including the cardiovascular, neuropsychiatric, renal, and musculoskeletal systems. Optic neuritis and intracranial hypertension are conditions that rarely occur in SLE, and their coexistence has not been reported to date. Herein, we report the first case of a patient who was diagnosed with SLE complicated by concurrent intracranial hypertension and bilateral optic neuritis. An 11-year-old Thai girl had a low-grade fever, discoid rash, oral ulcer, chronic headache, and fluctuating diplopia. She experienced bilateral vision loss just before presentation. She was diagnosed with juvenile SLE. We believe that her headache, which was probably a symptom of optic disc edema, was due to intracranial hypertension. Furthermore, she exhibited vision loss and color vision deficit and was diagnosed with bilateral optic neuritis. Her condition improved on treatment with corticosteroids (intravenous pulse methylprednisolone for 3 days, followed by 1 mg/kg/day oral prednisolone tapered over 3 months). The occurrence of optic neuritis and intracranial hypertension during an active SLE inflammation and a rapid response to high-dose corticosteroids support the fact that SLE was the etiology of these neuropsychiatric conditions. Early diagnosis and prompt treatment in such cases can lead to favorable outcomes.

Keywords: Systemic lupus erythematosus; idiopathic intracranial hypertension; optic neuritis.

Figure 1.

Gazes positions showing bilateral limited abduction in the left and right photos.

Figure 2.

Optic disc photo of both the eyes showing optic disc edema with peripapillary hemorrhage and cotton wool spots. Macular edema is noted on the left eyeground examination.

Figure 3.

Magnetic resonance imaging of the brain and orbit with gadolinium contrast showing faint enhancement of both optic nerves (top photo showing axial view, and the bottom photo showing coronal view).

Figure 4.

Optical coherence tomography angiography of both the eyes showing a normal foveal avascular zone (top photo representing the right eye and bottom photo representing the left eye). A hyporeflective space underneath the submacular area of the left eye is noted.

Figure 5.

Flash visual evoked potential (VEP) showing delayed P2 in both eyes with more prominence on the right. Top photo showing the VEP graph and bottom photo showing its results.