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A 30-year-old African-American woman with a 4.5-year history of systemic lupus erythematosus (SLE), and well-documented antiphospholipid syndrome for which she was taking warfarin, noted “spots” in her peripheral vision, first in the right eye, then in the left. She had neither headache nor other new systemic symptoms. She did not have an ophthalmological examination until 3 months later, at which time she had normal visual acuity, colour vision and visual fields by automated perimetry, but moderate bilateral optic disc swelling. No structural lesions or venous thromboses were seen on magnetic resonance imaging (MRI) and magnetic resonance venography (MRV). A lumbar puncture revealed an opening intracranial pressure (ICP) of 30 cm H2O with markedly elevated cerebrospinal fluid (CSF) protein of 700 mg/dl. The patient underwent four infusions of rituximab without response. She was prescribed prednisone 5 mg/day and continued warfarin for antiphospholipid antibody syndrome.
Over the next 5 months, the patient’s CSF pressure and protein remained elevated. Although the appearance of her optic discs remained unchanged, her visual fields showed enlargement of the blind spots and she began to experience transient visual obscurations; surgical intervention (optic nerve sheath fenestration (ONSF) or CSF shunting) was proposed. The patient was referred to the Neuro-Ophthalmology Division of the Wilmer Eye Institute at Johns Hopkins Hospital for further evaluation.
At the time of her assessment in our clinic in April 2007, the patient’s visual acuity was 20/25 right eye (OD), 20/20 left eye (OS). Colour vision was normal, but static perimetry showed an enlarged blind spot in the visual field of the right eye associated with significant superior and inferior nasal steps (fig. 1A). The left eye also showed an …