Nathan H. Kung, Collin M. McClelland, Gregory P. Van Stavern, MD, Associate Professor, Ophthalmology & Visual Sciences and Neurology, Washington University School of Medicine
A 29-year-old woman was referred to neuro-ophthalmology clinic for 1 year of headaches and papilledema discovered 2 months earlier. She complained of recently blurred vision but no positional headache, pulsatile tinnitus, transient visual obscurations, or other neurologic issues. She used no medications and denied any recent illnesses except for thrombocythemia (591k) on recent blood work, and had no significant past medical history. Her initial examination in 4/2009 revealed normal acuity with 20/20 VA OU, equal pupils without RAPD, and severe grade 4 papilledema OU with several choroidal folds through both maculae. No hemorrhages were noted. All other portions of the examination were normal. Her BMI was 27. Humphrey SITA Standard visual fields were performed and showed slight enlargement of the blind spot OU with a nasal step in the L eye. An MRI of the brain and orbits with and without gadolinium was normal with normal venous flow voids. CSF analysis showed an opening pressure of 26 cm H2O with 1 RBC, 1 WBC, Protein 75, Glucose 69, and no abnormalities on cytology. Although she had atypical features, a preliminary diagnosis of Probable Pseudotumor Cerebri was made and she was initiated on acetazolamide, with improved papilledema over the next several months. Over the next three years, however, she developed worsening vision with multiple additional symptoms.