This case involves a 36-year-old male patient who presented to his primary care physician with headaches. The patient was found to have papilledema optic disc swelling caused by intracranial pressure. The patient was immediately referred to a neuro-ophthalmologist for further review. The neuro-ophthalmologist discovered that the patient had CSF opening pressure and diagnosed the patient with idiopathic intracranial hypertension. In spite of this diagnosis, the nuero-ophthalmologist did not initiate the acetazolamide treatment soon enough and the patient eventually became blind in both eyes as a result. An expert in ophthalmology was sought to review the records and opine on the standard of care.
Question(s) For Expert Witness
- 1. What steps would you take in order to manage a patient with IIH?
Expert Witness Response E-079532
I actively manage more 200 patients with this condition and I have lectured on this topic at least 20-30 times over the last two decades. The most important step is to make certain that the CSF formula is normal. If complete CSF studies are not done then repeat the LP. I would begin acetazolamide at a dose of 500 mg q am of the extended release type (specific generic medications would be requested). If only plain diamox is available, then I would prescribe a 250 mg bid and after 4 days increased to 250 mg q 6 hrs. The patient would be contacted in 7 days regarding their headache, pulsatile tinnitus, and/or any visual obscurations. If these symptoms persist, the dose would be increased to 500 mg ER q12 hrs or if plain diamox is available, 500 mg q 6 hrs. I would typically reevaluate the patient after 6 to 8 wks (visual fields OCT and dilated fundus exam) and adjust dose accordingly. The next visit thereafter at would occur after 3 months. Lab values should be checked while the patient is taking diamox. The patient should have an earlier visit if they experience persistent or increasing symptoms. I have most definitely seen patients with the described outcome. Most of these were either missed diagnosed by the original treating physician or under treated by the original treating MD. I have one or two patients I have managed who have had what I would call a malignant pseudotumor. They have failed to respond to even very aggressive treatment with acetazolamide. They have required either optic nerve fenestration or a VP shunt, but have lost significant vision in either one or both eyes prior to the procedure.