Acetazolamide
The first-line medical therapy is acetazolamide, which reduces CSF production by the choroid plexus. In the recent IIH Treatment Trial, acetazolamide, in addition to weight loss, was found to be more effective than weight loss alone in patients with IIH and mild visual loss. The starting dose of 500 mg twice daily can be increased up to 3-4 g daily depending on the severity of the visual loss, papilledema grade, and response to lower doses. Acetazolamide is less effective for the treatment of headaches associated with IIH. Patients with concomitant migraine or tension-type headaches may benefit from treatment by a headache specialist.
Topiramate
Topiramate has a similar effect to acetazolamide on CSF production and is also an effective treatment for migraine headaches, which often co-occur in patients with IIH.
Furosemide
Furosemide may be tried in patients intolerant to acetazolamide or topiramate, but has less effect on CSF production.
Weight Loss
Weight loss is important in reversing the underlying pathophysiology of IIH. Loss of only 6-10% of body weight is beneficial, and often patients can be tapered off of acetazolamide once this weight loss target is reached. Consultation with a nutritionist or involvement in an intensive weight-loss program may be helpful.
Surgical Treatments
Traditional surgical treatments, such as CSF diversion (ventriculoperitoneal shunt or lumboperitoneal shunt) and optic nerve sheath fenestration, are appropriate for patients with progressive visual loss due to papilledema that is unresponsive to medical therapy or weight loss.
Transverse cerebral venous sinus stenting is an endovascular treatment for medically refractory patients, but it has not been studied in a randomized-controlled clinical trial. Preliminary case series suggest efficacy in select patients with appropriate venous anatomy.
Robert M. Mallery, MD
Assistant Professor of Neurology, Harvard Medical School
Department of Neurology, Brigham and Women’s Hospital
Department of Ophthalmology, Mass. Eye and Ear