Pseudotumor cerebri

  • aka idiopathic intracranial hypertension
  • pt:
    • women, childbearing age, obese
    • Vitamin A excess (isotretinoin for acne)
    • Danazol (old synthetic steroid used to suppress gonadotrophins, replaced by GnRH agonists). Glucocorticoids
    • OCP
    • Tetracyclines
  • patho: not understood
  • sx:
    • chronic pulsatile headache, variable, visual disturbances, nausea.
    • blurry vision, diplopia
    • Pulsatile tinnitus: whooshing sounds, wind linke.
    • No alterations in mental status
  • dx:
    • ophthalmoscopic exam for papilledema
    • imaging (MRI) to exclude other causes of increased pressures but will normally show normal finding
    • LP: An opening pressure greater than 20-25 cmH2O can help to confirm the diagnosis of pseudotumor cerebri.
  • Treatment options for idiopathic intracranial hypertension include:
    • Acetazolamide
    • Topiramate
    • Weight loss
    • Invasive procedures (lumbar puncture, shunt placement, optic nerve fenestration) LP relieves pressure

For patients with symptoms refractory to medical therapy or those with progressive vision loss, surgical intervention with optic nerve sheath decompression or lumboperitoneal shunting is recommended. Short-term use of corticosteroids or serial lumbar puncture (LP) can serve as bridging therapy for patients awaiting definitive surgical treatment. These are not recommended as primary intervention due to side effects associated with long-term corticosteroid use and the complications associated with LPs.

Untreated idiopathic intracranial hypertension can lead to blindness.