Pseudotumor Cerebri

Bilateral papilledema in a young woman with pseudotumor cerebri. Note the small retinal ripples, Paton's lines (white arrowheads) and nerve fiber hemorrhage (yellow arrowhead) in the right eye. 

  • A syndrome that is defined by:
    • Papilledema occurs in over 90% of patients
    • Elevated intracranial pressure with normal CSF composition
    • Normal neuroimaging studies
    • Normal neurologic exam
  • Occurs primarily in young obese females

Clinical features:

  • Most common symptom is headache.
  • Other symptoms include normal or uncommonly decrease visual acuity, transient obscurations of vision, photopsias, diplopia, nausea, vomiting, pulsatile tinnitus and visual field loss secondary to optic disc swelling
  • Signs:
    • Bilateral optic disc edema
    • May have abducens nerve palsy

Work up:

  • Complete ophthalmological and neurological examinations including visual acuity, color vision, pupillary reactivity, fundus examination and visual field study of patients with papilledema
  • Neuroimaging study and lumbar puncture
  • Fundus photographs are also important to monitor disc swelling

Management:

  • Depends on visual status and whether or not the associating cause can be identified or treated
  • Treatment of underlying causes
  • Weight-loss program for obese patients
  • Cessation of substances causing PTC, such as vitamin A, tetracycline, etc.