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Updated: Feb 12 2017

Pseudotumor Cerebri

Snapshot
  • An obese 31-year-old female presents to the neurologists office complaining of severe headaches, visual obscurations, ringing in the ears and what her primary care doctor called "papilledema". She has been taking large amounts of vitamin A because a friend told her that it would give her more energy. Examination of the patient shows limited abduction of both eyes.
Introduction
  • Idiopathic intracranial hypertension
    • characterized by headache, increased ICP, and papilledema
      • usually not explained by any other identifiable cause
  • Epidemiology
    • incidence
      • 1 per 100,000
    • demographics
      • more common in younger women
      • median age is approximately 30 years old
    • risk factors
      • child-bearing aged women
      • obesity
      • medications
        • excessive vitamin A or D intake
        • growth hormone
        • OCPs
        • discontinuation of steroids
  • Associated conditions
    • cushings
    • steroid use
    • pregnancy
  • Prognosis 
    • does not seem to alter life expectancy
Presentation
  • Symptoms
    • pulsatile tinnitus
    • 6th nerve (abducens) palsies 
    • severe headaches
    • visual disturbances
  • Physical exam
    • inspection
      • papilledema on fundoscopic exam
Evaluation
  • Imaging studies
    • show normal or "slit" ventricles
  • Diagnosis
    • spinal tap 
      • elevated CSF pressure (usually > 50 cm)
      • normal CSF profile
    • PE
      • papilledema
      • abducens nerve palsies
    • imaging
      • slit-like ventricles
      • otherwise normal brain MRI
Treatment
  • Conservative
    • weight loss
  • Pharmacologic
    • acetazolamide
      • outcomes
        • reduce CSF production
      • side effects
        • abdominal pain
        • kidney stones
        • hypokalemia
          • extremity paresthesias
          • muscle weakness
    • lasix (furosemide)
      • indications
        • when acetazolamide isn't working well enough
      • outcomes
        • reduce fluids by increasing urine output
  • Operative
    • spinal taps or shunts
      • outcomes
        • symptoms can immediately improve by reducing CSF pressure
    • optic nerve sheath fenestration
      • outcomes
        • cut a window into the membrane surrounding the optic nerve
        • allows excess CSF to evacuate
        • visual stabilization
  • Untreated pseudotumor cerebri may lead to development of complete blindness 
Question
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