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Updated: Oct 14 2020

Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

  • Snapshot
    • A 28-year-old woman is referred to a headache neurologist for throbbing left-sided headaches that are refractory to numerous analgesics. Her headache is associated with episodes of double vision and "vision blurriness." She also endorses worsening headache with a cough and has experienced nausea. Her BMI is 32 kg/m2. Neurological examination is notable for a left-sided sixth nerve palsy, decreased visual fields, and papilledema on fundoscopy. She undergoes an MRI brain with MR venography, which is unremarkable. A lumbar puncture demonstrates a significant opening pressure with normal protein, glucose, and cells. She is started on acetazolamide and is referred to an Ophthalmologist for possible optic nerve sheath fenestration.
  • Introduction
    • Definition
      • signs and symptoms consistent with increased intracranial pressure without an identifiable cause
        • a diagnosis of exclusion
  • Epidemiology
    • Risk factors
      • female gender in childbearing age
      • obesity
      • oral contraceptive pills
      • vitamin A  (isotretinoin)
  • Pathogenesis
    • Believed to be due to reduced cerebrospinal fluid absorption
  • Presentation
    • Symptoms
      • headache
        • typically lateralized and pulsatile in nature
      • nausea and vomiting
      • pulsatile tinnitus
      • diplopia
    • Physical exam
      • papilledema (hallmark)
      • sixth nerve palsy
      • visual field loss
  • Imaging
    • MR brain with MR venography
      • indication
        • preferred initial imaging choice to exclude secondary causes of increased intracranial pressure
      • findings
        • typically normal; however, there are findings suggestive of idiopathic intracranial hypertension
          • posterior sclera flattening
          • vertical tortuosity of the orbital optic nerve
          • perioptic subarachnoid space distension
  • Studies
    • Lumbar puncture
      • most accurate diagnostic test for IIH
      • indication
        • performed after secondary causes of increased intracranial pressure has been excluded on neuroimaging
      • findings
        • elevated opening pressure
        • otherwise normal CSF profile (protein, cells, and glucose)
      • method
        • perform in the lateral decubitus position with the legs extended
    • Ophthalmic examination
      • indication
        • to determine the extent of optic nerve damage from the increased intracranial pressure
  • Differential
    • Migraine
      • differentiating factor
        • no papilledema on fundoscopy
    • Secondary causes of increased intracranial pressure
      • differentiating factor
        • the presence of a malignancy, cerebral sinus thrombosis, or other space occupying lesions on neuroimaging
  • Treatment
    • Medical
      • carbonic anhydrase inhibitors
        • indication
          • first-line treatment for idiopathic intracranial hypertension
        • medication
          • acetazolamide
            • a loop diuretic can also be added as adjunctive therapy
        • mechanism
          • decreases the rate of CSF production
    • Operative
      • optic nerve sheath fenestration
        • indication
          • in patients who fail medical management
  • Complications
    • Blindness
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