Intern Report 2/9 – Idiopathic Intracranial Hypertension (IIH) aka Pseudotumor Cerebri

Teaching Points:

  • Disorder defined by clinical criteria that include:
    • symptoms and signs isolated to those produced by increased intracranial pressure (headache, papilledema, vision loss)
    • elevated intracranial pressure with normal CSF composition
    • no other causes of intracranial hypertension evident on neuroimaging
  • Clinical Manifestations
    • Young obese female of childbearing age complaining of headaches found to have papilledema on fundoscopic exam.
    • Symptoms include headache, transient visual obscurations, intracranial noises (pulsatile tinnitus), photopsia, back pain, retrobulbar pain, diplopia, sustained vision loss.
  • Exam Findings
    • Most common findings are papilledema, visual field loss, sixth nerve palsy.
  • Evaluation
    • Urgent neuroimaging is required to exclude secondary causes of intracranial hypertension.
      • MRI brain with MR venography
    • If imaging is negative, LP is performed to check opening pressure.
  • Differential Diagnosis (Headache and papilledema)
    • Intracranial mass lesions
    • Obstruction of venous outflow
      • Venous sinus thrombosis
      • Jugular vein compression
      • Neck surgery
    • Obstructive hydrocephalus
    • Decreased CSF absorption
      • bacterial meningitis causing arachnoid granulation adhesions
    • Increased CSF production
      • Choroid plexus papilloma
    • Malignant systemic hypertension
  • Treatment
    • Weight loss, treat OSA or anemia if present, Avoid exacerbating drugs (tetracycline derivatives, Vit A analogs, etc)
    • Acetazolamide. May consider Lasix if symptoms persist
    • Surgical shunting

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