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