Idiopathic Intracranial Hypertension (IIH)

Case Overview

• A 24-year-old woman presented with a 3-week history of worsening headaches and recent blurry vision.
• Symptoms included:
  • Pulsating, throbbing headache
  • Slight improvement with OTC ibuprofen
  • Worse headaches when lying down
  • Pulsation sensation in ears
• No history of migraines, vision issues, or relevant medical conditions.
• Examination:
  • Obese, BMI: 32 kg/m²
  • Normal vital signs
  • 20/100 vision with glasses, mild optic disc blurring
  • Neurologically intact

Discussion

Definition

• Pseudotumor cerebri: Another name for IIH
• Characterized by high intracranial pressure without causative abnormalities in imaging or CSF analysis.
• Symptoms: headache, tinnitus, papilledema, vision loss.
• Not benign: untreated can lead to permanent vision damage.

Clinical Course

• Unpredictable; vision loss varies in progression.
• Some patients find relief with lumbar puncture (LP), others do not.

Etiology

• Rare condition: affects 1 in 100,000 annually, mostly obese women of childbearing age.
• Cause unknown, possibly linked to a congenital venous sinus malformation.

Symptoms

• Headache: pressure-like, throbbing
• Pulse-synchronous tinnitus
• Eye pain, photophobia, blurry vision
• May be relapsing/remitting

Diagnosis

Imaging

• Noncontrast CT: typically normal
• MRI: subtle findings possible

Lumbar Puncture

• Hallmark: high CSF opening pressure (>25 cm H₂O)
• Normal CSF composition

Differential Diagnosis

• Diagnosis of exclusion after ruling out other causes of ICP increase.
• Considerations include meningitis, cerebral edema, venous sinus thrombosis.

Venous Sinus Thrombosis

• Rare, serious condition; must be considered in differential diagnosis.
• Risk factors: thrombophilias, recent pregnancy, contraceptive use, dehydration, among others.

Management

General

• IIH rarely requires hospital admission; outpatient neuro follow-up essential.

Treatments

• Lumbar Puncture: May relieve symptoms; up to 30 mL of CSF can be safely removed.
• Weight Loss: Rapid weight loss (diet/surgery) can improve symptoms.
• Pharmacotherapy: Acetazolamide as first-line treatment, cautious use of loop diuretics/corticosteroids.

Refractory Cases

• Consider surgical intervention: CSF shunt, optic nerve fenestration, venous sinus stent.

Case Conclusion

• Patient underwent LP; 19 mL of CSF removed, pressure normalized, symptoms resolved.
• CT venogram ruled out venous sinus thrombosis.
• Discharged with acetazolamide prescription and neurology follow-up.

Summary

• IIH: Rare but serious headache disorder
• Diagnosis: High ICP without evidence on CT/CSF analysis
• Treatment: Weight loss, acetazolamide, LP, and possible surgical intervention
• Importance of ruling out venous sinus thrombosis.

References

• Detailed list of studies and papers cited in the original case report.