What is the treatment for idiopathic intracranial hypertension (IIH)?

Treatment of Idiopathic Intracranial Hypertension (IIH)

The first-line treatment for idiopathic intracranial hypertension is acetazolamide combined with weight loss for overweight patients, with surgical intervention reserved for cases with progressive visual loss or medication-refractory disease. 1

Medical Management

First-Line Pharmacotherapy

• Acetazolamide: Start at 250-500mg twice daily, maximum 4g daily as tolerated
  • Works through carbonic anhydrase inhibition to reduce intracranial pressure
  • Should not be discontinued without careful consideration of visual risk 1

Alternative Medications

• Topiramate: Consider when acetazolamide is not tolerated
  • Start at 25mg daily with weekly escalation to 50mg twice daily
  • Has dual benefit of weight loss and ICP reduction
  • Caution: Side effects include depression, cognitive slowing, reduced contraceptive efficacy 1
• Zonisamide: Second-line option when topiramate has excessive side effects 1
• Corticosteroids: Only for severe visual loss requiring rapid intervention
  • Not recommended for long-term use due to side effects 1

Lifestyle Modifications

• Weight loss: Essential for patients with BMI >30 kg/m²
  • Target 5-15% reduction in body weight 1
• Dietary changes:
  • Regular meals
  • Limited caffeine intake
  • Low-salt regimen 1, 2
• Exercise program as tolerated 1
• Improved sleep hygiene 1

Pain Management

• Short-term headache relief:

  • NSAIDs or paracetamol
  • Indomethacin may have additional benefit due to ICP-reducing effect
  • Triptans for migraine-like attacks (limit to 2 days/week or maximum 10 days/month) 1

• Avoid:

  • Opioids
  • Medication overuse (simple analgesics >15 days/month or triptans >10 days/month) 1

Surgical Interventions

When to Consider Surgery

• Medically refractory IIH
• Progressive visual loss
• Fulminant IIH with rapid symptom worsening 1, 3

Surgical Options

1. CSF Diversion Procedures:

   • Ventriculoperitoneal (VP) shunt: Preferred due to lower revision rates 1
   • Lumboperitoneal shunt: Alternative option 1

2. Optic Nerve Sheath Fenestration (ONSF):

   • Primarily for visual symptoms
   • Improves papilledema in 90.5% of cases 1, 4

3. Venous Sinus Stenting:

   • For patients with venous sinus stenosis who have failed medical therapy
   • Requires demonstration of significant pressure gradient across stenosis
   • Can rapidly improve symptoms and abolish papilledema 1, 4

Monitoring and Follow-up

• Regular ophthalmologic evaluations:

  • Visual acuity
  • Visual fields
  • Fundoscopy for papilledema
  • Optical coherence tomography (OCT)
  • Frequency based on severity of papilledema 1

• Long-term follow-up:

  • Extended follow-up (>5 years) is essential
  • Late recurrences can occur (10-18% rate) 1

Treatment Algorithm

1. Initial presentation:

   • Start acetazolamide + weight loss program (if BMI >30)
   • Initiate headache management with NSAIDs/paracetamol

2. If inadequate response:

   • Increase acetazolamide dose or switch to topiramate/zonisamide
   • Intensify weight loss efforts

3. For progressive visual loss or refractory cases:

   • Consider surgical intervention:
     • VP shunt if headache predominant
     • ONSF if visual loss predominant
     • Venous sinus stenting if venous stenosis present 1, 4

Important Considerations

• Visual monitoring is critical: The main morbidity of IIH is visual loss, which can usually be reversed if recognized early and treated appropriately 1

• Fulminant IIH: Some patients experience rapid worsening of symptoms over days, requiring prompt surgical intervention to prevent vision loss 3

• Surgical option selection: Recent evidence suggests venous sinus stenting may provide the best results for headache resolution and visual outcomes with lower complication and failure rates compared to other surgical options 4