What is the management for idiopathic intracranial hypertension?

Management of Idiopathic Intracranial Hypertension

The management of idiopathic intracranial hypertension (IIH) should begin with acetazolamide at 250-500mg twice daily (titrated up to 4g/day as tolerated) combined with weight loss of 5-15% for patients with BMI >30 kg/m², with surgical intervention reserved for cases with progressive visual loss despite maximal medical therapy. 1

Initial Treatment Approach

Medical Management

1. First-line medication: Acetazolamide

   • Starting dose: 250-500mg twice daily
   • Maximum dose: Up to 4g/day based on tolerance
   • Duration: Initial treatment phase typically lasts 3-6 months 1

2. Alternative medications (if acetazolamide is not tolerated):

   • Topiramate
   • Zonisamide
   • Corticosteroids (only for severe visual loss requiring rapid intervention; not for long-term use) 1

3. Pain management:

   • NSAIDs for short-term pain relief (with gastric protection as needed)
   • Avoid opioids for headache management
   • Caution regarding medication overuse headache (MOH) with analgesics used >15 days/month or triptans >10 days/month 1

Lifestyle Modifications

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

Monitoring Protocol

Frequency of follow-up depends on papilledema severity:

Papilledema Grade	Affected but Stable	Affected but Improving	Affected but Worsening
Mild	3-4 months	3-6 months	Within 4 weeks
Moderate	1-3 months	1-3 months	Within 2 weeks
Severe	Within 1 week	Within 4 weeks	Immediate

Regular monitoring should include:

• Visual field testing
• Fundoscopy
• Visual acuity assessment
• Optical coherence tomography (OCT) 1

Surgical Interventions

Surgical options should be considered when:

1. Visual deterioration occurs despite maximum medical therapy
2. Rapid, severe visual decline is present 1

Surgical Options:

1. CSF diversion procedures:

   • Ventriculoperitoneal (VP) shunt (preferred due to lower revision rates)
   • Lumboperitoneal shunt 1

2. Optic nerve sheath fenestration:

   • Particularly useful for precipitous visual decline 2

3. Venous sinus stenting:

   • For patients with confirmed transverse sinus stenosis
   • Criteria: Failed medical therapy and weight loss, significant pressure gradient (≥8 mmHg) across stenosis, threatened vision or intolerable symptoms 1

Special Considerations

• Serial lumbar punctures are not recommended for long-term management 1
• Extended follow-up (>5 years) is essential as late recurrences can occur (10-18% recurrence rate) 1
• IIH without papilledema can occur and should be considered in obese women with chronic daily headache and symptoms of increased intracranial pressure 3

Treatment Algorithm

1. Initial presentation:

   • Begin acetazolamide + weight loss program
   • Monitor according to papilledema severity schedule

2. If symptoms improve:

   • Continue medical management
   • Gradually extend monitoring intervals

3. If symptoms worsen or vision deteriorates:

   • Increase acetazolamide dose (up to 4g/day)
   • Consider alternative medications
   • If vision continues to deteriorate, proceed to surgical intervention

4. For fulminant IIH or precipitous visual decline:

   • Intravenous steroids and acetazolamide
   • Urgent surgical intervention (preferably optic nerve sheath fenestration for isolated visual loss; CSF diversion if severe headache accompanies vision loss) 1, 2

The Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) has provided strong evidence supporting the efficacy of acetazolamide (up to 4g daily) combined with weight loss for treating mild vision loss in IIH, with associated improvements in papilledema, intracranial pressure, and quality of life 4.