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

Treatment of Idiopathic Intracranial Hypertension (IIH)

The treatment of idiopathic intracranial hypertension should begin with weight loss (5-15% reduction) for patients with BMI >30 kg/m² and acetazolamide as first-line pharmacological therapy, starting at 250-500mg twice daily (maximum 4g daily as tolerated), with surgical interventions reserved for cases with declining visual function despite maximal medical therapy. 1

First-Line Management

Weight Loss

• Recommended for patients with BMI >30 kg/m²
• Target: 5-15% reduction in body weight
• Supported by clinical evidence showing effectiveness in IIH 1

Medical Therapy

1. Acetazolamide (First-line)

   • Starting dose: 250-500mg twice daily
   • Maximum dose: 4g daily (as tolerated)
   • Mechanism: Reduces CSF production 1
   • Caution: Do not discontinue without careful consideration of visual risk

2. Topiramate (Alternative)

   • Use when acetazolamide is not tolerated
   • Starting dose: 25mg daily
   • Titration: Weekly escalation to 50mg twice daily
   • Added benefit: May assist with weight loss and has migraine-preventive properties 1

Symptom Management

• Regular meals and limited caffeine intake
• Improved sleep hygiene
• Exercise program as tolerated
• For headache relief:
  • NSAIDs or paracetamol for short-term relief
  • 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)
  • Avoid opioids 1

Surgical Management

Indications for Surgery

• Declining visual function despite maximal medical therapy
• Evidence of visual field defects and papilledema
• Rapid progression of symptoms (fulminant IIH) 1, 2

Surgical Options

1. CSF Diversion Procedures

   • Ventriculoperitoneal (VP) shunt (preferred due to lower revision rates)
   • Lumboperitoneal shunt
   • Indicated for cases refractory to medical management or with threatened vision 1

2. Optic Nerve Sheath Fenestration (ONSF)

   • Specifically indicated when visual loss is the main morbidity
   • Should not be delayed in fulminant cases to prevent vision loss 1, 2
   • Note: Can have serious complications that may result in severe vision loss 3

3. Transverse Sinus Stenting

   • Indicated for patients who have failed medical therapy and weight loss
   • Requires demonstration of significant pressure gradient across transverse sinus stenosis
   • Can immediately eliminate pressure gradients and rapidly improve symptoms 1

Monitoring and Follow-up

• Regular ophthalmologic evaluations to monitor:
  • Papilledema
  • Visual acuity
  • Visual fields
  • Optical coherence tomography (OCT)
• Frequency based on severity of papilledema and visual field status 1

Special Considerations

• Corticosteroids (e.g., IV dexamethasone) may be considered for severe visual loss requiring rapid intervention
• Not recommended for long-term use due to side effects 1
• Migraine preventatives (candesartan or venlafaxine) may be considered for patients with coexisting chronic migraine 1

Important Pitfalls to Avoid

1. Delaying surgical intervention in fulminant cases with rapid visual deterioration 2
2. Discontinuing acetazolamide without considering visual risk 1
3. Using opioids for headache management 1
4. Failing to monitor visual function regularly, as visual loss is the primary morbidity 1
5. Overlooking the possibility of recurrence, as IIH may recur throughout life 4