What are the treatment options for Idiopathic Intracranial Hypertension (IIH)?

Treatment Options for Idiopathic Intracranial Hypertension (IIH)

Weight loss combined with acetazolamide is the first-line treatment for idiopathic intracranial hypertension (IIH), with a structured weight loss program targeting 5-15% reduction in total body weight being the only disease-modifying therapy. 1

First-Line Medical Therapy

Weight Management

• Weight loss of 5-15% can lead to disease remission, particularly important for patients with BMI >30 kg/m² 1
• Structured weight loss program should be implemented as the foundation of treatment

Pharmacological Treatment

• Acetazolamide:

  • Starting dose: 250-500 mg twice daily
  • Titrate up as tolerated to maximum 4 g daily 1
  • In the IIHTT trial, 44% of participants achieved 4 g/day dosage 2
  • Patients should be warned about side effects including:
    • Gastrointestinal: diarrhea, nausea, vomiting, dysgeusia
    • Neurological: paresthesia, fatigue, tinnitus
    • Other: depression, renal stones 2, 1

• Topiramate (alternative if acetazolamide not tolerated):

  • Starting dose: 25 mg with weekly escalation to 50 mg twice daily 2, 1
  • Dual benefit: carbonic anhydrase inhibition and appetite suppression 1
  • Important counseling needed regarding:
    • Reduced efficacy of hormonal contraceptives
    • Potential side effects: depression, cognitive slowing
    • Teratogenic risks 2

Headache Management in IIH

Acute Treatment

• NSAIDs or paracetamol for acute headaches
• Indomethacin may be advantageous due to ICP-reducing effects 2, 1
• Triptans may be used for migrainous attacks
• Opioids should be avoided 2

Preventive Treatment

• Early introduction of migraine preventatives should be considered 2
• Weight-neutral options like candesartan or venlafaxine may be preferred
• Botulinum toxin A may be useful for those with coexisting chronic migraine 1
• Patients must be informed about medication overuse headache risk (use of simple analgesics >15 days/month) 2

Surgical Interventions

When to Consider Surgery

• Visual function deterioration despite maximal medical therapy 1
• Imminent risk of vision loss 1

Surgical Options

1. CSF Diversion Procedures:

   • Ventriculoperitoneal shunt preferred over lumboperitoneal shunt (fewer revisions) 1
   • Note: 68% of patients continue to have headaches at 6 months post-shunting 1

2. Optic Nerve Sheath Fenestration (ONSF):

   • Consider for asymmetric papilledema causing visual loss in one eye
   • Should be performed by experienced clinicians 1

3. Venous Sinus Stenting:

   • Investigational treatment
   • Requires documented pressure gradient >8 mmHg across stenosis
   • Requires long-term antithrombotic therapy for >6 months
   • Not currently recommended for headache management alone 1

Follow-up and Monitoring

Follow-up intervals based on papilledema grade and visual field status:

• Severe papilledema: Every 1-3 months
• Moderate papilledema: Every 3-4 months
• Mild papilledema: Every 6 months 2

Adjust intervals based on:

• Worsening: More frequent monitoring
• Improvement: Less frequent monitoring 2

Important Considerations and Pitfalls

1. Serial lumbar punctures are not recommended for ongoing management 1

2. Medication overuse headache is common in IIH patients and must be addressed 1

3. Pregnancy considerations:

   • Risk-benefit assessment needed for acetazolamide during pregnancy
   • Manufacturers do not recommend acetazolamide use during pregnancy
   • Topiramate should not be used in pregnancy due to higher rate of fetal abnormalities 2

4. CSF diversion procedures should not be routinely undertaken for headache alone unless there is papilledema and risk of visual deterioration 2

5. Lifestyle modifications are essential:

   • Limit caffeine intake
   • Ensure regular meals and adequate hydration
   • Establish exercise program
   • Implement sleep hygiene 2, 1

By following this structured approach to IIH management, focusing first on weight loss and acetazolamide, and escalating to surgical options when necessary, patients can achieve optimal outcomes with reduced morbidity from this challenging condition.