What is the management of benign intracranial hypertension?

Management of Benign Intracranial Hypertension

Weight loss is the only disease-modifying therapy in benign intracranial hypertension (BIH) for patients with BMI >30 kg/m², and should be implemented as first-line treatment along with appropriate medical therapy to protect vision and reduce headache morbidity. 1

Diagnostic Approach

• Confirm diagnosis with:
  • Evidence of increased intracranial pressure (elevated opening pressure)
  • Normal neuroimaging (no mass lesions)
  • Normal CSF composition
  • Normal neurological examination (except for papilledema and possible sixth nerve palsy) 2
• CT or MRI venography is mandatory to exclude cerebral sinus thrombosis 1

Management Algorithm

Primary Treatment: Weight Management

• For patients with BMI >30 kg/m²:
  • Target 5-15% weight reduction 1, 2
  • Refer to structured weight management program (community or hospital-based) 1
• For non-obese patients, investigate secondary causes 1

Medical Management

1. First-line medication: Acetazolamide

   • Starting dose: 250-500 mg twice daily
   • Maximum dose: 2-4 g daily 2
   • Administer every 8 hours to respect its kinetics 3
   • Continue for several months with gradual dose reduction 3
   • Monitor for side effects (hypokalemia - prevent with oral potassium) 3

2. Alternative medication: Topiramate

   • Consider when acetazolamide is not tolerated
   • Contraindicated in pregnancy 2

3. Other options:

   • Furosemide (as adjunct or alternative to acetazolamide) 3
   • Role of steroids is debatable and generally not recommended 3

Management of Imminent Visual Loss

When there is evidence of declining visual function:

1. Emergency measures:

   • Temporary lumbar drain as a temporizing measure 1

2. Surgical interventions:

   • CSF diversion procedures:
     • Ventriculoperitoneal (VP) shunt (preferred due to lower revision rates) 1
     • Lumboperitoneal (LP) shunt as an alternative 1
     • Use adjustable valves with antigravity or antisiphon devices to reduce low-pressure headaches 1
   • Optic nerve sheath fenestration 4

3. Emerging intervention:

   • Neurovascular stenting for transverse sinus stenosis (role not yet established)
   • Requires long-term antithrombotic therapy for >6 months 1

Monitoring and Follow-up

• Regular ophthalmologic assessment:
  • Visual acuity
  • Pupil examination
  • Formal visual field assessment
  • Dilated fundal examination 2
• Follow-up schedule:
  • Early review after intervention: 24-48 hours
  • Intermediate follow-up after procedures: 10-14 days (blood patch) or 3-6 weeks (surgery)
  • Late follow-up: 3-6 months 1

Special Considerations

• Pregnancy: Use acetazolamide with caution; topiramate contraindicated 2
• Medication overuse headache: Monitor and manage appropriately 1
• Post-treatment rebound headache: Evaluate for secondary intracranial hypertension 1

Pitfalls and Caveats

1. Never perform lumbar puncture if signs of raised intracranial pressure until imaging is performed 2
2. CT scan alone is not reliable for diagnosing raised intracranial pressure 2
3. Surgical treatment failure rates include worsening vision in 34% at 1 year and 45% at 3 years 1
4. Headache may persist in one-third to one-half of patients despite adequate treatment of increased pressure 1
5. Avoid medications that potentially lower CSF pressure (topiramate, indomethacin) or reduce blood pressure (candesartan, beta blockers) in patients with persistent symptoms 1

By following this structured approach to management, the risk of permanent visual loss and persistent headaches can be minimized in patients with benign intracranial hypertension.