What is the treatment for Pseudo tumor cerebri (Idiopathic Intracranial Hypertension)?

Treatment of Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

Weight loss is the only disease-modifying therapy for typical IIH and should be the foundation of treatment for all patients with BMI >30 kg/m², with a goal of 5-15% weight reduction to achieve remission. 1

First-Line Management

Weight Management

• All patients with BMI >30 kg/m² should be advised on weight management programs
• Low-salt diet is recommended alongside weight loss
• Weight loss of 5-15% of body weight may be necessary to achieve remission 1

Pharmacological Treatment

1. Acetazolamide (first-line medication):

   • Initial dose: 250-500 mg twice daily
   • Can be increased up to 2-4 g daily according to tolerance
   • Proven effective in the Idiopathic Intracranial Hypertension Treatment Trial 1, 2

2. Topiramate (alternative to acetazolamide):

   • Multiple beneficial mechanisms:
     • Promotes weight loss
     • Controls migraine headaches
     • Has carbonic anhydrase inhibition properties
   • Contraindicated in pregnancy due to higher rates of fetal abnormalities 1

Surgical Interventions

Indicated when:

• Medical therapy fails
• Visual function deteriorates rapidly
• Headaches persist despite medical management

Surgical Options:

1. CSF Shunting (preferred due to lower revision rate):

   • Ventriculoperitoneal or lumboperitoneal shunting
   • Effectively reduces intracranial pressure

2. Optic Nerve Sheath Fenestration:

   • Particularly useful for patients with severe or progressive visual loss
   • Protects vision but may not relieve headache symptoms

3. Transverse Sinus Stenting:

   • Emerging treatment option
   • Current role remains unclear and requires further research 1, 3

Special Considerations

Fulminant IIH (Rapid Visual Decline)

• Requires urgent surgical intervention
• Serial lumbar punctures may be used as a temporizing measure until definitive treatment 1

Pregnancy

• Multidisciplinary communication among experienced clinicians is crucial
• Acetazolamide is not recommended during pregnancy according to manufacturers
• No specific mode of delivery should be suggested based on IIH diagnosis
• Weight management should follow appropriate guidelines for gestational age
• For acute exacerbations with imminent risk to vision, consider serial lumbar punctures as a temporizing measure until longer-term interventions can be implemented 4, 1

IIH Without Papilledema (IIHWOP)

• Risk of vision loss has not been identified in these patients
• Headache is the principal morbidity
• Manage as typical IIH with weight management counseling
• Surgical management to control elevated intracranial pressure should not routinely be considered 4

Monitoring and Follow-up

All patients with papilledema should have documented:

1. Visual acuity
2. Pupil examination
3. Formal visual field assessment
4. Dilated fundal examination to grade papilledema
5. BMI calculation

Serial imaging of the optic nerve head (photographs or OCT) is useful for monitoring 4

Follow-up Schedule:

• Early review after intervention: 24-48 hours
• Intermediate follow-up: 10-14 days or 3-6 weeks
• Late follow-up: 3-6 months 1

Common Pitfalls and Caveats

• Failure to diagnose IIH early can lead to irreversible vision loss
• Non-obese patients should be investigated for secondary causes of intracranial hypertension
• Headache management often requires targeted treatment beyond ICP-lowering therapies
• Visual function can deteriorate rapidly in fulminant cases, requiring urgent intervention
• Regular ophthalmological monitoring is essential even when symptoms improve

The prognosis is generally good with accurate diagnosis and timely treatment, particularly regarding preservation of vision 1.