What is the treatment for Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)?

Treatment of Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

The treatment of Pseudotumor Cerebri (Idiopathic Intracranial Hypertension) should be stratified based on visual function, with weight loss as first-line treatment for all patients, acetazolamide as medical therapy, and surgical interventions reserved for cases with imminent risk of vision loss. 1

Initial Assessment and Risk Stratification

• All patients with IIH should have thorough documentation of visual acuity, pupil examination, formal visual field assessment, dilated fundal examination to grade papilloedema, and BMI calculation 1
• Risk stratification should be based on the presence and severity of papilloedema and visual field defects 1
• MRI brain with venography is mandatory to exclude secondary causes of raised intracranial pressure, particularly cerebral venous sinus thrombosis 2, 3

First-Line Treatment for All IIH Patients

• Weight loss program with low-salt diet should be initiated for all overweight IIH patients, with a goal of 5-10% weight loss 1, 3, 4
• Weight loss is the only disease-modifying treatment and should be emphasized even when other treatments are initiated 1, 3
• Secondary causes of IIH should be identified and addressed, including discontinuation of medications that may exacerbate the condition (vitamin A derivatives, steroids) 3, 5

Medical Management

• Acetazolamide should be the first-line medical therapy for symptomatic patients or those with evidence of visual loss 1, 6, 4
• Acetazolamide works by reducing CSF production and has been proven effective in the Idiopathic Intracranial Hypertension Treatment Trial 6
• Furosemide may be used as a second-line agent when acetazolamide is not tolerated or insufficient 7, 4
• Topiramate can be considered as it has dual benefits of carbonic anhydrase inhibition (reducing intracranial pressure) and appetite suppression (promoting weight loss) 3, 8

Surgical Management for Vision-Threatening IIH

• When there is evidence of declining visual function or severe visual loss at presentation, urgent surgical treatment is required to preserve vision 1, 4
• A temporizing lumbar drain may be used while planning definitive surgical intervention 1
• Ventriculoperitoneal (VP) shunt is the preferred CSF diversion procedure due to lower reported revision rates per patient 1
• Lumboperitoneal (LP) shunt is an alternative option, particularly when headache symptoms are prominent 1, 7
• Optic nerve sheath fenestration is effective for protecting vision and may be repeated if initially unsuccessful 7, 4
• Adjustable valves with antigravity or antisiphon devices should be considered for CSF shunts to reduce the risk of low-pressure headaches 1

Special Considerations

Neurovascular Stenting

• The role of neurovascular stenting in IIH is not yet established 1
• Many IIH patients have stenosis of the dominant or both transverse sinuses, but the degree of stenosis does not uniformly correlate with intracranial pressure or visual loss 1
• Long-term antithrombotic therapy is required for at least 6 months following neurovascular stenting 1

Pregnancy

• Multidisciplinary communication among relevant experienced clinicians should occur throughout pregnancy, peridelivery, and postpartum period 1
• Acetazolamide should be used with caution during pregnancy after a clear risk-benefit assessment 1
• If IIH is active with imminent risk of vision loss during pregnancy, serial lumbar punctures may be used as a temporizing measure until definitive treatment can be implemented 1
• Topiramate should not be used in pregnancy due to higher rates of fetal abnormalities 1

Treatment Failure and Follow-up

• Treatment failure rates include worsening vision after initial stabilization in 34% of patients at 1 year and 45% at 3 years 1, 3
• Headache may fail to improve in one-third to one-half of patients despite adequate ICP control 1
• Follow-up intervals should be based on papilloedema grade and visual field status, with more frequent monitoring for severe or worsening cases 1, 3

Common Pitfalls

• Focusing only on ICP-lowering treatments for headache when many IIH patients develop migrainous headaches requiring specific treatment approaches 3, 4
• Delaying surgical intervention when there is progressive vision loss despite maximal medical therapy 1, 4
• Failing to recognize that IIH without papilloedema exists and requires the same weight management approach as typical IIH 1
• Overlooking the need for long-term monitoring as visual deterioration can occur even after initial stabilization 1