Management and Follow-up for Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)
Regular interval follow-up with visual field testing and fundoscopic examination is essential for patients with pseudotumor cerebri, with frequency determined by disease severity and treatment response. 1, 2
Initial Assessment
- MRI of the head and orbits is the most useful imaging modality for initial evaluation of patients presenting with papilloedema and signs of raised intracranial pressure 1
- CT venography (CTV) or MR venography (MRV) should be performed to evaluate cerebral venous sinuses 1
- Diagnostic criteria include papilloedema, normal neurological examination, normal brain parenchyma on imaging, normal cerebrospinal fluid composition, and elevated lumbar puncture opening pressure 1
Treatment Algorithm
First-line Treatment
- Weight loss is the first-line treatment for pseudotumor cerebri in overweight patients 1, 3
- Acetazolamide is the primary medical therapy for patients with mild visual loss, with a starting dose that can be gradually increased as needed and tolerated 1, 4
- Medications that might cause or exacerbate IIH (tetracyclines, vitamin A, retinoids, steroids, growth hormone, thyroxine, and lithium) should be identified and discontinued if possible 1, 5
Second-line Treatment
- Furosemide may be used as a second-line agent when acetazolamide is not tolerated 5
- Topiramate may help with weight loss by suppressing appetite and have an effect on reducing ICP through carbonic anhydrase inhibition 6
- Zonisamide may be an alternative where topiramate has excessive side effects 6
Surgical Interventions (for progressive or severe visual loss)
- Ventriculoperitoneal (VP) shunt should be the preferred CSF diversion procedure due to lower reported revision rates 1
- Optic Nerve Sheath Fenestration (ONSF) is effective and safe for patients with progressive visual loss despite maximal medical therapy 1, 5
- Neurovascular stenting may lead to improvement in symptoms but has potential complications 1
- Serial lumbar punctures are not recommended for long-term management of IIH 1
Follow-up Schedule
Based on papilloedema grade and visual field status 6:
Severe papilloedema:
- Normal visual fields: Every 1-3 months
- Improving visual fields: Within 4 weeks
- Stable visual fields: Within 1 week
- Worsening visual fields: Immediate intervention
Moderate papilloedema:
- Normal visual fields: Every 3-4 months
- Improving visual fields: Every 1-3 months
- Stable visual fields: Every 1-3 months
- Worsening visual fields: Within 2 weeks
Mild papilloedema:
- Normal visual fields: Every 6 months
- Improving visual fields: Every 3-6 months
- Stable visual fields: Every 3-4 months
- Worsening visual fields: Within 4 weeks
Atrophic papilloedema:
- Normal visual fields: Every 4-6 months
- Improving visual fields: Within 4 weeks
Monitoring Parameters
- Visual acuity 3, 5
- Visual fields (quantitative perimetry) 5, 7
- Fundoscopic examination for papilloedema 3, 8
- Headache symptoms 9, 8
- Other symptoms: pulsatile tinnitus, diplopia, dizziness 9
- Weight monitoring for patients on weight loss programs 3
Important Considerations and Pitfalls
- Treatment failure rates include worsening vision after stabilization in 34% of patients at 1 year and 45% at 3 years 1
- Failure to improve headache occurs in one-third to one-half of treated patients 1
- Systemic hypertension is a significant risk factor for visual loss in patients with pseudotumor cerebri 7
- ONSF should be performed only by an experienced clinician trained in this technique 1
- CSF shunting to exclusively treat headache has limited evidence - 68% will continue to have headaches at 6 months and 79% by 2 years 6
- Patients with IIH often develop migrainous headaches superimposed on headaches from raised intracranial pressure, requiring specific treatment approaches 6
- Once papilloedema has resolved, visual monitoring within hospital services may no longer be required, but caution is needed for asymptomatic patients at presentation who may remain asymptomatic during recurrence 6