Treatment of Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)
Weight loss is the first-line treatment for overweight patients with pseudotumor cerebri, while acetazolamide should be initiated simultaneously as primary medical therapy for patients with mild visual loss. 1
Initial Diagnostic Confirmation
Before initiating treatment, confirm the diagnosis requires:
- MRI of the head and orbits (most useful imaging modality) to exclude mass lesions, hydrocephalus, or abnormal meningeal enhancement 1, 2
- CT or MR venography within 24 hours to exclude cerebral venous sinus thrombosis 2
- Lumbar puncture in lateral decubitus position showing opening pressure >200 mm H₂O with normal CSF composition 1, 2
- Fundus examination confirming papilledema 2
- Blood pressure measurement to exclude malignant hypertension 2, 3
Treatment Algorithm Based on Severity
Mild Visual Loss (First-Line Treatment)
Medical Management:
- Acetazolamide is the primary medication, with dosing gradually increased as needed and tolerated 1, 4
- Weight loss of 5-10% combined with low-sodium diet for all overweight patients 4
- Topiramate may be considered as it suppresses appetite for weight loss and reduces intracranial pressure through carbonic anhydrase inhibition 1, 3
- Zonisamide serves as an alternative when topiramate causes excessive side effects 1
Critical Medication Review:
- Immediately discontinue medications that may cause or exacerbate IIH: tetracyclines, vitamin A, retinoids, steroids, growth hormone, thyroxine, and lithium 1, 5
Severe or Rapidly Progressive Visual Loss (Surgical Intervention)
When visual loss is severe at diagnosis or progressive despite maximal medical therapy, surgical options include:
Preferred Surgical Options:
- Ventriculoperitoneal (VP) shunt is the preferred CSF diversion procedure due to lower reported revision rates 1
- Optic Nerve Sheath Fenestration (ONSF) is effective and safe, may be repeated if initially unsuccessful, and has fewer complications than CSF diversion procedures 1, 5
- Must be performed only by an experienced clinician trained in this technique 1
Emerging Option:
- Neurovascular stenting may improve symptoms but carries risks including headache, stent-adjacent stenosis, and rare serious complications 1
Headache Management
Important caveat: Patients with IIH often develop migrainous headaches superimposed on headaches from raised intracranial pressure, requiring specific migraine treatment approaches beyond ICP-lowering therapies 1, 6
- Failure to improve headache occurs in one-third to one-half of treated patients 1
- CSF shunting exclusively for headache has limited evidence: 68% continue having headaches at 6 months and 79% by 2 years 1
Monitoring and Follow-Up
Visual monitoring frequency should be based on papilledema grade and visual field status 3
Expected treatment failure rates:
Caution: Once papilledema resolves, hospital-based visual monitoring may no longer be required, but asymptomatic patients at presentation may remain asymptomatic during recurrence 1
Critical Pitfalls to Avoid
- Never use serial lumbar punctures for IIH management 1
- Never combine acetazolamide with furosemide in preterm infants as this increases mortality and neurological morbidity 1
- Never perform routine ventricular puncture as it increases risk of CSF infection and loculated hydrocephalus 1
- Patients must inform driving agencies following VP shunt placement 1
- Patients cannot donate blood during or for at least 1 year after discontinuing acetazolamide if they were on retinoid-class medications 1