What is the best management approach for pseudotumor cerebri (idiopathic intracranial hypertension)?

Management of Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

The best management approach for pseudotumor cerebri (idiopathic intracranial hypertension) is a stepwise treatment algorithm that begins with weight loss and acetazolamide as first-line interventions, followed by surgical procedures for refractory cases or those with severe vision loss. 1

Diagnosis

• Diagnosis requires papilledema, normal neurological examination, normal brain parenchyma on imaging, normal cerebrospinal fluid composition, and elevated lumbar puncture opening pressure 1
• MRI of the head and orbits is the most useful initial imaging modality for evaluation of patients presenting with signs of raised intracranial pressure 1
• CT or MR venography should be performed within 24 hours to exclude cerebral venous sinus thrombosis 2
• Typical patients are female, of childbearing age, with BMI >30 kg/m² 2

Treatment Algorithm

First-line Interventions

• Weight loss is the first-line treatment for overweight patients with a goal of 5-10% weight loss, along with a low-salt diet 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, 3
• Medications that might exacerbate IIH (tetracyclines, vitamin A, retinoids, steroids, growth hormone, thyroxine, and lithium) should be identified and discontinued if possible 1

Alternative Medical Therapies

• Topiramate may be considered as it helps with weight loss through appetite suppression and has carbonic anhydrase inhibition properties that may reduce intracranial pressure 1
• Zonisamide may be an alternative when topiramate has excessive side effects 1

Surgical Interventions (for severe or progressive vision loss)

• 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, with fewer complications than CSF diversion procedures, and may be repeated if initially unsuccessful 1
• Neurovascular stenting may improve symptoms but has potential complications including headache and stent-adjacent stenosis 1

Special Considerations

• Serial lumbar punctures are not recommended for long-term management of IIH 1
• CSF shunting exclusively for headache has limited evidence, with 68% of patients continuing to have headaches at 6 months 1
• Patients with IIH often develop migrainous headaches superimposed on headaches from raised intracranial pressure, requiring specific treatment approaches 1

Monitoring and Follow-up

• 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
• Once papilledema has resolved, visual monitoring within hospital services may no longer be required, but caution is needed for asymptomatic patients who may remain asymptomatic during recurrence 1

Pitfalls and Caveats

• ONSF should be performed only by an experienced clinician trained in this technique 1
• The term "benign intracranial hypertension" should be abandoned since permanent visual impairment can complicate the condition 4
• Patients should inform the relevant driving agency following VP shunt placement 1
• Viral-induced intracranial hypertension can mimic pseudotumor cerebri but with pleocytosis in the CSF, requiring different management 5