What is the treatment for pseudotumor cerebri?

Treatment of Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

The first-line treatment for pseudotumor cerebri includes weight loss for overweight patients and acetazolamide as the primary medical therapy for patients with mild visual loss, with surgical interventions reserved for those with severe or progressive visual decline. 1, 2

Diagnosis and Initial Assessment

• MRI of the head and orbits is the most useful initial imaging modality for evaluating patients with suspected pseudotumor cerebri 3, 1
• CT venography (CTV) or MR venography (MRV) should be performed to evaluate cerebral venous sinuses 1, 2
• Diagnostic criteria include papilledema, normal neurological examination, normal brain parenchyma on imaging, normal cerebrospinal fluid composition, and elevated lumbar puncture opening pressure (>280 mm CSF in children or >250 mm CSF if not sedated and not obese) 1, 2

Treatment Algorithm

Step 1: Weight Management

• Weight loss is the first-line treatment for overweight patients (BMI >30 kg/m²) 1, 2
• Target 5-15% weight loss, which has been shown to put IIH into remission 2
• Consider referral to community or hospital-based weight management programs 2
• Bariatric surgery may be considered for appropriate candidates to achieve sustained weight loss 2, 4

Step 2: Medical Management

• Acetazolamide is the first-line medication, with a starting dose that can be gradually increased as needed and tolerated 1, 5
• Topiramate may be considered as it helps with weight loss through appetite suppression and reduces intracranial pressure through carbonic anhydrase inhibition 1
• Zonisamide may be an alternative where topiramate has excessive side effects 1
• Medications that might exacerbate IIH should be identified and discontinued if possible, including tetracyclines, vitamin A, retinoids, steroids, growth hormone, thyroxine, and lithium 3, 1

Step 3: Surgical Management (for severe or progressive visual loss)

• Urgent surgical intervention is required for patients with imminent risk of visual loss or rapidly progressive visual decline 2, 6
• A temporizing lumbar drain may protect vision while planning definitive surgery 2
• Ventriculoperitoneal (VP) shunt is the preferred CSF diversion procedure due to lower revision rates 1, 2
• Optic Nerve Sheath Fenestration (ONSF) is an effective and safe procedure that may be repeated if initially unsuccessful, with fewer complications than CSF diversion procedures 1, 4
• Neurovascular stenting may lead to improvement in symptoms but has potential complications including headache and stent-adjacent stenosis 1, 4

Management of Associated Symptoms

• Patients with IIH often develop migrainous headaches superimposed on headaches from raised intracranial pressure, requiring specific treatment approaches 1
• CSF shunting to exclusively treat headache has limited evidence, with 68% of patients continuing to have headaches at 6 months 1, 7
• Serial lumbar punctures are not recommended for long-term management of IIH 1

Monitoring and Follow-up

• Regular ophthalmologic assessments to monitor papilledema and visual function 2, 5
• If significant deterioration of visual function occurs, consider diagnostic lumbar puncture 2
• Treatment failure rates include worsening vision after stabilization in 34% of patients at 1 year and 45% at 3 years 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
• Patients should inform the relevant driving agency following VP shunt placement 1
• Failure to improve headache occurs in one-third to one-half of treated patients despite adequate ICP control 1, 7
• For non-obese patients, secondary causes should be thoroughly investigated 2, 8