What is the treatment for pseudotumor cerebri (idiopathic intracranial hypertension)?

Treatment of Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

Weight loss is the first-line treatment for pseudotumor cerebri in overweight patients, while acetazolamide should be initiated as the primary medical therapy for patients with mild visual loss. 1, 2, 3

Initial Assessment and Management

• MRI of the head and orbits is the most useful imaging modality for initial evaluation of patients presenting with papilledema 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 papilledema, normal neurological examination (except possible sixth nerve palsy), normal brain parenchyma on imaging, normal cerebrospinal fluid composition, and elevated lumbar puncture opening pressure 1, 4

Treatment Algorithm

First-Line Approaches:

1. Weight Management:

   • All overweight patients should be encouraged to enter a weight-management program with a goal of 5-10% weight loss 3
   • Low-salt diet should be implemented alongside weight loss efforts 3

2. Medical Therapy:

   • Acetazolamide is the first-line medication for patients with mild visual loss 1, 2, 3
   • Starting dose can be gradually increased as needed and tolerated 2
   • Furosemide may be considered as a second-line agent when acetazolamide is insufficient or poorly tolerated 2

3. Headache Management:

   • Mild headaches can be treated with nonsteroidal anti-inflammatory drugs (NSAIDs) or migraine prophylactic agents 2
   • Headache often requires focused treatment beyond therapies that lower intracranial pressure 5

Surgical Interventions (for progressive or severe visual loss):

When visual function is declining despite maximal medical therapy, surgical intervention is necessary 1:

1. CSF Diversion Procedures:

   • Ventriculoperitoneal (VP) shunt should be the preferred CSF diversion procedure due to lower reported revision rates 1
   • Lumboperitoneal (LP) shunt is an alternative option 1
   • Adjustable valves with antigravity or antisiphon devices should be considered to reduce the risk of low-pressure headaches 1

2. Optic Nerve Sheath Fenestration (ONSF):

   • Effective and safe procedure that may be repeated if initially unsuccessful 2
   • Has fewer complications than CSF diversion procedures 1
   • May be considered as first treatment in fulminant cases or for asymmetric papilledema causing visual loss in one eye 1

3. Neurovascular Stenting:

   • Role in IIH treatment is not yet established 1
   • Requires long-term antithrombotic therapy for longer than 6 months 1
   • May lead to improvement in symptoms but has potential complications including headache, stent-adjacent stenosis, and rare serious complications 1

Special Considerations

• Serial lumbar punctures are not recommended for management of IIH 1
• Bariatric surgery can be effective for severely obese patients but is not useful as an acute intervention 2
• Medications that might cause or exacerbate IIH (tetracyclines, vitamin A, retinoids, steroids, growth hormone, thyroxine, lithium) should be identified and discontinued if possible 1, 2

Monitoring and Follow-up

• Visual acuity, visual fields, and ocular fundus appearance should be closely monitored in all patients 2
• Some patients with minimal symptoms and no evidence of vision loss may not require additional treatment beyond weight loss 2
• Treatment failure rates include worsening vision after stabilization in 34% of patients at 1 year and 45% at 3 years 1

Pitfalls and Caveats

• Permanent severe visual loss occurs in 25% of untreated cases 6
• Failure to improve headache occurs in one-third to one-half of treated patients 1
• CSF shunting has significant complication and failure rates 2
• ONSF should be performed only by an experienced clinician trained in this technique 1
• Patients in the UK should inform the Driver and Vehicle Licensing Agency following VP shunt placement 1