What is the management of pseudotumor cerebri (idiopathic intracranial hypertension)?

Management of Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

The management of pseudotumor cerebri (idiopathic intracranial hypertension) should prioritize weight loss as the primary disease-modifying therapy, with medical and surgical interventions based on vision status and symptom severity. 1

Diagnostic Approach

• MRI brain with and without contrast is the preferred initial imaging within 24 hours to exclude secondary causes of raised intracranial pressure 1, 2
• CT or MR venography is mandatory to exclude cerebral sinus thrombosis within 24 hours 1, 2
• Diagnostic criteria include:
  • Papilledema (typically present) 3
  • Normal neurological examination (except possible sixth nerve palsy) 1
  • Normal brain parenchyma on imaging (no mass, hydrocephalus, or abnormal meningeal enhancement) 1, 3
  • Normal cerebrospinal fluid composition 3
  • Elevated lumbar puncture opening pressure >280 mm CSF in children (or >250 mm CSF if not sedated and not obese) 1, 3

Treatment Algorithm

Step 1: Disease Modification Through Weight Loss

• All patients with BMI >30 kg/m² should be counseled about weight management at the earliest opportunity 1
• Target 5-15% weight loss, which has been shown to put IIH into remission 1, 4
• Refer to community or hospital-based weight management program 1
• Consider bariatric surgery for sustained weight loss in appropriate candidates 1

Step 2: Medical Management

• Acetazolamide is first-line medical therapy for patients with mild visual loss 4, 5
• Other medications may be added or substituted when acetazolamide is insufficient or poorly tolerated 4
• Low-salt diet should be recommended alongside weight management 4

Step 3: Surgical Management (Based on Vision Status)

For Imminent Risk of Visual Loss or Rapidly Progressive Visual Decline:

• Urgent surgical intervention is required 1
• A temporizing lumbar drain may protect vision while planning definitive surgery 1
• Ventriculoperitoneal (VP) shunt is the preferred CSF diversion procedure due to lower revision rates 1
• Lumboperitoneal shunt is an alternative option 1
• Optic nerve sheath fenestration may be considered, especially for predominant visual symptoms 5, 6

For Surgical Technique Considerations:

• Neuronavigation should be used to place VP shunts 1
• Adjustable valves with antigravity or antisiphon devices should be considered to reduce low-pressure headaches 1
• Patients should be informed about driving restrictions following VP shunt placement (UK specific) 1

Emerging Treatment Option:

• Endovascular stenting of transverse sinus stenoses has shown benefit in selected patients 5, 6
• This approach may be considered when dural sinus stenosis is identified as a contributing factor 6

Monitoring and Follow-up

• Regular ophthalmologic assessments to monitor papilledema and visual function 1
• If significant deterioration of visual function occurs, consider diagnostic lumbar puncture 1

Special Considerations

• For non-obese patients, secondary causes should be thoroughly investigated 1
• Secondary causes include medications (tetracyclines, Vitamin A, retinoids, steroids, growth hormone, thyroxine, lithium), endocrine disorders, and cerebral venous abnormalities 1, 3

Treatment Pitfalls to Avoid

• Delaying treatment in patients with progressive visual loss 4, 7
• Failing to recognize that "benign intracranial hypertension" is a misnomer as permanent visual impairment can occur without proper treatment 8
• Overlooking the importance of weight loss as the only disease-modifying therapy 1, 4