What is the treatment for idiopathic intracranial hypertension?

Treatment of Idiopathic Intracranial Hypertension (IIH)

Weight loss is the only disease-modifying therapy for idiopathic intracranial hypertension and should be the primary treatment approach for all patients with BMI >30 kg/m². 1

Primary Treatment Approach

Weight Management

• All patients with BMI >30 kg/m² should be counseled about weight management at the earliest opportunity 1
• Target weight loss of 5-15% of total body weight is recommended to put IIH into remission 1, 2
• Patients should be referred to a community weight management program or hospital-based weight program 1
• For sustained weight loss, bariatric surgery may be considered in appropriate candidates 1, 3

Medical Therapy

• Acetazolamide is the first-line medication for patients with mild visual loss 4
• Dosing should be gradually increased as needed and tolerated 4, 2
• Topiramate may be considered as an alternative when acetazolamide is insufficient or poorly tolerated 5
  • Topiramate has the added benefits of promoting weight loss and improving migraine headaches, which are common in IIH 5

Management Based on Disease Severity

Mild to Moderate IIH

• Weight loss program plus acetazolamide is the standard approach 1, 4, 2
• Regular ophthalmology assessments to monitor visual function 1
• If visual function deteriorates, consider diagnostic lumbar puncture to reassess intracranial pressure 1

Severe or Rapidly Progressive Visual Loss

• Urgent surgical intervention is required 1
• A temporizing lumbar drain may be used to protect vision while planning definitive surgical treatment 1
• Surgical options include:
  • CSF diversion procedures (preferred in the UK) 1
    • Ventriculoperitoneal (VP) shunt is preferred due to lower revision rates 4
  • Optic nerve sheath fenestration (ONSF) - effective for cases with precipitous visual decline 4, 6
    • Should only be performed by experienced clinicians trained in this technique 4

Monitoring and Follow-up

• Regular assessment of visual function is essential 1
• Treatment failure rates include worsening vision in 34% of patients at 1 year and 45% at 3 years 4
• Failure to improve headache occurs in one-third to one-half of treated patients 4

Special Considerations

• Medications that might exacerbate IIH (tetracyclines, vitamin A, retinoids, steroids, growth hormone, thyroxine, and lithium) should be identified and discontinued if possible 4
• Serial lumbar punctures are not recommended for long-term management 4
• For atypical IIH (patients who are not female, not of reproductive age, BMI <30 kg/m²), secondary causes should be revisited 1

Emerging Treatments

• Recent research suggests GLP-1 agonists may reduce cerebrospinal fluid secretion and intracranial pressure, representing a potential therapeutic strategy 3
• Neurovascular stenting may lead to symptom improvement but has potential complications including headache and stent-adjacent stenosis 4

Common Pitfalls

• Failure to address weight management as the primary disease-modifying intervention 1
• Relying solely on medical therapy without addressing the underlying cause 1
• Delaying surgical intervention when there is evidence of declining visual function 1
• Not recognizing that IIH symptoms may return if weight loss is not maintained 7