What is the initial management for Idiopathic Intracranial Hypertension (IIH)?

Initial Management of Idiopathic Intracranial Hypertension (IIH)

The initial management for Idiopathic Intracranial Hypertension (IIH) should focus on weight loss as the primary disease-modifying therapy, along with appropriate medical therapy based on symptom severity and risk to vision. 1

Diagnostic Confirmation

Before initiating treatment, confirm diagnosis with:

• Urgent MRI brain within 24 hours (if unavailable, CT brain followed by MRI) 1, 2
• CT or MR venography to exclude cerebral sinus thrombosis 1, 2
• Lumbar puncture to confirm elevated opening pressure and normal CSF composition 1

Risk Stratification

Classify patients into one of three categories to guide management:

• Fulminant IIH: Vision at imminent risk 1
• Typical IIH: Female of reproductive age with BMI ≥30 kg/m² 1
• Atypical IIH: Not female, not of reproductive years, BMI <30 kg/m² 1

Treatment Algorithm

Step 1: Weight Management (Primary Disease-Modifying Therapy)

• All patients with BMI >30 kg/m² should be counseled about weight management immediately 1, 3
• Target 5-15% weight loss, which has been shown to put IIH into remission 1, 3
• Refer to community or hospital-based weight management program 1
• Consider bariatric surgery for sustained weight loss in appropriate candidates 1, 4

Step 2: Medical Management

For patients without imminent risk of visual loss:

• First-line: Acetazolamide (starting at low dose and gradually increasing) 1, 5
• Alternative: Topiramate if acetazolamide is not tolerated or contraindicated 1, 6
  • Topiramate has additional benefits of weight loss and migraine control 6
  • Side effects include cognitive slowing, depression, and reduced contraceptive efficacy 1

Step 3: Surgical Management

For patients with imminent risk of visual loss or rapidly progressive visual decline:

• Emergency measure: Temporizing lumbar drain to protect vision while planning definitive surgery 1, 3
• Preferred surgical procedure: Ventriculoperitoneal (VP) shunt due to lower revision rates 1, 7
• Alternative procedure: Optic nerve sheath fenestration, especially in cases of precipitous visual decline 7, 4

Headache Management

If headache persists despite ICP management:

• Identify headache phenotype (often migrainous) 1
• Avoid medication overuse by limiting acute medications to 2 days per week 1
• Consider migraine preventatives based on individual factors 1

Monitoring and Follow-up

• Regular ophthalmologic assessments to monitor papilledema and visual function 1, 3
• If significant deterioration of visual function occurs, consider diagnostic lumbar puncture 1

Common Pitfalls and Caveats

• Failure to recognize imminent vision loss requiring urgent surgical intervention 1, 7
• Inadequate weight loss counseling, which is the only disease-modifying therapy 1, 5
• Medication overuse headache can develop and prevent optimization of preventative treatments 1
• Surgical treatment failure rates include worsening vision in 34% at 1 year and 45% at 3 years 1