What are alternative treatments for Idiopathic Intracranial Hypertension (IIH) if acetazolamide or topiramate are not tolerated?

Alternative Treatments for Idiopathic Intracranial Hypertension (IIH) When Acetazolamide or Topiramate Are Not Tolerated

For patients who cannot tolerate acetazolamide or topiramate, alternative treatments for IIH include zonisamide, furosemide, weight loss interventions, and surgical options such as CSF diversion procedures or optic nerve sheath fenestration in cases with progressive visual loss. 1, 2

Medical Management Options

Alternative Medications

• Zonisamide may be used as an alternative to topiramate when side effects are excessive, as it has similar carbonic anhydrase inhibition properties but potentially fewer side effects 3
• Other diuretics such as furosemide, amiloride, and coamilofruse are used by some clinicians, though evidence for their efficacy is uncertain 3
• Indomethacin may have advantages for headache management due to its effect on reducing ICP 3
• For patients with migrainous features, candesartan can be a useful alternative due to its lack of weight gain and depressive side effects 3
• Venlafaxine is weight neutral and helpful for patients with comorbid depression symptoms 3

Weight Management

• Weight loss remains the cornerstone of IIH management and should be emphasized even when medications cannot be tolerated 1, 2
• A structured weight loss program should be implemented as this can lead to significant improvement in ICP and symptoms 2

Headache Management

• Migraine-specific therapies should be considered as 68% of IIH patients have migrainous headache phenotypes 3
• Acute migraine attacks may benefit from triptan therapy used in combination with either NSAIDs or paracetamol and an antiemetic (limited to 2 days/week or maximum 10 days/month) 3
• Botulinum toxin A may be useful in those with coexisting chronic migraine, though specific studies in IIH are lacking 3
• Patients must be warned about medication overuse headache risk (use of simple analgesics >15 days/month or opioids/triptans >10 days/month) 3

Procedural and Surgical Options

For Visual Preservation

• Optic nerve sheath fenestration (ONSF) should be considered for patients with asymmetric papilledema or as first-line treatment in fulminant cases with rapid visual decline 3, 2
• ONSF has fewer complications than CSF diversion but should be performed by experienced clinicians 3

For Refractory Cases

• CSF diversion procedures (shunting) should be considered for patients with progressive visual loss despite medical therapy 2
• Ventriculoperitoneal (VP) shunt is the preferred CSF diversion procedure due to lower revision rates 2
• Lumboperitoneal (LP) shunts are an alternative option 2
• Adjustable valves with antigravity or antisiphon devices should be used to reduce low-pressure headaches 2

Important Considerations and Pitfalls

• Serial lumbar punctures are not recommended for long-term management despite providing temporary relief, as CSF is produced at 25 mL/hour and rapidly replaces the removed volume 3, 2
• CSF diversion is generally not recommended for headache management alone, as 68% continue to have headaches at 6 months and 79% by 2 years after the procedure 3, 2
• Neurovascular stenting is not currently recommended as a treatment for IIH headache without visual deterioration 1
• Medication overuse must be addressed, as it can prevent optimization of preventative treatments 3
• Beware of attributing all headaches to IIH; many patients develop migrainous headaches that persist even after ICP normalization 1

Follow-up Recommendations

• More frequent monitoring is required if there are any signs of recurrence or worsening symptoms 1
• Visual field testing should be performed at each follow-up visit to detect early changes 1
• Patient education about symptoms that warrant urgent evaluation is essential 1