What are the treatment options for a patient with Idiopathic Intracranial Hypertension (IIH) experiencing intractable headaches?

Management of Intractable Headache in Idiopathic Intracranial Hypertension (IIH)

For patients with IIH experiencing intractable headaches, a stepwise approach starting with migraine-targeted therapies is recommended, with topiramate as a preferred option due to its dual benefits of carbonic anhydrase inhibition and weight loss properties. 1, 2

First-line Approaches

Medical Management

• Acetazolamide is the first-line pharmacological treatment for IIH with a starting dose of 250-500mg twice daily, titrating up as needed to a maximum of 4g daily 2
• Topiramate should be strongly considered as an alternative or adjunct therapy due to its multiple benefits:
  • Carbonic anhydrase inhibition (reduces ICP)
  • Weight loss promotion through appetite suppression
  • Migraine prophylaxis effects 1, 3
• When using topiramate, start with 25mg and escalate weekly to 50mg twice daily, with appropriate counseling regarding side effects and contraceptive interference 1
• Where topiramate has excessive side effects, zonisamide may be an alternative 1

Acute Headache Management

• Short-term analgesics may be helpful in the first few weeks following diagnosis, including NSAIDs or paracetamol 1
• Indomethacin may be particularly beneficial due to its effect on reducing intracranial pressure 1, 2
• For migrainous attacks, triptans may be used in combination with either NSAIDs or paracetamol and an antiemetic with prokinetic properties 1
• Limit triptan use to 2 days per week or a maximum of 10 days per month to prevent medication overuse headache 1
• Opioids should NOT be prescribed for headaches in IIH 1, 2

Second-line Approaches

Additional Preventative Medications

• For patients with migrainous features (present in 68% of IIH patients with headache), consider migraine preventatives 1, 4
• Candesartan can be a useful alternative due to its lack of weight gain and depressive side effects 1
• Venlafaxine is weight neutral and helpful with depression symptoms, which is a frequent comorbidity in IIH 1
• Botulinum toxin A may be useful in those with coexisting chronic migraine 1
• AVOID medications that could increase weight (beta blockers, tricyclic antidepressants, sodium valproate, pizotifen and flunarizine) 1

Management of Medication Overuse

• Medication overuse is common in IIH patients and must be addressed 1
• Non-opioids and triptan medications may be stopped abruptly or weaned down within a month 1
• Opioid medications should be gradually removed, with at least 1 month painkiller-free to determine effectiveness 1

Interventional Approaches

CSF Diversion Surgery

• CSF diversion is generally NOT recommended as a treatment for headache alone in IIH 1
• Following CSF diversion, 68% will continue to have headaches at 6 months and 79% by 2 years 1
• 28% can develop iatrogenic low-pressure headaches after shunting 1
• If considered, CSF diversion procedures should only be carried out in a multidisciplinary setting and following a period of intracranial pressure monitoring 1

Neurovascular Stenting

• Neurovascular stenting is not currently recommended as a treatment for headache in IIH 1
• The evidence for stenting is limited by non-randomized studies, small sample sizes, and lack of long-term follow-up 1, 5

Non-Pharmacological Approaches

Lifestyle Modifications

• Implement strategies to limit caffeine intake 1
• Ensure regular meals and adequate hydration 1
• Establish an exercise program and sleep hygiene 1
• Behavioral and stress management techniques can be implemented such as yoga, cognitive-behavioral therapy, and mindfulness 1

Special Considerations

Persistent Post-IIH Headache

• Despite resolution of papilledema and normalization of raised ICP, a majority of patients suffer persistent post-IIH headache 6, 4
• Recent research suggests CGRP monoclonal antibodies may be effective for persistent post-IIH headache, though more studies are needed 6, 7

Follow-up Recommendations

• Follow-up intervals should be based on papilloedema grade and visual field status 1
• Patients need clear explanation of how their headaches may change over time and how to minimize the risks of developing medication overuse headache 1

Common Pitfalls to Avoid

• Failing to recognize medication overuse headache, which can prevent optimization of preventative treatments 1
• Using medications that promote weight gain, which can worsen IIH 1
• Relying on CSF diversion procedures for headache management alone without addressing the migrainous component of headaches 1
• Not starting preventative medications early enough (they can take 3-4 months to reach maximal efficacy) 1