Which specialist should a patient with idiopathic intracranial hypertension (IIH) be referred to?

Referral for Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

Patients with pseudotumor cerebri (idiopathic intracranial hypertension) should be referred to a neurologist as the primary specialist, with urgent referral within 2-4 weeks for most cases, or sooner if visual symptoms are severe. 1

Initial Referral Pathway

• Refer to a local neurologist as the first-line specialist for suspected IIH, with referral urgency based on symptom severity 1
• Referral timeframe should be:
  • 2-4 weeks for patients able to care for themselves with mild to moderate symptoms 1
  • Within 48 hours for patients unable to care for themselves but who have help 1
  • Emergency admission for patients unable to care for themselves without help 1

Specialist Collaboration Required

• Ophthalmology consultation is essential for all patients with IIH to monitor visual function and papilledema 2, 3
• The optimal management approach involves both neurologists and ophthalmologists working together to monitor disease progression and guide treatment decisions 3

Indications for Referral to Tertiary Neuroscience Center

• Diagnostic uncertainty despite initial evaluation 1
• Failure of first-line treatments 1
• Rapid clinical deterioration 1
• Serious complications such as subdural hematoma with mass effect 1
• Severe or progressive visual loss requiring surgical intervention 4

Services Required at Specialist Centers

• Neuroradiological investigations including CT myelography and/or digital subtraction myelography 1
• Specialist clinical expertise in IIH diagnosis and treatment 1
• Multidisciplinary team meetings for complex cases 1
• Surgical expertise for CSF diversion procedures or optic nerve sheath fenestration when needed 1, 4

Common Pitfalls in Specialist Referral

• Delaying ophthalmology consultation, which is critical for monitoring visual function 2, 3
• Failing to recognize fulminant IIH (rapid visual decline within 4 weeks), which requires emergency referral 1
• Not considering atypical presentations of IIH (patients who are not female, not of childbearing age, or with BMI <30 kg/m²) that may require more extensive investigation 1, 5
• Overlooking the need for venography (CT or MR) to exclude cerebral sinus thrombosis 1, 6

Special Considerations

• For IIH without papilledema (IIHWOP), referral should still be to a neurologist, but surgical management is not routinely considered unless advised by experienced clinicians in a multidisciplinary setting 1
• Patients with IIH in ocular remission (resolved papilledema) may still have ongoing headache morbidity requiring neurological management 1
• Weight management counseling should be initiated for all overweight IIH patients, regardless of specialist referral 4

Following this referral pathway ensures appropriate specialist care while minimizing delays in diagnosis and treatment that could lead to permanent visual loss in patients with pseudotumor cerebri.