To review the most relevant developments in the understanding of headache in idiopathic intracranial hypertension (IIH).
The phenotype of the typical IIH headache is diverging from the historical thinking of a raised intracranial pressure headache, with the majority being classified as having migraine. A larger proportion of those with IIH have a past medical history of migraine, compared with the general population, highlighting the importance of re-examining those who have a change or escalation in their headache. The mechanisms underlying headache in IIH are not understood. Additionally, factors which confer a poor headache prognosis are not established. It is clear, however, that headache has a detrimental effect on all aspects of the patient's quality of life and is currently ranked highly as a research priority by IIH patients to better understand the pathophysiology of headache in IIH and identification of potential headache specific therapeutic agents.