Bilateral paroxysmal cephalalgia: a novel indomethacin-responsive primary headache syndrome?

Paroxysmal hemicrania (PH) is a primary headache disorder characterised by frequent, short-lasting, very severe, strictly unilateral attacks occurring in association with cranial autonomic features. A striking feature of this disorder is its clear response to indomethacin.

In contrast to this stereotypic presentation, we describe a man with indomethacin-responsive headaches that have a temporal profile similar to that of PH but whose attacks have a bilateral distribution without associated cranial autonomic features.

There have been several case reports of patients presenting with short-lasting, frequent, bilateral headaches responding to indomethacin, without cranial autonomic features. These cases have been described as representing bilateral PH although strict unilaterality of pain and cranial autonomic phenomena are cardinal features of PH. These cases may represent a novel indomethacin-responsive syndrome and therefore, for now, should be studied separately from PH until their pathophysiological basis is better understood.