Long-Term Outcome of Indomethacin Treatment in Pediatric Patients with Paroxysmal Hemicrania-A Case Series

Paroxysmal Hemicrania is a rare form of primary headache in children and adolescents, belonging to the group of trigeminal autonomic cephalalgias. Patients suffer from severe, shortlasting unilateral headaches accompanied by symptoms of the autonomic system on the same side of the head. The short duration of attacks distinguishes Paroxysmal Hemicrania from other trigeminal autonomic cephalalgias. Indomethacin is the treatment of choice, and its effectiveness provides a unique diagnostic criterion. However, the long-term outcomes in children are highly underreported. In this case-series, n = 8 patients diagnosed with Paroxysmal Hemicrania were contacted via telephone 3.1 to 10.7 years after initial presentation. A standardized interview was conducted. n = 6 patients were headache-free and no longer took indomethacin for 5.4 +/- 3.4 years. The mean treatment period in these patients was 2.2 +/- 1.9 years. Weaning attempts were undertaken after 1.7 +/- 1.3 months; in n = 3 patients, more than one weaning attempt was necessary. n = 2 patients were still taking indomethacin (4.5 and 4.9 years, respectively). Both unsuccessfully tried to reduce the indomethacin treatment (two and six times, respectively). Adverse effects appeared in n = 6 (75%) patients and led to a discontinuation of therapy in n = 2 patients. Our long-term follow-up suggests that in a substantial proportion of pediatric patients, discontinuing indomethacin therapy is possible without the recurrence of Paroxysmal Hemicrania.