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, short-lasting
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.