Indomethacin-responsive refractory headache: Two case reports in children after hemispherectomy for Rasmussen's encephalitis

Abstract

Background: Indomethacin-responsive headaches occur in youth and include primary headache syndromes such as hemicrania continua and paroxysmal hemicrania. Both are trigeminal autonomic cephalalgias (TACs). In pediatrics TACs are rare. Moreover, they may differ in their phenotypes and response to indomethacin compared to adults. Secondary causes for side-locked headaches can have vascular, neoplastic, and inflammatory etiologies, emphasizing the importance of imaging in the evaluation of these headache types. Post-craniotomy indomethacin-responsive headaches have been described in adults, but not in children. Methods: Written consent was obtained from the patients’ families and written assent from the two children for publication. Results: We report the course of two children, both with a history of Rasmussen’s encephalitis treated with functional hemispherectomies, who subsequently developed debilitating, side-locked, medically refractory headaches several months after surgery. Headaches were on the same side of their encephalitis and surgery. In both instances, the headaches were exquisitely and rapidly responsive to indomethacin. Headache freedom was maintained on low doses of indomethacin (0.14-0.5 mg/kg/day). Conclusion: In cases of refractory side-locked headaches following a craniotomy or neuroinflammatory condition in children, one may consider indomethacin as a treatment option after evaluation for other secondary etiologies.