Hemicrania continua (HC) is an indomethacin responsive primary chronic headache disorder which is currently classified as a subtype of trigeminal autonomic cephalalgias (TACs). It is not very uncommon. There are >1000 cases of HC in the literature, and it constitutes 1.7% of total headache in the clinic settings. Misdiagnosis for HC is very common at all clinical settings. A diagnosis of HC is missed even by neurologists and headache specialists. It is characterized by a continuous strictly unilateral headache with superimposed exacerbations. Just like other TACs, exacerbations are associated with cranial autonomic symptoms and restlessness. A large number of patients may have migrainous features (nausea, vomiting, photophobia, and phonophobia) during exacerbations phase. The "key" feature of HC is persistent featureless background headaches. However, patients and physicians may focus only on the exacerbation part. As durations, frequency and associated symptoms of exacerbations are highly variables; it may mimic a large number of primary and secondary headache disorders. Migraine and cluster headache are two most common misdiagnosed conditions. Another specific feature of HC is remarkable repose to indomethacin. A "complete" response to indomethacin is as "sine qua non" for HC. However, a few other medications may also be effective in a subset of HC patients. Various surgical procedures have been tried with mixed results in patients who were intolerant to indomethacin or other drugs.
Keywords: Hemicrania continua; indomethacin; indomethacin-responsive headache; trigeminal autonomic cephalalgias.