Hemicrania continua: clinical review, diagnosis and management

Hemicrania continua (HC) is an indomethacin-responsive primary headache disorder which is currently classified under the heading of trigeminal autonomic cephalalgias (TACs). It is a highly misdiagnosed and underreported primary headache. The pooled mean delay of diagnosis of HC is 8.0 ± 7.2 years. It is not rare. We noted more than 1000 cases in the literature. It represents 1.7% of total headache patients attending headache or neurology clinic. Just like other TACs, it is characterized by strictly unilateral pain in the trigeminal distribution, cranial autonomic features in the same area and agitation during exacerbations/attacks. It is different from other TACs in one aspect. While all other TACs are episodic, HC patients have continuous headaches with superimposed severe exacerbations. The central feature of HC is continuous background headache. However, the patients may be worried only for superimposed exacerbations. Focusing only on exacerbations and ignoring continuous background headache are the most important factors for the misdiagnosis of HC. A large number of patients may have migrainous features during exacerbation phase. Up to 70% patients may fulfill the diagnostic criteria for migraine during exacerbations. Besides migraine, its exacerbations can mimic a large number of other primary and secondary headaches. The other specific feature of HC is a remarkable response to indomethacin. However, a large number of patients develop side effects because of the long-term use of indomethacin. A few other medications may also be effective in a subset of patients with HC. Various surgical interventions have been suggested for patients who are intolerant to indomethacin. Several aspects of HC are still not defined. There is a great heterogeneity in types of patients or articles on the HC in the literature. Diagnostic criteria have been modified several times over the years. The current diagnostic criteria are too restrictive in some aspects. We suggest a more accommodating type of criteria for the appendix of International Classification of Headache Disorder (ICHD).