The hemicrania continua diagnosis

More than 16 years after the first description of hemicrania continua (HC), its aetiology and pathogenesis remain obscure. Clinically, HC is considered a syndrome with two pivotal characteristics: (i) strictly unilateral (moderate, fluctuating, relatively long-lasting) headache; and (ii) absolute response to indomethacin. HC is further characterized by some ancillary, but mostly "negative", features such as: (iii) relative paucity of accompaniments; and (iv) lack of precipitating factors. The female preponderance is also remarkable, although not diagnostic in the solitary case. Finally, a non-specific, but remarkable feature is the temporal pattern. HC may present as a remitting or chronic (continuous) headache. In HC, unilaterality and absolute response to indomethacin are considered crucial diagnostically. Existing controversy, such as regarding atypical features, particularly the so-called "HC resistant to indomethacin", is discussed. The nature of hemicrania with negative indomethacin response remains most unclear; it may not belong to the HC cycle at all. Accordingly, we propose that the typical clinical picture of HC, including an absolute response to indomethacin, be termed Hemicrania continua vera. More or less analogous, but indomethacin-resistant, clinical pictures can provisionally be termed Hemicrania generis incerti (of undetermined nature), provided other diagnostic possibilities have been ruled out. The differential diagnosis of HC vs. other unilateral headaches is commented on. Previous attempts at classification of HC into the group chronic daily headache (CDH) are discussed. The only acceptable "link" of HC with the other headaches classified as CDH is the temporal pattern (which is a non-specific feature). HC is probably pathophysiologically different from the others disorders classified under CDH. Conversely, HC and chronic paroxysmal hemicrania share many common features, including the absolute response to indomethacin. HC should probably be included in the IHS group 3.