Functional brain imaging in hemicrania continua: implications for nosology and pathophysiology

Hemicrania continua is a strictly unilateral, continuous headache of mild to moderate intensity, with superimposed exacerbations of moderate to severe intensity that are accompanied by trigeminal autonomic features and migrainous symptoms. The syndrome is exquisitely responsive to indomethacin. Its clinical phenotype overlaps with that of the trigeminal autonomic cephalalgias and migraine, in which the hypothalamus and the brain stem, respectively, have been postulated to play central pathophysiologic roles. A recent positron-emission tomography study of a cohort of patients with hemicrania continua demonstrated significant activation of the contralateral posterior hypothalamus and ipsilateral dorsal rostral pons in association with the headache of hemicrania continua. In addition, there was activation of the ipsilateral ventrolateral midbrain, which extended over the red nucleus and the substantia nigra and bilateral pontomedullary junction. No intracranial vessel dilatation was obvious.