Trigeminal autonomic cephalgias: diagnosis, therapy, atypical forms and pathophysiology

Trigeminal autonomic cephalgias; cluster headache, paroxysmal hemicrania, hemicrania continua and SUNCT (short-lasting unilateral neuralgiform headache attacks with conjuctival injection and tearing) syndrome are characterized by unilateral trigeminal distribution of pain and accompanying ipsilateral autonomic symptoms. Other than cluster headache, all of these syndromes have been described within last twenty years, and are found relatively less frequent and less known forms. Diagnosis of paroxysmal hemicrania and hemicrania continua, especially atypical forms, essentially depends on indomethacin responsiveness. For SUNCT syndrome, there is not such a drug which provides a practical approach to both diagnosis and therapy and its diagnosis depends on clinical features. So far, case reports from various countries helped us realize the existence of secondary forms of these syndromes and the necessity of imaging techniques, especially for recently described autonomic cephalgias.