The trigeminal autonomic cephalgias include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). The evidence for the current treatment options for each of these syndromes is considered, including oxygen, sumatriptan, and verapamil in cluster headache, indomethacin in paroxysmal hemicrania, and intravenous lidocaine and lamotrigine in SUNCT. Some treatments such as topiramate have an effect in all of these, as well as in migraine and other pain syndromes. The involvement of the hypothalamus in functional imaging studies implies that this may be a substrate for targeting treatment options in the future.
Key words: trigeminal autonomic cephalalgias, cluster headache, paroxysmal hemicrania, SUNCT, treatmentThe trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders characterized by unilateral head pain that occurs in association with ipsilateral cranial autonomic features.1,2 The TACs include cluster headache (CH), paroxysmal hemicrania (PH), and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). These headaches are grouped into section 3 of the revised International Classification of Headache Disorders (ICHD-II).3 The TACs differ in attack duration and frequency as well as the response to therapy. CH has the longest attack duration and relatively low attack frequency. PH has intermediate duration and intermediate attack frequency. SUNCT has the shortest attack duration and the highest attack frequency (Table 1). The importance of recognizing these syndromes resides in their excellent but highly selective response to treatment.
CLUSTER HEADACHECluster headache is a strictly unilateral headache that occurs in association with cranial autonomic features. It is an excruciating syndrome and is probably one of the most painful conditions known to humans with female patients describing each attack as being worse than childbirth. It is located mainly around the orbital and temporal regions though any part of the head can be affected. The headache usually lasts 45 to 90 minutes but can range from 15 minutes to 3 hours. In most patients, it has a striking circannual and circadian periodicity. It is not uncommon by neurological standards at about 1 patient per 1,000 of the population. About 80% to 90% of patients have episodic cluster headache (ECH), which consists of recurrent bouts or cluster periods, each with a duration of more than a week and separated by remissions lasting more than 4 weeks. The cluster periods occur typically once or twice a year. The remaining 10% to 20% of patients have chronic cluster headache (CCH) in which either no remission occurs within 1 year or the remissions last less than 1 month.
3Triggers.-CH attacks are typically triggered by alcohol, with 90% of patients reporting sensitivity to alcohol during their cluster bout. 4 Anecdotal evidence suggests that volatile substances, such as solvents and oil-based paints, can al...