PURPOSE OF REVIEW: This article covers the clinical features, differential diagnosis, and management of the trigeminal autonomic cephalalgias (TACs). The TACs are composed of five diseases: cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA), and hemicrania continua.

RECENT FINDINGS: New classifications for the TACs have two important updates; chronic cluster headache is now defined as remission periods lasting less than 3 months (formerly less than 1 month), and hemicrania continua is now classified as a TAC (formerly classified as other primary headache). The first-line treatments of TACs have not changed in recent years: cluster headache is managed with oxygen, triptans, and verapamil; paroxysmal hemicrania and hemicrania continua are managed with indomethacin; and SUNCT and SUNA are managed with lamotrigine. However, advancements in neuromodulation have recently provided additional options for patients with cluster headache, which include noninvasive devices for abortive therapy and invasive devices for refractory cluster headache. Patient selection for these devices is key.

SUMMARY: The TACs are a group of diseases that appear similar to each other and to other headache disorders but have important differences. Proper diagnosis is crucial for proper treatment. This article reviews the pathophysiology, epidemiology, differential diagnosis, and treatment of the TACs.

Address correspondence to Dr Mark Burish, Will Erwin Headache Research Center, 6400 Fannin St, Suite 2010, Houston, TX 77030, mark.j.burish@uth.tmc.edu.

RELATIONSHIP DISCLOSURE: Dr Burish has received personal compensation for serving as a lecturer for the Midwest Pain Society and the North American Neuromodulation Society and has received research/grant support from the American Headache Society, the National Headache Foundation, and the Will Erwin Headache Research Foundation.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Burish discusses the unlabeled/investigational use of baclofen, corticosteroids, deep brain stimulation, lithium, occipital nerve stimulation, oxygen, sphenopalatine ganglion stimulation, sumatriptan, topiramate, valproate, verapamil, and zolmitriptan for the treatment of cluster headache; indomethacin, topiramate, and verapamil for the treatment of paroxysmal hemicrania; carbamazepine, duloxetine, gabapentin, lamotrigine, lidocaine, oxcarbazepine, and topiramate for the treatment of short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing; and celecoxib, gabapentin, ibuprofen, indomethacin, melatonin, occipital nerve stimulation, onabotulinum toxin injections, topiramate, and verapamil for the treatment of hemicrania continua.

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