Cluster Headache and Other Trigeminal Autonomic Cephalalgias

Stephanie J. Nahas, MD, MSEd, FAHS, FAAN Headache p. 633-651 June 2021, Vol.27, No.3 doi: 10.1212/CON.0000000000000965
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PURPOSE OF REVIEW The trigeminal autonomic cephalalgias (TACs) are relatively rare, but they represent a distinct set of syndromes that are important to recognize. Despite their unique features, TACs often go undiagnosed or misdiagnosed for several years, leading to unnecessary pain and suffering. A significant proportion of TAC presentations may have secondary causes.

RECENT FINDINGS The underlying pathophysiology of TACs is likely rooted in hypothalamic dysfunction and derangements in the interplay of circuitry involving trigeminovascular, trigeminocervical, trigeminoautonomic, circadian, and nociceptive systems. Recent therapeutic advancements include a better understanding of how to use older therapies more effectively and the identification of new approaches.

SUMMARY TAC syndromes are rare but important to recognize because of their debilitating nature and greater likelihood for having potentially serious underlying causes. Although treatment options have remained somewhat limited, scientific inquiry is continually advancing our understanding of these syndromes and how best to manage them.

Address correspondence to Dr Stephanie J. Nahas, Jefferson Headache Center, 900 Walnut St., Ste 200, Philadelphia, PA 19107, [email protected].

RELATIONSHIP DISCLOSURE: Dr Nahas serves on advisory boards for Allergan/AbbVie Inc and Zosano Pharma Corporation and as a consultant for Alder BioPharmaceuticals, Inc/Lundbeck; Allergan/AbbVie Inc; Amgen Inc/Novartis AG; Biohaven Pharmaceuticals; Impel NeuroPharma, Inc; Lilly; Nesos Corp (formerly Vorso Corporation); Supernus Pharmaceuticals, Inc; Teva Pharmaceutical Industries Ltd; Theranica Bio-Electronics Ltd; and Zosano Pharma Corporation. Dr Nahas serves on the editorial board of Current Pain and Headache Reports and UpToDate, Inc and as a contributing author for the Massachusetts Medical Society, Springer Nature, and Wolters Kluwer. Dr Nahas has received personal compensation for speaking engagements from Allergan/AbbVie Inc, Amgen Inc/Novartis AG, Lilly, and Teva Pharmaceutical Industries Ltd and research/grant support from Teva Pharmaceutical Industries Ltd.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Nahas discusses the unlabeled/investigational use of medications for the treatment of cluster headache and other trigeminal autonomic cephalalgias, of which only galcanezumab, sumatriptan, and the noninvasive vagus nerve stimulator are approved or cleared by the US Food and Drug Administration for this indication.

© 2021 American Academy of Neurology.