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Cluster headache

Subjects

Abstract

Cluster headache is an excruciating, strictly one-sided pain syndrome with attacks that last between 15 minutes and 180 minutes and that are accompanied by marked ipsilateral cranial autonomic symptoms, such as lacrimation and conjunctival injection. The pain is so severe that female patients describe each attack as worse than childbirth. The past decade has seen remarkable progress in the understanding of the pathophysiological background of cluster headache and has implicated the brain, particularly the hypothalamus, as the generator of both the pain and the autonomic symptoms. Anatomical connections between the hypothalamus and the trigeminovascular system, as well as the parasympathetic nervous system, have also been implicated in cluster headache pathophysiology. The diagnosis of cluster headache involves excluding other primary headaches and secondary headaches and is based primarily on the patient's symptoms. Remarkable progress has been achieved in developing effective treatment options for single cluster attacks and in developing preventive measures, which include pharmacological therapies and neuromodulation.

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Figure 1: Schematic pathway representation summarizing the trigeminal-parasympathetic reflex.
Figure 2: Schematic peripheral and central pathway representation summarizing the pathogenesis of cluster headache.
Figure 3: Neuromodulation therapies for cluster headache.

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Contributions

Introduction (A.M.); Epidemiology (P.P.-R. and D.M.); Mechanisms/pathophysiology (A.M. and S.-J.W.); Diagnosis, screening and prevention (T.J.S.); Management (S.E. and D.M.); Quality of life (P.P.-R.); Outlook (S.-J.W. and A.M.); Overview of the Primer (all authors).

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Correspondence to Arne May.

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Competing interests

A.M. has been a consultant or speaker for Allergan, Autonomic Technologies, Desitin, Electrocore and Teva and is the Editor in Chief for Cephalalgia. T.J.S. has acted as a consultant or has served on the advisory boards of Allergan, Amgen, Autonomic Technologies, Avanir, Dr. Reddy's, Nocira, Novartis and Teva; has stock options in Nocira and Second Opinion; and has received royalties from UpToDate. D.M. has received research and travel grants from ElectroCore LLC. P.P.-R. has received honoraria as a consultant or speaker from Allergan, Almirall, Chiesi, Eli Lilly, Novartis and Teva. S.E. has received honoraria as a speaker from or is a member of an advisory board for Allergan, Johnson & Johnson, Novartis, Reckitt Benckiser and TAD Pharma. S.-J.W. has served on the advisory boards of Daiichi-Sankyo, Eli Lilly and Taiwan Pfizer and has received honoraria as a moderator from the Taiwan branches of Bayer, Eisai, Eli Lilly and Pfizer.

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May, A., Schwedt, T., Magis, D. et al. Cluster headache. Nat Rev Dis Primers 4, 18006 (2018). https://doi.org/10.1038/nrdp.2018.6

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