Cluster headache

Peter J Goadsby MD PhD (

Dr. Goadsby of King’s College London and the University of California, San Francisco, received fees from Aeon Biopharma, Alder Biopharmaceuticals, Allergan, Autonomic Technologies Inc, Biohaven Pharmaceuticals Inc, Clexio, Electrocore LLC, eNeura, Epalex, GlaxoSmithKline, Impel Neuropharma, Lundbeck, MundiPharma, Novartis, Pfizer, Sanofi, Santara Therapeutics, Satsuma, Teva Pharmaceuticals, Trigemina Inc, and WL Gore; consulting grants and fees from Amgen and Eli-Lilly; and a grant from Celgene.

)
Shuu-Jiun Wang MD, editor. (

Dr. Wang of the Brain Research Center, National Yang-Ming University, and the Neurological Institute, Taipei Veterans General Hospital, received consulting fees from Eli Lilly and Novartis for advisory board membership and honorariums from AbbVie as a speaker.

)
Originally released February 14, 1994; last updated December 25, 2019; expires December 25, 2022

Overview

Cluster headache is a primary headache disorder characterized by severe, unilateral pain that is orbital, supraorbital, or temporal and 15 minutes to 3 hours in duration occuring 1 to 8 times a day. Accompanying symptoms include ipsilateral: conjunctival injection, lacrimation, nasal congestion, rhinorrhea, facial sweating, eyelid edema, and miosis. The current update provides the latest understanding and management of this disorder, including the latest clinical trial data.

Key points

 

• Cluster headache is a relatively common, very severe form of primary headache that belongs to the family of trigeminal autonomic cephalalgias.

 

• Cluster headache involves dysfunction of central nervous system elements concerned with pain control and with links to circadian and circannual mechanisms.

 

• Acute cluster headache can be treated with oxygen, intranasal triptans (sumatriptan and zolmitriptan), and injected sumatriptan, and for episodic cluster headache noninvasive vagal nerve stimulation.

 

• Medicines or strategies useful in the preventive management of cluster headache include verapamil, lithium, corticosteroids, topiramate, melatonin, greater occipital nerve injection, the calcitonin gene-related peptide (CGRP) monoclonal antibody galcanezumab, and noninvasive vagal nerve stimulation.

 

• Sphenopalatine ganglion stimulation has been demonstrated to be useful for the treatment of medically refractory chronic cluster headache.

Historical note and terminology

Cluster headache has been recognized for over 350 years (Koehler 1993); an excellent clinical description can be found in Van Swieten's textbook (Isler 1993) and a reasonable description in the Spanish literature (Palma and Palma 2011). It has been known by many names, notably Horton headache in North America (Horton et al 1939; Horton 1952; Horton 1956) and migrainous neuralgia in the United Kingdom (Harris 1936; Bickerstaff 1959; Lance and Anthony 1971; Boes et al 2002). Other names that have probably described substantially similar syndromes, perhaps now called the trigeminal autonomic cephalalgias (Lance and Goadsby 2005), include ciliary neuralgia (Romberg 1840), erythroprosopalgia of Bing (Bing 1930), and hemicrania periodic neuralgiforms (Olivarius de Fine 1971). Symonds recognized it as a particular variety of headache (Symonds 1956). Sphenopalatine ganglion neurosis (Sluder 1910) and Vidian neuralgia (Vail 1932) were described as affecting females and were probably migraine with a facial distribution and cranial autonomic symptoms, given data that confirm the common appearance of cranial autonomic features in migraine (Obermann et al 2007). The periodicity of the attacks led to the current nomenclature (Headache Classification Committee of The International Headache Society 1988; Headache Classification Committee of The International Headache Society 2004; Headache Classification Committee of The International Headache Society 2018), cluster headache (Kunkle et al 1952), which seems to describe it well and respect its biology.

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