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  • Updated 01.17.2021
  • Released 02.14.1994
  • Expires For CME 01.17.2024

Cluster headache

Introduction

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 (164); an excellent clinical description can be found in Van Swieten’s textbook (152) and a reasonable description in the Spanish literature (269). It has been known by many names, notably Horton headache in North America (150; 148; 149) and migrainous neuralgia in the United Kingdom (137; 24; 176; 27). Other names that have probably described substantially similar syndromes, perhaps now called the trigeminal autonomic cephalalgias (178), include ciliary neuralgia (292), erythroprosopalgia of Bing (25), and hemicrania periodic neuralgiforms (265). Symonds recognized it as a particular variety of headache (342). Sphenopalatine ganglion neurosis (330) and Vidian neuralgia (353) 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 (263). The periodicity of the attacks led to the current nomenclature (138; 139; 140), cluster headache (172), which seems to describe it well and respect its biology.

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