ABSTRACT
Figure 6.1 SUNCT is marked by very short lasting attacks (5 to 250 s) of headache and associated autonomic symptoms. Adapted with permission from Pareja JA, Shen JM, Kruszewski P, et al. SUNCT syndrome: duration, frequency, and temporal distribution of attacks. Headache 1996;36:161-5
Figure 6.2 Duration of individual attacks of chronic paroxysmal hemicrania (CPH) in minutes. Most attacks will be 15 to 20 min in duration. Adapted with permission from Antonaci F, Sjaastad O. Chronic paroxysmal hemicrania (CPH): a review of the clinical manifestations. Headache 1989;29: 648-56
Figure 6.3 Duration of cluster attacks.Typical attack duration is 1 h or less. Adapted with permission from Russell D. Cluster headache: Severity and temporal profiles of attacks and patient activity prior to and during attacks. Cephalalgia 1981;1:209-16
Vascular
Infection
Trauma
Neoplasm
Figure 6.4 Cluster headache is considered a primary headache disorder, so there are no underlying secondary causes. In rare instances cluster headache has been linked to various secondary causes including: aneurysms, head trauma, orbital enucleation, sphenoid sinusitis, parasellar tumors, cervical cord mengiomas or infarction, subdural hematomas and arteriovenous malformations. Adapted from the Neurology Ambassador Program with permission from the American Headache Society
Attacks daily (up to 8 attacks/day)
Peak time periods
Figure 6.5 Cluster headache has a distinct circadian periodicity to its attacks. Cluster patients will get attacks at the same time each day and cluster periods at the same time each year. This suggests that the hypothalamus (suprachiasmatic nucleus) or circadian clock is playing a role in cluster genesis. A hallmark of cluster is for the patient to awaken with a cluster headache 1.5 to 2 h after falling asleep (first REM period of the night); typically these night-time attacks are the most painful.Adapted from the Neurology Ambassador Program with permission from the American Headache Society
Episodic Chronic
Cluster headache
Episodic (90%)
Related syndromes Figure 6.6 Most cluster patients have the episodic subtype so they will have periods of remission sometimes for years at a time. Adapted from the Neurology Ambassador Program with permission from the American Headache Society
Figure 6.7 Cluster periods plotted against month of the year and mean monthly daylight duration. Cluster periods appear to occur during the longest and shortest days of the years. Adapted with permission from Kudrow L. The cyclic relationship of natural illumination to cluster period frequency. Cephalalgia 1987;7(Suppl 6):76-8
Melatonin
Suprachiasmatic nucleus
Hypothalamus
Pineal gland
Figure 6.8 The hypothalamus or circadian clock must be involved in cluster genesis. Cluster headaches have a circannual and circadian rhythmicity, a seasonal predilection for cluster periods and there is altered secretion of hypothalamic hormones in cluster patients (testosterone, melatonin). Adapted from the Neurology Ambassador Program with permission from the American Headache Society
Optic chiasm
Pituitary
Other hypothalamic nuclei
Mamillary body
Suprachiasmatic nucleus
Figure 6.9 The suprachiasmatic nucleus is the human circadian clock
Figure 6.10 An actual cluster patient of Bayard T. Horton. Horton is considered the ‘father of cluster headache’; not only did he describe the cardinal features of cluster headache but he was also the first to use oxygen in its therapy. The image shows a male undergoing a right-sided cluster headache with associated autonomic symptoms. The patient has some of the typical ‘leonine facies’ features recognized in cluster headache: deep nasolabial folds, peau d’orange skin and squared jaw. Reproduced with permission from Horton BT. The use of histamine in the treatment of specific types of headaches. JAMA 1941;116:377-83
Figure 6.11 Age of cluster onset. (a) Most men develop their first ever cluster attack in their twenties or thirties (standard deviation = 13.47, mean = 31.3, n = 69); (b) women with cluster headaches have two age peaks of cluster onset (unlike men), one in their late teens or twenties and a second when they are 50 or 60 years of age (standard deviation = 15.89, mean = 29.4, n = 32). Late age of onset of female cluster headaches needs to be recognized by the treating physician. Adapted from Rozen TD, Niknam RM, Shechter AL, et al. Cluster headache in women: clinical characteristics and comparison with cluster headache in men. J Neurol Neurosurg Psychiatr 2001;70:613-7, with permission from the BMJ Publishing Group
Prevalence
Predominantly male (male: female ratio)
Mean age of onset
Rare before the age of 10 years
Figure 6.12 Cluster predominantly occurs in men, although more women are being diagnosed with cluster headache. The prevalence of cluster headache is 0.4% of the population. Adapted from the Neurology Ambassador Program with permission from the American Headache Society
Figure 6.13 Reduction in pain severity after administration of sumatriptan injectable to patients with cluster headache. A significant response was observed by 10 and 15 min versus placebo. Adapted with permission from Olesen J, Tfelt-Hansen P, Welch KMA, eds. The Headaches, 2nd edn. Philadelphia, PA: Lippincott Williams & Wilkins, 2000:733
Figure 6.14 Presently the most effective surgical therapy for cluster is radiofrequency lesioning of the gasserian (trigeminal) ganglion. Under radiographic control a device is inserted into the cheek and directed through the foramen ovale into the area of the gasserian ganglion where a specific denervating agent (radiofrequency, glycerol) is then used. Adapted from the Neurology Ambassador Program with permission from the American Headache Society
Figure 6.15 SUNCT is considered a disorder of the elderly, although there are some reported cases younger than age 40. Adapted with permission from Pareja JA, Sjaastad O. SUNCT syndrome. A clinical review. Headache 1997;37:195-202
Figure 6.16 SUNCT pain is located in or around the eye and/or the forehead region. It can spread to extra-trigeminal innervated areas.Adapted with permission from Pareja JA, Sjaastad O. SUNCT syndrome. A clinical review. Headache 1997;37:195-202
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Time (h)
Combined temporal distribution of SUNCT attacks
6 a.m.
