Definition

The best known TAC condition is cluster headache. It was first described in 1939 as “Horton’s histamine headache,” so named after the researcher. Horton described a specific form characterized by headache, facial pain, tearing and running nose, and swelling around the eye that could be induced by subcutaneous injection of histamine. A wide variety of names were subsequently used, probably for the same diagnosis, which included among others “ciliary neuralgia,” “erythromelalgia of the head,” “Sluder’s neuralgia,” “sphenopalatine neuralgia,” and “migrainous neuralgia.” In 1952, it was first referred to as a cluster headache.

Cluster headache is a primary headache disorder characterized by recurrent attacks of severe unilateral pain, which usually involve the orbital or periorbital region which is served by the first (ophthalmic) division of the trigeminal nerve. The classic signs and symptoms that accompany the pain on the same side are:

  • tearing
  • red eye
  • nasal congestion or runny nose (or both)
  • droopy eyelid or pupil constriction (or both)
  • periorbital swelling

The term cluster headache originates from the tendency of attacks to cluster together into bouts that last several weeks. In the episodic form of the disorder, the bouts can occur at certain times of year. They are separated by periods of remission, which last at least a month. However, about 10% of patients have the chronic form of the disorder and have continuous attacks with no respite.

Episodic cluster headache: Attacks occurring in periods that last seven days to one year separated by pain-free periods that last one month or longer

Chronic cluster headache: Attacks that occur for more than one year without remission or with remissions that last less than one month

Less frequent bouts of attacks and more prolonged, and sometimes permanent, periods of remission can occur with advancing age.

Symptoms

The pain of cluster headache is unilateral in at least 97% of people with episodic disease and mainly focused behind the eye over the temple, or over the jawbone, although it may extend to other areas of the head and neck. Between 14% and 18% of patients report that the pain shifts sides between bouts of attacks and less commonly during a bout, but never during the attack itself. Patients often describe the pain as a sharp, piercing, burning, or pulsating sensation like “having a red hot poker forced through my eye,” and they report that the intensity is so extreme it is unlike anything they have ever experienced (“11 out of 10.”)

Attacks should last between 15 and 180 minutes, although on rare occasions they can last longer.

The onset of pain is rapid, and the sensation increases from serious discomfort to excruciating pain over the course of a few minutes. The pain usually stays at maximal intensity for the duration of the attack, although it may wax and wane slightly, or be punctuated by super-intense stabs of pain. The attack will often end as abruptly as it started.

The frequency of attacks varies from one attack every 48 hours to eight separate attacks in 24 hours, although less frequent attacks may occur at the beginning and end of bouts.

Most patients describe a sense of restlessness and agitation during an attack and will often pace, rock back and forth, and bang their heads. Most patients wish to isolate themselves and seek a cold environment. Between 28% and 50% report nausea, and a further 23% may vomit during an attack. More than half of patients have extreme sensitivity to light, often limited to the same side as the pain, with slightly fewer reporting an aversion to loud noise or strong smells during the attack.

Aura phenomena, similar to those experienced during migraine, including visual phenomena and abnormal sensations, precede attacks by up to 60 minutes in 14% of patients.

Patients commonly have tenderness and allodynia at and around the site of pain between attacks, including over the greater occipital nerve.

Most people with cluster headache experience one bout a year. However, patients may go for several years without a bout (up to 20 in some cases), and others may have more frequent bouts each year.

Diagnostic criteria:
A At least five attacks fulfilling B–D
B Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15–180 minutes if untreated
C Headache is accompanied by at least one of the following:
    (1) Ipsilateral conjunctival injection and/or lacrimation
    (2) Ipsilateral nasal congestion and/or rhinorrhoea
    (3) Forehead and facial sweating
    (4) Ipsilateral eyelid oedema
    (5) Ipsilateral forehead and facial sweating
    (6) Ipsilateral miosis and/or ptosis
    (7) A sense of restlessness or agitation
D Attacks have a frequency from one every other day to eight per day
E Not attributed to another disorder
Episodic cluster headache
Description: Occurs in periods lasting seven days to one year separated by pain free periods lasting one month or more
Diagnostic criteria:
    A All fulfilling criteria A–E of CH Diagnostic Criteria
    B At least two cluster periods lasting from 7 to 365 days and separated by pain free remissions of one month or more
Chronic cluster headache
Description: Attacks occur for more than one year without remission or with remissions lasting less than one month
Diagnostic criteria:
    A All alphabetical headings of CH Diagnostic Criteria
    B Attacks recur for more than one year without remission periods or with remission periods lasting less than one month

Table: J Neurol Neurosurg Psychiatry 2005;76:301-305 doi:10.1136/jnnp.2004.036012
Some material excerpted from:
– BMJ. Clinical Review: Cluster Headache. 2012; 344 doi: http://dx.doi.org/10.1136/bmj.e2407
– Zakrzewska and Jensen. History of facial pain diagnosis. International Headache Society, 2017. DOI: 10.1177/0333102417691045 https://doi.org/10.1177/0333102417691045


Further Reading:
Balla JI, Walton JN. Periodic migrainous neuralgia. BMJ1964;1:219-21. Full text
Headache Classification Committee of the International Headache Society. The international classification of headache disorders (second edition). Cephalalgia 2004; 24(suppl 1):1-160. Full text
Bahra A, May A, Goadsby PJ. Cluster headache: a prospective clinical study in 230 patients with diagnostic implications. Neurology 2002; 58:354-61. Full text
Donnet A, Lanteri-Minet M, Guegan-Massardier E, Mick G, Fabre N, Geraud G, et al. Chronic cluster headache: a French clinical descriptive study. J Neurol Neurosurg Psychiatry 2007; 78:1354-8. Full text
Cittadini E, Matharu MS, Goadsby PJ. Paroxysmal hemicrania: a prospective clinical study of thirty-one cases. Brain 2008; 131:1142-55. Full text
Cohen AS, Matharu MS, Goadsby PJ. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or cranial autonomic features (SUNA). A prospective clinical study of SUNCT and SUNA. Brain 2006; 129:2746-60. Full text
Drummond PD. Mechanisms of autonomic disturbance in the face during and between attacks of cluster headache. Cephalalgia 2006; 26:633-41. Full text
Fischera M, Marziniak M, Gralow I, Evers S. The incidence and prevalence of cluster headache: a meta-analysis of population-based studies. Cephalalgia 2008; 28:614-8. Full text
Irimia P, Cittadini E, Paemeleire K, Cohen AS, Goadsby PJ. Unilateral photophobia or phonophobia in migraine compared with trigeminal autonomic cephalalgias. Cephalalgia 2008; 28:626-30. Full text
Marmura MJ, Pello SJ, Young WB. Interictal pain in cluster headache. Cephalalgia 2010; 30:1531-4. Full text
Chervin RD, Zellek N, Lin X, Hall JM, Sharma N, Hedger KM. Sleep disordered breathing in patients with cluster headache. Neurology 2000; 54:2302-6. Full text
P J Goadsby. Trigeminal autonomic cephalalgias: fancy term or constructive change to the IHS classification? J Neurol Neurosurg Psychiatry 2005;76:301-305 doi:10.1136/jnnp.2004.036012
T. Sprenger, MD, H. Boecker, MD, T. R. Tolle, MD PhD, G. Bussone, MD, A. May, MD and M. Leone, MD, Specific hypothalamic activation during a spontaneous cluster headache attack. Neurology February 10, 2004 vol. 62 no. 3 516-517.
Benoliel, Rafael. Trigeminal Autonomic Cephalgias. British Journal of Pain 6.3 (2012): 106–123.
Michele Viana, Cristina Tassorelli, Marta Allena, Giuseppe Nappi, Ottar Sjaastad, Fabio Antonaci. Diagnostic and Therapeutic Errors in Trigeminal Autonomic Cephalalgias and Hemicrania Continua: a Systematic Review The Journal of Headache and Pain, 14.1 (2013): Page 1
Leonne, M. Bussone, G. Pathophysiology of Trigeminal Autonomic Cephalalgias Lancet Neurology 8.8 (2009): 755-64

 

Rev. 07/17