Cluster Headache: The Essentials

Cluster Headache, nicknamed “suicide Headache,” is a neurological disease that involves, as its most prominent feature, excruciating pain. “Cluster” refers to the tendency of these Headaches to occur periodically, with active periods interrupted by spontaneous remissions. The cause of the disease is currently unknown.

“There is no more severe pain than that sustained by a cluster headache sufferer and if not for the rather short duration of attacks most cluster sufferers would choose death rather than continue suffering.”   — Todd Rozen, MD

Cluster Headaches are classified as a trigeminal autonomic cephalalgias (TAC). Other Headache disorders in this same classification are SUNCT (Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing), SUNA (Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms), Hemicrania Continua, and Paroxysmal Hemicrania. All of these disorders activate the trigeminal parasympathetic reflex, resulting in one-sided involuntary tears, nasal congestion, runny nose, dropping eyelid, or redness and swelling of the eyelid.

ICHD-3 Classification of Cluster Headache

Description:

Attacks of severe, strictly unilateral pain which is orbital, supraorbital, temporal or in any combination of these sites, lasting 15-180 minutes and occurring from once every other day to eight times a day. The pain is associated with ipsilateral conjunctival injection, lacrimation, nasal congestion, rhinorrhoea, forehead and facial sweating, miosis, ptosis and/or eyelid oedema, and/or with restlessness or agitation.

Diagnostic criteria:

1. At least five attacks fulfilling criteria B-D
2. Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 minutes (when untreated)
3. Either or both of the following:
   1. at least one of the following symptoms or signs, ipsilateral to the headache:
      • conjunctival injection and/or lacrimation
      • nasal congestion and/or rhinorrhoea
      • eyelid oedema
      • forehead and facial sweating
      • miosis and/or ptosis
   2. a sense of restlessness or agitation
4. Occurring with a frequency between one every other day and 8 per day
5. Not better accounted for by another ICHD-3 diagnosis.

3.1.1 Episodic cluster headache

Description:

Cluster headache attacks occurring in periods lasting from 7 days to one year, separated by pain-free periods lasting at least 3 months.

Diagnostic criteria:

1. Attacks fulfilling criteria for 3.1 Cluster headache and occurring in bouts (cluster periods)
2. At least two cluster periods lasting from 7 days to 1 year (when untreated) and separated by pain-free remission periods of ≥3 months.

3.1.2 Chronic cluster headache

Description:

Cluster headache attacks occurring for one year or longer without remission, or with remission periods lasting less than 3 months.

Diagnostic criteria:

1. Attacks fulfilling criteria for 3.1 Cluster headache, and criterion B below
2. Occurring without a remission period, or with remissions lasting <3 months, for at least 1 year.

Sources:

1. Cluster Headache. American Migraine Foundation. 28 Nov 2017. Retrieved 11 April 2018 from  https://americanmigrainefoundation.org/understanding-migraine/cluster-headache/.
2. International Headache Society (2013-2018). 3.1 Cluster Headache. Retrieved 16 April 2018 from https://www.ichd-3.org/3-trigeminal-autonomic-cephalalgias/3-1-cluster-headache/.
3. Robbins M, Starling A, Pringsheim T, et al. (2016), Treatment of Cluster Headache: The American Headache Society Evidence‐Based Guidelines. Headache, 56: 1093-1106. doi:10.1111/head.12866
4. Rozen T and Fishman R (2012), Cluster Headache in the United States of America: Demographics, Clinical Characteristics, Triggers, Suicidality, and Personal Burden. Headache, 52: 99-113. doi:10.1111/j.1526-4610.2011.02028.x