Cluster headache has now been grouped with cluster and the autonomic cephalagias (see Table 1).  The diagnostic criteria for cluster headache are outlined in Table 7 and episodic cluster in Table 8.

Table 7.                   Cluster headache

3.1           Cluster headache   Previously used terms: Ciliary neuralgia, erythro-melalgia of the head,    erythroprosopalgia of Bing, hemicrania angioparalytica, hemicrania neuralgiformis    chronica, histaminic cephalalgia, Horton’s headache, Harris-Horton’s disease,     migrainous neuralgia (of Harris), petrosal neuralgia (of Gardner).                   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 8 times a day.  The attacks are associated with one or more of the following, all of which are ipsilateral:  conjuctival injection, lacrimation, nasal congestion, rhinorrhoea, forehead and facial sweating, miosis, ptosis, eyelid oedema.  Most patients are restless or agitated during an attack.   Diagnostic criteria A.            At least five attacks fulfilling B-D   B.            Severe or very severe unilateral obital, supraorbital and/or temporal pain lasting 15-180 minutes if untreated1   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.             ipsilateral eyelid oedema 4.             ipsilateral forehead and facial sweating 5.             ipsilateral miosis and/or ptosis 6.             a sense of restlessness or agitation   D.            Attacks have a frequency from one every other day to 8  per day2                   E.            Not attributed to another disorder3

¹ During part (but less than half) of the time-course of cluster headache, attacks may be less severe and/or

  of shorter or longer duration.

² During part (bust less than half) of the time-course of cluster headache, attacks may be less frequent.

³ History and physical and neurological examinations do not suggest any of the disorders listed in groups

  5-12, or history and/or physical and/or neurological examinations do sugges such disorder but it is ruled out

  by appropriate investigations, or such disorder is present but attacks do not occur for the first time in close

  temporal relation to the disorder.

Table 8.     Episodic Headache

3.1.1        Episodic headache   Description                 Cluster headache attacks occurring in periods lasting 7 days to 1 year separated by pain-                 free periods lasting 1 month or longer.   Diagnostic criteria A.            Attacks fulfilling criteria A-E for 3.1 Cluster headache                   B.            At least two cluster periods lasting 7-365 days1 and separated by pain-free                                      remission periods of >1 month.                   Comment                 The duration of the remission period has been increase in this second edition to a minimum of 1 month.   3.1.2        Chronic cluster headache   Description                 Cluster headache attacks occurring for more than 1 year without remission or with remissions lasting less than 1 month.   Diagnostic criteria A.            Attacks fulfilling criteria A-E for 3.1 Cluster headache   B.       Attacks recur over >1 year with remission periods or with remission periods lasting <1 month                   Comment                 Chronic cluster headache may arise de novo (previously referred to as primary chronic cluster headache) or evolve from the episodic subtype (previously referred to as  secondary chronic cluster headache).  Some patients may switch from chronic to episodic cluster headache.

¹ Cluster periods usually last between 2 weeks and 3 months.

The phrase "cluster headache" denotes a characteristic type of cephalgia defined as a severe unilateral head or facial pain, which lasts minutes to hours; it is often associated with ipsilateral lacrimation, nasal congestion, and facial flushing. The headaches tend to occur in separate bouts or clusters in one or more attacks daily for periods of weeks  to months.^162-167  Often during an attack, and sometimes persisting following the episode, there is ipsilateral miosis and ptosis, ie, and oculosympathetic paresis (Horner syndrome). 

Romberg in 1840 described "ciliary neuralgia" as recurrent pain in the eye with injection and pupillary constriction.¹⁶⁸  Harris described the same syndrome in 1926 as "periodic migrainous neuralgia"¹⁶⁹ and later (1928) as ciliary (migrainous) neuralgia.¹⁷⁰  Multiple redescriptions and rediscoveries of the syndrome have resulted in a host of synonymous terms as well as confusion with other entities not related to this particular headache syndrome. (Table 9)

Table 9.            Cluster Headache and Variants

Synonyms Periodic migrainous neuralgia Ciliary neuralgia             Harris’ neuralgia             Symonds’ “particular variety of headache”             Cluster headaches             Autonomic faciocephalgia             Erythromelalgia of the head             Histaminic cephalgia             Horton’s cephalgia or syndrome             Petrosal neuralgia             Raeder’s neuralgia (type 2) Syndromes Bearing Possible Relationship             Erythroprosopalgia             Some cases of vidian neuralgia             Some cases of Sluder’s neuralgia             Some cases diagnosed as “supraorbital neuralgia” Syndromes Bearing No Relationship             Reader’s neuralgia             Most cases of vidian neuralgia             Most cases of Sluder’s neuralgia Trigeminal neuralgia Atypical facial pain Tension headaches

(Modified from Bickerstaff EB: Cluster headaches. In Vinken PJ,

Bruyn GW (eds): Handbook of Clinical Neurology, Vol. 5, Headache

and Cranial Neuralgias.  New York, American Elsevier, 1968)

 

The pain in cluster headache is very severe, being described as "boring," "sharp," "unbearable," and "the worst pain I have ever felt."  The headache usually appears in or around one eye or on the cheek and then can spread to the temple, frontal region, occiput, or the ipsilateral neck.  It rapidly builds to a peak within a few minutes, with an intensity greater than most other headache varieties.  The usual duration is from 30 minutes to a few hours.  Rather than lying down in seclusion, as preferred by most migraine sufferers, the cluster victim often paces about, holds onto his face, or applies very hot or cold water to the affected region, even to the point of injuring himself.  The attacks usually occur once in a 24‑hour period, often at a specific time, and frequently in the early morning hours awakening the patient from sleep.  Men are affected much more commonly than women, in a ratio of approximately 5:1.  Usually the episodes begin in the second or third decade and, according to Bickerstaff,¹⁶³ frequently are ipsilateral to previous head trauma.  The headache tends to remain on the same side in a given cluster but rarely may alternate in subsequent clusters.  Typical migraine headache is frequently found in family members.  While most patients have clusters occurring over weeks or months (often at the same season of year), other patients have sporadic attacks or irregular episodes for indeterminate periods of time. 

Typical migraine headaches may occur in the same individual with cluster, sometimes waning as the cluster commences.  In the cluster attacks as described, it is extremely rare that any organic pathology is ever demonstrated; however, the variants of the syndrome require more detailed analysis.  When the pain becomes persistent, becomes bilateral, or additional neurologic abnormalities are present (such as fifth nerve or optic nerve involvement), other conditions must be ruled out. 

Regarding the mechanism of cluster headache, few specifics are known.  However, Ekbom and Greitz¹⁷¹ have demonstrated a localized narrowing of the extradural portion of the internal carotid artery distal to its exit from the carotid canal in a patient during an attack of cluster headache.  They speculated that, in addition to the headache, the partial Horner syndrome might be due to repeated dilatation and edema of the internal carotid artery resulting in damage to the sympathetic nerves surrounding the vessel. 

Bickerstaff¹⁶³ has reviewed the variety of terms used to describe cluster headache and the relationship to other types of facial and cranial neuralgias.  Vidian neuralgia as described by Vail¹⁷² referred to recurrent aching pain affecting the nose, eye, face, neck, and shoulder on one side, but he also included recurrent episodes which would clearly conform to the cluster headache syndrome.  The facial pain described originally by Sluder included clinical variants but primarily referred to a constant pain affecting eye, upper jaw, hard palate, and teeth on one side, usually in menopausal  women.¹⁷³

In 1924, Raeder described 5 patients with Horner syndrome but without facial anhydrosis (oculosympathetic paresis), with pain or numbness in the distribution of the ophthalmic branch of the trigeminal nerve, which he designated as "paratrigeminal neuralgia";¹⁷⁴ however, some of his patients had other cranial nerve signs.  Additional findings suggested specific intracranial lesions

Boniuk and Schlezinger¹⁷⁵ divided Raeder syndrome into two groups:  1) characterized by hemicrania, ipsilateral oculosympathetic paresis, and parasellar cranial nerve (III, IV, V, and VI) involvement, the additional cranial nerve signs suggesting disease in the middle cranial fossa and indicating appropriate diagnostic studies, and 2) hemicrania and group the most common cause is a cluster headache  variant,^162,176,177 and further  diagnostic studies including arteriography are unwarranted.  However, in  Raeder type 2 neuralgia, aneurysm,^178,179 and fibromuscular dysplasia of the carotid artery¹⁸⁰ have been reported. In the patients with such demonstrable lesions, unilateral facial pain was persistent rather than the episodic excruciating variety described in the classic cluster headache syndrome. 

Headaches with similar characteristics of cluster and associated signs and symptoms similar to cluster, but with a shorter burst of pain, occurring more frequently and responding to indomethacine are known as paroxysmal hemicrania headaches.(Table 10)  The usual dose of indomethacine is 25 to 50 mg per day.  Failure to respond makes the diagnosis questionable.

Therapy recommended for cluster headache has been quite variable and often was tailored to the suspected condition. Harris' method was to inject the gasserian ganglion.¹⁷⁰  Horton tried to desensitize his patients to histamine,¹⁸¹ and others have recommended sphenopalatine ganglionectomy.¹⁸²  Histamine is indeed elevated in serum during a cluster attack, with minimal change in serotonin (as contrasted to depressed levels of serotonin in typical migrainous episodes).¹⁸³ Ergotamine preparations have been widely used, parenterally in the acute attack and orally as a prophylactic agent.¹⁸³  The prophylactic use of methysergide (Sansert) has been employed successfully,¹⁸⁴ and currently propranolol (Inderal), 20 to 40 mg b.i.d. to q.i.d, may serve as an effective preventive.¹⁸⁵  Calcium channel blocking agents may also be effective in cluster headache, particularly nimodipine.¹⁸⁶ Therapy of cluster headaches and their relationship to other forms of vascular headache has been reviewed by Lance.¹⁶⁰