Cluster Headaches: Introduction and Epidemiology



Cluster Headaches: Introduction and Epidemiology


Peter J. Goadsby

Peer Tfelt-Hansen



CLASSIFICATION AND SHORT DESCRIPTION

Cluster headache (CH) falls into group 3 of the International Headache Society (IHS) classification, the trigeminal autonomic cephalalgias, and is coded 3.1 (6) (Table 85-1). In the tenth revision of the International Classification of Diseases (ICD-10) of the World Health Organization (WHO), it is coded G44.0 (episodic CH-G44.01, chronic CH-G44.02) (21).

Cluster headache is characterized by attacks of strictly unilateral severe pain with orbital, supraorbital, or temporal location. Attacks typically last 15 to 180 minutes and usually occur one or several times per day, especially at night. They are accompanied by ipsilateral conjunctival injection, lacrimation, rhinorrhea or nasal congestion, eyelid edema, miosis, and low-grade ptosis. There is a clear male preponderance.

Two main clinical forms of CH may be identified: episodic and chronic. The most common form is the episodic form, which affects 80% to 90% of patients. It is characterized by periods of attacks (bouts, cluster) and periods of remission. In the cluster period, the patient experiences from one to eight attacks per day (days without headache may occur); this period may last from 7 days to 1 year. Between cluster periods, patients are usually completely pain free, but short-lasting pain periods with isolated short attacks may occur.

The chronic form lacks the remissions and is diagnosed after 1 year without remission or if remissions have lasted less than one month. The patient may suffer from the chronic form from the beginning of the disease (chronic CH unremitting from onset), or it may develop from the episodic form (chronic CH evolved from episodic). In clinical practice, the essential differentiation between episodic and chronic cluster headache, in terms of how they are managed, is whether there are sufficiently long breaks to allow preventive treatment to be discontinued. Apart from these main clinical forms of CH, which affect the vast majority of patients, there are atypical cases, coded 3.4 in the IHS classification. These patients present some CH symptoms but differ in clinical course, additional signs, or both. These forms constitute the borderland of CH (also called variants).


Other Terms

Previously used terms include erythroprosopalgia of Bing (7), ciliary or migrainous neuralgia (Harris), erythromelalgia of the head, Horton’s headache, histaminic cephalgia, petrosal neuralgia (Gardner), sphenopalatine, Vidian and Sluder’s neuralgia, and hemicrania periodica neuralgiformis. The term “cluster headache” was introduced relatively recently, in 1952, by Kunkle and colleagues (11,12). By then, CH certainly had been known for a long time under other names (10), as demonstrated by the striking similarities between this condition and most other syndromes listed in Table 85-2. The abundance of eponyms and synonyms used in the past to describe the same form may have been indirectly responsible for the late recognition of CH as such. In fact, according to Koehler (9), the first report on CH dates to 1641, when Nicolaas Tulp, a famous Dutch physician, published his Observationes Medicae, in which he describes the case of Isaak van Halmaal, who “in the beginning of the summer season, was afflicted with a very severe headache, occurring and disappearing daily on fixed hours. For rarely it lasted longer than two hours. This recurring pain lasted until the fourteenth day.…” Another probable case from the 17th century was described by Thomas Willis, who, in De Anima Brutorum, mentioned a headache whose attacks tended to recur more severely at the time of the sun’s solstice and equinox, although in most cases it struck people in between, at preferred hours during the 24-hour day (16). Nappi and Manzoni (16) also argued
that CH and chronic paroxysmal hemicrania were both described in 1747 when Oppermann, in his doctoral dissertation on “hemicrania horologica,” described the case of a 35-year-old woman who, since the age of 29 years, had been suffering from daily 15-minute attacks every hour of the day and night with such regularity as to tell the hour more accurately than the clock in the city square.








TABLE 85-1 International Headache Society Classification of Cluster Headache (6a)































































3.1 Diagnostic criteria:



A.


At least five attacks fulfilling B through D



B.


Severe or very severe unilateral orbital, supraorbital, and/or temporal pain lasting 15 to 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 rhinorrhea



  3. Forehead and facial sweating



  4. Ipsilateral eyelid edema



  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


3.1.1


Episodic cluster headache



Description: occurs in periods lasting 7 days to 1 year separated by pain-free periods lasting ≥ month or more



Diagnostic criteria:



A.


All fulfilling criteria A through E of 3.1



B.


At least two cluster periods lasting from 7 to 365 days and separated by pain-free remissions of ≥1 month


3.1.2


Chronic cluster headache



Description: Attacks occur for >1 year without remission or with remissions lasting <1 month.



Diagnostic criteria:



A.


All alphabetical headings of 3.1



B.


Attacks recur for >1 year without remission periods or with remission periods <1 month









TABLE 85-2 Cluster Headache Synonyms and Related Syndromes



























































Previous Terms


Authors


Year


Red migraine


Moellendorf


1867


Angioparalytic hemicrania


Eulenburg


1883


Sphenopalatine ganglion neuralgia


Sluder


1910


Erythrosopalgia


Bing


1913


Syndrome de vasodilatation hemicéphalique d’origine sympathique


Vallery-Radot and Blamoutier


1925


Ciliary neuralgia


Harris


1926


Syndrome du nerf nasal


Charlin


1931


Vidian neuralgia


Vail


1932


Autonomic faciocephalalgia


Brickner and Riley


1935


Migrainous neuralgia


Harris


1936


Erythromelalgia


Horton et al.


1939


Greater superficial petrosal neuralgia


Gardner et al.


1947


Histaminic cephalgia


Horton


1952

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Jun 21, 2016 | Posted by in PAIN MEDICINE | Comments Off on Cluster Headaches: Introduction and Epidemiology

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