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); Sluder’s neuralgia; spheno-palatine neuralgia; vidian neuralgia.

Coded elsewhere:
Symptomatic cluster headache, secondary to another disorder, is coded as a secondary headache attributed to that disorder.

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:
A. At least five attacks fulfilling criteria B-D
B. Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 min (when untreated)1
C. Either or both of the following:

    1. at least one of the following symptoms or signs, ipsilateral to the headache:

      a) conjunctival injection and/or lacrimation
      b) nasal congestion and/or rhinorrhoea
      c) eyelid oedema
      d) forehead and facial sweating
      e) forehead and facial flushing
      f) sensation of fullness in the ear
      g) miosis and/or ptosis

    2. a sense of restlessness or agitation

D. Attacks have a frequency between one every other day and 8 per day for more than half of the time when the disorder is active
E. Not better accounted for by another ICHD-3 diagnosis.

Note:
1. During part (but less than half) of the time-course of 3.1 Cluster headache, attacks may be less severe and/or of shorter or longer duration.

Comments:
Attacks occur in series lasting for weeks or months (so-called cluster periods) separated by remission periods usually lasting months or years. About 10-15% of patients have 3.1.2 Chronic cluster headache, without such remission periods. In a large series with good follow-up, one quarter of patients had only a single cluster period. Such patients meet the criteria for and should be coded as 3.1 Cluster headache.

The pain of 3.1 Cluster headache is maximal orbitally, supraorbitally, temporally or in any combination of these sites, but may spread to other regions. During the worst attacks, the intensity of pain is excruciating. Patients are usually unable to lie down, and characteristically pace the floor. Pain usually recurs on the same side of the head during an individual cluster period. During a cluster period in 3.1.1 Episodic cluster headache, and at any time in 3.1.2 Chronic cluster headache, attacks occur regularly and may be provoked by alcohol, histamine or nitroglycerin.

Age at onset is usually 20-40 years. For unknown reasons, men are afflicted three times more often than are women.

Acute attacks involve activation in the region of the posterior hypothalamic grey matter. 3.1 Cluster headache may be autosomal dominant in about 5% of cases.

Some patients have been described who have both 3.1 Cluster headache and 13.1 Trigeminal neuralgia (sometimes referred to as cluster-tic syndrome). They should receive both diagnoses. The importance of this observation is that both conditions must be treated for the patient to become headache free.