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Marja-LiisaSumelahti

Cluster Headache (Horton's Syndrome)

Essentials

  • Cluster headache consists of extremely severe, brief attacks of pain that may recur several times a day. The symptoms affect the patient's working capacity and functional ability.
  • Various studies have been performed on the efficacy of different products and forms of treatment used either for attacks or prophylactically. A plan for effective treatment should be drawn up by a neurologist with expertise in pain management.

Definition and epidemiology

  • A trigeminal autonomic cephalalgia with very intense attacks lasting 15-180 min
  • Its prevalence on population level is about 0.1%. Affects mainly men.
  • The symptoms often begin at the age of 30-40 years.
  • In the episodic form of the disease, periods of pain may last several weeks.
  • In the rarer, chronic form, periods of pain will last a year or longer.

Symptoms and diagnostics

  • The diagnostic criteria for cluster headache are defined in the International Classification of Headache Disorders (ICHD-3) http://ichd-3.org/.
  • The clinical picture is clearly identifiable. There is unilateral deep, pulsating, tearing, burning, drilling pain in the eye area that is very severe and lasts 15-180 min. As an autonomic feature, there is ipsilateral lacrimation and additional symptoms including nasal congestion, forehead sweating, miosis, eyelid oedema and ptosis. Agitation and restless movement are typical.
  • Attacks usually occur over periods lasting several weeks that can be followed by symptomless periods.
  • There may be as many as 8 pain attacks per day, pain causing the patient to wake up at night after a couple of hours of sleep, some attacks being associated with REM sleep. Some attacks occur during waking hours.
  • The headache is different from migraine headache; there are no prodromal symptoms or nausea.
  • The diagnosis is based on the patient's description. If the neurological status between attacks is normal, no imaging is needed.

Differential diagnosis

  • Migraine begins at a younger age, attacks are less frequent and are associated with prodromal symptoms, physical activity aggravates the pain, pain attacks are longer (4-72 h) and there may be nausea; see Migraine.
  • In female patients, cluster headache and migraine often coexist or alternate.
  • In trigeminal neuralgia, pain attacks in the trigeminal nerve region are set off by touching the skin or teeth on the side of the pain, are shorter in duration and electric shock-like; see Trigeminal Neuralgia and other Facial Pain.
  • Atypical facial pain is milder, continuous pain, often following dental or facial surgery.
  • Tension-type headache may be located unilaterally and temporally, causing milder, continuous pain alleviated by physical activity.
  • In differential diagnosis, other rare trigeminal autonomic cephalalgias should be taken into consideration, such as chronic, paroxysmal hemicrania which responds to indomethacin http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=157835.
  • SUNCT (Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing) http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Expert=57145&lng=EN

Treatment

Acute attack at home and in emergency care

Prophylaxis

  • Verapamil starting with 80 mg 3 times daily, increasing up to 600 mg/day or even more, if no cardiovascular contraindications are present (ECG)
  • Methylprednisolone 100 mg p.o. - 500 mg i.v. for 5 days, thereafter reducing the dose over a period of one week
  • Propranolol 40 mg 3 times daily, atenolol 100 mg once daily may be tried if previous treatments have failed or are contraindicated.
  • Topiramate 100-150 mg daily divided into 2 doses may be tried if previous treatments have failed or are contraindicated.
  • Lithium (requires monitoring of blood concentrations as even a short-term overdosage may cause renal damage; aim at the lowest effective dose). Simultaneous use of NSAIDs should be avoided.
  • Serial suboccipital glucocorticoid injections and occipital nerve block have also been used.
  • Studies on prophylactic CGRP monoclonal antibodies, which have been shown to be effective in migraine prophylaxis, are ongoing.

    References

    • May A, Leone M, Afra J et al. EFNS guidelines on the treatment of cluster headache and other trigeminal-autonomic cephalalgias. Eur J Neurol 2006;13(10):1066-77. [PubMed]
    • Fischera M, Marziniak M, Gralow I et al. The incidence and prevalence of cluster headache: a meta-analysis of population-based studies. Cephalalgia 2008;28(6):614-8. [PubMed]
    • Robbins MS, Starling AJ, Pringsheim TM et al. Treatment of Cluster Headache: The American Headache Society Evidence-Based Guidelines. Headache 2016;56(7):1093-106. [PubMed]