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Headaches - cluster - Cluster Headaches

Description

An in-depth report on the causes, diagnosis, treatment, and prevention of cluster headaches.

Cluster Headaches:

Cluster headaches are among the most painful, and least common, of all headaches. The pain can be so excruciating that they are sometimes referred to as "suicide headaches." Their signature is a pattern of periodic cycles ("clusters") of headache attacks, which may be either:

  • Episodic. Attacks occur regularly for 1 week to 1 year, separated by long pain-free periods that last at least 1 month. Between 80 - 90% of patients have episodic cycles. A significant percentage of people who experience a first cluster attack do not have another one.
  • Chronic. Attacks occur regularly for more than 1 year, with pain-free periods lasting less than 1 month. Between 10 - 20% of patients have chronic cluster headaches. The chronic form is very difficult to treat.

Symptoms of Cluster Headaches

Cluster headaches usually strike suddenly and without warning, although some people experience a migraine-type aura before the attack. The pain is deep, constant, boring, piercing, or burning in nature, and located in, behind, or around the eye. The pain then spreads to the forehead, jaw, upper teeth, temples, nostrils, shoulder or neck. The pain and other symptoms usually remain on one side of the head.

The pain generally reaches very severe levels within 15 minutes. Patients may feel agitated or restless during an attack and often want to isolate themselves and then move around. Gastrointestinal symptoms are not very common.

Other typical symptoms include:

  • Swollen or droopy eyelid
  • Watery, tearing eye
  • Contraction of the eye pupil
  • Stuffy or runny nose
  • Forehead and facial sweating
  • Restlessness and agitation
  • Nausea and vomiting
  • Intolerance to light and sound
The symptoms of a cluster headache include stabbing severe pain behind or above one eye or in the temple. Tearing of the eye, congestion in the associated nostril, and pupil changes and eyelid drooping may also occur.
Pain of cluster headache

Typical Cluster Cycles

Timing of an Attack. Headache attacks tend to occur with great regularity at the same time of day. (For this reason, cluster headaches are sometimes referred to as "alarm clock" headaches.) About 75% of attacks occur between 9 p.m. - 10 a.m. Attacks may also peak between 1 - 3 p.m.

Duration of an Attack. A single cluster attack is usually brief but extremely painful, lasting about 15 minutes - 1.5 hours if left untreated.

Number of Attacks per Day. During an active cycle, people can experience as few as 1 attack every other day to as many as 8 attacks a day.

Duration of Cycles. Attack cycles cycles typically last 6 - 12 weeks with remissions lasting up to 1 year. In the chronic form, attacks are ongoing and there is little remission. Attacks cycles tend to occur seasonally, most often in the spring and autumn.

Primary Headaches That Resemble Cluster Headaches

Chronic Paroxysmal Hemicrania. Chronic paroxysmal hemicrania is a close relative of cluster headache and very similar. It causes multiple, short, and severe daily headaches with similar symptoms. Unlike cluster headaches, the attacks are shorter (1 - 2 minutes) and more frequent (occurring an average of 15 times a day). This headache is even rarer than cluster headache, tends to occur in women, and always responds to treatment with indomethacin.

Hemicrania Continua. Hemicrania continua occurs mostly in women. The patient generally experiences continuous low-level headache always on one side of the face. Periodic attacks can last days to weeks, which can be mild to severe, and may resemble migraines. (About 10% of patients experience remissions.) The headache can usually be treated successfully with indomethacin, which helps differentiate it from cluster and migraine headaches.

SUNCT Syndrome. A disorder called SUNCT syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) causes stabbing or burning eye pain that may resemble cluster headaches, but attacks are very brief (lasting about a minute) and may occur more than 100 times per day. Red and watery eyes, sweating forehead, and congestion are typical. This rare headache is more common in men and does not respond to other headache treatments.

Resources

References

Beck E, Sieber WJ, Trejo R. Management of cluster headaches. Am Fam Physician. 2005; 71(4): 717-24.

Burns B, Watkins L, Goadsby PJ. Treatment of medically intractable cluster headache by occipital nerve stimulation: long-term follow-up of eight patients. Lancet. 2007 Mar 31;369(9567):1099-106.

Cittadini E, May A, Straube A, Evers S, Bussone G, Goadsby PJ. Effectiveness of intranasal zolmitriptan in acute cluster headache: a randomized, placebo-controlled, double-blind crossover study. Arch Neurol. November 2006. [Epub ahead of print 11 September 2006]

Magis D, Allena M, Bolla M, De Pasqua V, Remacle JM, Schoenen J. Occipital nerve stimulation for drug-resistant chronic cluster headache: a prospective pilot study. Lancet Neurol. 2007 Apr;6(4):314-21.

May A. Cluster headache: pathogenesis, diagnosis, and management. Lancet. 2005; 366(9488): 843-55.

Rapoport AM, Mathew NT, Silberstein SD, Dodick D, Tepper SJ, Sheftell FD, Bigal ME. Zolmitriptan nasal spray in the acute treatment of cluster headache: a double-blind study. Neurology. 2007 Aug 28;69(9):821-6.

Rose KM, Wong TY, Carson AP, Couper DJ, Klein R, Sharrett AR. Migraine and retinal microvascular abnormalities: the Atherosclerosis Risk in Communities Study. Neurology. 2007 May 15;68(20):1694-700.

Schurks M, Kurth T, de Jesus J, Jonjic M, Rosskopf D, Diener HC. Cluster headache: clinical presentation, lifestyle features, and medical treatment. Headache. 2006 Sep;46(8):1246-54.

Silberstein SD, Young WB. Headache and facial pain. In: Goetz CG, eds. Textbook of Clinical Neurology. 3rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 53.

Sostak P, Krause P, Forderreuther S, Reinisch V, Straube A. Botulinum toxin type-A therapy in cluster headache: an open study. J Headache Pain. 2007 Sep 24; [Epub ahead of print]

Van Vliet JA, Eekers PJ, Haan J, Ferrari MD; Dutch RUSSH Study Group. Evaluating the IHS criteria for cluster headache -- a comparison between patients meeting all criteria and patients failing one criterion. Cephalalgia. 2006 Mar;26(3):241-5.

  • Reviewed last on: 9/9/2008
  • Harvey Simon, MD, Editor-in-Chief, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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