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Cluster Headache

NORD is very grateful to Olivia Lanes, NORD Intern and Frederick R. Taylor, MD, Park Nicollet Headache Center, Adjunct Professor of Neurology, University of Minnesota, for assistance in the preparation of this report.

Synonyms of Cluster Headache

  • familial cluster headaches
  • histamine cephalalgia
  • vasogenic facial pain

Disorder Subdivisions

  • chronic cluster headache
  • episodic cluster headache

General Discussion

Summary
Cluster headaches (CH) are an uncommon, severe form of primary neurovascular headaches. CH are the most painful form of headaches, with the pain occurring on one side of the head and behind or above the eye or at the temple most commonly. The pain has been described as searing, burning and stabbing. The age of onset of CH is most often between 20 and 40, and they are more common in men than women with a ratio of 2:1. CH attacks also include one or more cranial autonomic symptoms (CAS) on the same side of the head as the pain (ipsilaterally) such as red eye (conjunctival injection), eyelid swelling (edema), forehead and facial sweating, tearing (lacrimation), abnormally small size of the pupil (miosis), nasal congestion, runny nose (rhinorrhea), and drooping eyelid (ptosis). CH is divided into episodic and chronic. Episodic cluster headache patients usually suffer from 1 to 4 short headaches a day that can individually last between 15-120 minutes when they are having a series of attacks. These attacks (cluster periods) last for weeks or months and are separated by months or years of remission periods where the patients are pain-free. Chronic cluster headache patients suffer without remissions for 1 year or more or with remissions so brief they do not even span a month. Less than 20% of cluster headache patients have the chronic form. It is not yet clear what causes CH, but scientists have discovered a lot of recent evidence that links it to the part of the brain called the hypothalamus. There is no cure, and treatment is determined on an individual basis. However, the two most effective types of acute or symptomatic treatment, high-flow inhaled oxygen and injections of subcutaneous sumatriptan, have been proven to be effective in reducing the pain from CH.

Introduction
Cluster headaches were first completely described by the London neurologist Wilfred Harris in 1926. At that time, it was believed that nearly 90% of CH patients were men, while women instead suffered with migraine headaches. Although it has been confirmed that men are more at risk for developing CH, that ratio has decreased significantly. Researchers theorize that this is because women have long been misdiagnosed with migraines instead. Treatment for migraine headaches differ in several ways from CH, so an accurate diagnosis is important.

Symptoms

In most cases, cluster headaches occur more often at night than during the day, and more often in the spring and fall. Headaches also often occur at the same time every day. The headaches usually last approximately 30 minutes, but can last as long as two hours and uncommonly longer. The pain of a cluster headache is deep, agonizing, usually non-throbbing and affects only one side of the head (unilateral). The area of the head or face involved is always the same from day to day, but occasionally switches to the other side before a cluster ends entirely. The cluster can switch sides with the next onset of attacks. The pain usually occurs in the eye socket area (orbit) and spreads to affect the face, temple and/or forehead. Often the headache is tolerated better by moving, pacing, rocking or even banging their head and usually not by remaining still. They may be irritable and aggressive. When the headache passes, affected individuals may fall into a deep sleep only to be awakened again by another headache. This may continue several times a day, sometimes for weeks or months. Some affected individuals may be unable to continue a normal work schedule. It is important to note that both drinking alcohol and smoking can act as triggers and can bring on attacks in persons who suffer from CH.

Other symptoms include excessive watering of the eye and nose on the same side of the head as the pain occurs. Facial sweating, nasal congestion, drooping eyelids (ptosis), and eyelid swelling (edema) are also common. Rarely before an attack patients will report seeing an aura or having other visual disturbances.

Chronic and episodic CH patients both experience the same type of severe, unilateral pain, which generally occurs 1 to 4 times a day for about 30 minutes. However, chronic CH patients do not go through periods of remission or these periods are shorter than 1 month, while cyclic CH patients may experience episodic attacks for weeks or months but are then relieved by symptom-free periods that last months or years.

Causes

While the underlying cause of CH is not known, the direct cause of the pain is due to the dilation of blood vessels which creates pressure on the trigeminal nerve. Recently, position emission tomography (PET) scans have shown activity in the hypothalamus, the part of the brain responsible for circadian rhythms, or the biological clock, during CH attacks. This reinforces a previous hypothesis that the hypothalamus may be the cluster headache generator.

Although originally not thought to be a genetic disease, researchers now know that genetic factors are involved in approximately 10% of CH cases. It has been suggested that the disorder can be inherited as an autosomal dominant trait that appears to skip generations (incomplete penetrance) or as an autosomal recessive trait.

Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary to cause a particular disease. The abnormal gene can be inherited from either parent or can be the result of a new mutation (gene change) in the affected individual. The risk of passing the abnormal gene from affected parent to offspring is 50% for each pregnancy. The risk is the same for males and females.

Recessive genetic disorders occur when an individual inherits two copies of an abnormal gene for the same trait, one from each parent. If an individual receives one normal gene and one gene for the disease, the person will be a carrier for the disease but usually will not show symptoms. The risk for two carrier parents to both pass the defective gene and have an affected child is 25% with each pregnancy. The risk to have a child who is a carrier like the parents is 50% with each pregnancy. The chance for a child to receive normal genes from both parents and be genetically normal for that particular trait is 25%. The risk is the same for males and females.

All individuals carry 4-5 abnormal genes. Parents who are close relatives (consanguineous) have a higher chance than unrelated parents to both carry the same abnormal gene, which increases the risk to have children with a recessive genetic disorder.

CH has also been associated with tobacco smoking as well as alcohol consumption.

Affected Populations

Cluster headaches affect males more often than females with a ratio that is 2:1. Symptoms usually start to appear between the ages of 20-40 with a mean age of onset of 30.

Related Disorders

Symptoms of the following disorders can be similar to those of cluster headaches. Comparisons may be useful for a differential diagnosis:

Migraine headaches are most commonly mistaken for CH. Migraines usually cause pain on only one side of the head as well, although this side can switch from one attack to the next while it remains constant in CH. Auras are much more likely to be experienced with migraines than CH. Migraines are also much longer in duration than CH, lasting hours up to days, however, they can also occur multiple times a week and then disappear for a period of time, much like CH. One large distinction between migraine headache patients and cluster headache patients is the way they behave during an attack. Migraine patients usually wish to lie down, not move, and remain quiet until the headache has passed. Patients who suffer from CH typically display opposite behaviors such as pacing, restlessness, and aggressive behavior. CH patients are also cognitively alert, unlike migraine patients who may show mental slowing and fatigue.

Tolosa-Hunt syndrome is a rare disorder characterized by severe headaches and pain around the sides and back of the eye, along with weakness and paralysis (ophthalmoplegia) of certain eye muscles. Symptoms usually affect only one side of the head (unilateral). In most cases, affected individuals experience intense sharp pain and paralysis of muscles around the eye. Symptoms subside without intervention and recur without a distinct pattern. In addition, affected individuals may exhibit paralysis (palsy) of certain facial nerves and drooping of the upper eyelid (ptosis). Other symptoms may include double vision, fever, chronic fatigue, headaches, a feeling that one's surroundings are spinning (vertigo), pain in the joints (arthralgia), and/or abnormal protrusion of one or both eyeballs (exophthalmos). The exact cause of Tolosa-Hunt syndrome is not known, but the disorder is thought to be associated with inflammation of the areas behind the eyes (cavernous sinus and superior orbital fissure). (For more information on this disorder, choose "Tolosa-Hunt" as your search term in the Rare Disease Database.)

Trigeminal neuralgia, also known as tic douloureux, is a disorder of the fifth cranial nerve (trigeminal nerve) characterized by attacks of intense, stabbing pain affecting the mouth, cheek, nose, and/or other areas on one side of the face. This pain is usually triggered by chewing, brushing the teeth, touching the lip. If the trigger is repeated and repeated very close together the pain by not respond to the trigger (become refractory for a short period of time). The exact cause of trigeminal neuralgia is not fully understood. (For more information on this disorder, choose "trigeminal neuralgia" as your search term in the Rare Disease Database.)

Giant cell arteritis is s a chronic inflammatory disease characterized by the progressive inflammation of many arteries of the body (panarteritis). Granular material and abnormally large cells (giant cells) accumulate in the elastic lining of the arteries. Chronic inflammation is sometimes confined to the different branches of the heart's main artery (aorta) and any large arteries can become inflamed. However, the temporal arteries of the head are most frequently affected (temporal arteritis). In rare cases, veins may also be affected by giant cell arteritis. The symptoms of giant cell arteritis may include stiffness, muscle pain, fever, and/or headaches. The exact cause of this disease is not fully understood, although it is thought to be an autoimmune disease that occurs when the body's own immune system attacks healthy tissue. (For more information on this disorder, choose "arteritis, giant cell" as your search term in the Rare Disease Database.)

Paroxysmal hemicrania is another rare form of headache where patients experience severe headaches of short duration multiple times per day. The pain can be described as throbbing or clawing. These headaches can last anywhere from 2-45 minutes and can occur 2-40 times per day. However, unlike cluster headaches, paroxysmal hemicrania does not occur in recognizable time patterns and is also much more common in women.

SUNCT (short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing) syndrome is a disorder marked by extremely short bursts of moderate to severe pain on one side of the head typically around the eye area. These attacks can last for as little as a few seconds and as long as a few minutes. Patients will usually experience around 6 attacks per hour during the day, but hardly ever at night.

Standard Therapies

Diagnosis
Currently there are no diagnostic tests to confirm CH.

According to the criteria of the International Classification of Headache Disorders, at least 5 attacks meeting the following are required for diagnosis:

1 - Severe unilateral, orbital (around the eye) and/or temporal (around the temple) pain lasting 15-180 minutes if untreated.

2 - Headache must be accompanied by at least one of the following:

·Red eye or tearing on the side of the headache
·Nasal congestion or runny nose on the side of the headache
·Eyelid swelling on the side of the headache
·Forehead and facial sweating on the side of the headache
·Small pupil or eyelid droop on the side of the headache
·A sense of restlessness or agitation

3 - Attacks have a frequency from one every other to 8 per day

Clinical Testing and Work-Up
Brain imaging with MRI with and without contrast is used at presentation of the very first cycle or when CH patients do not respond well to any of the common treatments, in order to exclude an alternative cause. Imaging studies in CH patients are normal most of the time, but lesions responsible for the headache can be seen leading to diagnosis of a secondary cluster headaches.

Treatment
Treatment for CH varies greatly from patient to patient, depending on what works best to relieve pain for each individual. A common form of acute treatment at the first sign of pain symptoms is pure oxygen inhalation at 7-15 liters per minute by facial mask. Often this is successful at aborting an attack. Another effective, acute treatment is subcutaneous sumatriptan. When injected or inhaled, this drug acts on several different processes involving blood vessels and pain nerves. Both of these treatments narrow or constrict blood vessels, but the exact mechanism by which headache is stopped is not known. . Drugs that are used to end the cluster episode are grouped as transitional and chronic prevention. Occipital nerve block may terminate an episode. Corticosteroids are commonly used transitionally for up to three weeks. Verapamil is the customary chronic preventative, while others include antiepileptic drugs and melatonin. Transitional rugs are slowly tapered off even with continued headaches, while preventatives discontinued about two weeks after the last headache for episodic cluster. They are continued long term in chronic cluster. In cases of chronic CH only, lithium may be used.

Investigational Therapies

In extreme cases, electrical stimulation of the occipital nerve, deep brain stimulation or surgery to destroy certain facial nerves (radiofrequency retrogasserian rhizotomy) may provide relief.

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com.


Contact for additional information about cluster headache:

Frederick R. Taylor, MD FAAN, FAHS
Park Nicollet Headache Center
Adjunct Professor of Neurology
University of Minnesota
Minneapolis, Minnesota 55426
Frederick.Taylor@parknicollet.com

Organizations related to Cluster Headache

References

INTERNET
Sargeant LK, Blanda M. Headache, Cluster. Emedicine. http://emedicine.medscape.com/article/792150-overview. Updated May 20, 2012. Accessed May 25, 2012.

Robert T. Cluster Headache. The American Headache Society. http://www.achenet.org/resources/cluster_headache/. Edited August 14, 2010. Accessed May 25, 2012.

Leroux E, Ducros A. Cluster headache. Orphanet Journal of Rare Diseases.http://www.ojrd.com/content/3/1/20. Published July 23, 2008. Accessed May 25, 2012.

Cluster Headaches. National Headache Foundation. http://www.headaches.org/education/Headache_Topic_Sheets/Cluster_Headaches. Accessed May 25, 2012.

Online Mendelian Inheritance in Man (OMIM). The Johns Hopkins University.
Cluster Headache, Familial. Entry No: 119915. Last Edited October 4, 2007. Available
at: http://www.ncbi.nlm.nih.gov/omim/. Accessed May 25, 2012.

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2012/05/29 00:00:00 GMT+0

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