Cluster headache

Title

Not a rare disease

Categories:

Nervous System Diseases

Summary Summary

Cluster headaches are a form of headache notable for their extreme pain and their pattern of occurring in "clusters", usually at the same time(s) of the day for several weeks. The headaches are accompanied by autonomic symptoms, and some people experience restlessness and agitation.

A cluster headache begins with severe pain strictly on one side of the head, often behind or around one eye. In some people, it may be preceded by a migraine-like "aura." The pain usually peaks over the next 5 to 10 minutes, and then continues at that intensity for up to three hours before going away. Typical attacks may strike up to eight times a day and are relatively short-lived. On average, a cluster period lasts 6 to 12 weeks. Autonomic symptoms may include: conjunctival injection (bloodshot eyes), swelling under or around the eye, excessive tearing of the eyes, drooping of the eyelid, runny nose and/or nasal congestion, and forehead and facial sweating. These symptoms generally occur only during the pain attack and are on the same side as the headache pain.

Cluster headaches usually begin between the ages of 20 and 50, although they can start at any age. Males are more commonly affected than females. Treatment can be divided into acute therapy aimed at stopping symptoms once they have started and preventive therapy aimed at preventing recurrent attacks during the cluster period. [1] [2] [3]

Last updated: 7/14/2015

Symptoms Symptoms

People with cluster headaches describe the pain as piercing and unbearable. The headaches occur in "clusters" usually at the same time of the day and night for several weeks. The symptoms are usually experienced on one side of the head, often behind or around the eye. The nose and the eye on the affected side of the head may also get red, swollen, and runny. Some people will experience nausea; restlessness; changes in blood pressure and heart rate; and agitation, or sensitivities to light, sound, or smell. Most affected individuals have one to three cluster headaches a day and two cluster periods a year, separated by periods of freedom from symptoms.

A small group of people develop a chronic form of the disorder, characterized by bouts of cluster headaches that can go on for years with only brief periods (2 weeks or less) of remission.^[1]^[2]

Last updated: 4/1/2016

The Human Phenotype Ontology (HPO) provides the following list of features that have been reported in people with this condition. Much of the information in the HPO comes from Orphanet, a European rare disease database. If available, the list includes a rough estimate of how common a feature is (its frequency). Frequencies are based on a specific study and may not be representative of all studies. You can use the MedlinePlus Medical Dictionary for definitions of the terms below.

Signs and Symptoms	Approximate number of patients (when available)
Agitation	-
Autosomal dominant inheritance	-
Edema	-
Hyperhidrosis	-
Miosis	-
Ptosis	-

Last updated: 9/1/2016

Cause Cause

Scientists aren't sure what causes cluster headaches, although there are currently several theories. The tendency of cluster headaches to occur during the same time(s) from day to day, and more often at night than during the daylight hours, suggests they could be caused by irregularities in the body’s circadian rhythms, which are controlled by the brain and a family of hormones that regulate the sleep-wake cycle. The development of cluster headaches may additionally be related to the body's release of histamine (chemical released in the body during an allergic response) or serotonin (chemical made by nerve cells). It is also possible that a problem in a part of the brain called the hypothalamus may be involved. ^[1]^[2]

Alcohol (especially red wine) provokes attacks in more than half of those with cluster headaches, but has no effect once the cluster period ends. Cluster headaches are also strongly associated with cigarette smoking.^[2] Glare, stress, or certain foods may also trigger an attack.^[1]

An increased familial risk of these headaches suggests that there may be a genetic cause, though more studies are needed to confirm this suspicion and identify specific genetic changes associated.^[2]

Last updated: 4/1/2016

Treatment Treatment

Treatment does not cure cluster headaches. The goal of treatment is to relieve symptoms. Spontaneous remission may occur, or treatment may be required to prevent headaches.^[1]

There are medications available to lessen the pain of a cluster headache and suppress future attacks. Oxygen inhalation and triptan drugs (such as those used to treat migraine) administered as a tablet, nasal spray, or injection can provide quick relief from acute cluster headache pain. Lidocaine nasal spray, which numbs the nose and nostrils, may also be effective. Ergotamine and corticosteroids such as prednisone and dexamethasone may be prescribed to break the cluster cycle and then tapered off once headaches end. Verapamil may be used preventively to decrease the frequency and pain level of attacks. Lithium, valproic acid, and topiramate are sometimes also used preventively.^[2]

More detailed information on medications can be found in the treatment and management sections of Medscape Reference's article on cluster headache.

Last updated: 11/1/2013

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Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.
ClinicalTrials.gov lists trials that are studying or have studied Cluster headache. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Organizations Organizations

Nonprofit support and advocacy groups bring together patients, families, medical professionals, and researchers. These groups often raise awareness, provide support, and develop patient-centered information. Many are the driving force behind research for better treatments and possible cures. They can direct people to research, resources, and services. Many groups also have experts who serve as medical advisors. Visit their website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

American Headache Society Committee for Headache Education (ACHE)
19 Mantua Road
Mt. Royal, NJ 08061
Telephone: 856-423-0043
Fax: 856-423-0082
E-mail: achehq@talley.com
Website: http://www.achenet.org
American Migraine Foundation (AMF)
19 Mantua Rd.
Mount Royal, NJ 08061 United States
Telephone: 856-423-0043
Fax: 856-423-0082
E-mail: amf@talley.com
Website: https://americanmigrainefoundation.org/
M.A.G.N.U.M. (The National Migraine Association)
100 N Union Street
Suite B
Alexandria, VA 22314
Website: http://www.migraines.org/
Migraine Research Foundation
300 East 75th Street
Suite 3K
New York, NY 10021
Telephone: 212-249-5402
Fax: 212-249-5405
E-mail: contactmrf@migraineresearchfoundation.org
Website: http://www.migraineresearchfoundation.org
National Headache Foundation
820 North Orleans
Chicago, IL 60610-3132
Toll-free: 888-643-5552
E-mail: info@headaches.org
Website: http://www.headaches.org
Organization for Understanding Cluster Headaches (O.U.C.H.)
3225 Winding Way
Round Rock, TX 78664
Telephone: 214-783-1899
E-mail: contact_ouch@ouch-us.org
Website: http://www.ouch-us.org/index.shtml

Do you know of an organization? Send us your suggestions.

Living With Living With

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Genetics Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by GeneTests, the American College of Medical Genetics, and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
PubMed is a searchable database of medical literature and lists journal articles that discuss Cluster headache. Click on the link to view a sample search on this topic.

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