Rare Pulmonology News

Disease Profile

Cluster headache

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

#N/A

ICD-10

#N/A

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Categories

Nervous System Diseases

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.[1][2][3]

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.[1][2][3]

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]

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]

 

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
Percent of people who have these symptoms is not available through HPO
Agitation
0000713
Autosomal dominant inheritance
0000006
Cluster headache
0012199
Edema
Fluid retention
Water retention

[ more ]

0000969
Hyperhidrosis
Excessive sweating
Increased sweating
Profuse sweating
Sweating
Sweating profusely
Sweating, increased

[ more ]

0000975
Miosis
Constricted pupils
Pupillary constriction

[ more ]

0000616
Pain
0012531
Ptosis
Drooping upper eyelid
0000508
Rhinorrhea
Runny Nose
0031417

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]

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.

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s 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

    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.

        References

        1. Campellone JV. Cluster headache. MedlinePlus. 11/05/2014; https://www.nlm.nih.gov/medlineplus/ency/article/000786.htm.
        2. Headache: Hope Through Research. National Institute of Neurological Disorders and Stroke (NINDS). 11/03/2015; https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Hope-Through-Research/Headache-Hope-Through-Research.
        3. May A. Cluster headache: Epidemiology, clinical features, and diagnosis. UpToDate. 05/22/2014; https://www.uptodate.com/contents/cluster-headache-epidemiology-clinical-features-and-diagnosis.

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