Cluster headache
310429
225420814
2008-07-13T16:28:27Z
Chris 73
26995
Reverted edits by [[Special:Contributions/75.38.232.210|75.38.232.210]] ([[User talk:75.38.232.210|talk]]) to last version by Rsagall
{{DiseaseDisorder infobox |
Image = |
Caption = |
DiseasesDB = 2850 |
Name = Cluster headache |
ICD10 = {{ICD10|G|44|0|g|40}} |
ICD9 = {{ICD9|346.2}} |
ICDO = |
OMIM = |
MedlinePlus = |
eMedicineSubj = EMERG |
eMedicineTopic = 229 |
eMedicine_mult = {{eMedicine2|NEURO|67}} |
MeshID = D003027 |
}}
'''Cluster headache''' is a neurological disease that involves, as its most prominent feature, an immense degree of pain. "Cluster" refers to the tendency of these [[headaches]] to occur periodically, with active periods interrupted by spontaneous remissions. The cause of the disease is currently unknown.
==Signs and symptoms==
Cluster headaches are extremely painful, unilateral headaches of a piercing quality. The duration of the common attack is 15 minutes to three hours. Onset of an attack is rapid, and most often without the preliminary signs that are characteristic of a [[migraine]]. However, some sufferers report preliminary sensations of diverse description, often referred to as "shadows," that may warn them an attack is imminent. Though the headaches are almost exclusively unilateral, there are many documented cases of "side-shifting" between cluster periods, or, even rarer, simultaneously (within the same cluster period) bilateral headache. They are often initially mistaken for brain tumors and [[multiple sclerosis]] often until patients are treated with corticosteroids and then imaged. [[Trigeminal neuralgia]] can also bring on headaches with similar qualities.
===Pain===
The degree of pain involved in cluster headaches is markedly greater than in other headache conditions, including migraine. It has been described by female patients as being more severe than childbirth. The pain is lancinating or boring in quality, and is located behind the eye or in the temple, sometimes radiating to the neck or shoulder. Analogies frequently used to describe the pain are a red-hot poker inserted into the eye, or a spike penetrating from the top of the head, behind one eye, radiating down to the neck, or sometimes having a leg amputated without any anaesthetic. The condition was originally named Hortons Neuralgia after Dr. B.T Horton who postulated the first theory as to their pathogenesis. His original paper describes the severity of the headaches as being able to take normal men and force them to suicide. Indeed, cluster headaches are also known by the nickname "suicide headaches".
From Horton's 1939 original paper on cluster headache:
{{quote|Our patients were disabled by the disorder and suffered from bouts of pain from two to twenty times a week. They had found no relief from the usual methods of treatment. Their pain was so severe that several of them had to be constantly watched for fear of suicide. Most of them were willing to submit to any operation which might bring relief}}
===Other symptoms===
The cardinal symptoms of the cluster headache attack are ptosis (drooping eyelid), conjunctival injection (redness of the conjuctiva - the covering of the eyeball), lacrimation (tearing), rhinorrhea (runny nose), and, less commonly, facial blushing, swelling, or sweating. These features are known as the autonomic symptoms. The attack is also associated with restlessness, the sufferer often pacing the room or rocking back and forth. Less frequently, he or she will have an aversion to bright lights and loud noise during the attack. Nausea is not typical of cluster headache, though it has been reported. The neck is often stiff or tender in the aftermath of a headache, with jaw or tooth pain sometimes present.
===Cyclical recurrence and regular timing===
Cluster headaches are occasionally referred to as "alarm clock headaches", because of the regularity of its timing and its ability to wake a person from sleep. Thus it has been known to strike at the same time each night or morning, often at precisely the same time during the day a week later. This has prompted researchers to speculate an involvement of the brain's "biological clock" or [[circadian rhythm]].
In some cases, cluster headaches remain "steady" without cyclical ups and downs for days.
====Episodic or chronic====
In episodic cluster headache, these attacks occur once or more daily, often at the same times each day, for a period of several weeks, followed by a headache-free period lasting weeks, months, or years. Approximately 10–15% of cluster headache sufferers are [[chronic (medicine)|chronic]]; they can experience multiple headaches every day for years.
Cluster headaches occurring in two or more cluster periods lasting from 7 to 365 days with a pain-free remission of one month or longer between the clusters are considered episodic. If the attacks occur for more than a year without a pain-free remission of at least one month, the condition is considered chronic.<ref>[http://ihs-classification.org/en/02_klassifikation/02_teil1/03.01.02_cluster.html IHS Classification ICHD-II 3.1.2 Chronic cluster headache]</ref>
Chronic clusters run continuously without any "remission" periods
between cycles. The condition may change from chronic to episodic and from episodic to chronic. Remission periods lasting for decades before the resumption of clusters have been known to occur.
==Other names==
Cluster headaches have been called by several other names in the past including Erythroprosopalgia of Bing, Ciliary neuralgia, Migrainous neuralgia, Erythromelagia of the head, Horton's headache (named after Bayard T. Horton, an American neurologist who was the first to accurately describe the headache in 1939<ref>[http://www.mayoclinicproceedings.com/inside.asp?AID=3032&UID= Mayo Clinic Proceedings<!-- Bot generated title -->]</ref>), Histaminic cephalalgia, Petrosal neuralgia, sphenopalatine neuralgia, Vidian neuralgia, Sluder's neuralgia, and Hemicrania angioparalyticia.<ref>Stephen D. Silberstein, Richard B. Lipton. Peter J. Goadsgy. "Headache in Clinical Practice." Second edition. Taylor & Francis. 2002. </ref>
Sluder's neuralgia (syndrome) and cluster pain can often be temporarily stopped with nasal lidocaine spray. If successful, outpatient nasal [[septoplasty]] and splinting can resolve the condition.<ref>[http://ihs-classification.org/en/02_klassifikation/05_anhang/11.05.01_anhang.html IHS Classification ICHD-II A11.5.1 Mucosal contact point headache]</ref>
<div align="center">
<gallery heights="175" widths="175">
image:Robert Bing.jpg|Robert Bing (1878–1956)
image:BT_Horton.jpg|Bayard T. Horton (1895–1980)
</gallery>
</div>
==Prevalence==
While [[migraines]] are diagnosed more often in women, cluster headaches are diagnosed more often in men. The male-to-female ratio in cluster headache ranges from 4:1 to 7:1. It primarily occurs between the ages of 20 to 50 years. [http://www.diamondheadache.com/article_archives/cluster_headache.html]This gap between the sexes has narrowed over the past few decades, and it is not clear whether cluster headaches are becoming more frequent in women, or whether they are merely being better diagnosed. Limited epidemiological studies have suggested prevalence rates of between 56 and 326 people per 100,000.<ref>{{cite journal | author = Torelli P, Castellini P, Cucurachi L, Devetak M, Lambru G, Manzoni G | title = Cluster headache prevalence: methodological considerations. A review of the literature | journal = Acta Biomed Ateneo Parmense | volume = 77 | issue = 1 | pages = 4–9 | year = 2006 | pmid = 16856701}}</ref>
[[Latitude]] plays a role in the occurrence of cluster headaches, which are more common as one moves away from the [[equator]] towards the poles.
==Pathophysiology==
{{imageStackRight|270|[[Image:Gray778.png|thumb|right||[[Gray's Anatomy|Gray's]] FIG. 778– Trigeminal nerve is shown in yellow.]][[Image:Gray777.png|thumb|right|[[Gray's Anatomy|Gray's]] FIG. 777– Detailed view of ophthalmic nerve, shown in yellow.]]}}
Cluster headaches are classified as [[vascular headache]]s. The intense pain is caused by the [[dilation]] of blood vessels which creates pressure on the [[trigeminal nerve]]. While this process is the immediate cause of the pain, the [[etiology]] (underlying cause or causes) is not fully understood.
===Hypothalamus===
Among the most widely accepted theories is that cluster headaches are due to an abnormality in the [[hypothalamus]]; Dr Goadsby, an Australian specialist in the disease has developed this theory. This can explain why cluster headaches frequently strike around the same time each day, and during a particular season, since one of the functions the hypothalamus performs is regulation of the [[Circadian rhythm|biological clock]]. [[Metabolic]] abnormalities have also been reported in patients.
The hypothalamus is responsive to light—[[Circadian rhythm|daylength and photoperiod]]; [[olfactory]] stimuli, including [[pheromone]]s; [[steroid]]s, including [[sex steroids]] and [[corticosteroid]]s; neurally transmitted information arising in particular from the [[heart]], the [[stomach]], and the [[reproductive system]]; [[autonomic]] inputs; [[blood]]-borne stimuli, including [[leptin]], [[ghrelin]], [[angiotensin]], [[insulin]], [[pituitary]] [[hormone]]s, [[cytokine]]s, [[blood plasma]] concentrations of [[glucose]] and [[Osmole (unit)|osmolarity]], ''etc''.; and [[Stress (medicine)|stress]]. These particular sensitivities may underlay the causes, triggers, and methods of treatment of cluster headache.
<gallery heights="150" widths="150" perrow="3" caption="Neuroimaging">
image:PET1.jpg
image:PET2.jpg
image:PET3.jpg
image:VBM1.jpg
image:VBM2.jpg
image:VBM3.jpg
</gallery>
''top row:'' '''Functional Magnetic Resonance Imaging (fMRI) shows brain areas being activated during pain'''<!-- I'm sure there's a way to incorporate these descriptions into the gallery -->
''bottom row:'' '''Voxel-based morphometry (VBM) shows brain area structural differences'''<!-- I'm sure there's a way to incorporate these descriptions into the gallery -->
The above [[Positron emission tomography]] pictures indicate the brain areas which are activated during pain only, compared to the pain free periods. These pictures show brain areas which are always active during pain in yellow/orange colour (called "pain matrix"). The area in the centre (in all three views) is specifically activated during cluster headache only. The bottom row [[Voxel-based morphometry]] (VBM) pictures show structural brain differences between cluster headache patients and people without headaches; only a portion of the [[hypothalamus]] is different.<ref>May et al.: PET and MRA findings in cluster headache and MRA in experimental pain ''Neurology'' 2000;'''55''':1328-1335, PMID 11087776.</ref><ref> Dasilva AF, Goadsby PJ, Borsook D: Cluster headache: a review of neuroimaging findings. ''Curr Pain Headache Rep.'' 2007;'''11(2)''':131-6. PMID 17367592.</ref>
===Genetics===
There is a [[genetics|genetic]] component to cluster headaches, although no single gene has been identified as the cause. First-degree relatives of sufferers are more likely to have the condition than the population at large.<ref>{{cite journal | author = Pinessi L, Rainero I, Rivoiro C, Rubino E, Gallone S | title = Genetics of cluster headache: an update | journal = J Headache Pain | volume = 6 | issue = 4 | pages = 234–6 | year = 2005 | month = September 2005 | pmid = 16362673 | doi = 10.1007/s10194-005-0194-x}} </ref>
However, genetics appears to play a much smaller role in cluster headache than in some other types of headaches.
===Triggers===
[[Nitroglycerin]] (glyceryl trinitrate) can sometimes induce cluster headaches in sufferers in a manner similar to spontaneous attacks. Ingestion of [[Ethanol|alcohol]] or chocolate is recognized as a common trigger of cluster headaches when a person is in cycle or susceptible. Exposure to [[hydrocarbon]]s (petroleum solvents, perfume) is also recognized as a trigger for cluster headaches. Some patients have a decreased tolerance to heat, and becoming overheated may act as a trigger. Napping causes a headache for some sufferers, while for others lack of sleep triggers them. The role of diet and specific foods in triggering cluster headaches is controversial and not well understood.
===Smoking===
Nicotine may trigger cluster headaches, and the affliction is often found in people with a heavy addiction to cigarette smoking. Some sufferers report that quitting smoking has brought about an end to their cluster headaches.
==Treatment==
{{Refimprovesect|date=March 2008}}
Cluster headaches often go undiagnosed for many years, being confused with migraine or other causes of headache.<ref>{{cite news | author= | title=Vast Majority of Cluster Headache Patients Are Initially Misdiagnosed, Dutch Researchers Report | url=http://www.w-h-a.org/wha2/Newsite/resultsnav.asp?color=C2D9F2&idContentNews=595 | date=2003-08-21 | publisher=World Headache Alliance | accessdate=2006-10-08}}</ref>
Medically, cluster headaches are considered [[benign]], but because of the extreme and often debilitating [[pain]] associated with them, a severe attack is nevertheless treated as a [[medical emergency]] by doctors who are familiar with the condition. Because of the relative rareness of the condition and ambiguity of the symptoms, some sufferers may not receive treatment in the [[emergency room]] and patients may even be mistaken as exhibiting [[drug-seeking behavior]].
[[Over-the-counter substance|Over-the-counter]] pain medications (such as [[aspirin]], [[paracetamol]], and [[ibuprofen]]) typically have no effect on the pain from a cluster headache. Unlike other headaches such as migraines and tension headaches, cluster headaches do not respond to [[biofeedback]].
Some have reported partial relief from [[narcotic]] pain killers. Anecdotal evidence indicates that cluster headaches can be so excruciating that even [[morphine]] does little to ease the pain. However, some newer medications like [[fentanyl]] (and Percocet) have shown promise in early studies and use. {{Fact|date=April 2008}}
Medications to treat cluster headaches are classified as either [[Abortive medication|abortives]] or [[Prophylaxis|prophylactics]] (preventatives). In addition, short-term transitional medications (such as steroids) may be used while prophylactic treatment is instituted and adjusted. With abortive treatments often only decreasing the duration of the headache and preventing it from reaching its peak rather than eliminating it entirely, preventive treatment is always indicated for cluster headaches, to be started at the first sign of a new cluster cycle.
In many cases, some doctors have tried the use of [[beta blockers]] as a treatment.
Some non-narcotic treatments that have shown mixed levels of success are botox injections along the occipital nerve,{{Fact|date=March 2008}} as well as sarapin (pitcher plant extract) injections.{{Fact|date=March 2008}} Some people have claimed relief from kudzu,{{Fact|date=March 2008}} but the evidence is purely anecdotal at this point.
Recently, researchers have linked low testosterone as a possible cause of cluster headaches,{{Fact|date=March 2008}} which can be especially troublesome since the most effective pain medications for clusters, like fentanyl, reduce testosterone levels.{{Fact|date=March 2008}}
===Abortive treatment===
During the onset of a cluster headache, some patients respond to rapid inhalation of pure [[Oxygen therapy|oxygen]] (12-15 litres per minute in a non-rebreathing apparatus).<ref name="EFNS">{{cite journal | author = May A, Leone M, Afra J, Linde M, Sándor P, Evers S, Goadsby P | title = EFNS guidelines on the treatment of cluster headache and other trigeminal-autonomic cephalalgias | journal = Eur J Neurol | volume = 13 | issue = 10 | pages = 1066–77 | year = 2006 | month = Oct | pmid = 16987158 | doi=10.1111/j.1468-1331.2006.01566.x}} [http://www.efns.org/files/guideline_49.pdf Free Full Text (PDF)]</ref>
When used at the onset this can abort the attack in as little as 5 minutes. Once an attack is at its peak, oxygen therapy appears to have little effect. Alternative first-line treatment is subcutaneous administration of [[triptan]] drugs, like [[sumatriptan]] and [[zolmitriptan]].<ref name="EFNS"/> Because of the rapid onset of an attack, the triptan drugs are usually taken by [[subcutaneous injection]] rather than by mouth. While available as a nasal spray, these are seldom effective to sufferers of cluster headaches due to the swelling of the nasal passages during an attack.
[[Lidocaine]] and other topical anesthetics sprayed into the nasal cavity may relieve or stop the pain,<ref>{{cite journal | author = Mills T, Scoggin J | title = Intranasal lidocaine for migraine and cluster headaches | journal = Ann Pharmacother | volume = 31 | issue = 7-8 | pages = 914–5 | year = 1997 | month = Jul-Aug| pmid = 9220056}}</ref> normally in a few minutes, but long term use is not suggested due to the side effects and possible damage to the nasal cavities.
Previously, vaso-constrictors such as [[ergot]] compounds were also used, and sufferers report a similar relief by taking strong cups of coffee immediately at the onset of an attack.
Sometimes, lying in a dark room will help a person if the pain is a side effect of [[Horner's Syndrome]], but is not recommended. If no Horner's syndrome is present, lying down is not recommended. The cluster sufferer is best off sitting upright, lying down will intensify and exacerbate the pain. A cluster sufferer sitting upright, though still in excruciating pain, is better able to endure, and the pain level does not increase as rapidly or exponentially as when lying down.
Other abortive remedies that work for some include ice, hot showers, breathing cold air, caffeine, and drinking large amounts of water in the early stages of an attack. Vigorous exercise has been shown in some cases to be very effective in relieving and aborting an acute attack by increasing the levels of oxygen within the body. This could also be due to an increase in adrenaline and changes in blood pressure. Some people report that sexual intercourse and specifically orgasm may terminate an attack possibly by acutely modulating hypothalamic function.<!--
--><ref>{{cite journal | author = Ekbom K, Lindahl J | title = Effect of induced rise of blood pressure on pain in cluster headache | journal = Acta Neurol Scand | volume = 46 | issue = 4 | pages = 585–600 | year = 1970 | pmid = 4994083}}</ref><!--
--><ref>{{cite journal | author = Atkinson R | title = Physical fitness and headache | journal = Headache | volume = 17 | issue = 5 | pages = 189–91 | year = 1977 | month = Nov | pmid = 924787 | doi = 10.1111/j.1526-4610.1977.hed1705189.x}}</ref><ref>{{cite journal | author = Gotkine M, Steiner I, Biran I. | title = Now dear, I have a headache! Immediate improvement of cluster headaches after sexual activity | journal = J Neurol Neurosurg Psychiatry. | volume = 77 | issue = 11 | pages = 1296 | year = 2006 | month = Nov | pmid = 17043304 | doi = 10.1136/jnnp.2006.092643}}-
[http://jnnp.bmj.com/cgi/content/extract/77/11/1296 Abstract]</ref>
===Prophylactic treatment===
A wide variety of prophylactic medicines are in use, and patient response to these is highly variable. Current European guidelines suggest the use of the [[calcium channel blockers|calcium channel blocker]] [[verapamil]] at a dose of at least 240 mg daily. [[Steroid]]s, such as [[prednisolone]], are also effective, with a high dose given for the first five days before tapering down. [[Methysergide]], [[lithium]] and the [[anticonvulsant]] [[topiramate]] are recommended as alternative treatments.<ref name="EFNS"/>
[[Muscle relaxant]]s and atypical [[Antipsychotic|anti-psychotics]] have also been used.
[[Magnesium]] supplements have been shown to be of some benefit in about 40% of patients. [[Melatonin]] has also been reported to help some.
[[Other]] neuropathic pain alleviating agents can also be used such as the [[Tricyclic antidepressants]] including [[Amitriptyline]] and [[Nortriptyline]]
===Non-established and research approaches===
There is substantial anecdotal evidence that [[serotonergic psychedelics]] such as [[psilocybin]] (mushrooms) and [[LSD]] and [[ergine|LSA]] [[Lysergic acid|d-Lysergic acid amide]] ([[Rivea corymbosa]] seeds) abort cluster periods and extend remission periods.<!--
--><ref>{{cite web | title=Hallucinogenic Treatment of Neuro-Vascular Headaches | url=http://www.clusterbusters.com | publisher=ClusterBusters | accessdate=2006-09-22}}</ref><!--
--><ref>{{cite news | author=Mark Honigsbaum | title=Headache sufferers flout new drug law - Calls for clinical trials and rethink of legislation as patients claim that magic mushrooms can relieve excruciating condition | url=http://www.maps.org/sys/nq.pl?id=571&fmt=page | date=2005-08-02 | publisher=The Guardian | accessdate=2006-09-22}} [reprint by Multidisciplinary Association for Psychedelic Studies]</ref> [[Melatonin]], [[psilocybin]], [[serotonin]], and the triptan abortive drugs are closely-related [[tryptamines]].
Dr. Andrew Sewell and [[Dr. John Halpern]] at McLean Hospital in Boston have investigated the ability of low doses of psilocybin ("magic mushrooms") to treat cluster headaches. Dr. Sewell examined medical records of 53 patients who had taken hallucinogenic mushrooms and reported in Neurology that the majority of them found partial or complete relief from cluster attacks.<!--
--><ref>{{cite journal | author = Sewell R, Halpern J, Pope H | title = Response of cluster headache to psilocybin and LSD | journal = Neurology| volume = 66 | issue = 12 | pages = 1920–2 | year = 2006 | month = Jun | pmid = 16801660 | doi = 10.1212/01.wnl.0000219761.05466.43}} - a Brief Communications, also presented as:<br>Sewell, R. Andrew, M.D.; Halpern, John M., M.D. "The Effects Of Psilocybin And LSD On Cluster Headache: A Series Of 53 Cases." Abstract. Presented to the National Headache Foundation’s Annual Headache Research Summit. February, 2006.</ref> A [[clinical study]] of these treatments under the auspices of [[Multidisciplinary Association for Psychedelic Studies|MAPS]] is being developed by researchers at [[Harvard Medical School]], [[McLean Hospital]].<!--
--><ref>{{cite web | author= | title= Research into psilocybin and LSD as potential treatments for people with cluster headaches | work=LSD and Psilocybin Research | url=http://www.maps.org/research/cluster/psilo-lsd/index.html | date= | publisher=Multidisciplinary Association for Psychedelic Studies | accessdate=2006-09-22}}</ref><!--
--><ref>"Response of Cluster Headache to Psilocybin and LSD" Chapter 5 in "Psychedelic Medicine: New Evidence for Hallucinogens as Treatment" Michael Winkelman and Thomas B. Roberts (editors) (2007) Westport, CT: Praeger/Greenwood.</ref>
Within the United States, the Controlled Substances Act (CSA) of 1970 makes it illegal to possess hallucinogens (including psilocybin and LSD), classifying them as Schedule I drugs with no legitimate medical use. Patients who use psilocybin to treat their symptoms face legal prosecution, although there are no known convictions.
==Other types of headache==
*[[Chronic Paroxysmal Hemicrania]] (CPH) is a condition similar to cluster headache, but CPH responds well to treatment with the [[anti-inflammatory]] drug [[indomethacin]] and the attacks are very much shorter, often lasting only seconds.<ref>{{eMedicine|NEURO|67}}</ref>
*Some people with extreme headaches of this nature (especially if they are not unilateral) may actually have an [[ictal headache]]. [[Anti-convulsant]] medications can significantly improve this condition so sufferers should consult a physician about this possibility.<ref>{{cite web | author= | title=Seizures and Headaches: They Don't Have to Go Together | url=http://www.epilepsy.com/articles/ar_1063754930.html | date=2003-09-16 | publisher=Epilepsy.com | accessdate=2006-09-22}}</ref>
*It is also possible to have two or more different types of headaches, complicating diagnosis and treatment.
== See also ==
* [[Headache]]
* [[Tension headaches]]
* [[Migraine]]
* [[Rebound headaches]]
* [[Hemicrania continua]]
* [[Trigeminal neuralgia]]
* [[Chronic Paroxysmal Hemicrania]]
* [[Organization for Understanding Cluster Headaches]]
==References==
{{Reflist|2}}
== External links ==
<!--Please do not insert commercial links here or any external links selling anything. Your link will be deleted and your account will be blocked if you repeat. -->
* [http://www.guideline.gov/algorithm/4002/NGC-4002_5.gif Algorithm for diagnosis and treatment] from [[National Guideline Clearinghouse]] ([[DHHS]])
* [http://ihs-classification.org/en/02_klassifikation/02_teil1/03.01.00_cluster.html The International Headache Society (IHS): 2nd Edition of The International Headache Classification (ICHD-2) - 3.1 Cluster Headache]
<!--Please do not insert commercial links here or any external links selling anything. Your link will be deleted and your account will be blocked if you repeat. -->
{{Diseases of the nervous system}}
[[Category:Ailments of unknown etiology]]
[[Category:Headaches]]
[[Category:Neurological disorders]]
[[Category:Vascular disorders]]
[[de:Cluster-Kopfschmerz]]
[[es:Cefalea en racimos]]
[[fr:Algie vasculaire de la face]]
[[it:Cefalea a grappolo]]
[[hu:Cluster-fejfájás]]
[[nl:Clusterhoofdpijn]]
[[no:Clusterhodepine]]
[[pl:Klasterowy ból głowy]]
[[pt:Cefaléia em salvas]]
[[sv:Hortons huvudvärk]]