Alternative names: Suicide Headaches.
Cluster headache (CH), also known as histamine headache, is a specific vascular headache syndrome and one of the most severe forms of headache. The underlying cause is still poorly understood but it is thought that the pain may be related to the dilation/alteration of blood vessels or to inflammation of nerves behind the eye.
Cluster headaches affect approximately 8-10% of all persons who suffer from headaches, or about 1-in-1000 people. They most commonly occur in men (six times more frequently than in women) between the ages of 20 and 40. Most sufferers are smokers.
Cluster headaches are associated with dilation (widening) of blood vessels and inflammation of nerves behind the eye. In both cluster and migraine headaches blood vessels dilate, but in cluster headaches only the blood vessels behind the eyes pulsate. This leads some experts to believe that part of the pain is caused by dilation in branches of the carotid artery (a major artery that supplies the brain with blood). Some research suggests that a sensitivity to histamine, a chemical found in all body tissue, may play some role. Histamine opens blood vessels and can cause swollen membranes. Because such dilation appears to follow, not precede, pain, however, some researchers believe that some other process involving the central nervous system in the brain is more likely to be the primary cause.
Abnormalities in the Hypothalamus
The root cause of cluster headaches is unknown but researchers are zeroing in on areas in the brain where abnormalities occur. Evidence suggests that abnormalities in the hypothalamus of the brain may play a role. The hypothalamus is a complex brain structure located deep in the brain. It controls the pituitary gland (which is responsible for reproductive function) and also regulates body temperature, emotion, hunger, sleep, and the body's biologic rhythms (its circadian rhythms). In studying cluster headaches, researchers are particularly interested in the circadian rhythms because of the periodic nature of cluster attacks. Within the hypothalamus are small clusters of nerves that act like biologic clocks, the most important being one called the suprachiasmatic nuclei (SCN). It appears to help coordinate the body's activities (sleep/wake) with the environment (dark/light). Some studies support the idea that some impairment in this biologic pacemaker may cause these terrible attacks. (Cluster attacks often follow the seasonal increase in warmth and light, beginning in summer and ending in the fall.)
The hypothalamus is also involved in the regulation of many important hormones, including serotonin, norepinephrine, and cortisol (stress hormones), melatonin (related to sleep), and beta endorphins (involved with pain). As with other headaches, particularly migraines, alterations in serotonin are of particular interest. This neurotransmitting hormone (chemical messenger in the brain) affects, among other functions, well-being, sleep, and appetite. In cluster patients, there is some evidence of abnormal serotonin levels (although not as pronounced as in migraines). Some research suggested that serotonin may play an important role in the way circadian rhythms are expressed.
There are no abnormalities to be found upon a physical or laboratory investigation other than Horner's syndrome occasionally. In approximately 70% of patients with cluster headaches, the carotid artery is palpably tender at several points in the neck (Raskin and Prusiner, 1977).
Cluster headaches are observed more frequently among those who smoke and consume alcohol.
Each headache episode generally lasts from fifteen minutes to three hours. Many such episodes may occur during a day. They tend to occur in cycles, often during the months of the year that are warmer and have more daylight. Persons who suffer from CH tend to be sociable, active and responsible and, for this reason, CH are sometimes called "the executive headache".
Only the CH victim can understand the excruciating pain and discomfort that characterize this disorder. Fortunately, only a small percentage of CH cases complain of the chronic form. Chronic CH is distinguished by its lack of a remission period lasting more than 14 days, or the absence of a remission period for more than one year.
The telltale signs of a cluster headache are distinct yet remarkably similar among CH sufferers everywhere. Here is a list of commonly reported symptoms associated with this condition:
Many sufferers report that they have been to numerous doctors, neurologists and other specialists over a period of years, prior to being accurately diagnosed. In addition to their pain, they have incurred great frustration in seeking answers to their little-known condition, as well as untold expense, and have been subjected to a number of inappropriate treatments.
Treatment of CH generally aims at prevention of the attacks. Since CH generally appears over several days at around the same time each day, it is possible to prevent these headaches by taking timely remedies.
Ergotamine. Dihydroergotamine (DHE) injections have stopped cluster attacks within five minutes in many patients. Ergotamine aerosols or ergotamine suppositories with caffeine may be useful. When using the aerosol the patient usually inhales two or three times. They should be sure to shake the canister vigorously and administer the spray while making an inhalation immediately after a forced exhalation. The patient should then hold the breath for several seconds before slowly exhaling. Proper administration can produce an effective response 80% of the time. (Oral and under-the-tongue preparations of ergotamine are ineffective because of the brevity of cluster attacks.)
Triptans. Triptans are migraine agents that are proving to have a role in stopping a cluster attack. Injections of sumatriptan have been used the longest. In one 1998 analysis of 2,031 attacks in 52 patients, it was successful in 88% attacks, and 42% of patients were pain-free within 15 minutes in over 90% of their attacks. (The nasal spray form is not very effective.) Side effects are common but are usually only considered to be unpleasant; they include nausea, odd sensations (e.g., tingling and numbness), dizziness, fatigue, sensations in the jaw and chest, and a fall in blood pressure. Other triptans, including rizatriptan (Maxalt) and zolmitriptan (Zomig), may prove to be good alternatives. In one study, zolmitriptan brought significant relief within 30 minutes for many episodic (but not chronic) cluster patients.
Lidocaine, a local anesthetic, may be useful in nasal-spray or nasal-drop form for cluster attacks. Some reports suggest that it is helpful for about 60% of patients.
Methoxyflurane is an anesthetic. Inhaling about 10 to 15 drops applied to a cloth may help abort an attack.
Methysergide, which is also used to prevent migraine, is believed to prevent cluster headaches by constricting blood vessels and reducing inflammation. It has serious side effects, however, and can be used only for brief periods, generally for the length of a cluster period.
Corticosteroids Corticosteroids, particularly prednisone and methylprednisolone, are used for short-term cluster therapy, due to potential serious side effects with longer-term use.
Calcium Channel Blockers Calcium channel blockers, typically used in treating cardiovascular conditions, are used to treat cluster headache episodes and chronic cluster headaches. They may work by blocking the release of neurotransmitters (chemicals in the brain that stimulate nerve cells) involved in causing pain.
Many sufferers report that alcohol is an important trigger during a cluster period, but not during remission periods.
Those suffering from cluster headaches often have pregnenolone levels that are below normal.
Low levels of melatonin have been linked to cluster headaches, and melatonin supplements reduce cluster headaches in some patients.
Capsaicin cream has a significant success rate reported from one study where three applications (in a liquid form) per day were placed in the nose on the affected side. A significant downside must be that cayenne pepper in the nose has to hurt!
When painkillers or caffeine are taken daily, the drugs may lose effectiveness over time and headaches can become more frequent. When stopping the medication, a rebound headache can occur. Rebound headaches are frequently caused by dietary caffeine.
Both migraine and cluster headaches are associated with heavy caffeine intake, and caffeine withdrawal can cause a headache resembling a migraine. However, many people find that caffeine will help reduce the severity of a cluster headache. In some of these cases it is suspected that heavy use, temporarily suspended, causes a headache which can be aborted by renewed caffeine consumption. Caffeine is a constrictor of dilated arteries and should best be avoided as a dietary staple in vascular headaches.
Subcutaneously injected sumatriptan (6mg in 0.5ml) (Imitrex in the US) is the most effective, reliable, and rapid abortive therapy for cluster headache attacks. An injection (easily given by the patient) eliminates or markedly diminishes cluster headaches within 15 minutes in essentially all patients at every attack. Some patients have had headaches eliminated in as little as 7 minutes. This effect does not lessen with continued use. Some patients have had satisfactorily rapid results with sumatriptan nasal spray. The oral triptans are less effective, but some especially good responders with relatively milder and slower-developing headaches may prefer this route of administration.
Vigorous physical exertion at the earliest sign of an attack can, in some patients, be remarkably effective in ameliorating or even aborting an attack. [Atkinson, 1977; Ekbom and Lindahl, 1970]
A drop in nocturnal melatonin has been linked with cluster headaches, and melatonin supplementation has shown a low but significant preventive capacity for cluster headaches. In a blinded trial, 10mg of melatonin was given to 10 subjects and a placebo was given to 10 controls for 14 days. 5 out of 10 treated patients reported a decline in attack frequency after 3-5 days of treatment and then experienced no further attacks until melatonin was discontinued. The melatonin was taken in a single evening dose. [Cephalalgia. 1996;16: pp.494-496]
People who suffer from cluster headaches often have low blood levels of magnesium, and preliminary trials show that intravenous magnesium injections may relieve a cluster headache episode. Magnesium is a relaxant of smooth muscles. [Headache 1995;35: pp.597-600, 1996;36: pp.154-60]
Lithium carbonate, orotate or aspartate has been found to be effective in treating chronic cluster headaches, possibly due to its ability to impact the electrical system within the brain. The usual dose for the carbonate form is 300mg 2-3 times daily. Lithium levels should be checked and kept within, or even slightly below, the therapeutic range for bipolar disorder, namely 0.5 to 1.5 milliequivalents per liter.
Breathing pure oxygen (by face mask at a flow rate of 7 liters per minute for 15 minutes or less) provides considerable, rapid relief to most cluster headache sufferers by relaxing constricted blood vessels and thereby raising blood-oxygen levels. This treatment is both effective and safe, benefiting the majority of patients who use it.
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