Headache
Cluster Headache
By: Frederick G. Freitag, DO, FAHS
Cluster headache is a significant headache disorder that differs in many aspects from the other primary headache disorders such as migraine and tension-type headache. As such, it is classified separately from them. The term “cluster headache” may actually represent several different kinds of headaches, which may afflict different age groups and genders preferentially. One of the most important factors necessary in diagnosing cluster headache, as is true for any of the primary headaches, is to assure that there is no underlying, potentially serious or curable cause for the headaches. This is accomplished through the history, a careful examination and appropriate diagnostic studies.
When we discuss cluster headache, we most commonly are referring to the episodic variety. However, about 10% of all patients with cluster headache will have a period in which the disease becomes chronic and does not remit for a year or more. This can either occur right from the first attacks or develop in the latter years of having them, which is the more common. Chronic paroxysmal hemicrania is another form of cluster headache in which the attacks are much shorter but far more frequent. This can also occur in a number of variants and may be referred to as the “stab and jab syndrome” and “ice pick headache,” among others. One last type of headache classified with cluster headache is called short-lasting unilateral neuralgiform headache with conjunctival injection, or SUNCT. Obviously you can see why, in fact, the pain in this syndrome may last less time than it takes to pronounce the full diagnosis.
Typical episodic cluster headache begins in the late 20s. It has rarely been seen in children and almost invariably begins before the Medicare years. For decades, we have cast it as being a male dominant disorder as compared to migraine, which is more common among women. However increasingly, the studies show that many women also experience these headaches.
It has been said that a picture is worth a thousand words, so I use a little mnemonic for cluster headache that shows a bunch of grapes and a clock. The reason for this relates to the timing of the headaches. Cluster headaches do occur in bunches – every day or several per day for periods of weeks to months, only to spontaneously disappear and then recur with set regularity months to years later based on the person. During a bout of cluster headache, the attacks typically occur at almost the exact same time of day, every day in the cycle, and this is often near the end of a sleep cycle. The average headache lasts 15 minutes to two hours and occurs once or twice a day for a period of several months before remitting for about two years.
The first description of cluster headache was by the Swiss physician Gerhard van Swieten. In 1745 he reported, “A healthy robust man of middle age was suffering from troublesome pain, which came on every day at the same hour at the same spot above the orbit of the left eye.”
Not only does cluster headache occur are the same time of day each day in a bout, but also the bouts of cluster typically occur at the same time of year in a given person. There are two peak times for these cycles to begin. These are within two weeks of the shortest and longest days of the year -December and June.
The pain of cluster headache is extremely intense and is described as a hot burning poker by some, occurring in or around the eye. The attacks do not switch sides of the head like in migraine but are always on the same side. While migraine suffering patients retreat to a dark quiet bed, cluster patients typically pace, rock back and forth and cannot lie down because the headache gets worse. Patients may rant and rave and even risk harm to themselves by hitting their head on the wall. Patients with migraine are sensitive to their environmental stimuli and experience nausea and vomiting. Patients with cluster headache develop what is called a Horner’s Syndrome in which the eyelid on the side of the headache droops, the pupil constricts and the eye becomes red and tearing. Additionally, the nose becomes stuffy and may have a runny discharge. Patients with migraine are pale; cluster patients flush and sweat. The occurrence of the nasal symptoms and the seasonality lead many to think this is sinus-related, which it is not, even if there is a partial response to nasal decongestants since these drugs work in many vascular headache disorders to a small degree.
Our knowledge about cluster headache causation lags behind migraine because comparatively there are far fewer patients, the attacks occur in their well-spaced series compared to the regular recurrence of migraine among other reasons. We recognize, however, that many of the signs and symptoms are due either to a partial paralysis of a portion of the nervous system called the sympathetic nervous system or from an excessive stimulation of its counterpart, the parasympathetic system – all of this being regulated deep in the brain by the hypothalamus and adjacent structures.
As I mentioned, some patients will develop a phase of cluster headache where there is no let up from the daily barrage of headaches. This is termed chronic cluster. Patients with this have similar headaches but many more of them. Instead of occurring at the same time of day and same time of year, they can happen any old time of the day or year. They also become more resistant to treatment over time.
While medicines are needed to treat individual attacks of cluster headache, the mainstay of treatment rests on preventing the occurrence of the headaches. The most uniformly successful medication for acute relief of cluster headache is the use of a high flow of pure oxygen delivered via a facemask. This works in minutes in the majority of patients. Other treatments include drugs such as sumatriptan, zolmitriptan and dihydroergotamine, which work on the serotonin receptors in the brain and blood vessels. Because the attacks are brief, the medicine must be delivered quickly. This eliminates pills but is ideal for shots under the skin or in nasal spray routes. Other treatments have also been tried but lack consistency or tolerability.
Preventive treatment of cluster headache resembles that of migraine in many aspects. Several differences, though, include stopping the medicine when the cycle ends and using medicines that can be taken for short periods safely but that would be problematic if used over a long period as would be needed in migraine. Our old standby methysergide is not sold in the US any longer, so often we will use a related drug called methergine to prevent the attacks. Other top choices include the calcium channel blocker Verapamil, which has great safety, tolerability and efficacy. Anti-convulsant medications like valproic acid and topiramate are better tolerated in cluster than in migraine. Short courses of a month or two of one of the long-acting triptans such as frovatriptan or naratriptan are also successful. A few weeks of a cortisone-type medication almost invariably brings relief within hours. However if used for long periods, these medications can lead to recurrence of the headaches and significant side effects.
Some patients have benefited from having nerve blocks done in the upper neck at the base of the skull for prevention. These are relatively straightforward and well tolerated by most.
Some cluster headache patients who are resistant to treatment benefit from infusions or injections of histamine to break the cycle of headaches. This was pioneered at Mayo Clinic in the 1940s and remains a useful therapy today. Surgery has also been done for cluster headache, but the results are often transient and lead to recurrence of the clusters on the opposite side or complications from the procedures.
Along with chronic paroxysmal hemicrania are groups of disorders in cluster headache that are characterized by their uniform response to the anti-inflammatory medicine Indomethacin. All of these syndromes may have symptoms resembling cluster headache but are typically briefer, have more variability of the associated symptoms and are more common among women. Indomethacin works rapidly within weeks often to produce a complete remission. Sometimes small doses will keep the attacks at bay indefinitely. Stomach and kidney problems may occur with this medicine, necessitating close follow-up.
In summary, cluster headache is a briefer headache than migraine that occurs with a comparatively higher frequency of events. The attacks have more localized symptoms to the area of pain than in migraine but lend themselves readily to treatment. Prevention is the key in cluster headache as compared to migraine where the acute treatments are the mainstay. Most patients with this cluster headache can be managed in a straightforward manner.
Signs and Symptoms
When we discuss cluster headache, we most commonly are referring to the episodic variety. However, about 10% of all patients with cluster headache will have a period in which the disease becomes chronic and does not remit for a year or more. This can either occur right from the first attacks or develop in the latter years of having them, which is the more common. Chronic paroxysmal hemicrania is another form of cluster headache in which the attacks are much shorter but far more frequent. This can also occur in a number of variants and may be referred to as the “stab and jab syndrome” and “ice pick headache,” among others. One last type of headache classified with cluster headache is called short-lasting unilateral neuralgiform headache with conjunctival injection, or SUNCT. Obviously you can see why, in fact, the pain in this syndrome may last less time than it takes to pronounce the full diagnosis.
Typical episodic cluster headache begins in the late 20s. It has rarely been seen in children and almost invariably begins before the Medicare years. For decades, we have cast it as being a male dominant disorder as compared to migraine, which is more common among women. However increasingly, the studies show that many women also experience these headaches.
It has been said that a picture is worth a thousand words, so I use a little mnemonic for cluster headache that shows a bunch of grapes and a clock. The reason for this relates to the timing of the headaches. Cluster headaches do occur in bunches – every day or several per day for periods of weeks to months, only to spontaneously disappear and then recur with set regularity months to years later based on the person. During a bout of cluster headache, the attacks typically occur at almost the exact same time of day, every day in the cycle, and this is often near the end of a sleep cycle. The average headache lasts 15 minutes to two hours and occurs once or twice a day for a period of several months before remitting for about two years.
The first description of cluster headache was by the Swiss physician Gerhard van Swieten. In 1745 he reported, “A healthy robust man of middle age was suffering from troublesome pain, which came on every day at the same hour at the same spot above the orbit of the left eye.”
Not only does cluster headache occur are the same time of day each day in a bout, but also the bouts of cluster typically occur at the same time of year in a given person. There are two peak times for these cycles to begin. These are within two weeks of the shortest and longest days of the year -December and June.
The pain of cluster headache is extremely intense and is described as a hot burning poker by some, occurring in or around the eye. The attacks do not switch sides of the head like in migraine but are always on the same side. While migraine suffering patients retreat to a dark quiet bed, cluster patients typically pace, rock back and forth and cannot lie down because the headache gets worse. Patients may rant and rave and even risk harm to themselves by hitting their head on the wall. Patients with migraine are sensitive to their environmental stimuli and experience nausea and vomiting. Patients with cluster headache develop what is called a Horner’s Syndrome in which the eyelid on the side of the headache droops, the pupil constricts and the eye becomes red and tearing. Additionally, the nose becomes stuffy and may have a runny discharge. Patients with migraine are pale; cluster patients flush and sweat. The occurrence of the nasal symptoms and the seasonality lead many to think this is sinus-related, which it is not, even if there is a partial response to nasal decongestants since these drugs work in many vascular headache disorders to a small degree.
Our knowledge about cluster headache causation lags behind migraine because comparatively there are far fewer patients, the attacks occur in their well-spaced series compared to the regular recurrence of migraine among other reasons. We recognize, however, that many of the signs and symptoms are due either to a partial paralysis of a portion of the nervous system called the sympathetic nervous system or from an excessive stimulation of its counterpart, the parasympathetic system – all of this being regulated deep in the brain by the hypothalamus and adjacent structures.
As I mentioned, some patients will develop a phase of cluster headache where there is no let up from the daily barrage of headaches. This is termed chronic cluster. Patients with this have similar headaches but many more of them. Instead of occurring at the same time of day and same time of year, they can happen any old time of the day or year. They also become more resistant to treatment over time.
Treating Cluster Headaches
While medicines are needed to treat individual attacks of cluster headache, the mainstay of treatment rests on preventing the occurrence of the headaches. The most uniformly successful medication for acute relief of cluster headache is the use of a high flow of pure oxygen delivered via a facemask. This works in minutes in the majority of patients. Other treatments include drugs such as sumatriptan, zolmitriptan and dihydroergotamine, which work on the serotonin receptors in the brain and blood vessels. Because the attacks are brief, the medicine must be delivered quickly. This eliminates pills but is ideal for shots under the skin or in nasal spray routes. Other treatments have also been tried but lack consistency or tolerability.
Preventive treatment of cluster headache resembles that of migraine in many aspects. Several differences, though, include stopping the medicine when the cycle ends and using medicines that can be taken for short periods safely but that would be problematic if used over a long period as would be needed in migraine. Our old standby methysergide is not sold in the US any longer, so often we will use a related drug called methergine to prevent the attacks. Other top choices include the calcium channel blocker Verapamil, which has great safety, tolerability and efficacy. Anti-convulsant medications like valproic acid and topiramate are better tolerated in cluster than in migraine. Short courses of a month or two of one of the long-acting triptans such as frovatriptan or naratriptan are also successful. A few weeks of a cortisone-type medication almost invariably brings relief within hours. However if used for long periods, these medications can lead to recurrence of the headaches and significant side effects.
Some patients have benefited from having nerve blocks done in the upper neck at the base of the skull for prevention. These are relatively straightforward and well tolerated by most.
Some cluster headache patients who are resistant to treatment benefit from infusions or injections of histamine to break the cycle of headaches. This was pioneered at Mayo Clinic in the 1940s and remains a useful therapy today. Surgery has also been done for cluster headache, but the results are often transient and lead to recurrence of the clusters on the opposite side or complications from the procedures.
Along with chronic paroxysmal hemicrania are groups of disorders in cluster headache that are characterized by their uniform response to the anti-inflammatory medicine Indomethacin. All of these syndromes may have symptoms resembling cluster headache but are typically briefer, have more variability of the associated symptoms and are more common among women. Indomethacin works rapidly within weeks often to produce a complete remission. Sometimes small doses will keep the attacks at bay indefinitely. Stomach and kidney problems may occur with this medicine, necessitating close follow-up.
In summary, cluster headache is a briefer headache than migraine that occurs with a comparatively higher frequency of events. The attacks have more localized symptoms to the area of pain than in migraine but lend themselves readily to treatment. Prevention is the key in cluster headache as compared to migraine where the acute treatments are the mainstay. Most patients with this cluster headache can be managed in a straightforward manner.