Primary headache disorders are idiopathic disorders such as migraine and tension-type headache. The latter are warning symptoms of other diseases, such as sinusitis, tumour or cerebral bleeding. Although there are many different possible causes of symptomatic headaches, primary headache disorders are much more frequent, accounting for about 95% of all headaches.
To make the correct diagnosis, the physician needs a detailed account of the headache characteristics. He will ask you to describe the headache location (on one side or both sides of the head), headache intensity, type of pain (dull, stabbing, throbbing) and accompanying symptoms (e.g., nausea, increased sensitivity to light or sounds, fatigue, red eyes). The duration of attacks, triggering factors (e.g., stress, menstruation), attack frequency (daily, two times per month, etc.), headache history (new headache vs. long-standing headache) and present and previous treatment (during attacks, prophylactic) are also important.
Some patients suffer from more than one type of headache. When headaches are long-standing, keeping a headache diary for some weeks may be useful before an appointment at the doctor’s.
A physical examination is performed before making a diagnosis. In primary headaches, the physical examination is usually normal, or (abnormal) findings are not related to the headache. If headache symptoms are typical for a primary headache type (e.g. migraine) and physical examination is normal, no further examinations (e.g. electroencephalography, imaging of the brain) are necessary.
Additional investigations, as recommended by the doctor, are only necessary when a symptomatic headache is suspected. Not all symptomatic headaches can be diagnosed using computed tomography or magnetic resonance imaging of the brain. Sometimes blood tests, ultrasound examinations or a spinal tap are required.
Questions and Answers
Headaches are among the most frequent health complaints throughout the population. Epidemiologic surveys show that around 70% of the population have a headache at least once a year, with women more often affected than men. Headache disorders are divided into primary and secondary (symptomatic) forms.
Please find below a description of the various types of headaches:
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Many types of headache are unproblematic and can be treated with over-the-counter drugs as long as they occur only occasionally. When headaches occur on a regular basis, or do not respond satisfactorily to analgesics or increase in frequency, a doctor should be consulted. Headache patients that regularly take painkillers on more than 8 to 10 days per month should also make a doctor’s appointment.
A symptomatic headache is suspected when a new headache occurs on a daily or almost daily basis from the beginning, when the character of pre-existing headaches changes, or when fever or neurological symptoms occur (e.g. muscle weakness or palsy, sensory disturbances, changes in personality). Extremely intense headaches that reach their maximum within seconds or minutes indicate an emergency that requires immediate medical attention. The same is true when headache occurs together with meningism, significant fever, epileptic seizures or other disturbances of consciousness or acute neurologic deficits.
Migraine is among the most frequent headache disorders in Germany and throughout the world. About 10-15% of the population suffers from migraine.
Migraine can start in childhood. In children, migraine attacks are often less distinctive and shorter than in adults. Abdominal pain, nausea or dizziness may be the main symptoms. Before puberty, migraine affects boys and girls similarly. After puberty, three times more women than men suffer from migraine. Most often, migraine starts between 20 and 30 years of age. In many patients, there are significant fluctuations of migraine activity during lifetime, with good times and bad times. Often, migraine activity is highest between 40 and 50 years of age. In old age, migraine often becomes less frequent and less severe, and sometimes stops altogether. Most often, migraine is an episodic disease with recurrent attacks that may occur several times a month but usually not several times a week.
A small proportion of patients suffer from the chronic form of migraine. In this case, headache is present on 15 days per month or more, with typical migraine characteristics on many but not necessarily all of these days. Chronic migraine can be diagnosed only when there is no concomitant overuse of acute headache medication or if headache does not improve after withdrawal of acute headache medication.
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Migraine is characterized by recurrent headache attacks. Migraine headache is often unilateral but in some cases may be bilateral. The pain is described as dull and pressing and usually becomes stabbing or throbbing with physical activity. Pain intensity is usually high enough to lead to a significant impairment in daily life. In adults, migraine attacks last for a few hours up to 3 days if not treated. Rarely, migraine attacks may last longer than 3 days. Migraine has typical accompanying features, such as nausea, vomiting, increased sensitivity to light, sounds and odours. Many migraine patients experience fatigue during their attacks. They are pale and want to be alone, preferably to lie down in a quiet, dark room. Sleep is often experienced as helpful.
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15-25% of migraine patients experience an aura, mostly before the headache starts. The aura is characterised by neurological symptoms, particularly visual disturbances with scintillations (flickering) or scotomas (parts of the visual field are missing). There may also be sensory disturbances on one side of the body or speech difficulties. Typically these symptoms develop slowly over minutes and regress within one hour. Many patients suffer from migraine attacks both with and without aura.
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Migraine is a disorder of the brain. During a migraine attack, pain-processing brain areas are activated and pain-related signalling molecules (neurotransmitters and neuromediators) are released. These signalling molecules lead to a sterile inflammatory reaction of the blood vessels in the meninges. The normal pulsations of the blood vessels then lead to stretching of the inflamed vessel wall, explaining the typical throbbing character of migraine headache. According to current knowledge, there is also a genetic predisposition for migraine. The brain of predisposed persons reacts to certain triggers or stress situations with a migraine attack. About two thirds of migraine patients have family members who also suffer from migraine attacks.
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Principally, one has to distinguish between treatment of the attack itself and preventive treatment. There are both pharmacological and non-pharmacological methods of migraine therapy. During the attack, common painkillers such as acetylsalicylic acid or ibuprofen are used, in combination with antiemetics (drugs to treat nausea) if necessary. Moreover, there are also migraine-specific drugs, so-called triptans. Most triptans are prescription drugs. All acute migraine drugs work best when taken early in the attack. Painkillers may not work when taken during the aura phase. Triptans should not be taken during the aura phase for safety reasons. In order for the acute medication to be most effective, it is recommended that patients take a break and lie down if possible. However, all painkillers, including triptans, may also lead to worsening of migraine when taken too regularly over a longer period of time. Therefore, common painkillers must not be taken on more than 10 to 15 days per month. Triptans must not be taken on more than 8 to 10 days per month.
If migraine attacks occur more than 3 or 4 times per month, or regularly last for more than three days, or do not react well to acute therapy, a preventive treatment (migraine prophylaxis) should be started. Daily intake of preventive medication may reduce migraine frequency and severity. This is sometimes the only way to prevent frequent intake of painkillers or triptans. The drugs used for preventive migraine treatment are drugs that are also used for treatment of arterial hypertension or epilepsy. Dietary supplements or magnesium may also be useful in selected patients. It is important to know that the effect of a preventive treatment may set in only after 4-6 weeks. The goal of preventive treatment is a reduction of migraine attacks by at least 50 %. It is usually not possible to completely stop migraine using preventive treatment. An effective preventive treatment should be continued for at least 6 to 9 months. After that, dose reduction and ultimately discontinuation of the drug can be attempted. -
Non-pharmacological methods play an important role in reducing the frequency and severity of migraine. Scientific evidence indicates that migraine can be reduced by regular physical exercise, especially in the form of endurance sport and by muscle relaxation training. Biofeedback may also be helpful. Many patients know that there are certain factors, such as lack of sleep, irregular meals, dehydration or stress, that trigger their migraine attacks. In these cases behavioural interventions, such as cognitive behavioural therapy with the goal of stress reduction, may be very effective in reducing the number of migraine attacks.
Most people have experienced a tension-type headache at some point in their life. Tension-type headache is the most frequent type of headache, but in most of the affected subjects it occurs only occasionally.
Tension-type headache is usually bilateral, dull and pressing. Pain intensity is low to moderate, annoying but not leading to severe impairment in daily life. Accompanying symptoms such as nausea and light and noise intolerance, which are typical of migraine, are usually not present in tension-type headache. Many patients describe tension-type headache as feeling like the head is being squeezed in a vice, or like a tight band around the head. Some patients also have a feeling of drowsiness. Tension-type headache usually does not get worse with physical exercise; on the contrary, it may improve with outdoor activity. The duration of a tension-type headache attack may vary greatly, ranging from half an hour to several days.
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Tension-type headache may be episodic or chronic. In the chronic form, headaches are present on 15 days or more per month for at least three months. In the episodic form, the number of headache days is less than 15 per month. In chronic tension-type headache, slight nausea or increased sensitivity to light or sounds may occur. Vomiting or worsening by physical activity are typical migraine symptoms, which are not present in tension-type headache. Episodic tension-type headache is very frequent. Normally, it does not cause significant impairment in daily life and is well controlled by standard painkillers. In contrast, chronic tension-type headache is rare and, in most cases, develops from the episodic form. An important risk factor for the development of chronic tension-type headache is regular intake of painkillers. It is therefore important to rule out medication overuse before making the diagnosis and starting the treatment (see below).
Moreover, genetic factors may play a role in the development of chronic tension-type headache. The risk of developing chronic tension-type headache is three times higher in families with other affected members. Epidemiologic studies have shown that persons with chronic tension-type headache suffer from comorbid depression, anxiety or panic attacks more often than persons without headache. It is not clear if there is a causal relationship here. On the one hand, depression increases the risk of suffering from headache. On the other hand, frequent headaches and concomitant reduction of quality of life also lead to an increased risk of depression.
Simultaneous occurrence of migraine and tension-type headache is a frequent diagnostic problem. In these cases it is often not clear if the less severe, bilateral, dull or pressing headache indeed corresponds to a tension-type headache or instead to a less severe migraine attack. There are several arguments in favour of this view. First, the presumed tension-type headache may evolve into a full migraine attack within hours. Second, application of migraine-specific drugs (triptans) is often successful in these headaches but not in true tension-type headache. -
Although tension-type headache is a frequent affliction, its origin is not clear. Possibly, there are several different factors that lead to a common type of headache described as tension-type headache. The most common hypothesis is that increased tension of the neck muscles leads to increased sensitisation of pain-processing centres in the brain if continuously present. No alteration of the muscles themselves has been found.
When alteration of the central pain processing structures has set in, tension-type headache is more difficult to treat. The ongoing muscle tension further reinforces these mechanisms, resulting in a vicious circle. However, these mechanisms are not dangerous and physical damage is not encountered. -
If possible, the diagnosis of tension-type headache should be made by a headache specialist. The diagnosis is supported by the typical history and a normal physical examination that does not give reason to suspect other causes of headache. There is no further examination that might corroborate the diagnosis of tension-type headache. Additional examinations such as a computed tomography or magnetic resonance imaging are necessary only if it is suspected that there is another disorder underlying the headache.
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Treatment of the acute headache attack has to be differentiated from preventive treatment. Most common painkillers, such as acetylsalicylic acid 500 mg, paracetamol 500 mg or ibuprofen 400 mg are effective in tension-type headache. Alternatively, essential oils such as peppermint oil can be used. The oil should be spread generously on the forehead, temples and neck. A major problem is that frequent intake of painkillers on more than 10 days per month can lead to an exacerbation of headache; thus it is recommended that painkillers are used restrictively, especially in chronic tension-type headache. Therefore, it is important to keep track of the number of medication days per month.
Preventive treatment first includes general measures such as ensuring that the day is structured, with breaks and sufficient sleep and the reduction of stress factors. Moreover, regular exercise, e.g. cycling, swimming, running or Nordic walking, if possible three times per week for at least 30 min is recommended as well as relaxation techniques such as progressive muscle relaxation (PMR) that is effective in both migraine and tension-type headache.
When there is a continuous increase of headache days, with the risk of a previously episodic headache becoming chronic, preventive medication should be started in addition to the general measures described above. Preventive drugs are not direct painkillers. In contrast, they are supposed to act on pain processing in the brain and thereby lead to a reduction of tension-type headache. The exact mechanism of action is not known. Preventive medication has to be taken regularly, i.e. daily. Its action sets in only after about 4-6 weeks. In tension-type headache, mainly antidepressant drugs are used that act not only on headache but also on other types of pain. For preventive treatment of headache, lower doses are used compared to the treatment of depression. The first choice is amitriptyline, alternatively other types of antidepressants such as mirtazapine or venlafaxine can be used. Tizanidine and valproate are used as drugs of second choice, they are not antidepressant drugs. Botulinum toxin is not effective in chronic tension-type headache.
All of these drugs are prescription drugs and blood tests or ECG controls are required during the initial dosing phase. If the dose is increased slowly, these drugs are usually well tolerated. If there is a significant reduction of headache days and severity, the preventive drug is maintained for at least 6-9 months.
There are also non-pharmacological methods of tension-type headache treatment, including the general measures described above (regular structure of the day, stress reduction and stress coping, physical exercise and relaxation techniques). Most of the time, a combination of pharmacological and non-pharmacological methods is more effective than only using a pharmacological method. Acupuncture can be tried; however, results vary between studies and are less pronounced than with the methods described above.
Cluster headache is a rare headache disorder, that manifests with strictly unilateral, very severe pain attacks that are most pronounced around the temple and eye.
It is called cluster headache because it typically occurs in clusters of several weeks or months that may periodically recur, often with a seasonal preference in spring or autumn. Between these clusters, patients experience pain-free periods that often last for months or years.
The severe, unilateral pain attacks of cluster headache mostly last for 15 to 180 minutes. They often occur spontaneously, without apparent triggers. However, they tend to occur around the same time of the day every day, often waking patients from sleep. Cluster headache attacks can be triggered by alcohol, drugs (especially drugs derived from glyceryl trinitrate) and exposure to high altitudes. During an episode of cluster headache, attacks occur with a varying frequency of between once every second day and eight per day. Pain is usually side-locked in cluster headache.
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Cluster headache pain is described as excruciating, gnawing, drilling, sometimes also burning. The main area of pain is usually located around or behind the eye. Some patients have the feeling that the pain radiates from their neck. Pain may also radiate towards the upper jaw.
In addition to the typical circadian periodicity, there are several accompanying symptoms that are limited to the side of the pain and are typical of cluster headache:- Reddening of the eye
- Tearing of the eye
- Drooping of the eyelid
- Running or stuffy nose
- Sweating of the forehead or entire side of the face
- Restlessness or agitation
For diagnosis, at least one of these symptoms must be present on the side affected by the pain. Moreover, patients may suffer from symptoms as found in migraine, such as nausea, increased sensitivity to light and sounds.
These accompanying symptoms are typical of the group of so-called trigeminoautonomic headache disorders that constitute a group within the International Headache Classification. Apart from cluster headache, this group includes very rare headache disorders such as paroxysmal hemicrania and SUNCT syndrome. These headache disorders are characterized by shorter but more frequent pain attacks. They do not respond to treatments known to be effective in cluster headache.
Sometimes, cluster-like headaches occur in diseases of the ocular region or in sinusitis. Very rarely, tumours, e.g. of the pituitary gland may be at the base of cluster-like headaches. To rule out a symptomatic headache, brain imaging (magnetic resonance imaging or computed tomography) is usually performed once during the course of the disease. If both neurological examination and imaging results are normal, no additional examinations are required. -
The mechanisms underlying cluster headache are currently not known. There is some evidence that a particular brain structure, the hypothalamus, plays an important role in cluster headache. The hypothalamus is thought to constitute our “body clock” and is held responsible for the circadian rhythm and the seasonal clustering of attacks. In addition, during the attack there is activation of the pain-related structures of the trigeminal nerve and of the autonomic nervous system. The trigeminal nerve is responsible for perception of touch and pain in the face and head.
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In most patients, cluster headache is episodic with cluster attacks occurring for periods of weeks or months, separated by pain-free periods of months or years. Rarely, cluster headache takes a chronic course, with cluster episodes lasting longer than a year or separated by pain-free periods shorter than a month. About 10% of patients suffer from a chronic cluster headache from the beginning, and about 5% of patients develop a chronic cluster headache from the episodic form.
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Cluster headache is a primary headache disorder, and there is presently no permanent cure for this disease. However, pain intensity and attack frequency can be effectively reduced by pharmacological treatment in most cases. As in other types of headache, treatment of the acute attack is differentiated from preventive treatment.
A cluster headache attack does usually not respond to normal, over-the-counter painkillers. Many patients can stop an acute attack by inhalation of pure oxygen. This treatment is free of side effects and can be prescribed for use at home. In addition, rapidly acting triptans or nasal application of lidocaine can be used in the treatment of the acute attack.
Usually, preventive treatment is the mainstay of cluster headache therapy, as it is the only way to reduce the frequency of the extremely severe attacks. Drugs from different classes can be used. The way in which these drugs act to reduce cluster headache attacks is not known. Because cluster headache is a rare disorder, preventive treatment should be performed by a neurologist with experience in the treatment of cluster headache. Selection of the preventive drugs depends on the clinical course and on possible concomitant diseases. Frequently used drugs include verapamil, topiramate, methysergide and lithium. The effect usually sets in at 1-2 weeks. To bridge this time gap, glucocorticoids are often used. Drugs may also be used in combination. Preventive treatment is maintained until the end of the cluster episode and then tapered. As an alternative to oral drugs, repeated occipital nerve blocks (the nerve responsible for the sensation of touch and pain at the back of the head) can be performed, using local injections of local anaesthetics and glucocorticoids on the side of the pain.
Only if pharmacotherapy is not effective, operative therapies may be considered in selected patients with a severe, chronic course of the disease. Currently, there is no established operative method that allows cluster attacks to be stopped reliably and with long-lasting success in every patient. Electric stimulation of the greater occipital nerve is a promising therapeutic strategy that is currently under scientific evaluation. Before initiating an operative treatment of cluster headache, the pros and cons must be carefully considered by both the doctor and the patient and a cluster headache specialist should be involved.
All patients suffering from a primary headache disorder such as migraine or tension-type headache run the risk of developing a so-called medication overuse headache if they use analgesic medication too often. When medication overuse headache sets in, headache attacks may last longer and longer, more and more painkillers may be necessary to reduce headache, and the number of headache days often increases progressively. Many patients complain of ongoing headache, without completely pain-free days.
Patients who regularly take simple analgesics (e.g. acetylsalicylic acid, ibuprofen, paracetamol) on more than 15 days per month, or triptans, opioids or combination analgesic medication on more than 10 days per month run the risk of developing medication overuse headache. Medication overuse headache generally develops only if frequent intake of analgesics is continued for months or even years. The day limits given above are based on the International Headache Classification. Clinical experience shows that some patients may develop medication overuse headache even without explicitly reaching these limits.
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It is only partially understood how overuse of analgesics and triptans leads to headache exacerbation and increase in headache frequency. Both modification of neurobiological pain processing and psychological factors seem to contribute. Especially the strong effect of triptans on migraine headache may induce an accelerated learning process that may lead to more and more frequent intake of the medication. Studies have shown that triptans may cause medication overuse headache more rapidly and in lower dosages than other drugs. However, medication overuse headache is usually not a dependence disorder as is the case with illicit drugs.
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With medication overuse, the pre-existing headache, most often a migraine, often changes in character. The headache may be more often bilateral, more dull than throbbing, and less frequently accompanied by additional symptoms such as nausea, vomiting and increased sensitivity to light and sounds. Sometimes the headache may thus resemble a tension-type headache or a combination of migraine and tension-type headache. To make the diagnosis it is important that the headache has developed from a primary headache disorder, with an increasing headache frequency, eventually exceeding 15 days per month, and during frequent, sometimes even prophylactic intake of acute headache medication that shows an increasing lack of effect.
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Some analgesics, if taken very frequently, can impair normal body functions (impairment of kidney function, high blood pressure) or interact with other drugs (e.g. impair the blood thinning effect of acetylsalicylic acid). Moreover, the effect of acute headache medication in patients suffering from medication overuse headache is often unsatisfactory. In addition, the high cost of triptans may be a problem for patients buying these drugs over the counter.
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It was long thought that preventive drugs do not work as long as the patient is overusing acute headache medication. In contrast, several studies have shown that withdrawal of the overused acute pain medication leads to a marked improvement in headache. However, recent studies now show that certain preventive migraine medicines (topiramate, botulinum toxin for chronic migraine) are effective even in the presence of medication overuse. It is currently not clear if this is true for all preventive migraine treatments. Therefore, the current standard procedure is to recommend withdrawal and at the same time start patients on a preventive medication. For patients who do not tolerate withdrawal, preventive medication should be started anyway.
When stopping the overused acute headache medication, withdrawal symptoms can occur. There may be an exacerbation of headache for some days, possibly with increased accompanying symptoms such as nausea and fatigue. In addition, autonomic symptoms such as restlessness, sweating and heart palpitations can occur. In most cases, these symptoms recede within a few days and patients are then markedly improved. Withdrawal of acute headache medication is usually done in an out-patient or day-clinic setting. An in-patient treatment should be reserved for patients who need special support because of psychosocial constellations (e.g. high stress load at work or at home), or who have psychological comorbidities such as depression or anxiety disorders, or who overuse not only simple analgesics and triptans but also opioids and tranquilizers or if previous attempts to withdraw acute headache medication were not successful.
One third of the patients who successfully terminate medication overuse relapse within one year. Therefore, treatment by a headache specialist should be continued even after an initially successful withdrawal. In this way, medication intake can be monitored and adapted to the patient’s needs. In addition, the patient may then benefit from other therapeutic options, including patient education and treatment of concomitant disorders. Non-pharmacological approaches and integration of these measures into daily life are important. These include training in a relaxation technique, regular sleeping times, regular breaks and physical activity. In this way, patients acquire additional strategies for managing their headache disorder, learn to use their acute headache medication responsibly, and are able to recognize impending medication overuse.
However, the most important message is that medication overuse headache can be treated effectively. In over 80% of the patients who successfully stop their overuse of acute headache medication, headache improves significantly. In most cases, after stopping medication overuse, headache reverts to its original pattern with isolated headache attacks that can be treated effectively with acute headache medication.
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