Cluster headaches are still a relatively unknown complaint even though they have received increasing media attention in recent years. This special form of headache disease is characterized by the most violent, relapsing, one-sided headache . By the time people have been diagnosed with this problem, they have often already had to go through a lengthy medical odyssey. A time in which many patients threaten to despair in the face of massive pain.
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Cluster headache is a periodic headache that occurs repeatedly without any apparent connection to an existing disease. In contrast to this primary headache disease, secondary forms of headache have to be distinguished, which can be very similar to cluster headaches, but are a symptom of an existing disease. The term “cluster” refers to the periodic manifestation of the disease. After quite lengthy symptom-free periods, the cluster headache occurs in concentrated time intervals (“cluster”: cluster, group, bundle). The up to three-hour acute headache episodes can usually be observed increasingly over a period of several days before a symptom-free period follows.
In addition to pain, according to the definition of the International Headache Society (IHS), at least one of the following accompanying symptoms of cluster headache can be observed: tears of the eye (lacrimation), reddening of the eyes, drooping eyes (ptosis), swelling of the eyelids (edema of the eyelids), narrowing of the pupil (miosis ), increased secretion of nasal secretions (rhinorrhea), nasal congestion, restlessness with urge to move and / or profuse sweating , especially in the area of the forehead and face. According to the current medical classification, cluster headaches belong to the so-called trigeminal autonomous headache diseases. They are also known as erythroposopalgia, histamine headache and Bing-Horton headache.
Cluster headache is determined by very special pain symptoms, which – as already mentioned – are accompanied by various other symptoms. According to the German Migraine and Headache Society (DMKG), the pain attacks occur suddenly during the acute intervals and last for fifteen minutes to a maximum of 180 minutes. Afterwards, most patients show a symptom-free resting phase, which can last for different lengths of time (a few weeks to months or years) before the pain starts again.
The frequency of pain attacks in the acute phases can differ significantly from patient to patient. While some go through numerous attacks within a day (the DMGK reports up to eight attacks a day), others have one or two days of rest after a single occurrence. Typically there are longer symptom-free periods between the acute pain intervals, which can last for weeks and months. The patients are symptom-free for months or even years before the next acute phase begins. According to the information from the Pain Clinic Kiel, the complaints tend to be observed increasingly in the months of February, March, April and September, October, November.
Bing Horton neuralgia is most noticeable in the first few hours after falling asleep and in the early hours of the morning, but it can theoretically occur at any time of the day. Sufferers suffer extreme, one-sided pain, which mostly focuses on the area around the eyes, cheekbones and temples. For the majority of those affected, the complaints are always on the same page. The pain is so intense that it is not uncommon for patients to feel faint. On a pain scale from zero (no pain) to ten (extreme pain, with suicidal thoughts), cluster headache patients often choose the 10 to describe the pain intensity. Migraine patients, for example, usually choose lower values or a maximum of nine. This should not reduce the suffering of migraine patients, especially since the pain can last much longer. But it becomes clear how massive patients suffer.
The Pain Clinic Kiel, at whose center for rare headache diseases also cluster headache is an important topic, reports of a “devastating severity of pain that very often leads to suicide if the diagnosis and therapy are ineffective” Pain clinic “one of the most malignant and at the same time the most disabling pain diseases in humans.”
Accompanying the headache, those affected usually show extreme inner restlessness , bobbing back and forth with the upper body or continuously walking up and down. You also often start to sweat profusely during the pain attacks. Rather non-specific symptoms such as nausea or an excessive sensitivity to noise and bright light can also be part of the symptoms. The appearance of other accompanying symptoms in the area of the eye and / or the nose is characteristic of trigeminal autonomous headache disease. For example, the conjunctiva appears red in the patient, the eye begins to water, the pupil is narrowed, the eyelid hangs or is swollen. A stuffy or runny nose can also occur in connection with the cluster headache.
Since the pain often sets in during the night, many sufferers suffer from a lack of sleep or chronic fatigue during the acute phase . In addition, there are not infrequently further psychological impairments, whereby the pain clinic Kiel mentions “social isolation, personality changes, fear, depression, discouragement, anger, grief, despair and abandonment of the will to live” as possible consequences of the cluster headache. The fact that patients often suffer for years without a correct diagnosis not only increases psychological stress, but also often results in incorrect therapies, which in turn can also have negative effects on health.
Causes and triggers
Although various causes of symptomatic cluster headaches can be identified, little is known about the development of primary cluster headaches. Secondary headache attacks can be observed, for example, in the context of brain tumors, injuries in the area of the brain stem, inflammatory processes in the brain or as a result of a stroke. Trigeminal neuralgia (irritation of the trigeminal nerve) can also lead to one-sided pain attacks with accompanying symptoms such as watery eyes. However, the attacks do not usually last as long as with primary cluster headaches.
The Pain Clinic Kiel mentions the possible causes of the symptomatic cluster headache in particular as “upper cervical meningiomas, parasellar meningiomas, large arteriovenous malformations in the most diverse ipsilateral brain structures, ethmoidal cysts in the area of the clivus and in the area of the suprasellar cisterns, pituitary adenomas, calcifications in the area of the third. Ventricles, ipsilateral aneurysms and aneurysms of the anterior communicating artery. ”Although the terms are difficult for laypersons to understand, it can be said that the diseases all occupy space in the area of the midbrain line in the vicinity of the so-called cavernous sinus (enlarged vein space in the brain). Therefore, according to the Kiel Pain Clinic, the conclusion is obvious
On the basis of this thesis, numerous investigations have been carried out in recent years, which allow a closer look at the processes in the brain during the cluster attacks. For example, examinations of the blood flow in the affected person’s brain were carried out using so-called positron emission tomography (PET). These made it clear that changes in the area of the cavernous sinus can actually be observed. Magnetic resonance imaging also shows that “during a cluster headache attack, the uptake of contrast medium in the area of the cavernous sinus is increased,” reports the Pain Clinic in Kiel. These are indications of inflammatory processes taking place here during the pain attacks.
Signs of inflammatory events were also found in the affected persons in the cerebrospinal fluid and in the peripheral blood, and when “performing a phlebography (special X-ray examination procedure for assessing the veins) there were indications for the presence of venous vasculitis in the area of the cavernous sinus and the upper eye vein during the cluster period, ”explain the Kiel experts. In addition, vascular dilatations (vasodilation) can be observed in the area of the cavernous sinus, the arteria opthalmica, arteria cerebri anterior and the arteria cerebri media. The changes were found on the same side of the body (ipsilateral) as the headache attacks.
Vascular dilation was initially considered to be the cause for a long time before the knowledge prevailed in the specialist world that it should rather be seen as a result of the disease. The activation in the area of the hypothalamus, which can be demonstrated with the help of functional magnetic resonance tomography (fMRI), led to the presumption that the cause of the disease was to be found in this region of the brain. Especially since the sleep-wake rhythm is regulated here and this has striking connections with the occurrence of pain attacks. If science initially started with purely functional changes in the brain as the cause, studies “using voxel-based morphometry have shown a significant structural change in the density of the gray matter compared to healthy control persons”, reports the Pain Clinic Kiel.
However, the structural changes in gray matter in recent studies could only be confirmed for the central pain processing system, but not for the hypothalamus area. This suggests that “the morphological changes are the effects of acute pain and not the primary cause,” explains the Kiel Pain Clinic.
The evidence found for inflammatory processes in the cavernous sinus and in the area of the superior vena ophthalmica therefore became the focus of possible explanations for the occurrence of headache attacks. In this region, a wide variety of nerve fibers that supply the face, the eye and eyelid, the eye socket (orbit) and the retroorbital (behind the eye socket) vessels are very close together. In addition, there are the internal carotid artery and venous vessels, which serve to drain the orbit and face. Inflammatory processes occurring here could affect the nerve fibers as well as the arterial and venous vessels, which is a possible explanation for the cluster pain and the accompanying symptoms, reports the Pain Clinic Kiel.
The inflammatory basic reaction in the cavernous sinus would therefore be the cause of impairments of the carotid artery, optic nerves, eye nerves and facial nerve, which are all affected during the pain attacks, explains the Kiel Pain Clinic. This would also explain why vasodilators such as alcohol, nitroglycerin or histamine can provoke the attacks during acute intervals and why vasoconstricting substances such as oxygen or sumatriptan end the cluster pain.
Furthermore, it is understandable why the attacks occur more while lying down or sleeping – the venous drainage of the cavernous sinus is reduced when lying down due to the poorer hydrostatic conditions. Standing up, walking around and the need to observe movement are beneficial for the drainage of the cavernous sinus. Although plausible explanations for the occurrence of the pain attacks are provided here, a comprehensive, scientifically proven explanatory model has been lacking to date, which justifies the daily connections, the temporary concentration of the attacks, the one-sided localization, the sympathetic and parasympathetic activation as well as the accompanying symptoms that occur. Furthermore, the headache disease of science poses some riddles here.
Cluster headache trigger
In the acute periods, various triggers can trigger the pain attacks, whereby in addition to alcohol and nicotine, calcium antagonists such as nitroglycerin and a number of other substances such as the neurotransmitter histamine should be mentioned. In addition, those affected also report glaring, flickering light and heat or heat as possible trigger factors. Food additives such as glutamate or sodium nitrite are also increasingly being mentioned as possible triggers for cluster attacks. Foods such as tomatoes, citrus fruits and chocolate are also discussed as trigger factors.
The attacks are caused by particularly intense smells in some sufferers. Extreme physical stress as well as extreme psychological stress are also possible triggers for cluster headaches. Typically, the triggers only work during the acute periods and otherwise show no unusual effect in the remission phases. The relatively reliable trigger effect of nitroglycerin may also be used as part of the diagnosis, with sublingual (under the train) administration being used to specifically trigger a pain attack.
The basis of the diagnosis is a detailed survey of the patients on the intensity and duration of the headache attacks as well as the accompanying symptoms, whereby the symptoms are classified according to the IHS classification ICHD-II. The symptoms are still the only clue to the diagnosis, since cluster headaches cannot be determined with medical procedures such as laboratory examinations. However, it may be possible to initiate a pain attack with the sublingual administration of nitroglycerin during the acute phases, but this is not always possible reliably. Modern imaging methods such as computer tomography, magnetic resonance imaging, Doppler sonography or electroencephalography are used primarily in the context of diagnosis to:
Fortunately, cluster attacks affect only a minimal fraction of the population. The German Society for Neurology (DGN) reports a one-year prevalence of 0.1 percent to a maximum of 0.9 percent of the population. Men are affected three times more often than women. The first attacks usually show up at the age of 28 to 30 years and “up to 80 percent of patients”, according to the DGN, “still suffer from cluster episodes after 15 years.” However, the intensity and frequency of the attacks leave higher in some patients Age after. On the other hand, up to 12 percent of those affected change from a primary episodic to a chronic form, reports the DGN. Although an inheritable disease has not yet been assumed,
Cluster headaches are still not curable to this day, but acute pain attacks can be treated relatively reliably and there are a variety of preventive treatment options. As the patients are usually under considerable suffering, detailed information about the disease and possible therapeutic measures are of particular importance. Those affected should also be informed about the known triggers so that they can avoid them in case of doubt. Furthermore, it makes sense to create a headache calendar or a headache diary in which the attacks are recorded.
In its treatment recommendations for acute pain attacks, the German Society for Neurology mentions inhalation of 100 percent oxygen through a face mask in the first place. Since no systemic side effects are to be expected here, the treatment method should at least be tested in all patients, according to the DGN. However, the treatment usually only works after 15 to 20 minutes, which is why patients with short-term cluster attacks of just 15 minutes do not benefit here. According to DGN, oxygen inhalation only shows the desired effect in 60 percent to 70 percent of those affected.
If oxygen inhalation remains ineffective, according to the DGN Sumatriptan, which is administered by subcutaneous (under the skin) injections in doses of six milligrams, is the drug of choice. The patients can set the injections themselves with the help of so-called auto-injectors. Devices that do without a needle are now also available here. The tolerance of sumatriptan in cluster patients is “generally very good, even with the described overdoses of up to 8 injections within 24 hours,” reports the Pain Clinic in Kiel. However, the general contraindications to the use of sumatriptan should be noted. In the case of particularly long attacks, sumatriptan can also be administered nasally in a 20 milligram dose,
Zolmitriptan nasal spray in a dosage of five to ten milligrams is known as another, relatively effective medicine against the cluster attacks. Here, however, the effects usually do not occur as quickly as with subcutaneous sumatriptan use. In addition, according to the DGN, the “intranasal application of lidocaine” remains an option for the treatment of acute cluster attacks. The active ingredient is sprayed into the nostril in a four percent solution on the pain side. In the case of particularly long-lasting complaints that cannot be managed with the help of the methods mentioned, there is also the option of oral administration of triptans, the effects of which, however, only start to appear after a considerable delay.
In view of the high number of pain attacks during an active cluster period, prophylactic drug therapy is advisable with the aim of preventing the occurrence of renewed cluster attacks. Here, the German Society for Neurology names the active ingredient verapamil in a dosage of three to four times 80 milligrams a day as the first choice. If the application does not show the desired success, it is possible to switch to higher doses (maximum 960 milligrams per day). However, this requires an accompanying cardiac check (monitoring using an electrocardiogram; EKG) by experienced specialists.
If the affected persons are already in a cluster period and a renewed attack is to be expected due to the slow effect of the verapamil, according to the DGN, corticosteroids (prednisolone) are often used “in the sense of bridging therapy”. According to the DGN, the corticoids should be used in a dosage of at least one milligram per kilogram of the patient’s body weight and can be administered for two to five days. In view of the risk of side effects, dosing is urgently recommended.
Ergotamine and long-acting triptans such as naratriptan can be used as part of the prophylactic cluster treatment, but here too, only a limited use is possible. For longer-term use, according to the DGN, in addition to verapamil, lithium in particular is recommended in a dosage of 600 to 1,500 milligrams per day. Other active ingredients that can be used in cluster prophylaxis are, for example, topiramate, melatonin, methysergide, gabapentin, valproic acid, pizotifen, leuprorelin and capsaicin. According to the experience of the Pain Clinic Kiel, the administration of valproinate, topiramate and gabapentin in particular is not reliably effective. Although there are some positive case reports on these substances in the literature,
Which medicines are best suited for prophylactic treatment depends primarily on the patient’s individual symptoms and their physical requirements. Experienced neurologists should reserve and select the medication, especially since combinations of the above-mentioned medications often promise the greatest success in treatment, and some medications should never be used at the same time. The prophylaxis is maintained as long as the cluster periods of those affected normally last, which presupposes that their length can be traced, for example, using a headache diary. In the case of chronic pain, the breaks between the acute periods are often difficult to make out,
Operative procedures for cluster treatment
If a symptomatic cluster headache is ruled out and all medication measures are unsuccessful, there is the possibility of a surgical intervention to eliminate the cluster headache. However, the success rates are relatively low in relation to the treatment risks, which is why the surgical procedures are usually only considered in patients with severe forms of chronic cluster headaches.
The Pain Clinic Kiel opens the following calculation example: Chronic cluster headaches only suffer 27 percent of cluster patients (in 240,000 cluster patients in Germany 64,800 have chronic cluster headaches) and in only one percent of them (648 patients) the development of “therapy-refractory situations” is due expect. If of these “an estimated 50 percent are eligible for invasive surgery, such procedures are likely to be relevant for around 300 people in Germany,” reports the Pain Clinic in Kiel. This makes it clear that only a negligible proportion of those affected are considered for the operative procedures at all.
In general, according to the Kiel Pain Clinic, three different invasive therapy strategies for the treatment of cluster headaches can be distinguished: “Destructive procedures, local blockages and neuromodulatory procedures.” The chances of success are often extremely limited. For example, “destructive procedures such as the severing or decompression of the intermediate or superficial petrosus major and direct interventions in the area of the trigeminal nerve are only of historical importance due to unsatisfactory long-term results,” reports the Pain Clinic in Kiel.
Meanwhile, “neuromodulatory procedures have come to the fore because of the improved electronic stimulation possibilities.” In the blockade of certain nerve pathways, the blockage of the occipital nerve by the injection of local anesthetics and corticosteroids is a possible therapeutic approach, the operative methods such as the radiation of the entry zone of the nerve trigeminal nerve (gamma knife), which is strongly recommended for resections of the superficial petrosal nerve or sphenopalatine ganglion.
Possible neuromodulatory methods for cluster treatment are deep brain stimulation, occipital nerve stimulation and neurostimulation of the sphenopalatine ganglion. Deep brain stimulation concentrates on the area of the posterior, inferior hypothalamus and, according to the studies to date, can bring about an improvement in around 50 percent of the patients. However, the pain clinic in Kiel reported that massive side effects such as fatal intracranial bleeding had occurred in numerous patients. In addition, the procedure is associated with extremely high costs of over 30,000 euros and extensive post-operative treatment. “Based on the current data situation, neither a theoretically rational nor a practical reason for the use of deep brain stimulation in cluster headache can be understood,” explains the Pain Clinic Kiel. The previous data would not justify deep brain stimulation in therapy.
Occipital nerve stimulation is usually achieved by inserting a stimulation electrode in the area of the first cervical vertebra. Afterwards, a trial stimulation with an external pulse generator takes place over several weeks, “before a pulse generator is permanently implanted,” reports the Pain Clinic in Kiel. The effectiveness of the method is roughly equated by the experts with deep brain stimulation, whereby the occipital nerve stimulation offers the advantage that it is “less invasive and less complicated than deep brain stimulation”.
In 2011, neurostimulation of the sphenopalatine ganglion for the treatment of cluster headaches was also presented for the first time. This latest treatment method for cluster therapy is based on a tiny neurostimulator that is implanted in the gums without visible scars or cosmetic impairments and whose electrode tip attaches to the sphenopalatine ganglion (GSP) behind the cheekbones. An external remote control can be used to initiate stimuli that should alleviate the headache. So far, however, the method has only been little tested, so it is not possible to make a reliable statement about the effects and possible risks.
Overall, it can be said that the cluster headache is still not curable, but the intensity and frequency of the pain attacks can be reduced quite reliably based on various medicines. Over-the-counter pain relievers such as aspirin, paracetamol or ibuprofen have no effect and self-medication is strongly discouraged. Which agents are ultimately used for treatment is essentially determined by the clinical course, possible comorbidities and the general constitution of those affected. Surgery should only be considered for a minimal fraction of those affected.
A problem with the drug treatment approaches mentioned is that the drugs are often not approved for treatment in Germany. If they are nevertheless prescribed as part of a so-called off-label therapy, this can lead to difficulties in billing the health insurance companies. In order to prevent possible recourse claims, the DMGK provides the treating physicians with “evidence-based guidelines” and “valid publication lists for the individual indications” as argumentation aids. However, according to the German Society of Neurology, some medicines such as pizotifen and methysergide also have a procurement problem because they are no longer approved in Germany and can only be obtained as imported medicines.
While naturopathic treatment methods have promising approaches for other forms of headache, the options for cluster headaches are rather limited. Oxygen inhalation is a naturopathic approach that is also considered the treatment option of choice for acute cluster attacks in conventional medicine. However, other methods that are often used in naturopathy for headaches, such as relaxation procedures, stress management techniques, biofeedback, have magnetic therapy , acupuncture or dietary changes have no effect on cluster headache. Although those affected often associate the appropriate measures with the sudden disappearance of the complaints, the timing is purely coincidental.
When using homeopathic remedies, there are considerable doubts about the effect. In view of the low risk of side effects and the enormous pressure on the patient to suffer, an attempt at homeopathic treatment can nevertheless be considered if conventional therapeutic approaches do not achieve the desired success. Experienced therapists should, however, reserve the choice of the appropriate means.
A victim’s report
The individual medical history of the affected patients is often characterized by a long phase without an exact diagnosis, in which various therapeutic measures are tried out and the suffering of those affected increases significantly. I suffered my first cluster attack around 13 years ago at the age of 25. The pain affected the left side of my face and was incredibly intense. I started to sweat profusely, my nose was blocked and the eye watered slightly. I ran around the apartment uncontrollably for a little more than half an hour and tried desperately to get relief. In between, I felt like I was about to faint due to the pain. After the pain attack, I was initially completely symptom-free before two more episodes followed the next day.
A comparable pain, I had previously only experienced with an inflammation of the root, which remained untreated for far too long. However, the local pain was severely limited, while the cluster pain covered the area around the eyes and head. For me it was reasonable to assume that there might be a connection with the root canal treatment. I also thought of a possible outbreak of a previous sinus infection. However, since there were no further attacks for some time, I dismissed the complaints as a unique phenomenon. Unusual headache attacks were well known to me as a migraine patient. I only had real worries after the recurrence of an active cluster period with several pain attacks. The pain was simply unbearable.
I experience migraines with aura symptoms such as visual disturbances, numbness in the fingertips and an increased sensitivity to light. These can be determined before the onset of the headache and, as the course progresses, repeated vomiting usually follows. The symptoms can last for up to three days, whereby after the vomiting there is usually only an extreme headache and the aura symptoms subside. The temporal manifestation of migraines is therefore significantly more uncomfortable than with cluster pain. However, the intensity of the pain was much higher, which caused me considerable uncertainty.
An odyssey to various doctors began, in search of the causes of the complaints. First I went to an ear, nose and throat doctor to find out if there was any sinus infection. The stuffy nose during the pain attacks made me lean in that direction. But the otolaryngologist could not find any pathological events. It was also extremely difficult for me to describe the symptoms, since I no longer had any complaints when I went to the doctor. Next I remembered the previous root canal treatment and therefore went back to the dentist. Although there were no clear signs of an inflammatory event, he suspected an inflammation in the area of the treated tooth root as the cause of the symptoms.
It had been a good two years since the cluster headache first appeared and I had to deal with several other acute periods. The pressure of suffering increased and I underwent a root tip resection on the recommendation of my dentist, in the hope that the symptoms would be over. In fact, after the procedure, it took some time before the next acute period of cluster headache occurred, so I initially believed in a cure. The renewed pain attack after a year and a half was all the more frustrating. I contacted my family doctor again, who then referred me to a neurologist. After I finally got an appointment, he explained to me that he suspected cluster headache as the cause, but first a magnetic resonance imaging had to be done, to rule out more serious brain diseases. Again, I waited for an appointment for a while and then contacted the neurologist again. Around six years after the first pain attack, he then diagnosed me and prescribed a bottle of oxygen and two nasal sprays to treat the acute attacks.
Relieved by the final diagnosis, the question still arose how I should deal with the disease in the future. Because occasionally the pain attacks caught me during the day in quite unfavorable situations. Unfortunately, the oxygen did not have the desired effect for me, the nasal sprays did. However, the nasal sprays prescribed for me were intended for single use, i.e. an attack could be ended with their help, but for the next attack I needed a new nasal spray. Thus, theoretically, I should have been prescribed significantly more than two nasal sprays, especially since the number of pain attacks during the acute periods had meanwhile increased to more than four per day. However, I have a certain aversion to drugs and painkillers anyway, so I didn’t bother to prescribe any more. With the established diagnosis and the certainty that there was no more serious illness, the pain attacks, which were rather short compared to other cluster patients, were bearable without medication.
In addition, I had developed my own methods in the meantime to limit the duration and intensity of the pain attacks. Since I had heat as a trigger (more than 25 minutes in the warm bathtub most likely led to an attack), the conclusion was obvious that cold could provide relief. At the first sign of a cluster attack, I started rinsing my mouth with ice-cold water, gurgling with it, and leaning forward to draw in air against the resistance of the water.
If this did not alleviate the pain, I placed a cooling pillow from the freezer on the affected half of the face. In extreme cluster attacks, I took an ice cube and clamped it between my gums and cheek. Due to the cooling, the perceived pain intensity was significantly reduced and as soon as I could feel the pain of the ice on the tooth necks, the cluster headache was usually over. Today I only have a few cluster attacks per year that are not comparable in intensity to the initial attacks and that last for a maximum of half an hour. Often, with an appropriate early reaction or cooling, I can completely prevent the onset of an acute pain attack – but only if ice or very cold water is within reach.
I cannot say whether cooling can also help to relieve other patients. It also makes a difference whether an attack lasts a maximum of 30 minutes or 180 minutes. Ultimately, it should be noted for me that in rare cases the cluster headache still hinders me in everyday life, but overall I can live with the disease well today. Especially since theoretically there is also the option to tackle the pain with medication. The worst was the time without a confirmed diagnosis and possible countermeasures