Cluster headache

Mareike Müller is a freelance writer in the medical department and assistant doctor for neurosurgery in Düsseldorf. She studied human medicine in Magdeburg and gained a lot of practical medical experience during her stays abroad on four different continents.

More about the experts All content is checked by medical journalists.

Cluster headache (Bing-Horton syndrome) is characterized by extreme, strictly unilateral headache attacks. Typically, the headache is accompanied by symptoms such as watery eyes or a runny nose. Sometimes there can be months between individual attacks. The quality of life is often massively impaired by the cluster headaches. Some patients even develop depression as a result of the stress. Here you can read everything you need to know about cluster headaches.

ICD codes for this disease: ICD codes are internationally recognized codes for medical diagnoses. They can be found, for example, in doctor's letters or on certificates of incapacity for work. R51

Cluster headache: description

Cluster headache manifests itself in one-sided, very severe headache attacks. The attacks last between 15 and 180 minutes on average and can occur several times a day. But there can also be months between the cluster pain episodes. The pain is so severe that it causes extreme suffering. It is not uncommon for a persistent cluster headache to develop into depression.

In addition to the headache, cluster headache also occurs on the affected side of the head or face. These include red, watery eyes, a runny nose, and profuse sweating on the face. These accompanying symptoms are an automatic reaction to the severe pain and are controlled by the so-called autonomic (vegetative) nervous system.

In Germany around 120,000 people are affected by cluster headaches, three times more men than women. In about two to seven percent of patients with cluster headache, the disease occurs more frequently in the family. A genetic component therefore appears to contribute to the development of the disease. However, exactly which genes are involved is still the subject of research. In principle, cluster headaches can occur at any age. Men between the ages of 20 and 40 are most likely to get the disease, especially around the age of 30.

Cluster headache: symptoms

Cluster headache is attack-like and strictly one-sided. The pain is very intense and is perceived as piercing or cutting. The patients feel the greatest pain behind the eye. They often describe the cluster headache as "a red-hot knife in the eye" or "a burning thorn in the temple". The cluster headache never occurs on both sides of the head at the same time and usually remains confined to one half of the head for the duration of the disease. Only in a few cases does he change sides.

In addition to the pain, there are the following cluster headache symptoms in the affected half of the face:

• Watery eye
• Reddened conjunctiva of the eye
• Eyelid swelling
• Runny nose
• Sweating of the forehead or face
• Horner syndrome

In cluster headache, Horner's syndrome, characterized by three symptoms, is often observed on the side of the face affected by the pain. These include a narrowed pupil, a drooping upper eyelid and an eyeball that has sunk slightly into the eye socket. However, Horner's syndrome does not only occur with cluster headaches. It is also possible with numerous other diseases.

In addition, over 90 percent of patients are extremely restless during a cluster headache attack. This also distinguishes them from migraine patients. For example, they walk up and down the room or bob apathetically with their upper body (so-called pacing around). Migraine sufferers, on the other hand, seek absolute rest and try to move as little as possible.

Typically, cluster headaches always start at the same time of day, most commonly an hour or two after falling asleep or in the early hours of the morning. Many sufferers can “set the clock according to their headache”. They also occur more frequently in spring and autumn. The individual attacks last between 15 and 180 minutes. The intervals between attacks are very different. You can torture patients every other day or up to eight times a day. For some patients, there are weeks and months between episodes with cluster pain attacks in which they are symptom-free.

Some patients develop depression due to the severity of the pain and the deterioration in quality of life. In extreme cases, they see their last resort in suicide. It is therefore important that any emotional complaints the patient may have are taken seriously and treated professionally.

Cluster headache: causes and risk factors

The causes and mechanism of development of a cluster headache are currently not exactly known. Since the attacks occur in a certain rhythm of the day and the season (especially after falling asleep, in the early morning hours, in spring and autumn), it is assumed that the underlying biological rhythms are incorrectly controlled. The control of the sleep-wake rhythm regulates, among other things, the diencephalon, the hypothalamus. Experts suspect that the attacks originate in this region of the brain and that the autonomic nervous system and the trigeminal nerve maintain them. Studies have shown that the brain region around the hypothalamus is more active in cluster headache patients.

The inheritance of cluster headache is also discussed. First-degree relatives are about 18 times more likely to develop cluster headaches as well. It has not been conclusively clarified whether certain substances or situations trigger the respective cluster headache attack. However, doctors assume that alcohol and nicotine in particular, but also chocolate, nuts, cheese, foods containing histamine, flickering lights (TV, cinema), stays at high altitudes, physical exertion and vasodilating drugs (such as nitroglycerin) are the triggers for cluster headaches could.

Cluster headache: examinations and diagnosis

The right person to contact if you suspect cluster headache is your family doctor or a neurologist who specializes in headaches. The diagnosis of cluster headache is a purely clinical diagnosis. This means that the doctor can find out whether it is a cluster headache or not on the basis of the symptoms alone. The medical history (anamnesis) is particularly important for this. When you come to the practice with a headache, your family doctor will ask you these questions, among others:

• When did you have the headache for the first time?
• How often have you had this type of headache and at what interval?
• How long do the headache attacks last?
• Can you describe where exactly the pain is localized and how it feels?
• Did you notice any other symptoms during the headache, such as watery eyes or runny nose?
• Is there anything that you can do to relieve the pain?
• Is there a specific situation in which the headache occurs?

In addition, the doctor will examine you neurologically. As a rule, this examination is normal for cluster headaches. For example, the doctor checks the light reaction of your pupil in the eye and tests muscle strength and sensitivity in various places in the body.

The doctor can then use the diagnostic criteria of the International Headache Classification (ICHD-2) to determine whether a cluster headache is present. These cluster headache criteria are:

a) At least five attacks that meet criteria b to e
b) Strong or very strong unilateral pain attacks in the eye area that last 15 to 180 minutes if left untreated
c) In addition, at least one of the following cluster characteristics occurs on the painful side:

• Watery eyes and / or reddened conjunctiva
• Runny or stuffy nose
• Eyelid swelling
• Sweating of the forehead or face
• Narrowed pupil and / or drooping eyelid
• Physical restlessness

d) The frequency of attacks is between one attack every other day and one to eight attacks per day
e) The symptoms are not due to any other illness

If the pain occurs for the first time or if the doctor detects neurological deficits, computed tomography of the head (CCT) or magnetic resonance imaging (MRI) of the brain should be performed in order to rule out any inflammation, tumors or other causes of the symptoms. If necessary, the intraocular pressure is also measured during the first attack to rule out glaucoma. Other examinations, such as examining blood or nerve fluid (liquor) or recording brain waves with electroencephalography (EEG) are sometimes also necessary.

The three clinical pictures migraine, the rare, very similar form of headache paroxysmal hemicrania and trigeminal neuralgia, in which a diseased facial nerve causes hellish pain, must be distinguished from cluster headache. Primarily because atypical accompanying symptoms such as sensitivity to light can occur, the diagnosis of cluster headache cannot always be made immediately. Therefore, the actual disease is often only recognized late. Sometimes there is initially a suspicion of dental disease or sinus infections, which lead to incorrect or delayed therapy.

Cluster headache: treatment

Cluster headache treatment is often difficult. Those affected should definitely contact a doctor who specializes in this. The usual pain relievers for the treatment of headaches (aspirin, ibuprofen, diclofenac and even opioids) are usually absolutely ineffective for cluster headaches.

Medicines prescribed for cluster headache therapy suppress the pain, but do not in themselves lead to a cure. However, the drugs can reduce the strength and frequency of attacks. Most patients respond well to drug cluster headache therapy. Other methods are available for the remaining patients, some of which are still experimental.

Drug cluster headache therapy is a priority. Acupuncture, relaxation exercises or biofeedback are often less effective than migraine treatment.

In cluster headache treatment, a distinction is made between attack therapy (acute treatment) and preventive treatment (cluster headache prophylaxis). If those affected also suffer from emotional complaints (e.g. depression), these must also be treated in any case. Chronic pain such as cluster headaches also cause psychological problems for many people. Those affected should therefore definitely seek professional help to treat their mental complaints.

Acute therapy of a cluster headache attack (acute treatment)

The so-called triptans are very effective against cluster headache in acute attacks. This group of drugs is also used in the treatment of migraines. They are either injected into the subcutaneous fat tissue (sumatriptan) or given as a nasal spray (zolmitriptan). That way they work faster. Taking triptans as tablets only makes sense for a few patients. However, triptans are not suitable for prevention, as they can cause headaches themselves if they are taken continuously.

In a cluster headache attack, inhaling pure oxygen leads to freedom from pain in over half of the cases. The patient inhales oxygen through a face mask for 15 to 20 minutes. He sits with a slightly bent upper body. It is not known why oxygen works against cluster headache attacks and why it only helps some patients - and not always them either.

The situation is similar with local anesthetics (e.g. lidocaine), which are dripped or sprayed into the nostril of the painful half of the head. Lidocaine helps about 30 percent of the time by blocking the nerve pathways that transmit the pain. Even if oxygen and local anesthetic therapies do not help every patient, they should be tried at least once.

Prophylactic cluster headache therapy

There are effective drugs to reduce the frequency and severity of attacks. Above all verapamil. This calcium antagonist, which is mainly used for cardiac arrhythmias and high blood pressure, must be taken continuously. As a rule, it is well tolerated, but controls of the heart function (e.g. by means of an EKG) are necessary. Verapamil only starts to work after two to three weeks. To bridge this period of time, glucocorticoids can be taken initially. These should not be administered over a longer period of time, a maximum of four weeks. However, there are some patients for whom only glucocorticoids help in the long term.

Lithium, topiramate or, in exceptional cases, methysergide are available as further means of preventing cluster headaches. Due to their side effects and lower effectiveness compared to verapamil, they are only second choice.

Operative method of cluster headache therapy

If all drug therapy attempts fail, surgical procedures can be used to treat the cluster headache. Many of these methods are still experimental and no long-term observations are available. Structures can be permanently damaged by surgical interventions, which can lead to new complaints. Cluster headache operations should only be performed in specialized centers.

Occipital nerve stimulation (ONS): A comparatively less invasive surgical method is the blockage or stimulation of the occipital nerve. Among other things, this sensitizes parts of the hairy scalp. It can be blocked or stimulated using local anesthesia and cortisone injection or electrical stimulation therapy in order to provide temporary relief.

Deep brain stimulation: If this procedure does not provide sufficient relief, deep brain stimulation can also be used, which is also used, for example, to treat Parkinson's disease. In deep brain stimulation of the hypothalamus, electrodes are inserted into the part of the brain that is believed to be responsible for the cluster headache. This surgical therapy is riskier than treatments on the occipital nerve. All in all, all surgical interventions should only be carried out in specialized centers with a focus on headaches, and then only if drug therapy for cluster headache has failed in a patient.

Cluster headache: disease course and prognosis

Cluster headache symptoms occur in a certain rhythm in most patients. This means that headache attacks occur more frequently in spring and autumn. Another characteristic of the course of cluster headache is that the patients always have the attacks at the same time of day, mostly shortly after falling asleep or in the early morning hours.

The cluster headache is chronic and recurrent. That means, it keeps reappearing over many years. Around 80 percent of patients report that cluster headaches recur 15 years after the first attack. There are two types of cluster headache: episodic (80 percent of cases) and chronic (20 percent) cluster headache. In around twelve percent of cases, the episodic attack turns into a chronic headache.

In episodic cluster headache, the episodes of repeated headache attacks last a few weeks to months. This is followed by a period of months to years during which there are no symptoms. In chronic cluster headache, the pain periods last over a year or the symptom-free intervals between the individual periods are shorter than a month.

Many patients can be helped with suitable medication. The frequency and strength of the cluster periods are reduced. If the patient knows his individual trigger for the attacks (for example alcohol), he can avoid it and thus further reduce the frequency of pain periods. The cluster headache is currently not curable, but spontaneous healing is possible at any time.

Tags:  womenshealth first aid laboratory values