Cluster headache (Bing-Horton syndrome) is characterized by extreme, severe unilateral headache attacks. Typically, the headache is accompanied by symptoms such as watery eyes or runny nose. Months can sometimes be between attacks. Quality of life is often severely affected by cluster headaches. Some patients even develop depression because of the stress. Here you can read all important information about cluster headache.
Cluster headache: description
Cluster headache manifests in one-sided, very severe headache attacks. The seizures last on average between 15 and 180 minutes and can occur several times a day. But there may also be months between the cluster pain episodes. The pain is so severe that it causes extreme suffering. It is not uncommon for depression to develop with ongoing cluster headache.
In addition to the headache, cluster headache also has accompanying symptoms on the affected head or face. These include a reddened, watery eye, a runny nose, and profuse facial sweating. These concomitant symptoms are an automatic response to the severe pain and are controlled by the so-called autonomic (autonomic) nervous system.
In Germany, about 120,000 people are affected by cluster headache, three times more men than women. In about two to seven percent of cluster headache patients, the disorder is more common in the family. A genetic component therefore seems to contribute to the development of the disease. However, exactly which genes are involved is currently the subject of research. In principle, the cluster headache can occur at any age. The most common men are between the ages of 20 and 40, especially around the age of 30.
Cluster headache: symptoms
Cluster headache occurs attack-like and strictly one-sided. The pain is very intense and is perceived as boring or cutting. Patients feel the strongest pain behind the eye. They often describe the cluster headache as “glowing hot knife in the eye” or “burning spine in the temple”. The cluster headache never occurs at the same time on either side of the head and is usually limited 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 face:
- Bleeding eye
- Reddened conjunctiva of the eye
- eyelid
- Runny nose
- Sweating in the area of the forehead or face
- Horner’s syndrome
In cluster headache one often observes that characterized by three symptoms Horner’s syndrome on the face affected by the pain. These include a narrowed pupil, a drooping eyelid and an eyeball sunken into the eye socket. However, Horner syndrome does not only occur in cluster headache. It is also possible with many other diseases.
In addition, over 90 percent of patients are extremely restless during a cluster headache attack. This feature also distinguishes them from migraine patients. For example, they run up and down in the room or apathetically bobbing their heads (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 occur at the same time of day, most often one to two hours after falling asleep or early in the morning. Many sufferers can put “the clock after their headache”. In addition, they occur frequently in spring and autumn. The individual attacks last between 15 and 180 minutes. The distances between the attacks are very different. You can torture patients every other day or up to eight times a day. Between episodes of cluster pain attacks, some patients have weeks and months of absence from symptoms.
Some patients develop depression due to the severity of the pain and the quality of life. In extreme cases, they see their last resort in suicide. Therefore, it is important that any emotional complaints of the patient are taken seriously and treated professionally.
Cluster headache: causes and risk factors
Causes and development mechanism of a Cluster Headache are currently not known exactly. Since the attacks occur in a particular daily and seasonal rhythms (especially after falling asleep, in the early morning, in spring and autumn), it is assumed that a malfunction of biological rhythms underlies. The control of the sleep-wake rhythm regulates, inter alia, the diencephalon, the hypothalamus. Experts suggest that the attacks occur in this brain region and that the autonomic nervous system and the trigeminal nerve maintain it. Studies have shown that the brain region around the hypothalamus is more active in cluster headache patients.
In addition, an inheritance of cluster headache is discussed. For example, first-degree relatives are about 18 times more likely to develop cluster headaches as well. Whether certain substances or situations trigger the respective cluster headache attack is not conclusively clarified. Doctors assume, however, that especially alcohol and nicotine, but also chocolate, nuts, cheese, histamine-containing foods, flickering light (television, cinema), stays at high altitudes, physical exertion and vasodilator drugs (such as nitroglycerin) are triggers of cluster headache could.
Cluster headache: examinations and diagnosis
The right contact for suspected cluster headache is the family doctor or a specialist in headache specialized neurologist. The diagnosis cluster headache is a purely clinical diagnosis. This means that the doctor can find out from the symptoms alone whether or not it is a cluster headache. This is the history of the disease (anamnese) particularly important. If you come for a headache, your family doctor will ask you these questions:
- When did you first have a headache?
- How often have you had this type of headache already and at what distance?
- How long do the headache attacks last?
- Can you describe exactly where the pain is located and how it feels?
- Did you notice any other symptoms during the headache, such as a watery eye 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 examines you neurologically. Typically, this study is inconspicuous in cluster headache. The doctor, for example, examines the light reaction of her pupil in the eye and tests muscle strength and sensitivity at various points in the body.
Subsequently, the doctor can determine whether a cluster headache exists based on diagnostic criteria of the International Headache Classification (ICHD-2). These cluster headache criteria are:
a) at least five attacksthat meet the criteria b to e
b) Strong or very severe unilateral pain attacks in the eye area, which take 15 to 180 minutes if left untreated
c) Accompanying occurs at least one of the following cluster characteristics on the aching side:
- Bleeding eye and / or reddened conjunctiva
- Running or stuffy nose
- eyelid
- Sweating in the area of the forehead or face
- Narrowed pupil and / or hanging eyelid
- Physical restlessness
d) The attack frequency is between one attack every other day and one to eight attacks per day
e) The symptoms are not due to another disease
When the pain first occurs, or when the doctor detects neurological deficits, a computerized tomography of the head (CCT) or magnetic resonance imaging (MRI) of the brain should be used to rule out any inflammation, tumors or other causes of the condition. If necessary, the intraocular pressure is additionally measured during the first attack in order to rule out glaucoma. Other examinations, such as the examination of blood or cerebrospinal fluid (CSF) or the derivation of brain waves with electroencephalography (EEG) are sometimes also necessary.
In particular, the three clinical pictures of migraine, the rare, very similar headache form of paroxysmal hemicrania and trigeminal neuralgia, in which a diseased facial nerve causes infernal pain, are to be distinguished from cluster headache. Above all, because atypical concomitant symptoms such as photosensitivity may occur, the diagnosis of cluster headache is not always immediately obvious. Therefore, the actual disease is often recognized late. Sometimes there is a suspicion of dental disease or sinusitis, leading to wrong or delayed therapy.
Cluster headache: treatment
The treatment of Cluster Headache is often difficult. Patients should definitely contact a specialist doctor. The usual analgesics for the treatment of headaches (aspirin, ibuprofen, diclofenac and even opioids) are usually absolutely ineffective in a cluster headache.
Medications prescribed for cluster headache therapy suppress the pain, but do not actually cure it. However, the drugs can reduce the strength and frequency of attacks. Most patients respond well to cluster headache medication therapy. Other procedures are available for the remaining patients, some of which are still experimental.
The medicinal cluster headache therapy is paramount. Acupuncture, relaxation exercises or biofeedback are often less effective than migraine treatment.
In cluster headache treatment, a distinction is made between the treatment of an attack (acute treatment) and preventive treatment (Cluster headache prophylaxis). If those affected additionally suffer from emotional problems (such as depression), they must always be treated as well. Chronic pains such as cluster headaches also cause a lot of mental problems for many people. Patients should definitely seek professional help to treat their mental health problems.
Acute therapy of a cluster headache attack (acute treatment)
Well-effective against cluster headache are the so-called acute attacks triptans, This group of medicines is also used in the treatment of migraine. They are either injected into the subcutaneous fatty tissue (sumatriptan) or administered as a nasal spray (zolmitriptan). In this way they work faster. Taking triptans as tablets is useful only in a few patients. triptans are suitable, however not for preventionbecause they can cause a headache even if taken permanently.
In a cluster headache attack, in more than half of the cases inhalation of pure leads oxygen for freedom from pain. Through a face mask, the patient breathes oxygen for 15 to 20 minutes. He sits with slightly bent upper body. Why oxygen works against cluster headache attacks and why it only helps some patients, and not all of them, is not known.
The situation is similar with the local narcotics (for example lidocaine), which are dripped or sprayed into the nostril of the aching head half. Lidocaine helps in about 30 percent of cases by blocking the nerves that transmit the pain. Although the oxygen and local anesthetic therapies do not help every patient, they should be tried at least once.
Prophylactic cluster headache therapy
In order to reduce the frequency and strength of attacks, there are good effective medicines. First of all verapamil, This calcium antagonist, which is mainly used in cardiac arrhythmias and high blood pressure, must be taken permanently. He is usually well tolerated, but controls of cardiac function (for example by means of ECG) are necessary. The effect of Verapamil begins only after two to three weeks. To bridge this period, you may initially glucocorticoids be taken. These should not be given over a longer period of time, for a maximum of four weeks. However, there are some patients who only use glucocorticoids in the long run.
As other remedies that prevent cluster headaches, stand lithium, topiramate or in exceptional cases methysergide to disposal. Because of their side effects and lower efficacy compared to verapamil, they are only second-line drugs.
Surgical procedures of cluster headache therapy
If all drug therapy attempts fail, one can resort to surgical procedures for the treatment of cluster headache. Many of these methods are still experimental and there are no long-term observations. Through surgical interventions structures can be permanently damaged, which may cause new complaints. Cluster headache surgery should only be performed in specialized centers.
Occipital nerve stimulation (ONS): A comparatively less invasive surgical method is the blockade or stimulation of the occipital nerve. This sensitizes, among other things, parts of the hairy scalp. With local anesthesia and cortisone injection or stimulation current therapy, it can be blocked or stimulated to provide temporary relief.
Deep Brain Stimulation: If this procedure does not provide adequate relief, deep brain stimulation (deep brain stimulation), which is used, for example, for the treatment of Parkinson’s disease, is also suitable. In the deep brain stimulation of the hypothalamus, electrodes are introduced into the part of the brain that is responsible for the cluster headache. This surgical therapy is riskier than the treatments on the occipital nerve. Overall, all surgical procedures should only be performed in specialized centers with a focus on headache, and only then if a patient has failed the therapy of cluster headache.
Cluster headache: disease course and prognosis
Cluster headache symptoms appear in a certain rhythm in most patients. This includes headache attacks frequently occurring in spring and autumn. Characteristic of the course of the cluster headache is also that the patients always have the attacks at the same time of day, usually shortly after falling asleep or in the early morning hours.
The cluster headache is chronically recurrent. That means he keeps reappearing over many years. About 80 percent of patients report recurrence of cluster headache 15 years after the first attack. Cluster headache distinguishes two forms: the episodic (80 percent of the cases) and the chronic (20 percent) cluster headache. In about twelve percent of cases, episodic attacks turn into a chronic headache.
At the episodic Cluster headache episodes of repeated headache attacks last several weeks to months. This is followed by a period of months to years in which no complaints occur. At the chronic Cluster headache persists for more than a year, or the symptom-free intervals between each period are less than one month.
With appropriate drugs many patients can be helped. This reduces the frequency and strength of cluster periods. If the patient knows his individual trigger for the attacks (for example, alcohol), he can avoid this and thus additionally reduce the frequency of the pain periods. It is curable Cluster Headache at the moment, a spontaneous cure is possible at any time.