Cluster headache is characterized by periodic unilateral attacks of pain of the most severe intensity in the temporal and ocular regions, which primarily affect young men. Because of the unexplained etiology of cluster headache, there are no causal therapies. However, if therapy is started early, cluster headache can be treated well with medication.
What is cluster headache?
Infographic on the causes and symptoms of migraine and headache. Click image to enlarge. Cluster headache is the term used to describe an areal-dependent pain condition that is associated with severe hemifacial attacks of pain in the temples and eyes. The pain attacks occur in clusters up to eight times a day and can last 15 to 180 minutes if untreated. In 80 percent of cases, the periodic pain attacks (sometimes lasting weeks to months) alternate with symptom-free intervals lasting months to years (episodic cluster headache). In contrast, 20 percent of cases present with chronic cluster headache, which is characterized by pain attacks lasting more than a year without periods of remission (symptom-free periods of at least four weeks).
Causes
The causes of cluster headache are not well understood. Since the attacks occur more frequently in spring and autumn (meteorological transition phases), disturbances of the biorhythm are suspected as possible causes. Since cluster headache attacks occur more frequently at the same time of day, dysregulation in the brain areas that regulate the sleep-wake rhythm (hypothalamus) is suspected. In addition, genetic causes for cluster headache are assumed, although no single gene responsible for the disease has been identified so far. However, the likelihood of cluster headache is greatly increased (18-fold) if a first-degree relative has the disorder. Furthermore, specific trigger factors such as alcohol and nicotine use, bright or flickering light, altitude, physical exertion, and certain vasodilating agents (histamine, nitrates) can trigger attacks of cluster headache.
Symptoms, complaints, and signs
Cluster headache is characterized by sudden onset of severe drilling or cutting headaches. Patients experience stabbing pain behind the eye that feels like a red-hot knife in the eye. During a headache attack, only one side of the head is affected at a time. Simultaneous headaches on both sides never occur. In rare cases, however, the headache may switch sides of the head during an attack. In addition to the severe headache, there is also watery eyes, conjunctivitis, swelling of the eyelids, runny nose, profuse sweating of the face and forehead, and Horner’s syndrome on the affected side of the face. Horner’s syndrome is characterized by a drooping upper eyelid, a constricted pupil, and an eyeball that has sunk into the eye socket. Extreme restlessness during a cluster headache attack is also typical. This applies to more than 90 percent of those affected. This restlessness manifests itself in constant pacing back and forth as well as apathetic rocking with the upper body. The attacks occur especially in spring or autumn. Furthermore, the headaches always start at the same time often early in the morning or two hours after falling asleep. However, the intervals between headache attacks often vary. Sometimes there are weeks or months of symptom-free periods in between. A single attack can last between 15 and 180 minutes. Many patients also suffer from depression due to the severe attacks.
Diagnosis and course
Cluster headache is diagnosed on the basis of the typical symptoms. If, in addition to the characteristic pain attacks, redness of the eye and face, hyperhidrosis (excessive sweating), rhinorrhea (nasal secretion), lacrimation (lacrimation), miosis (pupil constriction), ptosis (drooping upper eyelid), eyelid edema (eyelid swelling), or restlessness of movement is noted during the attacks, cluster headache can be assumed. A nitroglycerine test (differentiation from migraine or trigeminal neuralgia) as well as neurological and imaging procedures (exclusion of tumors as the cause of the complaint) are used for confirmation and differential diagnosis.In addition, a glaucoma attack, which is similar to a cluster headache, should be excluded by an eye examination. Cluster headache usually has a chronic recurrent course, in which symptom-free intervals alternate with phases dominated by pain attacks even after years. However, cluster headache diminishes with age in terms of intensity and frequency of attacks.
Complications
Cluster headache causes the patient to experience a very uncomfortable headache. Usually, the person with a cluster headache can no longer concentrate or think clearly. The pain can also spread to the teeth, ears and neck, causing severe pain in these areas as well. Due to the cluster headache, ordinary work is no longer possible, so that the patient’s daily life is severely limited by these attacks. This can also have a negative effect on social contacts, when the patient withdraws or a slightly aggressive mood develops in the affected person due to the cluster headache. Often there is sweating, nausea and vomiting during these headaches. Due to the severe pain, there is also a physical restlessness until even panic attacks can be triggered. The eyes become red and irritated. In most cases, the cluster headache disappears by itself within a few minutes. However, the patient cannot work or perform any strong physical activities during this time. The treatment itself is usually carried out with painkillers or by the targeted addition of oxygen. If the pain is only temporary, there are no further complications. However, there is usually another cause for the cluster headache, so this can lead to unpredictable complications and other symptoms.
When should you see a doctor?
Headaches that last for several days or are severe in intensity should always be evaluated by a doctor. A visit to the doctor is also recommended if the pain spreads or other symptoms occur. Cluster headaches may cause impairment of other sensory modalities. Vision or hearing is often impaired. A physician should be consulted to determine the exact cause. If the unilateral pain leads to decreased performance or limitation in accomplishing everyday tasks, a physician should be consulted. A doctor should always be consulted before taking pain medication. If there are repeated sleep disturbances, fever or attention problems, a doctor should be consulted. If the headache triggers a bad posture of the head, muscle tension or hardening of the muscles in the neck, shoulder as well as back area can be triggered. In order to prevent the skeletal and muscular system from becoming ill, the complaints should be medically clarified and treated. If the headache results in reduced food intake, a doctor must be consulted if there is unwanted weight loss or a feeling of internal dryness. In addition, a visit to the doctor is necessary if psychological or emotional problems develop and the affected person finds the stresses very disturbing and debilitating.
Treatment and therapy
Therapy for cluster headache aims first to relieve pain during attacks (acute therapy) and second to prevent further attacks (prophylactic therapy or interval therapy). While conventional pain medication does not help or does not help enough in an acute cluster headache, inhalation of 100 percent oxygen usually provides pain relief after a few minutes. In some cases, sumatriptan (subcutaneous self-injection) or zolmitriptan (nasal spray) is used to treat acute cluster headache attacks. These two active substances, which belong to the group of triptans, suppress pain perception and block the release of pain-mediating neuropeptides. In addition to verapamil, lithium, glucocorticoids or methysergide are also used prophylactically during the pain-free interval in episodic cluster headache (interval therapy). In chronic cluster headache, verapamil or lithium is used as part of long-term therapy. A newer method is the so-called deep brain stimulation by a brain pacemaker.This procedure, known as neuromodulation, involves surgically inserting electrodes into the hypothalamus of the affected person, which modify the nerve signals in this area of the brain by means of appropriate electrical stimuli. However, this promising procedure, for which long-term results are not yet available, is used only when drug therapy has been ineffective or for chronic cluster headache with a very severe course.
Outlook and prognosis
The disease course of cluster headache is chronic-recurrent and is differentiated into a chronic and episodic course. Between episodes of pain attacks, there is complete freedom from symptoms. About 80% of patients have an episodic course. In some cases there is a transition from episodic to sporadic. The number of affected patients increases significantly in the spring and autumn seasons. Affected persons report always the same times of day when they suffer from the severe pain. They usually occur in the morning hours or immediately after falling asleep. In addition to the recurring characteristics of cluster headache, there may be long pauses between attacks of pain in an episodic course. Often patients are symptom-free for years until a new episode of cluster headache subsequently occurs. The episodic cluster headache lasts for a few weeks or months until it disappears completely. In a chronic course of the disease, the pain attacks last for more than a year until there is a period of freedom from symptoms. Here, the intervals until the next occurrence are limited to a few weeks. A permanent spontaneous healing of the cluster headache is possible at any time in both courses. During the periods of pain, medication is used to reduce the discomfort. If the individual trigger is known, this itself can be avoided.
Prevention
Because the causes of cluster pain are not clear, no preventive measures exist. However, trigger factors such as histamine– and tyramine-containing substances (contained in alcohol, nuts, cheese, chocolate, tomatoes, citrus fruits, among others) as well as bright or flickering light, high-altitude air, and nicotine consumption should be avoided to prevent a cluster headache attack.
Follow-up
During and after medically supervised therapy, measures should be taken at home to minimize cluster headache symptoms. Taking specific nutritional supplements is recommended:
Affected individuals are often deficient in magnesium. Taking magnesium supplements can minimize the frequency of cluster headaches. Vitamin B2 can reduce the severity and frequency of attacks. Incomplete relief from conventional treatment methods may be improved by taking melatonin. Lifestyle should be adjusted according to the disease. Thus, appropriate exercise is recommended. Regular exercise stimulates blood circulation and reduces stress. Yoga classes and meditation can also play their part in providing relief. Breathing exercises that ensure adequate intake of oxygen can ease the severity of an attack and reduce its duration. Sufferers should also ensure that sleep patterns are maintained and that they go to bed at consistent times. Ginger tea acts on the vanilloid receptors and reduces vomiting. It helps to relieve the attacks. It is recommended to drink one to two cups daily. It is also advisable to avoid addictive substances such as alcohol, tobacco or narcotics of any kind. This is especially true during an attack. The aforementioned substances aggravate the symptoms of cluster headaches.
This is what you can do yourself
Cluster headaches can only be completely cured in a few cases. However, there are a number of strategies that can help manage the condition. Since the headache attacks are often triggered by so-called triggers, a prudent approach to the symptoms is recommended in the first place. Do headaches occur after the consumption of alcohol? Or do the symptoms occur after taking medication, dietary supplements or certain foods? Affected persons should answer these questions with the help of a sickness diary. Appropriate precautions can then be taken to prevent further attacks or at least alleviate the symptoms.Typical trigger factors are also noise, flickering light, extreme heat or changes in altitude – these should be avoided at all costs. If the cluster headache does not subside despite all the measures taken, therapeutic advice is required. Accompanying acute attacks can be alleviated by sports, meditation or autogenic training. Constant monitoring of the triggers and effective countermeasures can at least reduce the symptoms in the long term. Sometimes, however, cluster headaches can be relieved by simple medications, so sufferers should consult a physician in any case.
