Bundle headache

Cluster headache is a primary form of cephalgia, manifested by attacks of very intense, strictly unilateral pain in the orbital, supraorbital, temporal or mixed localization, lasting 15-180 minutes, occurring daily with a frequency of once every 2 days to eight times a day. Attacks on the side of pain are accompanied by one or more of the following symptoms: conjunctival injection, lacrimation, nasal congestion, rhinorrhea, sweating of the forehead and face, miosis, ptosis, and eyelid edema. The clinical picture is the decisive criterion for diagnosis. To stop an attack, oxygen inhalation, triptans, ergotamine or a combination of them are used. To prevent attacks, verapamil, methysergide, lithium valproate or a combination of them are prescribed.

The incidence of cluster headache in the population is low - 0.5-1%. Men suffer 3-4 times more often than women, the onset of the disease occurs at 20-40 years. In 5% of patients, the disease is hereditary.

In the United States, the incidence is 0.4%. In most cases, cluster headaches are episodic; during cluster periods, the patient experiences daily attacks (one or more) of cluster headaches for 1-3 months, followed by a long remission of several months to several years. In some patients, cluster headaches occur without periods of remission.

The pathophysiology of cluster headache is not fully understood, but its periodicity suggests hypothalamic dysfunction. Alcohol consumption triggers headaches during cluster periods but not during remission.

Synonyms: cluster migraine, histamine cephalgia, Horton's syndrome, Harris's migraine neuralgia, ciliary neuralgia, erythromelalgia of the head, Bing's erythroprosopalgia.

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What causes cluster headaches?

It has been shown that the cluster period (including the first "bundle") can occur after a disruption of the usual daily rhythm: changing time zones during air travel, sleepless nights, a 24-hour work schedule, etc. During a painful "bundle", as well as in the chronic form of cluster headache, attacks can be provoked by alcohol, histamine or nitroglycerin. A close connection between attacks of cluster headache and night sleep has been noted: night attacks are considered obligatory for this form of cephalgia. It is curious that during the period of remission, no provocateurs are capable of causing an attack of cluster headache.

Symptoms of cluster headaches

The most typical characteristics of cluster headaches are their unbearable nature, repeated occurrence during the day and night, vivid vegetative manifestations on the face, and a peculiar course of the disease - the occurrence of pain attacks in series, or "clusters". The duration of a cluster is from several weeks to several months with a clearly expressed remission lasting from several months to several years (on average 2-3 years). 10-15% of patients have a chronic course without remissions, 27% develop only one episode of cluster headaches. Many patients are characterized by seasonal exacerbations: in spring and autumn. Unlike patients with migraine, a patient with cluster headache does not feel the desire to go to bed or retire to a quiet, darkened room, he is excited and restlessly walks around the room.

The pain is most often localized around the eye, in the brow, temple, or in several areas, but can spread to other areas of the head. Due to the unbearable pain, most patients experience motor restlessness, aggression, and agitation during an attack; suicide attempts are known during an attack of cluster headache. During a painful "cluster", the pain always occurs on the same side. Vegetative symptoms, including nasal congestion, rhinorrhea, lacrimation, facial flushing, and Horner's syndrome, are noted on the same side as the headache.

Patients with cluster headaches often have the so-called "lion and mouse" syndrome. Thus, men with this form of cephalgia usually have a characteristic appearance: an athletic, masculine build, thickened facial skin with telangiectasias and pronounced expression wrinkles - "lion face". At the same time, they are characterized by internal constraint, indecisiveness, they often have difficulty making decisions ("mouse heart").

Clinical types of cluster headache

The most common form of cluster headache is episodic, less common is chronic, when remissions are either absent or do not exceed 1 month. Chronic cluster headache (10-15% of cases) may develop de novo or originate from the episodic form. Some patients experience a transition from chronic to episodic. Some patients have described a combination of cluster headache and trigeminal neuralgia.

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Diagnosis of cluster headache

The diagnosis of "cluster headache" is based on the typical clinical picture (strictly one-sided pain in half of the face and head, accompanied by vegetative manifestations on the face: lacrimation, rhinorrhea, etc.) and on the characteristic course of the disease (alternation of painful periods, "clusters", with light intervals, remissions). Additional criteria of cluster headache are its unbearable nature and motor excitation, as well as the occurrence of attacks during night sleep. Traditional research methods (EEG, MRI, ultrasound Doppler) are uninformative. The diagnostic criteria of cluster headache are presented below.

3.1. Cluster headache (ICHD-4)

• A. At least five seizures meeting criteria for BD.
• B. Intense or extremely intense unilateral pain in the orbital, supraorbital and/or temporal region lasting 15-180 minutes without treatment.
• C. The headache is accompanied by at least one of the following symptoms on the side of the pain:
  • conjunctival injection and/or lacrimation;
  • nasal congestion and/or rhinorrhea;
  • swelling of the eyelids;
  • sweating of the forehead and face;
  • miosis and/or ptosis;
  • a feeling of restlessness (inability to remain still) or agitation.
• D. Frequency of attacks: from once every 2 days to eight times a day.
• E. Not associated with other causes (disorders).

Cluster headache is differentiated from other syndromes with unilateral headache and autonomic components, in particular chronic paroxysmal hemicrania with more frequent (>5 per day) and shorter (usually a few minutes) attacks and constant hemicrania characterized by moderately prolonged unilateral headache with overlapping short episodes of more intense pain. These two types of headache, unlike cluster headache and migraine, are effectively relieved by indomethacin, but at the same time respond poorly to other NSAIDs.

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Treatment for cluster headaches

Acute attacks of cluster headache can be treated with parenteral administration of a triptan or dihydroergotamine, as well as inhalation of 100% O2. Since cluster headaches significantly reduce work capacity due to the frequency and intensity of attacks, patients are recommended to take preventive drug therapy. For example, a single oral dose of prednisone (60 mg) will provide rapid protection until the effect of prophylactic drugs with a slower onset of action (verapamil, lithium, methysergide, valproate, topiramate) is evident.

During a painful "cluster", patients should avoid possible provoking factors: do not take alcohol and vasodilators, observe a sleep-wake regimen. In stopping attacks of cluster headaches, as well as migraine attacks, triptans (sumatriptan, eletriptan, zolmitriptan, etc.) have the greatest effect. Considering the frequency of attacks in cluster headaches (more than once a day) and the possibility of side effects, caution should be exercised when using triptans: do not exceed the permissible daily dose of drugs.

Basic approaches to treating cluster headaches

• Treatment of an attack (abortive therapy):
  • inhalation of oxygen;
  • triptans;
  • intranasal lidocaine.
• Prevention of an attack:
  • verapamil (80-240 mg/day);
  • lithium carbonate (300-900 mg/day);
  • valproic acid (600-2000 mg/day);
  • topiramate (50-100 mg/day);
  • gabapentin (1800-2400 mg/day).
• Surgical treatment:
  • radiofrequency thermocoagulation of the trigeminal ganglion;
  • radiofrequency rhizotomy:
  • microvascular decompression;
  • neurostimulation.

In case of episodic form and relatively mild course, lithium carbonate and verapamil have a good effect, if necessary, a combination of these drugs is possible. In case of more severe course (more than five attacks per day, long duration of pain bundle - more than 2 months) the use of anticonvulsants and gabapentin is indicated.

Treatment of chronic cluster headache is difficult. If the above approaches are ineffective, glucocorticoids may be used for chronic cluster headache. Along with surgical methods, neurostimulation methods are used to treat chronic cluster headache that is resistant to other types of therapy: deep stimulation of the posterior hypothalamic region, stimulation of the greater occipital and vagus nerves (Shoenen, 2007). According to the first few studies conducted in Europe, the duration of remission after hypothalamic neurostimulation can reach 9 months. Due to the invasive nature of the above interventions and the likelihood of complications, careful selection of patients for this type of treatment is necessary. Currently, criteria for selecting patients with cluster headache for neurostimulation are under development.