Cluster headache is a primary unilateral cephalgia accompanied by periodic recurrent attacks in the periorbital region and combined with ipsilateral disorders of the autonomic nervous system. Diagnosis is based on clinical symptoms, anamnesis data, differentiation with other hemicranias. Treatment is represented by drugs from the groups of triptans, anticonvulsants and calcium channel blockers, glucocorticosteroids, topical anesthetics and oxygen inhalations. Suboccipital blockade and percutaneous stimulation of the occipital nerve, radiofrequency rhizotomy and other techniques are used.
ICD 10
G44.0
General information
Cluster or bundle headache (CH) is a rare form of cephalgia diagnosed in 0.1-0.4% of the population. Previously used names are histamine cephalgia, Harris migraine neuralgia, ciliary neuralgia. The prevalence of pathology ranges from 55 to 200 cases per 100 thousand population. Hereditary character can be traced only in 2.5-4% of patients. CH is much more often detected in men – there is a 3-4-fold predominance of males among patients. Women are characterized by two peaks of morbidity at the age of 15 to 20 years and from 45 to 50 years, in men this variant of pathology usually debuts at 20-29 years.
Causes
The exact etiology of the disease has not been established. It is believed that one of the leading roles in the development of the disease is played by the pathological activity of the hypothalamus, which is indirectly confirmed by the rhythmicity of the occurrence of headache episodes. Trigger factors that can cause new seizures are individual for each patient. According to statistics, most often the provocation of pain is caused by:
- Alcohol intake. Exacerbation can be potentiated even by small doses of alcoholic beverages. Alcohol-induced seizures are not usually prevented by drugs that are effective for CH. Pain syndrome occurs only in the cluster period – during remission, alcohol is rarely the cause of relapse.
- Medicines. Sometimes the episode is caused by medications such as histamine (administered intravenously or subcutaneously) and nitroglycerin (sublingual form).
- Psychoemotional and physical stress. In some patients, pain exacerbation is noted when watching TV or working with a personal computer for a long time. Deterioration of the condition can be observed with any tension and effort simulating the Valsalva test (holding your breath after a deep breath).
Pathogenesis
The mechanism of cluster headache development has not been thoroughly studied. It is proved that cephalgia is accompanied by narrowing of the ocular and proximal parts of the internal carotid arteries in combination with an increase in the level of “painful” neuropeptides. This confirms the fact of activation of the fibers of the V pair of cranial nerves. Circadian autonomic changes, namely, disturbances in the release of testosterone, prolactin, cortisol and some other hormones, fluctuations in blood pressure and body temperature indicate a connection between CH and dysfunctions of the hypothalamic-pituitary system and adrenal glands. Another proof of this connection is the activation of the gray matter of the posterior part of the hypothalamus during seizures according to PET results. A certain role in the pathogenesis of cluster hemicrania is played by the brain stem, which is confirmed by the development of attacks during sleep, changes in its structure (loss of the “rem sleep” phase before the onset of cephalgia), episodes of respiratory arrest during this period.
Classification
Depending on the features of the flow, several forms of bundle hemicrania are distinguished. The systematization is based on the frequency of seizures, the clinical symptoms of the disease are almost identical. Thanks to the use of this classification, the tactics of patient management and the choice of therapeutic agents are simplified. There are two variants of CH, which over time can transform from one to the other:
- Episodic. It is diagnosed in 80-90% of patients. There are more than 2 cluster periods with a total duration of 7 days to 1 year (usually from 14 to 90 days), between which there are clinical remissions lasting at least 30 days.
- Chronic. It is detected in no more than 20% of patients. It is characterized by the development of episodes of cephalgia for 1 year or longer without clinical remissions or with remissions not exceeding 1 month. Resistance to treatment is often found.
Cluster headache symptoms
Patients have typical constitutional features: high height, well-developed skeletal muscles. A distinctive personal characteristic is excessive ambition combined with indecision and a sense of inner helplessness. With exacerbations, the headache occurs acutely, reaches its peak in 1-3 minutes. The average duration of an episode is less than 1 hour, can range from 15 to 180 minutes. The attack stops as quickly as it started. Often there are series (“bundles”, “clusters”) from one attack in 2 days to eight (on average – from two to four) per day. Seizures usually occur at the same time, mostly at night. In the typical course of the disease, the period of exacerbation lasts from 14 days to 2 months.
Clinically, each individual attack is characterized by unilateral, more often left–sided unbearable pain of a burning or stabbing nature with an epicenter in the eyeball area. Patients describe it as a feeling of “squeezing out the eye from the inside”, “a blow with a red-hot knife in the eye”, “tearing the eye”. There is irradiation in the cheek, ear and teeth from the side of the lesion. Trigger zones are identified, under pressure on which the pain syndrome increases: the belt muscles of the neck, the eponymous muscles of the head, the trapezius muscle. Rarely, the pain spreads to the neck, shoulder and shoulder blade ipsilaterally. A third of patients experience nausea followed by vomiting, photophobia.
Cluster headache is characterized by a specific behavior of the patient during an attack – the inability to maintain a stationary horizontal position. This is due to the partial relief of pain syndrome with moderate physical activity. With an exacerbation, the patient may rush from side to side, clap his hands or beat the wall with his fist. A mandatory sign of a CH attack is vegetative disorders by the type of increased tone of the parasympathetic and hypoactivation of the sympathetic nervous system. Clinically, this is manifested by nasal congestion and mucous discharge, hypersalivation, conjunctival hyperemia, hyperhidrosis in the orbital region, Gorner syndrome (ptosis, myosis and enophthalmos) ipsilateral.
Diagnostics
The diagnosis of bundle cephalgia is based on the analysis of clinical symptoms and differentiation with other forms of paroxysmal hemicrania. When interviewing a patient, a neurologist carefully details complaints, clarifies the presence of trigger factors and episodes of similar pathologies among blood relatives. In case of atypical clinical manifestations, suspected traumatic injury, tumors or other morphological changes in the central nervous system, hardware imaging methods – CT and MRI of the brain can be used. After the exclusion of organic pathology, the doctor compares the survey data with the diagnostic criteria of CH according to the International Classification of Headaches (ICHD-II), which include:
A. The presence of at least 5 seizures that fall under the criteria B-D.
B. Extremely severe unilateral pain of periorbital or temporal localization, without treatment persisting for 15-180 minutes.
C. In addition headache is detected by one or more of the following signs:
- conjunctival injection and / or excessive tear production;
- pronounced swelling of the nasal mucosa and/or rhinorrhea;
- puffiness of the eyelids;
- hyperhidrosis of the forehead and face;
- dilation of the pupil or drooping of the eyelid;
- inability to maintain a calm lying position or agitation.
D. Frequency of seizures — from 1 time in 2 days to 8 times a day.
E. Lack of reliable connection with other probable disorders.
Differential diagnosis of CH is performed with migraine, trigeminal neuralgia, paroxysmal hemicrania. Distinctive features of migraine are the patient’s tendency to rest during the period of cephalgia, the presence of aura and burdened family history. For trigeminal nerve neuropathy, short-term pain lasting several seconds is typical, which is described by patients as an “electric shock”, an exacerbation of pain when the chewing muscles are strained. Unlike cluster cephalgia, patients with trigeminal neuralgia are afraid to touch the face, because this causes new attacks of pain. Paroxysmal hemicrania is more typical for women, the duration of the attack with it is no more than 30 minutes, and the frequency can reach 40 attacks per day.
Cluster headache treatment
Etiotropic treatment of cluster headache is currently absent. The pharmacotherapeutic agents used are aimed at combating the already developed pain syndrome, reducing the frequency of attacks in the structure of the “cluster”, preventing new attacks during the period of exacerbation. Thus, the following basic approaches are distinguished in the treatment of cluster headache:
- Elimination of an attack of cephalgia. Effective drugs in the fight against CH are triptans. If this group is intolerant, inhalations of 100% oxygen are used, nasal mucosa is applied with local anesthetics, ergot preparations are used less often.
- Prevention of new attacks. The basis of preventive treatment is calcium channel blockers (verapamil). In case of resistance, the appointment of glucocorticosteroids, antiepileptic drugs is recommended.
Surgical treatment of CH is resorted to when various combinations of drug therapy are ineffective. Invasive techniques are represented by blockade and percutaneous neurostimulation of the great occipital nerve, stimulation of the hypothalamus, radiofrequency thermocoagulation of the ganglion of the trigeminal nerve, radiofrequency trigeminal rhizotomy.
Prognosis and prevention
The probability of recovery directly depends on the features of the pathology and the patient’s response to the therapy. Cases of complete clinical remission of the disease have been described in medical practice. Cluster headache is characterized by a decrease in the frequency of seizures and the degree of their severity with age. Specific prevention of cluster cephalgia has not been developed. There is no evidence base regarding the prevention of new exacerbations with prolonged use of medications. Non-specific preventive measures consist in excluding or minimizing the impact of trigger factors that can cause new headache attacks.
