Cluster headache

The term "trigeminal vegetative cephalgia" unites several rare forms of primary headache, combining both features of cephalgia and typical features of cranial parasympathetic neuralgia. Due to the lack of awareness of doctors, the diagnosis of trigeminal vegetative cephalgia often causes difficulties. Their classification is presented below.

Cluster headache and other trigeminal autonomic cephalgias (ICHD-2, 2004)

• 3.1. Cluster headache.
  • 3.1.1 Episodic cluster headache.
  • 3.1.2. Chronic cluster headache.
• 3.2. Paroxysmal hemicrania.
  • 3.2.1. Episodic paroxysmal hemicrania.
  • 3.2.2. Chronic paroxysmal hemicrania.
• 3.3. Short-lasting Unilateral Neuralgiform Headaches with Conjunctival Injection and Tearing (SUNCT) [CONX - from English Short-lasting Unilateral Neuralgiform Headache Attacks with Conjunctival Injection and Tearing (SUNCT)].
• 3.4. Possible trigeminal autonomic cephalgia.
  • 3.4.1. Possible cluster headache.
  • 3.4.2. Possible paroxysmal hemicrania.
  • 3.4.3. Possible short-term unilateral neuralgic headaches with conjunctival injection and lacrimation.

Among all trigeminal vegetative cephalgias, cluster headache is the most common. Less common are paroxysmal hemicrania and short-term unilateral neuralgic headaches with conjunctival injection and lacrimation.

Cluster headache is a type of pain (synonyms: cluster headache, cluster syndrome, cluster cephalgia, angioparalytic hemicrania, sympathetic hemicrania vasodilation, etc.) that got its name from the nature of the course, when attacks follow in series, in bunches (English cluster - bunch, group, bunch), occurring several times a day. There are two types of cluster headache: episodic and chronic. The transition from episodic to chronic occurs in a quarter of cases. The episodic type is characterized by attacks of pain for 1-3 months, followed by remission from several months to several years. Chronic paroxysmal hemicrania can be primary and secondary (after a period of episodic cluster headache).

This form of unilateral headache is much less common than migraine (0.4 to 6%), and is more common in men than in women. It begins between the ages of 27 and 31, about 10 years later than regular migraine, and is more common among blacks than whites. There is a genetic predisposition - it occurs 13 times more often in families with cluster headaches than in the general population.

An attack manifests itself in the sharpest pains of a burning, boring nature in the eye, frontal-orbital or temporo-orbital region, sometimes the pain radiates to the cheek, teeth, ear, less often to the neck, shoulder, shoulder blade. An attack of pain is accompanied by lacrimation, rhinorrhea, nasal congestion and hyperemia of the conjunctiva on the side of pain (in two thirds of cases). More than half of patients during an attack develop incomplete Bernard-Horner syndrome (ptosis, miosis), swelling of the eyelids, hyperhidrosis in the forehead or the entire half of the face appear. It is characteristic that patients cannot lie down during an attack. They are restless, tossing and turning, groaning from pain, the intensity of which is so great that cluster headache is called "suicidal". The state of psychomotor agitation distinguishes this form of headache from migraine, in which patients try to lie down and prefer peace, silence, a darkened room. The duration of pain varies from 10-15 minutes to 3 hours, on average, an attack of pain lasts 45 minutes. Nausea and vomiting are observed in a third of cases. Attacks are repeated in series, "clusters", usually from 1 to 4, but not more than 5 times a day, as a rule, at the same time (more often during sleep - "alarm clock" headache). Such attacks are repeated for 2-6 weeks or more, then disappear for several months or years. Exacerbations are more common in autumn or spring, often associated with seasonal changes in light activity: attacks of cluster headaches become more frequent with lengthening or shortening of daytime (which indicates the chronobiological nature of the disease).

The patients have a characteristic appearance: tall, athletic build, with transverse folds on the forehead (lion face), plethoric face, telangiectasias are not uncommon. By nature, such patients are often ambitious, prone to arguments, outwardly aggressive, but internally helpless, timid, indecisive ("the appearance of a lion, but the heart of a mouse").

Some vasoactive substances provoke headache attacks: nitroglycerin 1 mg sublingually, alcohol, subcutaneously administered histamine, etc. Paradoxically, drinking large amounts of alcohol prevents the development of an attack. This may explain the abuse of alcohol by a number of patients with cluster cephalgia.

In case of persistent headaches, a thorough examination of patients is necessary to exclude the primary cause in the form of aneurysm of the cerebral vessels, arteriovenous malformation, tumor process, diseases of the paranasal sinuses (ethmoiditis), glaucoma. It is also necessary to exclude migraine, trigeminal neuralgia, pheochromocytoma, paratrigeminal Raeder syndrome (in case of a pathological process in the area of the Gasserian node or pituitary fossa, it is characterized by boring pulsating pain in the eye area spreading to the entire half of the face, combined with miosis or Bernard-Horner syndrome, sometimes, diplopia, impaired eye movements, nausea, appears mainly in the morning, after sleep, however, there is no typical "bundling" and vegetative manifestations on the back, women are more often affected), temporal arteritis, Tolosa-Hunt syndrome, myofascial syndrome, etc.

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Causes and mechanism of development of trigeminal autonomic cephalgia

Experimental and functional neuroimaging studies have shown that trigeminal autonomic cephalgias are accompanied by activation of the trigeminoparasympathetic reflex with clinical signs of secondary sympathetic dysfunction. The mechanism of the actual pain attack in cluster headaches is similar to that in migraine: activation of the trigeminovascular system, release of pain neuropeptides, vasodilation. It is believed that the pathogenesis of cluster headaches is based on a violation of the pacemaker function of the hypothalamus, which determines the occurrence of painful periods and the seasonality of exacerbations, and is clinically manifested in the daily periodicity of attacks, the dependence of attacks on sleep periods, peculiar behavior of patients, as well as mixed sympathetic and parasympathetic dysfunctions during an attack. By a mechanism that remains unclear, peripheral or centrally conditioned triggers cause activation of certain areas of the hypothalamus (gray matter, including the suprachiasmatic nucleus), which corresponds to the period of the pain bundle. Rhythmic activation of the hypothalamic region, in turn, leads to activation of the trigeminovascular system, dilation of the vessels of the dura mater, release of pain neuropeptides (CGRP, substance P) and the actual pain attack. The abatement of the exacerbation and the onset of remission indicate normalization of hypothalamic activity. The nature of paroxysmal hemicrania and short-term unilateral neuralgic headaches with conjunctival injection and lacrimation remains unclear.

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Pathogenesis of cluster headache

The pathogenesis of cluster headache is not fully understood. It is believed that it is based on the deficiency of regional sympathetic innervation on the side of pain. The periodicity of the disease depends on the biorhythms of homeostasis with fluctuations in the level of vasoactive substances. Among biochemical disorders, great importance is attached to changes in histamine metabolism. During a pain attack, the excretion of histamine in the urine increases, the level of testosterone in the blood plasma decreases. Significant importance is attached to the functional activity of substance P in the neurons of the ipsilateral trigeminal nerve and its connections with the pterygopalatine ganglion and perivascular sympathetic plexus of the internal carotid artery. During an attack of cluster headache, the concentration of substance P decreases significantly. The substance P inhibitor somatostatin is effective in an attack of cluster headache. Treatment of cluster headache is carried out with ergotamine, methisegrid, lithium carbonate.