Cluster headache

The concept of "trigeminal vegetative cephalgia" combines several rare forms of primary headache, combining both features of cephalgia and typical features of cranial parasympathetic neuralgia. Due to insufficient knowledge of doctors, the diagnosis of trigeminal autonomic cephalalgia often causes difficulties. Their classification is presented below.

Cluster headache and other trigeminal vegetative cephalalgia (ICGS-2, 2004)

• 3.1. Cluster (bundle) headache.
  • 3.1.1. Episodic cluster headache.
  • 3.1.2. Chronic cluster headache.
• 3.2. Paroxysmal hemicranium.
  • 3.2.1. Episodic paroxysmal hemicrania.
  • 3.2.2. Chronic paroxysmal hemicrania.
• 3.3. Short-term unilateral neuralgic headaches with conjunctival injection and lacrimation [CONS - from the English. Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing (SUNCT)].
• 3.4. Possible trigeminal vegetative cephalalgia.
  • 3.4.1. Possible cluster headache.
  • 3.4.2. Possible paroxysmal hemicranium.
  • 3.4.3. Possible short-term unilateral neuralgic headaches with conjunctival injection and lacrimation.

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

Cluster headache is a kind of pain (synonyms: cluster headache, cluster syndrome, cluster cephalgia, angioparalytic hemicrania, sympathetic hemizepheal vasodilation, etc.) derives its name from the nature of the course when the attacks follow series, bundles (English cluster - bundle , group, bunch), arising several times a day. Two variants of the cluster headache are known: episodic and chronic. Transition episodic to chronic occurs in a quarter of cases. Episodic type is characterized by bouts of pain during 1-3 months with subsequent remission from several months to a number of years. Chronic paroxysmal hemicrania is primary and secondary (after a period of episodic cluster headache).

This form of unilateral headache is much less common than migraine (from 0.4 to 6%), the bowl is found in men than in women. It begins at the age of 27 to 31 years, approximately 10 years later than a common migraine, prevails among the black population in comparison with white. There is a genetic predisposition - occurs 13 times more often in families with a cluster headache than in the general population.

The attack manifests itself in the sharpest pains of burning, drilling character in the ophthalmic, frontal-ophthalmic or temporal-ophthalmic region, sometimes the pain irradiates to the cheek, teeth, ear, less often to the neck, shoulder, shoulder blade. The attack of pain is accompanied by lacrimation, rhinorrhea, nasal congestion and congestion hyperemia on the side of the pain (in two thirds of cases). More than half of the patients develop an incomplete Bernard-Horner syndrome (ptosis, miosis) during an attack, puffiness of the eyelids, hyperhidrosis in the forehead or the entire half of the face appear. It is characteristic that patients during an attack can not lie. They are restless, rushing, groaning in pain, the intensity of which is so great that the 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, and darkened premises. The duration of pain varies from 10-15 minutes to 3 hours, with an average attack of pain of 45 minutes. Nausea and vomiting are noted in a third of cases. Attacks are repeated in series, "bundles", usually from 1 to 4, but no more than 5 times a day, usually at the same time (more often during sleep - "alarm clock" headache). Such seizures are repeated for 2-6 weeks or more, then disappear for several months or years. Exacerbations more often in autumn or spring are often associated with seasonal changes in light activity: cluster headache attacks increase with lengthening or shortening of daytime (which indicates the chronobiological nature of the disease).

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

Provoke a seizure of some vasoactive substances: nitroglycerin 1 mg under the tongue, alcohol, subcutaneously administered histamine, etc. It is paradoxical that taking a lot of alcohol prevents the development of an attack. This can explain the abuse of alcohol by a number of patients with clustered cephalalgia.

With persistent headaches, a thorough examination of patients is necessary to exclude the primary cause in the form of cerebral artery aneurysm, arterio-venous malformation, the tumor process, diseases of the paranasal sinuses (etmoiditis), glaucoma. It is also necessary to exclude migraine, trigeminal neuralgia, pheochromocytoma, paratrigeminal syndrome of Raeder (in the pathological process in the area of the gasser node or pituitary fossa is characterized by drilling pulsating pains in the eye area with spreading to the entire half of the face, combined with miosis or Bernard-Horner syndrome, sometimes, by diplopia , a violation of eyeballs movements, nausea, appears mainly in the morning, after sleep, but there is no typical "beam" and vegetative manifestations on the back, the bowl is ill nschiny), temporal arteritis, Tolosa-Hunt syndrome, myofascial syndrome, and others.

[1], [2], [3], [4], [5], [6]

Causes and mechanism of development of trigeminal autonomic cephalothia

Experimental and functional neuroimaging studies have shown that trigeminal vegetative cephalgia is accompanied by activation of a trigeminopasimpatic reflex with clinical signs of secondary sympathetic dysfunction. The mechanism of the actual pain attack with beam headache is similar to that of migraine: activation of the trigeminovascular system, the isolation of painful neuropeptides, and vasodilation. It is believed that the basis of the pathogenesis of the bundle headache is a violation of the pacemaker function of the hypothalamus, which determines the onset of pain periods and seasonality of exacerbations, and is clinically manifested in the daily frequency of seizures, the dependence of attacks on sleep periods, the peculiar behavior of patients, and mixed sympathetic and parasympathetic dysfunctions in attack time. With the help of a mechanism that remains unexplored for the time being, peripheral or centrally conditioned triggers cause the activation of certain hypothalamic zones (gray matter, including the suprachiasmal nucleus), which corresponds to the period of the painful bundle. Rhythmic activation of the hypothalamic region, in turn, leads to the activation of the trigeminovascular system, dilatation of the vessels of the dura mater, the isolation of pain neuropeptides (CGRP, substance P), and the actual pain attack. Stihanie exacerbation and the onset of remission indicates the normalization of hypothalamic activity. The nature of paroxysmal hemicrania and short-term unilateral neuralgic headaches with conjunctival injection and lacrimation remains unclear.

[7], [8], [9], [10], [11], [12], [13],

Pathogenesis of cluster headache

The pathogenesis of the cluster headache is not well understood. It is believed that the basis is the inferiority of regional sympathetic innervation on the side of pain. 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 the pain attack increases the secretion of histamine in the urine, the testosterone level drops in the blood plasma. Substantial importance is attached to the functional activity of substance P in the neurons of the ipsilateral trigeminal nerve and its connections to the pterygoid ganglion and perivascular sympathetic plexus of the internal carotid artery. During the attack of cluster headache, the concentration of substance P is significantly reduced. The substance P inhibitor somatostatin is effective in case of an attack of cluster headache. Treatment of cluster headache is carried out with ergotamine, metisegridum, lithium carbonate.