Cluster headache and paroxysmal hemicrania. Hemicrania is a terrible and uncontrollable pain in one side of the head. What is the difference between CPH and cluster headache?

: more than 5 attacks per day (more than half of all days).

Night attacks are not typical.

The duration of attacks ranges from 2 to 45 minutes.

Associated symptoms include the same autonomic phenomena observed in cluster headaches.

Indomethacin - sensitive headache. Indomethacin is always effective at a dose of 75 to 150 mg/day 1 .

Episodic paroxysmal hemicrania (PH) and chronic paroxysmal hemicrania are rare syndromes characterized by the appearance of short-term frequent attacks of headache with accompanying autonomic symptoms. Clinically, attacks of PG resemble short attacks of cluster headaches, but occur with greater frequency throughout the day. Unlike cluster headaches, which are more common in men, PH is more common in women (sex ratio is 3:1). As with cluster headaches, if daily headache attacks occur over several months, followed by periods of remission, the episodic form of PH is diagnosed. In patients with continuous attacks for a year or with remission of less than 1 month, a chronic form of PG is established.

Clinical manifestations

The most important feature to differentiate paroxysmal hemicrania from cluster headache is the frequency of attacks during the day. With paroxysmal hemicrania, the frequency of attacks is more than 5 per day (lasting more than half of all days throughout the disease). Pain in PG is characterized by significant intensity and is described as boring or tearing. Typically, headache attacks last from 2 to 30 minutes. Associated symptoms include the same autonomic phenomena observed in cluster headaches. Most patients with chronic PH experience lacrimation (62%), nasal congestion (42%), инъекция!} conjunctiva and rhinorrhea (36%) or ptosis (33%).

Treatment

Paroxysmal hemicrania is one of the types of headaches that is characterized by high sensitivity to indomethacin in all cases. The usual starting dose of indo-methacin is 1 tablet (25 mg) 3 times a day for three days. If there is no complete cessation of pain, the dose is increased to 2 tablets (50 mg) 3 times a day. In most patients, the effective therapeutic dose is 150 mg/day. A very dramatic therapeutic response is possible with rapid and пол!} significant disappearance of headache and associated symptoms. A positive therapeutic response is usually achieved within 48 hours after administration of indomethacin at an adequate dose. If there is no therapeutic response when indomethacin is administered at a dose of 75 mg 3 times a day, it is necessary to reconsider Diagnosis, -a; m. A brief medical report about the disease and condition of the patient, made on the basis of anamnesis and a comprehensive examination. From Greek - recognition, diagnosis, -i; and. 1. A set of techniques and methods, including instrumental and laboratory ones, that allow one to recognize the disease and establish a diagnosis. From Greek - capable of recognizing. 2. Diagnosis, dialysis, -a; m. peritoneal dialysis. A method for correcting water-alectrolyte and acid-base balance and removing toxic substances from the body when a dialysate solution is introduced into the abdominal cavity.

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Paroxysmal hemicrania is an independent disease that relates to vascular headaches. The fact that this is a separate nosological form, and not one of the types of cluster pain, was known back in 1974. However, nothing has yet been clarified about the causes of the disease and its development. It is assumed that this disease is a transformation of other paroxysmal pains.

Most often, in a ratio of 8 to 1, this pathology manifests itself in women, which distinguishes this type from cluster cephalgia. Some authors even consider this disease as an analogue of cluster cephalgia, which in most cases is detected in men.

Symptoms

For the first time, signs of the disease are detected in adulthood, although sometimes, but very rarely, children can also suffer from this disease. The main symptom is daily, very severe attacks of burning, throbbing or boring pain. It always covers only one side and manifests itself in the area of ​​the eye sockets, forehead and temple. Other symptoms completely coincide with the manifestations of cluster cephalgia:

1. Facial redness.
2. Redness of the eyes.
3. Tearing.
4. Nasal congestion.

It turns out that this form of headache is very similar to cluster headache, this is especially noticeable if you compare its intensity and localization, as well as additional manifestations. However, paroxysmal hemicrania has its own characteristics. Her attacks most often last only a few minutes, and there can be up to 10 or even more of them per day. But the difference in treatment is especially noticeable. For example, there are cases where an attack completely went away after taking several indomethacin tablets, although before this the patient had complained of discomfort for several years.

Clinical varieties

There are 3 types of this disease. Most often you can find chronic paroxysmal hemicrania. In this case, pain in the head area is observed every day for many years, without a period of remission.

The episodic clinical variety is characterized by the fact that a person has frequent daily attacks, but there are also long periods of remission.

And finally, prechronic paroxysmal hemicrania. It begins with rare episodes of headaches, but then becomes chronic without a period of remission.

Diagnostics

When diagnosing this disease, it is important not to make a mistake and not confuse this type of headache with cluster headaches. There is a special table for this, the answers in which will help make the correct diagnosis. In some cases, diagnosis requires only interviewing the patient and visual examination. It is very important to understand that the attack is accompanied by at least one of the following symptoms:

1. Redness of the eyes.
2. Tearing.
3. Nasal congestion.
4. Swelling of the eyelids.
5. Facial sweating.
6. Miosis or ptosis.

If the patient has at least one of the above symptoms, then paroxysmal hemicrania can be suspected.

If a person does not have any of the above signs, then additional examination is necessary. This could be a CT or MRI, because similar signs occur in other serious conditions, such as brain tumors or cysts.

As for the episodic variety of this disease, here the patient complains that pain in the head appears only for a while, for example, a month or even a year. But then sometimes complete remission occurs, in which a person considers himself completely healthy.

Treatment

The only drug that helps cope with the disease is. You can take it both in the form of tablets and in the form of suppositories. At the same time, paroxysmal hemicrania, the symptoms of which tormented a person for many months, disappears after taking the drug literally within a few days.

Treatment should be started with a dose not exceeding 75 mg. You need to take this dose of the drug 3 times a day, with a gradual increase to 250 mg, but only if the pain attacks continue. After the attacks have stopped and have not occurred for several days, the dose can be reduced to a maintenance dose, which ranges from 12.5 to 25 mg per day.

If there are no contraindications to taking indomethacin, the drug should be taken for many months, since after the drug is abruptly discontinued, the disease may return again. However, indomethacin should not be used in the following cases:

1. Allergy to the drug.
2. Erosion or ulcer of the stomach and intestines (exacerbation).
3. Hematopoiesis disorder.
4. Heart failure.
5. Pancreatitis.
6. Pregnancy.
7. Liver dysfunction.
8. Renal dysfunction.

As practice shows, indomethacin is the only drug that can fight pain of this type. All other drugs, including analgesics, do not help here. Unfortunately, not everyone knows this, and most people with a similar diagnosis take analgin or spasgan for many years, but their attacks never go away.

Paroxysmal hemicrania is a fairly rare type of headache. Characterized by severe short (from 2 to 30 minutes) and numerous attacks throughout the day. It is usually unilateral in nature and can spread to the fundus, temple, ear, and involve part of the neck and shoulder.

Since 1974, paroxysmal headache has been classified as a separate group of vascular pain, although the etiology of the disease and pathogenesis have not yet been fully identified by doctors. It is much more common (about 8 times) in women than in men, and is considered to be similar to male cluster cephalgia. There is reason to believe that paroxysmal headache is transformed from other forms of pain.

Symptoms

As a rule, signs of the disease initially appear in adulthood (very rarely in children). They manifest themselves as daily, very strong, but short-lived headache attacks. The nature of the sensations: burning, boring, deep, pulsating, but always one-sided, covering the ocular and frontotemporal lobe.

May be accompanied by:

• redness of the skin of the face;
• redness of the eyeball;
• tearfulness;
• swelling of the eyelids;
• ptosis (drooping of the upper eyelid) and miosis (constriction of the pupil);
• nasal congestion and/or rhinitis;
• sweating and damage to the sympathetic nerves of the pathological area.

The frequency of attacks varies up to 5 times a day, usually there is no connection with other causes of headache.

Thus, the symptoms of paroxysmal hemicrania are very similar to chronic cluster pain: intensity, localization of sensations, vegetative signs, etc.

Distinctive features: an increase in the frequency of attacks several times, a much shorter duration of the attack, predominance in women. In addition, there is a lack of response to prophylactic drugs that relieve cluster pain, and the cessation of attacks 1-2 days after the start of indomethacin therapy.

Types of disease

Attacks of paroxysmal hemicrania occur in periods that can last from one week to one year. Sometimes periods of attacks are followed by periods of complete remission (duration from a month to 1 year), when the symptoms of the disease are completely absent.

Types of clinical manifestations:

• Chronic. It occurs much more often than the others. Characterized by recurrence of attacks for 1 year or more without periods of remission or with very short relief (up to 1 month).
• Episodic. Characterized by at least 2 periods of pain attacks over 1 year and periods of remission lasting at least 1 month.
• Pre-chronic. It begins with rare periods of attacks (less than 2 times a year), which gradually become more frequent and become chronic.

Diagnosis and treatment

Diagnosis of the disease begins with collecting a clinical picture and examining the patient. More detailed differentiation is carried out according to the international classification of headaches. Paroxysmal is established based on the following criteria:

• At least 20 attacks.
• Severe attacks of unilateral pain localized in the orbital, supraorbital and/or temporal region. Constantly cover the same side. Lasts from 2 to 30 minutes.
• Unpleasant sensations are accompanied by at least one of the above symptoms.
• The frequency of attacks is 2-5 times a day, sometimes more.
• Onset of relief after taking indomethacin.

As additional studies, a CT or MRI may be prescribed to exclude pathologies of the cervical spine or brain tumors.

Treatment

Indomethacin is considered the most effective treatment for paroxysmal hemicronia. Therapeutic doses start at 75 mg per day, divided into 3 doses. As needed, the dose is increased to 250 mg during attacks. After the attacks stop, a gradual transition to a maintenance dose of 12.5-25 mg/day is carried out.

If there are no health-related contraindications to long-term use of indomethacin, then it is advisable to extend therapy to several months to avoid resumption of attacks. The use of analgesics or antispasmodics is unjustified, as they do not provide adequate relief.

Contraindications to taking indomethacin: individual intolerance, peptic ulcer, diseases of the liver, kidneys, gastrointestinal tract, heart, blood, bronchial asthma, pregnancy, lactation.

Hemicrania is simply a migraine, that is, acute headache pain, accompanied by strong pulsation, radiating to one of the hemispheres of the brain. This pathology can drag on for three days and cause a lot of suffering to the patient.

Migraines are classified into two types, namely:

1. An ordinary migraine, which usually affects the temple, crown, eyeball, and then spreads to the entire half of the head. An artery begins to protrude on the temple, which pulsates strongly, and the skin on the face becomes very pale. The pain is often accompanied by short-term immobilization of the eyeball, double images, dizziness, disturbances in the functioning of the speech apparatus, as well as abdominal pain, vomiting with nausea.
2. Ophthalmic migraine - this type of pathology occurs rarely and accounts for approximately 10% of all such lesions. Associated symptoms should be considered: visual disturbances, namely blurred images, fogging and short-term blindness. Bright lights, too loud noises, sneezing and coughing provoke pain.

Causes of the disease

Some doctors are of the opinion that the main cause of hemicrania is disturbances in intracranial blood flow. Others believe that this is a pathology of platelets or even the influence of serotonin, which causes severe vasoconstriction. While a person drinks coffee or tablets that contain serotonin, its concentration in the plasma decreases and it enters the urine, the vessels dilate sharply, causing severe pain.

It is important! Additional reasons include: severe stress, overheating in the sun, fatigue, eating foods that provoke an attack, and dehydration.

Paroxysmal form of the disease, its differences

Paroxysmal hemicrania makes itself felt through attacks of acute pain, accompanied by additional manifestations. The distinctive symptoms of the lesion include: short duration of attacks, which are characterized by the presence of nausea.

This form of pathology is more common in women and begins in adulthood, but some cases of infection in children are known.

Symptoms of the disease are also characterized by the fact that the frequency of pain attacks can reach up to 5 times per day and they last for 2 to 30 minutes. The attack can be prevented by taking indomethacin at a therapeutic dose. Pathology does not correlate in any way with other disorders in the functioning of the human body.

Episodic and chronic paroxysmal hemicrania is classified when a person suffers from attacks for one year or longer with remissions lasting up to one month. There are cases when the disease is combined with the trigeminal form of neuralgia.

Headaches are usually localized in the ear area or a little further than the eye. The pain is one-sided and only in rare cases does the affected side change. Sometimes the pain radiates to the shoulder.

It is important! A typical attack lasts from two to thirty minutes and some patients complain of mild pain during the interval between attacks. Attacks can recur many times throughout the day, and the time of painful attacks cannot be predicted.

Treatment of paroxysmal hemicrania is based on the organization of therapy with indomethacin - it is administered orally or rectally at least 150 and 100 mg, respectively. For preventive therapy, smaller dosages of the drug are also effective.

Pain relief with indomethacin is unpredictable. And the lack of pain control sometimes makes doctors doubt the correctness of the final diagnosis.

The dosage of indomethacin, which allows you to bring pain under control, varies from 75 mg to 225 mg and is divided into three doses during the day. The analgesic effect of this drug usually lasts for many years of life.

Considering that the disease is chronic, long-term use of the product can cause intestinal and kidney dysfunction.

Preventive therapy is effective only for some patients. Other medications and occipital nerve blockade have also shown positive results in some patients.

Hemicrania continua and its distinctive features

Hemicrania continua is a rare disease that mainly affects the female body. The pain is localized in the temple or near the eye. The pain does not go away, only its intensity changes - from mild to moderate. The pain is one-sided and can rarely change the side of the lesion, and the intensity most often increases.

The frequency of attacks of pain varies from multiple cases over the course of one week to single cases over the course of a month. As the frequency of attacks increases, the pain becomes moderate or very severe. During this period, it is supplemented by symptoms similar to cluster headaches - drooping upper eyelid, lacrimation, nasal congestion, as well as symptoms characteristic of migraine itself - sensitivity to bright light, nausea with vomiting. Symptoms may also be accompanied by swelling and twitching of the eyelid.

Some patients develop migraine-like auras during severe pain. The time for increased pain can last from several hours to several days.

It is important! The prognosis and timing of onset of primary headaches remain unknown. Approximately 85% of patients suffer from chronic forms without remission. Due to the fact that the correct diagnosis is not always made, the exact prevalence of the pathology remains unknown.

Patient examination and prevention

Recurrent headaches should definitely be the reason for visiting a neurologist. Diagnosis consists of interviewing and examining the patient. But hemicrania may indicate the formation of a tumor in the brain and other serious disorders. For this reason, it is necessary to organize a thorough neurological diagnosis in order to exclude malignant processes. You will also need to go to an ophthalmologist who will examine a person’s visual field, visual acuity, conduct a computed tomography and MRI scan, and examine the fundus of the eye. Subsequently, the neurologist will prescribe specific medications to help prevent an attack and relieve pain.

Preventive drug therapy for hemicrania is developed taking into account all provoking factors of the pathology. Concomitant diseases and emotional and personal qualities of a person are also taken into account. For prevention, various blockers, antidepressants, serotonin antagonists and other medications are used.

is a type of unilateral trigeminal headache accompanied by autonomic disorders. Typical episodes of the disease are characterized by short-term attacks of very strong throbbing or aching pain in the temple and/or orbit. Diagnosis is based on anamnestic information and compliance of the clinical picture with ICHD II criteria. A specific diagnostic and therapeutic feature of the pathology is the complete elimination of pain when taking indomethacin. If you are allergic to this drug, other NSAIDs, calcium channel blockers, or steroids are used.

ICD-10

G44.0 Histamine headache syndrome

General information

Paroxysmal hemicrania (PH) is a relatively rare disease. According to research, its prevalence is approximately 1-2.5% in the population. The average incidence rate is 55-385 cases per 100,000 population. The pathology was first described by Norwegian neurologists O. Jastad and I. Dale in 1974. This headache variant is classically viewed as predominantly affecting women, with a female to male ratio of approximately 2.5 to 7:1. The onset of the disease can occur at any age, but usually the first attacks are observed in people of young and middle age - from 20 to 40 years.

Causes

The exact etiology of the disease has not yet been established, but factors that can cause episodes of hemicrania have been identified. These include sudden turns of the head, drinking alcohol, stress, psycho-emotional overexcitation, and relaxation reactions immediately after a stressful situation. Sometimes attacks occur in response to excessive visual stimulation (prolonged viewing of TV, use of a smartphone) or taking certain pharmacotherapeutic agents, for example, nitroglycerin. In women, episodes are more common during menstruation. A reliable connection between the development of the first attacks of headache and organic lesions of the central nervous system has not been determined. It has been established that such clinical manifestations often develop in patients who have suffered a stroke or traumatic brain injury, suffering from neurofibromatosis and arteriovenous malformations with localization of the pathological process in the posterior cranial fossa.

Pathogenesis

The mechanisms of pain syndrome have not been thoroughly studied. There are several hypotheses. The probable role of vasomotor disturbances is indicated by the results of transcranial Dopplerography of cerebral vessels, according to which, during attacks of paroxysmal hemicrania, the blood flow velocity in the middle cerebral artery basins decreases ipsilaterally. Evidence of the possible involvement of the hypothalamic-pituitary system in the pathogenesis of the disease is the contralateral or bilateral activation of the posterior part of the hypothalamus during an attack according to neuroimaging data. Dysfunction of the trigeminal system is confirmed by the results of an electrophysiological study - a decrease in the RIII flexor reflex and the latency of the early component of the blink reflex.

Disruption of the autonomic nervous system in the ictal period is manifested by fluctuations in intraocular pressure and corneal temperature, hyperhidrosis in the forehead on the affected side. The rate of development of these symptoms indicates a connection between the etiology of attacks and neurogenic activation of functionally associated suprasegmental structures of the autonomic nervous and nociceptive systems. This may be due to the release of neuropeptides: calcitonin gene-related peptide (sensory endings of the trigeminal nerve) and vasoactive intestinal peptide (parasympathetic fibers).

Classification

Depending on the frequency of episodes of pain and its clinical features, it is customary to distinguish several forms of paroxysmal hemicrania. The use of such a classification in clinical practice allows for reliable differentiation between pathological variants and other short-term cephalgia for an adequate choice of appropriate treatment. Taking into account the frequency of attacks of PG and their characteristics, the following forms of the disease are distinguished:

• Episodic. It is distinguished by at least two painful periods with a duration of exacerbations from 1 week to 1 year and clinical remissions of at least 30 days. Determined in 15-25% of patients. The main localization of pain is the temporal region.
• Paroxysmal. Attacks are observed for more than 1 year without pain-free periods or with remissions, the duration of which does not exceed 1 month. Diagnosed in 75-85% of patients. The epicenter of pain is the orbital-temporal region.

Symptoms of paroxysmal hemicrania

Symptoms of PG during exacerbations occur daily with a frequency of 1 to 40, on average 5-10 attacks per day. The total duration of one episode ranges from 5 to 45 minutes, the average is 13 minutes. Pain syndrome with hemicrania is exclusively unilateral. The side does not change from attack to attack. In typical cases, the maximum severity of pain is observed around the orbit, in the temple or retro-orbital region. Less commonly, in the frontal, occipital or parietal areas, in the area of ​​innervation of the middle branch of the trigeminal nerve, around the nose or on the neck. Sometimes there is irradiation into the shoulder or arm on the affected side. The intensity of pain is high, but the nature may vary. Patients describe the pain as unbearable: throbbing, burning, boring, aching, stabbing, or punch-like.

The clinical picture of paroxysmal hemicrania usually increases rapidly during the first 1-5 minutes. In addition to headaches during an attack, autonomic disorders are determined by the type of local activation of the parasympathetic division of the autonomic nervous system. Clinically, this is manifested by increased lacrimation, injection of conjunctival vessels, a feeling of hot flashes, nasal congestion and catarrhal discharge. Hyperhidrosis of the forehead, photophobia, edema, drooping of the eyelid and constriction of the pupil ipsilaterally, and moderate nausea also develop. In some patients, these symptoms may precede a headache attack. In rare cases, vegetative manifestations are persistent or absent altogether. In the interictal period of PG, cephalgia does not occur; only 1/3 of patients note some discomfort in the area of ​​localization of the pain syndrome.

Diagnostics

The diagnosis is made by a neurologist based on special criteria corresponding to the International Classification of Headache II Revision (ICHD II). The results of laboratory research methods, neuroimaging in the form of CT and MRI of the brain do not provide diagnostically valuable information and play an auxiliary role in differentiating from organic pathologies of the central nervous system. The patient examination program includes the following points:

• Survey. In a conversation with the patient, the doctor details the complaints, finds out the provoking factors, the frequency and duration of cephalalgia attacks. The specialist focuses on the accompanying manifestations of increased activity of the parasympathetic nervous system.
• Physical examination. An external examination reveals autonomic disorders. In the neurological status of patients with PG, there may be a decrease in pain, tactile sensitivity and allodynia in the innervation zone of the IV pair of cranial nerves on the affected side.
• Trial treatment. A distinctive feature and important diagnostic criterion for paroxysmal hemicrania is the complete relief of pain after taking a standard therapeutic dose of a drug from the NSAID group - indomethacin.

Differential diagnosis is carried out with other trigeminal autonomic cephalgia: cluster headache, SUNCT syndrome, hemicrania continua. In the first disease, the pain syndrome is localized in the periorbital region, the duration of the attack is from 15 to 180 minutes, the frequency does not exceed 10 times a day. With SUNCT syndrome, pain is of moderate intensity, piercing or pulsating in nature, attacks can be provoked by irritation of trigger zones in the face, their duration ranges from 5-240 seconds. Hemicrania continua is characterized by acute, constant pain in the orbital or temple area of ​​moderate intensity, which is often provoked by alcohol consumption.

Treatment of paroxysmal hemicrania

Treatment of the disease is exclusively medicinal, in most cases carried out on an outpatient basis. The duration of treatment depends on the form of HP; therapy can be carried out on an ongoing basis or in a short course, several days longer than the duration of the exacerbation. The main goal is to relieve attacks of cephalalgia and prevent their occurrence. The treatment program is represented by the following pharmacotherapeutic agents:

• Indomethacin. Drug of choice for paroxysmal hemicrania. A lasting therapeutic effect occurs 1-2 days after the start of administration. If indomethacin is ineffective, the diagnosis of PG should be reconsidered. An exact justification for this phenomenon has not been found.
• Alternative means. If you are allergic to indomethacin, the neurologist individually selects another drug. In some cases, medications from the groups of nonsteroidal anti-inflammatory drugs, calcium channel blockers, and steroids are effective.
• Symptomatic remedies. With constant use of indomethacin, the likelihood of the formation of peptic ulcers of the stomach and duodenum increases, so antacids, H2-blockers or proton pump blockers can be additionally used.

Prognosis and prevention

The prognosis for patients with paroxysmal hemicrania is favorable. Rational pharmacotherapy allows you to completely relieve pain and achieve stable clinical remission. Specific prevention of the disease consists of taking indomethacin or an alternative drug in a dose previously selected by the treating specialist. Nonspecific preventive measures involve stopping the use of alcoholic beverages, eliminating all factors that can provoke new attacks of cephalalgia - prolonged visual strain, stressful situations, emotional overexcitation, etc.