Paroxysmal hemicrania - what is this disease and how to treat it? What is hemicrania? What is dangerous about this violation Triggers of Paroxysmal Hemicrania.

Paroxysmal hemicrania is an independent disease that belongs 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 is known 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 variety from cluster cephalalgia. Some authors even consider this disease as an analogue of cluster cephalalgia, which in most cases is detected in men.

Symptoms

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

  1. Redness of the face.
  2. Redness of the eyes.
  3. Lachrymation.
  4. Nasal congestion.

It turns out that this form of headache is very similar to cluster headache, which is especially noticeable when comparing 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 pieces or even more per day. But the difference in treatment is especially noticeable. For example, there are cases when an attack completely disappeared from taking several tablets of indomethacin, although before that the patient complained of discomfort for several years.

Clinical varieties

There are 3 varieties of this disease. The most common is chronic paroxysmal hemicrania. At the same time, pain in the head area is observed every day for many years, without the presence of a remission period.

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 ailment, 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 to make the correct diagnosis. In some cases, diagnosis requires only a patient interview and visual examination. It is very important to understand that an attack is accompanied by at least one of the following symptoms:

  1. Redness of the eyes.
  2. Lachrymation.
  3. Nasal congestion.
  4. Puffiness of the eyelids.
  5. Perspiration of the face.
  6. miosis or ptosis.

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

If a person does not have any of the above signs, then an additional examination is necessary. This can be a CT or MRI, because other serious conditions have similar signs, for example, tumors or brain cysts.

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

Treatment

The only drug that helps to cope with the disease is this. You can take it both in the form of tablets and in the form of candles. 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.

It is necessary to start treatment 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 stop, and they will not be for several days, the dose can be reduced to maintenance, which is 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 abrupt withdrawal of the drug, the disease may return again. In this case, indomethacin can not be used in the following cases:

  1. Allergy to the drug.
  2. Erosion or ulcer of the stomach and intestines (exacerbation).
  3. Violation of hematopoiesis.
  4. Heart failure.
  5. Pancreatitis.
  6. Pregnancy.
  7. Impaired liver function.
  8. Impaired kidney function.

As practice shows, indomethacin is the only medicine, which is able to deal with the 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 have been taking analgin or spazgan for many years, while their seizures never go away.

Chronic paroxysmal hemicrania (CPH) was identified by the Norwegian neurologist Shosta in 1974.

The disease is characterized daily attacks of intense unilateral burning, boring, less often throbbing pain in the orbital, supraorbital or temporal region. Pain attacks in chronic paroxysmal hemicrania in terms of the nature of pain, localization and accompanying symptoms are in many ways reminiscent of a cluster headache. The duration of the attack is from 2 to 45 minutes, but their frequency can reach 10-30 times a day. Usually, the more frequent the attacks, the shorter they are. Patients do not have periods of remission.

The pain is accompanied by vegetative symptoms: conjunctival injection, lacrimation, nasal congestion, rhinorrhea, eyelid edema, miosis, ptosis. CPG occurs with a frequency of 0.03-0.05%. In contrast to bundle GB, women (1:8) aged 40 years and older are more likely to suffer. The disease usually rarely occurs at a young age. It should be especially noted that in CPH, an exceptional therapeutic effect of indomethacin administration is observed: many-month-long attacks disappear in 1-2 days. However, the use of drugs used to treat beam GB in CPH is ineffective.

So three diagnostic criteria distinguish this form of GB from bundle pain: the absence of bundle pain, the gender of the patient (mainly women suffer) and the high effectiveness of pharmacotherapy with indomethacin.

"Pain syndromes in neurological practice", A.M. Vein

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Stomatalgia, glossalgia The main complaints of patients are pain and paresthesia (rawness, burning, bursting, tingling) in various parts of the oral cavity: with glossalgia - in various parts of the tongue, with stomatalgia - in the gums, oral mucosa, sometimes pharynx. The severity of the noted sensations is different: from very weak to unbearably painful. As the disease progresses, the zone expands, capturing the entire mucosa ...

Atypical facial pain is a type of psychogenic pain in which there are no peripheral mechanisms for their implementation and the central mechanisms of pain acquire the main importance, which is closely associated with depression. Atypical facial pains are diverse in the nature of clinical manifestations and localization, but they have a number of typical signs. Missing clinical manifestations, characteristic of other types of pain (trigger zones, impaired sensitivity, myofascial, peripheral ...

Disease Frequency Duration Localization Intensity Nature of pain Accompanying symptoms Beam GB 1-3 times a day From 15 minutes to 3 hours Unilateral periorbital, forehead, temple. Painful Not pulsating, burning Lachrymation, rhinorrhea, injection, partial Horner Migraine 1-3 times a month 4-72 hours Unilateral, alternating sides, rarely bilateral Severe Throbbing 80% Nausea, vomiting, photophobia, phonophobia Trigeminal ...

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The nosological independence of this form of vascular headache was first reported by O. Sjaastad, J. Dale (1974).

It is manifested by daily, extremely strong bouts of burning, boring, rarely pulsating, always one-sided pain in the orbital and frontotemporal regions.

Sometimes the pain spreads to the entire half of the head.

There is no nocturnal prevalence of seizures, but the number of seizures can reach 10-16 per day. and some of them are at night. Usually, the more frequent attacks, the shorter they are, their average duration is 10-40 minutes.

Associated symptoms are the same as with Harris's migraine neuralgia: Horner's syndrome, redness of the eye and lacrimation, stuffy half of the nose.

Unlike migraine neuralgia, chronic paroxysmal hemicrania is much more common [approximately 8:1] in women. Another important feature is the "lightning" effect of indomethacin, when many months or many years of pain attacks disappear 1-2 days after the start of treatment.

Since there is a lot in common in the clinical picture of the so-called chronic form of migraine neuralgia and chronic paroxysmal hemicrania, differential diagnosis happens to be difficult. Sensitivity to indomethacin can serve as an important differential diagnostic feature. Treatment with indomethacin begins with 25 mg 3 times a day, after the cessation of attacks, they switch to a maintenance dose of 12.5-25 mg / day.

In the absence of contraindications to non-steroidal anti-inflammatory drugs, multi-month treatment is recommended, since after short-term courses, seizures can resume. D. Boghen, N. Desaulniers (1983) observed a patient, 20 years old, suffering from chronic paroxysmal hemicrania. After 3 weeks of treatment with indomethacin [25 mg 2 times a day], a remission occurred, lasting 2 years.

Until 1980, no more than 50 cases were described, which, with sufficient reliability, based on the characteristic clinical picture and sensitivity to indomethacin can be attributed to chronic paroxysmal hemicrania. A. Prusinsky (1979) considers this form as "a variant of Horton's syndrome with very frequent seizures." However, the difference in the response to indomethacin indicates peculiar neurohumoral disorders that are not characteristic of either migraine or migraine neuralgia.

The original observation is given by Yu.N. Averyanov et al. (1983). The patient attacks of paroxysmal vascular pain debuted at the age of 40 and at first resembled menstrual migraine. Short-term paroxysms, following one after another, merged into a painful cephalgic status, lasting several days. These conditions could be stopped by taking 1-2 tablets of indomethacin 3 times a day.

The authors called this form "indomethacin-sensitive variant of migraine neuralgia." In a reply to Yu.N. Averyanova O. Sjaastad (1984) emphasizes the originality of the given observation, which differs from chronic hemicrania by a cluster-like grouping of seizures with light intervals.

He believes that this is a kind of transitional variant between migraine neuralgia and the "Jebs and Jolts syndrome", described in 1979, which is characterized by multiple short-term [from 1-2 to several seconds] attacks of pain, conjunctival hyperemia, sweating in the forehead. However, all of these variants are insensitive to indomethacin.

It is clear that there cannot be absolutely similar cases of paroxysmal hemicrania in terms of the duration of one attack of pain and a series of attacks during the day, weeks and months. Nevertheless, we consider the options proposed in the "Classification-2003" far-fetched and useless.

So, it is proposed to refer to “episodic paroxysmal hemicrania” cases when a series of attacks last from 7 days to a year (!) with remission intervals of no more than a month. Such different timing of the duration of a series of attacks cannot be seriously discussed as criteria for diagnosis. Cases where remissions between periods with a series of attacks are rare and last less than a month are referred to as chronic paroxysmal hemicrania.

Poorly documented reports of cases of paroxysmal hemicrania, and especially without indicating a response to treatment with indomethacin, do not allow us to conclusively state that in all such cases we are dealing with a nosologically uniform form of the disease.

In the “Classification-2003”, the group of cluster-type headache includes a peculiar form of trigeminal autonomic neuralgia - “paroxysmal neuralgioform unilateral headache with lacrimation and conjunctival hyperemia” (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing - SUNCT).

SUNCT is manifested by very short attacks of unilateral pain in the eye and periorbital region, combined with scleral hyperemia and lacrimation. There can be from 3 to 200 attacks per day. Such a clinical characteristic allows us to consider SUNCT as one of the variants of trigeminal neuralgia (or ganglioneuralgia), i.e. one of the forms of facial (prosopalgia), but not a headache.

It is believed that chronic paroxysmal hemicrania "develops" from other forms of paroxysmal pain. A.M. Rapoport et al. report a patient who had suffered from the age of 7 classic ophthalmic migraine. From the age of 14 to 30 there was a complete spontaneous remission, after which the attacks resumed, became daily, but with 4-8-week light intervals, and from the age of 35 they were daily for 17 years.

Salicylates, ergotamine, methysergide, and amitriptyline did not alleviate the pain. Moderate improvement was observed during treatment with lithium carbonate 600 mg/day. After the first dose of 25 mg of indomethacin, the pain [lasting 19 years] was gone, the maintenance dose of 12.5 mg of the drug was sufficient to prevent the attacks from recurring.

The nosological independence of this form of vascular headache was first reported by O.Sjaastad, J.Dale in 1974. The etiology and pathogenesis of the disease have not been elucidated. It is believed that paroxysmal hemicrania is transformed from other forms of paroxysmal pain.

Much more often (at a ratio of 8:1), paroxysmal hemicrania, in contrast to cluster cephalgia, occurs in women and is considered by some authors as an analogue of male cluster cephalgia.

Symptoms

Paroxysmal hemicrania is manifested by daily, extremely severe attacks of burning, boring, rarely pulsating, always one-sided pain in the orbital and frontotemporal regions.

Associated symptoms are the same as in cluster cephalgia: Horner's syndrome, facial flushing, conjunctival injection, lacrimation, nasal congestion.

Thus, this form of vascular headache is similar to chronic cluster cephalgia in terms of intensity, localization of pain, and autonomic manifestations. The main difference is a significant increase in the frequency of attacks (from two to ten times more often), a shorter duration of a painful attack, and a predominance among sick women. In addition, there is no response to anti-cluster prophylactic agents, and, most characteristically, there is a very rapid cessation of attacks with indomethacin, with long-term pain attacks disappearing 1-2 days after the start of treatment.

Sensitivity to indomethacin can serve as an important differential diagnostic feature.

Diagnostics

In accordance with international classification headaches, the diagnosis of paroxysmal hemicrania is based on the following diagnostic criteria:

A. At least 20 attacks that meet the following criteria:

B. Attacks of severe unilateral headache in the orbital, supraorbital and/or temporal region, always on the same side, lasting 2 to 30 minutes.

C. The pain is accompanied by at least one of the following symptoms on the side of the pain:

  1. conjunctival injection
  2. lacrimation
  3. Nasal congestion
  4. Rhinorrhea
  5. Ptosis or miosis
  6. Edema of the eyelids
  7. Sweating on half of the face or forehead

D. The frequency of seizures is more than 5 times a day, sometimes less often.

E. Absolute efficacy of indomethacin (150 mg daily or less).

F. Not related to other causes.

Episodic paroxysmal hemicrania

Attacks of paroxysmal hemicrania occur in periods that last from one week to a year. Periods of headache are replaced by remission, when symptoms are absent. Remissions can last from one month or more.

Diagnostic criteria:

C. At least two periods of headache attacks lasting 7-365 days separated by pain-free remission periods of at least 1 month.

Chronic paroxysmal hemicrania

Attacks of paroxysmal hemicrania occur for more than a year without remissions. Painful periods are punctuated by pain-free periods of remission lasting one month or more.

Diagnostic criteria:

A. Seizures responding criteria A-F for 3.2. Paroxysmal hemicrania.

B. Attacks recur for more than 1 year without remissions or with remissions that last less than 1 month.

Treatment

the only effective tool The treatment for paroxysmal hemicrania is indomethacin.

Treatment begins with a dose of 75 mg / day in 3 doses with a gradual increase to 250 mg with continued attacks. After the cessation of seizures, they gradually switch to a maintenance dose of 12.5-25 mg / day.

Paroxysmal hemicrania presents with attacks with pain characteristics and associated symptoms similar to those of cluster headache. Distinctive symptoms are the short duration of attacks and their high frequency. Paroxysmal hemicrania is more commonly observed in women, usually the disease begins in adulthood, but cases have also been described in children. A specific sign of this form of cephalalgia is the effectiveness of indomethacin.

Diagnostic criteria are presented below.

3.2. Paroxysmal hemicrania (ICHD-4)
A. At least 20 seizures fulfilling criteria B-D.
B. Attacks of intense unilateral pain of orbital, supraorbital or temporal localization lasting 2-30 minutes.
WITH. Headache accompanied by at least one of the following symptoms:
1) ipsilateral conjunctival injection and/or lacrimation;
2) ipsilateral nasal congestion and/or rhinorrhea;
3) ipsilateral swelling of the eyelids;
4) ipsilateral sweating of the forehead and face;
5) ipsilateral miosis and/or ptosis.
D. The predominant frequency of seizures is more than five times a day, sometimes somewhat less frequently.
E. Seizures are completely prevented by taking indomethacin at a therapeutic dose.
F. Not related to other causes (violations).

As with cluster headache, episodic (with remissions of 1 month or more) and chronic forms of paroxysmal hemicrania are distinguished, in which stupas are repeated for more than 1 year without remissions or with remissions for less than 1 month. There are cases of paroxysmal hemicrania, combined with trigeminal neuralgia (the so-called paroxysmal hemicrania-tic syndrome).

Treatment

Specific therapy for paroxysmal hemicrania is the use of indomethacin (orally or rectally at a dose of at least 150 mg / day or at least 100 mg as an injection). For maintenance therapy, lower doses are often effective.