Algie vascular of the face

In Encyclopedia by Aimée Barthelemy Views: 7

The vascular algie of face (FTA) is an acute form of essential headache. It is a rare, extremely painful and disabling condition for the one that suffers. It occurs on one of the halves of the head. The Americans the cynically called “the Boss’ Headache” (evil head of the pattern). More dramatically, it the nickname “suicide headaches”, so the violence of the attacks and their frequency makes hell the lives of the people who suffer, or about 0.1% of the population.

Synonyms

• Horton headache (not to be confused with Horton disease);
• Horton headache;
• Cluster Headaches;
• Cluster headache;
• Headache or migraine suicidal;
• Histamine headache;
• Bing-Horton syndrome.

The first complete description of vascular postherpetic of the face given by the neurologist Wilfred Harris London in 1926. He named the disease migraine neuralgia.

Vascular face postherpetic were called by various other names in the past: érythroposopalgie of Bing, neuralgia nerve naso-ciliary of Charlin, érythromelalgie of head, headache Horton histamine, histamine headache, neuralgia petrosal neuralgia of the report sphéno-Palatine ganglion, the Vail vidien nerve neuralgia, Gardner pétreuse neuralgia, syndrome of vasodilation hémicéphalique Pasteur-Vallery-Radot, red migraine of Mollendorf, and Hémicranie angioparalytique.

• Robert Bing (1878-1956)

• Bayard t. Horton (1895-1980)

Signs and symptoms

Test diagnostic of the FTA on the IHS
A	At least five attacks fulfilling criteria B – D
(B)	Unilateral severe pain, orbital, supra-orbitaire and/or temporal, topography during 15 to 180 minutes without treatment
C	The headache is associated with at least one of the following, on the same side as the pain: conjunctival injection, lacrimation, nasal obstruction, runny nose, sweating of the front and side, miosis, ptosis, eyelid edema
(D)	Frequency of crises of 1 to 8 per day
E	At least one of the following characters: the history, physical and neurological examination do not organic disorder suggest the history, physical and neurological examination suggest an organic disorder, but it is rejected by the neuro-imaging or any other method of laboratory organic disorder exists, but crises of FTA did not appear for the first time in temporal association with

AVF sufferers typically have headache important nearly an eye, nose or the temple condition which lasts from 15 minutes to 3 hours. Headaches are unilateral, and are still located on the same side of the face, respecting the median line.

The pain is increasing gradually but rapidly (5 to 15 min), is asymmetric, type of swelling, broiement, necking, the part of the face suffering; headache is often associated with when the crisis became severe and signing the relatedness of the FTA with common migraine. A significant difference in the expression of pain is agitation, the patient desperately seeking a position or a place where the pain would be more bearable.

The FTA is often associated with at least one of the following symptoms: a fall of the eyelids, the eye returned, contracted pupils, (these three symptoms constituting of Claude Bernard-Horner syndrome), eye red and tearjerking, swelling of the eyelids, nasal congestion or a runny nose, and sweating on the area of the head where the pain occurs. The neck is often tense and pain in the jaws and teeth have been reported.

In 80-90% of cases, patients are agitated. They frequently groan, cry or howl. They are sometimes engaged in violent behaviour towards themselves. The sensitivity to light, sound sensitivity appear frequently (about 60% of cases) at FTA. In some less common cases, nausea and vomiting may also occur (approximately 28% of the cases). In some cases, the condition can even push to suicide to escape the pain in a desperate act.

Pain intensity

Comparison with any other headache is limited: however patients who have migraines say that pain in a FTA is significantly larger (with intensity perceived sometimes 100 times greater). An analogy is that of an ice pick burning that is departure repeatedly through the eye and the brain, a tear or a broiement.

Medicine believes that it is one of the most intense pain, and that it exceeds that of amputations without anesthesia. In this case, Peter Goadsby, Professor of Clinical Neurology at the College of the University of London, a leading researcher, commented:

“The vascular algie of the face is probably the worst pain that man has ever known.” I know that it is a high note, but if you ask patients suffering from FTA if they had a worse experience, they say that they have not had. Women suffering from FTA will tell you that the attack is even worse than childbirth. You can therefore imagine that these people give birth, without anaesthesia, once or twice a day, for six, eight or ten weeks, and then take a break. “This is simply terrible.”.

These symptoms were previously called Horton headache after the first theories postulated on the pathogenesis by Dr. B.T. Horton. The first article describes the severity of the pain as being able to take normal men and force them to commit suicide. Indeed, the vascular postherpetic of the face are also known under the nickname of “suicide headache”.

Extract from the first article of Horton (1939) on the vascular algie of the face:

Our patients were disabled by the disorder and suffered from pieces of bread from two to twenty times a week. They had found no. relief from the usual methods of treatment. Their bread was so severe that several of them had to be constantly watched for fear of suicide. Most of them were willing to submit to any operation which might bring relief.

Cycle and frequency

The FTA can be episodic or chronic (chronic form affects about 20% of patients). Considering that the FTA are episodic when they occur in groups during a period between 7 and 365 days with a remission for at least a month between each period. If pain occur over a longer period than a year, without a period of calm for at least a month, then the pathology is considered chronic. Sometimes that the nature of evil change of episodic to chronic and vice versa. He arrived that people are again with FTA after-free decades of attacks.

The periodic AVF are well imitations postal periods of the year (several weeks, followed by a quiet period, without headaches) and specific hours of the day. The pain may happen once a week to six times per day (with an average of two a day). They often strike at night, at the same time or may return to the same time a week later. These phenomena have led scientists to study the relationships with the biological clock of the brain and body (circadian rhythm). For the chronic form, patients undergo the pain every day for years.

However, the FTA may evolve over time. A 10-year study revealed that, in the case of FTA episodic: approximately 81% of patients remained episodic, about 13% have evolved to a chronic form and about 6% into an intermediate form. In the case of FTA chronic: about 52% remained chronic, about 33% have evolved into the episodic form and about 14% to a “combined” form. The evolution of the chronic episodic form is not yet known, but factors such as head injuries, smoking and alcohol consumption suggests having a negative influence.

Diagnosis

The positive diagnosis is only on discovery. The clinical examination is normal, except immediately after a crisis or between two closely spaced crises, where you can observe a redness of the eye, small eyelid edema and a sign of Claude-Bernard-Horner. It definitely persists in about 20% of cases [REF. necessary], especially in chronic AVF. Complementary examinations are normal.

The diagnostic difficulties concern the first crises since then missing the characteristic evolutionary pattern of affection. The average diagnosis is 44 months and it is greater than 4 years for 31% of the patients, 52% of patients had consulted at least 3 doctors before diagnosis.

Symptomatic AVF cases have been reported. This is the most often of “pseudo-FTA”, with clinical atypies as the persistence of a substance painful standing. Crises of recent installation and/or atypical semiology, the rule is to conduct additional reviews: Vascular explorations (cervical and Transcranial échoDoppler), a brain MRI/MRA (cases of internal carotid dissection, aneurysm, tumor, pituitary adenoma, cavernous sinus thrombosis), a scanner of the sinuses (sinusitis blocked case). In the case of FTA secondary, more frequently found etiologies are vascular (aneurysm, dural fistula), tumor (pituitary adenoma, meningiomas, paranasales structures or the subsequent pit carcinomas) and finally inflammatory or infectious (sphénoïdale aspergillosis). Moreover, the age of onset is late (on average 42 years) and the length of the crisis (often > 180 minutes).

Differential diagnoses

• Migraine
• chronic paroxysmal hémicrânie (HPC)
• cluster-tic
• SUNCT (Short – lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing)
• postherpetic manducator apparatus (ADAM or Costen syndrome)
• Essential neuralgia of the trigeminal (NET)
• Sinusitis

Epidemiology

Prevalence

While migraines appear more often in women, the FTA are rather a pathology affecting men. For a woman diagnosed with a FTA, there are 2.5 to 3 men suffering from the same condition.

However, this difference between the sexes is in decline, it is difficult to say if it is induced by improved diagnosis or if the condition is now appears more often in women. However, before the start of the 1960s, there were 6.2 men to 1 woman. Then the gender report evolved 5. 6: 1, 4. 3: 1, 3.0: 1 and 2.1: 1 respectively for the 1960s, 70, 80 and 90. This change is in close correlation with the evolution of the way of life of women (educational level, consumption of tobacco, coffee and alcohol).

The prevalence varies from 56 to 326 per 100,000 with fairly limited studies. Latitude appears to play a role: the AVF appear more often in northern populations than those who live near the equator. It is assumed that the duration of the day, which varies with latitude, is related to these headache [REF. necessary].

Pathophysiology

The AVF are primary headache of neuro-vascular origin. The pain is caused by the dilation of blood vessels which urge the trigeminal nerve [REF. necessary]. If the immediate reason for the pain is known, the causes of the condition remain in part overlooked. Nevertheless, the FTA is better and better understood: the various studies that have been listed show that the research is progressing.

The pathophysiology certainly involves the trigémino-vascular system and a “generator of the FTA” hypothalamic. The unilateralism of pain, its location in the territory of the trigeminal, the presence of parasympathetic signs (lacrimation, nasal congestion, conjunctival injection and swelling of the eyelid) and sympathetic (miosis, ptôsis and sweating of the front) suggest involvement trigémino-vascular system and the cavernous sinus, however by a mechanism which remains obscure.

The cyclical nature of the crises strongly suggest the involvement of circadian melatonin and gamma-aminobutyric acid (GABA). These two substances are highly concentrated in the hypothalamus. In addition there are hyperactivity of the hypothalamic nuclei of the same side as the pain crises.

Several testimonies collected by the AFCAVF association clearly show that the FTA also reached the very young children. A few adults diagnosed FTA say have had about 8-10 years of age, of strong “migraines” that, on reflection, seem have been crises of FTA. At least one case of teenager FTA which was certainly FTA when she was baby.

Hypothalamus

The theory that the FTA would come from an abnormality in the hypothalamus is one of the most accepted; Dr. Peter Goadsby, specialist James Australian developed this theory. This theory may explain why headaches occur with a regular and precise chronology. One of the roles of the hypothalamus is to regulate the circadian rhythm. Metabolic abnormalities have also been reported in patients.

Neuro-imaging

Functional magnetic resonance (fMRI) imaging shows the areas of the brain are activated during pain

Morphometry voxel by voxel (Modulaire) shows the structural differences of the areas of the brain

Positron emission tomography images indicate the areas of the brain which are activated during pain, from periods without pain. They show the brain regions that are always active during pain in yellow/orange (called “matrix-pain”). The area to the centre (in three views) is specifically enabled only during the crisis. The pictures on the bottom line (made by Modulaire) show structural differences between healthy persons and patients with FTA: only a portion of the hypothalamus is different.

There appears to be bilateral (in this case the existence of hypertrophy) hypothalamic microstructural abnormalities that known if these defects are the cause or the consequence of the repetition of crises.

Genetic factors

To date, no gene in the AVF was detected. However, direct descendants of a person suffering from AVF were more likely to develop the disease than the rest of the population. Unlike the other types of headache, the importance of the genetic factor seems less significant in the case of the FTA. Recently, the risk of FTA was related to polymorphism of a the 2 type hypocretin receptor gene (HCRTR2). A survey conducted by the AFCAVF association to its members (inquiry that led to the publication of a thesis) suggests that cases where a person with AVF has a child himself of AVF are rare (on the order of 2%)

Triggers

NITROGLYCERIN may induce FTA in patients who suffer from it, in a manner similar to the sudden attacks. Ingestion of alcohol is a known factor that creates the appearance of pain in people. The exposure to hydrocarbons (solvents, perfumes, etc.) can also lead to AVF. Some people have an intolerance to heat and too high temperature can produce an attack. Of FTA have also been noted in some patients after their NAP. The relationship between food and headaches is unknown and remains controversial.

Treatment

AVF attack is considered a medical because of the resulting pain emergency. However many physicians are not accustomed to this condition. This is why the FTA are often poorly or not diagnosed. For misunderstanding or diagnostic insufficient physician may neglect emergency treatment by the patient wait, it can assimilate wrongly to a drug addict in need.

Treatment of the crisis

Some treatments results satisfactory to deal with crises:

• Normobaric oxygen (6 to 8 L/min) gives good results: 75% of patients are relieved if treatment is started at the beginning of the crisis. However packaging, congestion and storage of oxygen in limited use. The interest of this treatment is the absence of adverse effects using repeated (more than two seizures a day). In France, the oxygen in the vascular algie of the face is reimbursed by health insurance, provided that the original prescription is made by a neurologist, an ENT or doctor of a specialized structure in the management of pain.

• According to the proposals of the journal prescribe sumatriptan injection (Imiject) is an alternative when the use of oxygen is impossible. Efficiency is the same level as that of oxygen therapy, although there is no test comparative oxygen therapy versus sumatriptan. There are however a number of adverse effects, as well as cardiovascular risk to take into account, which are against its use in patients with a history of cardiovascular.
• In Quebec and elsewhere in North America, began to testing of injection of botox to treat evil, as it did, a few years, with glycerol.

The drugs usually prescribed against headaches are ineffective (aspirin, paracetamol and ibuprofen). Migraine and other headaches, the FTA are insensitive to treatment by “biofeedback”. Some patients respond in part to the Narcotic Painkillers but the need to often makes them inadequate. They do not, moreover, to totally remove the pain. Some evidence that pain is sometimes so intense that even morphine penalty to combat. However, opiate powerful of short action such as fentanyl look promising but have yet to be the subject of research.

Background processing

The substantive treatment aims to reduce the frequency of crises in episodes or the chronic long-term course for FTA.

• Verapamil is widely used but does not have the marketing authorisation in this indication. It is currently considered the most effective treatment. The usual dosage is 120 mg, 3 to 4 times per day, on failure of much higher dosages are sometimes used (from 600 to 1,200 mg per day), but required close cardiovascular monitoring.

• Lithium carbonate is used outside AMM mean dose of 750 mg/d. Its effectiveness was demonstrated in a randomized, study methodology is however disputed. Renal and thyroid functions must be verified before the start of treatment, and then monitored regularly.

• Methysergide was a classic treatment which is hardly used because it is a vasoconstrictor contraindicating the administration of sumatriptan in treatment of crisis. Effective dosage ranges from 6 to 12 mg daily, and the treatment must be introduced very gradually. Main contraindications are coronary artery disease, severe HTA, Arteritis of the lower limbs and the hepatic or renal impairment severe.

• The ergotamine tartrate. As methysergide, this treatment is almost used more because it formally contre-indique the taking of sumatriptan.

• valproate sodium and other anti-epileptic treatment (gabapentin, topiramate) has not been demonstrated in the FTA. However, they are sometimes used in rebel forms to the isoptine and lithium.

• Indomethacin has a remarkable effect closer of that obtained in the chronic paroxysmal hémicrânie. In the absence of digestive contraindication, treatment should be started at high doses (150 to 200 mg per day) and then gradually decreased to the minimum effective dose.

• Corticosteroids (prednisone, prednisolone or methylprednisolone) have a spectacular efficiency in a few days in 70% of cases, but requires high doses (1 to 2 mg/kg daily in morning single decision-making) for a maximum 30-day limitation.
  Corticosteroids may also be used in local infiltration of large occipital nerve of Arnold with a same efficiency, during the period where a substantive treatment (by verapamil) who have initiated, it has not yet shown its effectiveness.

• The amitryptiline in infusion, dosage up to 150 mg per day, sometimes to overcome a difficult period, including a breach of treatment of crisis.

Alternative treatment

There is anecdotal evidence that the hallucinogenic serotonin as psilocybin contained in some hallucinogenic mushrooms, LSD and LSA (d-acid diethylamide amide, contained in the seeds of Rivea corymbosa) stop periods of crisis and prolong periods of remission.

Non-médicamenteux treatments

There are treatments non-médicamenteux, more or less invasive to the severe (unilateral) chronic forms and resistant.

• Alcohol of the sphéno-Palatine ganglion. The neurolysis is done by injection of 0.5 to 1 mL of 1% lidocaine followed by an equivalent volume of absolute alcohol. The improvement is often transitory (weeks to months) but the gesture may then be renewed.

• Selective Thermocoagulation of trigeminal ganglion.

• The injection of glycerol in the retro-gassérienne tank. Short-term failures are common.

• Selective injury of trigeminal by “gamma knife”. It is to injure by gamma radiation (70 Gy) the sensory root of trigeminal level TROZ (trigeminal root entry zone).

• Decompression microvascular trigeminal, without hypoesthesia in the trigeminal territory. Efficiency is not sustainable.

• Deep stimulation of the hypothalamus (by implantation of intracerebral electrodes). This technique appears to be very promising, but the decline is still insufficient.

• Stimulation of the occipital nerve, the technique is the implementation of an internal Stimulator with stimulation electrodes are laid on the two occipital nerves. This technique, which is less invasive than hypothalamic stimulation, would be improved by an average of 60% of patients with a chronic AVF pharmaco-resistant.

• The infiltration of the greater occipital nerve of Arnold. The injection is done with 160 mg of methylprednisolone.

• The port of a bite during the night, the vascular algie gutter can be a symptom of a syndrome called S.A.D.A a.m.: the mandible algo dysfunctional syndrome.

History

Greek and Roman literature ancient highlights various headaches, but no indication of the existence of vascular algie of the face. The Dutch physician Nicolaes Tulp, famous for “The Anatomy read” painted in 1632 by Rembrandt Harmenszoon van Rijn (1606-1669), described in 1641 (in “Observationes Medica”) two recurrent types of headaches: migraine, and probably the vascular algie of the face:

… In the beginning of the summer season, [he] was afflicted with a very severe headache, occurring and disappearing daily on fixed hours, with such intensity that he often assured me that he could not bear the pain anymore or he would succumb shortly. Rarely it lasted for longer than two hours. And the rest of the day there was no fever, not discomfort of the urine, no. any pour of the pulse. Purpose this recurring pain lasted until the fourteenth day… He nature asked for help,… and lost a great amount of fluid from the nose… [and] was relieved in a short period of time…

Thomas Willis also probably described in 1672 the vascular algie of the face.

Gerard van Swieten, physician Marie Thérèse of Austria and founder of the school of Vienna in 1745 documented a case of vascular algie of episodic face. This description corresponds to the criteria of current diagnosis of the International Headache Society (IHS-II-ICHD classification 3.1):

A healthy robust man of middle age [was suffering from] troublesome bread which came on every day at the same hour at the same spot above the orbit of the eye, where the nerve emerges from the opening of the left frontal bone; After a short time the left eye began to redden, and to overflow with tears; then he felt as if his eye was slowly forced out of its orbit with so much pain, that he nearly went mad. After a few hours all these evils ceased, and nothing in the eye appeared at all changed.

• Nicolaes Tulp (1593-1674)

• Thomas Willis (1621-1675)

• Gerard van Swieten (1700-1772)

References in Films or books

The film π of Darren Aronofsky.