Rare diseases are very numerous, but it is a subject rarely covered by the mainstream media. Take a cluster headache, which has the particularity of causing one of the worst pains that humans have ever known. Patients who suffer from it find themselves in unimaginable distress, especially if it is the chronic form of the disease.
Vascular cluster headache (AVF) is a rare disease about which little is said and yet it affects nearly 80,000 people in France . This syndrome is characterized by repetitive severe pain in the face, most of the time in one eye, but also in the nose and temple. This pain takes the form of seizures occurring over a period that can vary from fifteen minutes to several hours.
These headaches are unilateral, that is, they occur always on the same side of the face respecting the midline. The pain is accompanied by one or more of the following symptoms:red and watery eye, edema of the eyelids, nasal congestion or even a runny nose. In many cases, it can also be a question of tension in the neck and pain in the jaws and teeth.
One of the main characteristics of AVF is none other than the intensity of the pain . Thus, the comparison with other headaches has its limits. Peter Goadsby, professor of clinical neurology at King's College London (UK), stated in a 2001 study that "Cluster headache is probably the worst pain that man has ever known. » According to some testimonies, the pain experienced can be hundred times greater than migraines classics, comparable for example to an ice pick or a screwdriver that one would push repeatedly through the eye and the brain. Let us mention in passing the most recent testimony published in France, namely that of Diane Wattrelos, published by Ouest France on February 7, 2021.
In eight to nine out of ten cases, patients are agitated during seizures . Some scream, howl or at best moan. Others remain stoic or behave violently towards themselves. In some cases, the patient has suicidal thoughts and may go so far as to take action in a desperate desire to free themselves terrible pain.
In addition, you should know that AVF has two forms:episodic or chronic . The episodic form occurs "in groups" for a period ranging from 7 to 365 days with a remission of at least one month between each period. In the case of a duration longer than one year without any lull period of at least one month, it becomes a question of a chronic form, which corresponds to approximately 20% of cases.
Cluster headache is a difficult disease to diagnose, mainly because it is relatively unknown. In addition, the delay for this diagnosis is long:on average 44 months. No less than 31% of patients are diagnosed four years after the onset of the disease . The French Society for the Study of Migraines and Headaches (SFEMC) further explains that the cause of AVF remains unknown to date. However, we know that alcohol consumption or even irregular sleep can promote seizures.
Several studies have focused on the hypothalamus, which plays an important role in sleep-wake cycles and the secretion of certain hormones. During a crisis, an area related to the hypothalamus functions abnormally. It then activates the structures responsible for the pain of the face and, by reflex, the vegetative nervous system. The latter causes watery eyes, nasal congestion and the other symptoms mentioned above.
Several treatments exist to relieve seizures, such as injectable Sumatriptan. This is the most effective treatment known with a disappearance of the crisis in less than fifteen minutes in 74% of cases . However, this would not be effective in all patients.
A petition is currently circulating in France concerning Aimovig, a very promising new treatment. Authorized in the United States and Canada, Aimovig recently received European marketing authorization . However, it is very expensive and is still not available in France. It is therefore not subject to reimbursement by Social Security. Already signed by more than 67,000 people, the petition will be addressed directly to the Minister of Health Olivier Véran as soon as the 75,000 signatures are reached.
