Headbutts against the floor and the wall.
Screaming in pain.
Desperate.
This is how Josep Riba, a 55-year-old from Badalona, spent the episodes of cluster headache that he suffered every day.
From the age of 21, "six, seven, eight" times a day, those intense headaches appeared that could last up to two hours each.
“They were like continuous punctures in the face and on the same side of the brain and I ended up on the floor lying like a cockroach.
He screamed, I wanted to die.
It was impossible to sleep or live, ”he recalls of the worst episodes.
Cluster headache is rare and of unknown origin, but the pain it causes is such, neurologists explain, that it incapacitates patients and leads them to profound despair, even with suicidal thoughts.
There are treatments to try to control it, from high-dose corticosteroids to Botox or lithium,
but they don't always work.
In the most extreme cases, such as Riba's, which is refractory to all treatments, there is only one last bullet left: placing electrodes in the brain to try to control the intensity of the pain.
"Now, after the operation, at least, I no longer throw myself on the floor or scream," he says resignedly.
He has half a dozen headaches every night, lasting about 20 minutes each.
More information
Discover the new section EL PAÍS Health and Wellbeing
When the American doctor Bayard T. Norton named this strange headache in 1939, he defined it as "a pain so severe that patients should be monitored for risk of committing suicide."
And it was even colloquially baptized as “suicide headache”.
It is not frivolous, explains Dr. Robert Belvís, head of the Headache Unit at Hospital Sant Pau in Barcelona: “They call it that because of the risk involved.
The suicide attempt is not so much, it is more suicidal behavior: they are patients who verbalize that they want to rush, but they do not do it, although they are very elaborate behaviours”.
Patients suffering from this ailment, in fact, are supervised by the suicide prevention units of the psychiatric services.
03:24
Cluster headache surgery
Video: GIANLUCA BATTISTA
The origin of cluster headaches, which affect one in 1,000 people (three men for every woman), is unknown.
There is no family relationship nor have associated genes been found yet, Belvís points out.
The first center that is activated when a patient suffers an attack is the hypothalamus, but they do not know why: “The genes of the hypothalamus are being looked at because substances called orexins are produced there and they have looked to see if there was any kind of relationship, but no Nothing has been found."
In addition, it is difficult to diagnose in emergencies or among neurologists: in Spain, the average diagnostic delay is four years, but in the United States it is six and in Norway, 11, says Belvís.
Each patient is different: a third of them usually have attacks seasonally (especially in spring or autumn), but the rest suffer pain in an anarchic way, without a defined pattern.
All of them, in any case, begin with episodic pain of severe or very severe intensity – migraines are of a moderate or severe level.
“One day you start doing a cluster of several pain attacks that last from two weeks to three months.
From a visual point of view, it is as if you had punched him in the eye: he cries at you, turns red inside, his eyelid swells and falls, his forehead swells and mucus and tears fall only on that side.
The patient feels that his ear is clogged”, specifies Belvís.
And all this symptomatic picture is accompanied by episodes of agitation, adds the neurologist: "There are people who start hitting themselves,
who self-harm, so as not to have that pain.
Patients often say that it is as if their eye was ripped out, it is a very serious pain, from screaming.
The therapeutic arsenal is extensive, but it does not always work: for crises, they use triptans, the most aggressive family of drugs against migraines, and 100% oxygen for half an hour —in attacks of asthma or bronchitis 28% is used at over several hours.
“Oxygen is a vasoconstrictor and prevents the production of nitric oxide, which is one of the molecules that produces pain in the brain”, justifies the neurologist.
High-dose cortisone is also administered and there are drugs to try to control the intensity and periodicity of the attacks, such as lithium (an aggressive antipsychotic that is only used for this condition and bipolar disorder), some antiepileptic drugs, Botox injections or a monoclonal antibody used for migraine.
10% of patients, however, do not respond to any of the pharmacological treatments and require surgery.
The least invasive is the radiofrequency of the sphenopalatine ganglion, which enters the nerve through the mouth, heats it and removes pain.
If that doesn't work either, neurosurgeons opt to incorporate an occipital nerve stimulator, which already requires a more complex intervention.
But there are patients with chronic cluster headaches who do not respond either, and for them, there is only one solution left: deep brain stimulation.
It is the last bullet to attack this strange and painful headache.
In the scientific literature, only 94 cases have been described that have undergone this operation, explains Belvís.
In his hospital, a reference for these interventions, they have 14 and go to two or three per year, adds Rodrigo Rodríguez, neurosurgeon and deputy and coordinator of the Tactical Functional Unit of Hospital Sant Pau: "The technique consists of the introduction of an electrode in an area of the brain that does not respond correctly [in this case, the hypothalamus] in order to create an electrical change that will affect the connectivity and electrical flow of different brain structures to normalize brain function”, he explains.
It is already done more commonly for other ailments, such as Parkinson's, but to treat cluster headaches it is still anecdotal.
less intense attacks
After this intervention, 80% of patients improve their clinical picture.
As in the case of Riba, the headaches do not completely remit, but the attacks are reduced by up to 50% and are less intense, says Rodríguez, a neurosurgeon at Sant Pau who is also in charge of these interventions.
The operation, adds the specialist, is very safe and hardly reports complications.
Among other things, he explains, because "the operation begins before entering the operating room."
“There is a large part of pre-planning in which we analyze the pain circuit and see which networks are affected.
This part of planning is fundamental because it gives security to the procedure inside the operating room”.
When the neurosurgeons proceed to drill holes in the skull to place the electrodes at the base of the hypothalamus, they go for a steady shot.
All this prior planning allows them to make a map of the exact route they have to take and with the help of a kind of crown that marks the exact spatial coordinates of the point of intervention, they insert the electrical devices.
There is hardly any margin for error with such precision, but to make sure, they do an intraoperative CT scan that confirms, indeed, that the electrodes are placed in the right place.
In most cases, the number of headaches or the intensity of the pain is reduced.
There is a substantial change compared to before the operation.
However, the specialists admit, there is always a percentage of patients who do not respond to this therapeutic approach either, and the alternative is very limited: try combinations of treatments and drugs that reduce headaches in some way, accompany the patients and continue researching to find new options.
Much remains to be known about this disease, they admit.
