Susannah Cahalan was 24 years old when one day she discovered that the world had become a “brighter, louder and more painful” place.
It was just the beginning of a few delirious weeks in which everything changed in the life of this journalist from
The New York Post
.
Sometimes she saw insects that bit her.
She felt numb on the left side of her body.
She couldn't sleep despite being exhausted.
And her behavior became erratic.
The night she suffered a major crisis at her boyfriend's house marked the beginning of a long and erroneous list of diagnoses: alcohol withdrawal syndrome, bipolar disorder, schizophrenia...
My brain on fire
(Kailas editorial)
is the eloquent title of the book, later turned into a film, in which this woman narrates the devastation caused by a disease as rare as it is terrifying, autoimmune encephalitis, caused by an attack by the immune system against the neurons of the brain.
The estimated incidence of the disease is one case per year per 100,000 people.
Cahalan suffered his crisis in 2009 and medicine was able to find a diagnosis thanks to the fact that Josep Dalmau (Sabadell, 70 years old), a neurologist who then worked at the hospital of the University of Pennsylvania, had discovered only two years earlier the mechanisms behind it. behind a type of encephalitis called “due to antibodies against the NMDA receptor.”
A look at the walls of Dalmau's current office at the Idibaps-Hospital Clínic (Barcelona) gives an idea of the global impact that the breakthrough had, a milestone that would lead this scientist and his team of researchers to describe 12 of the 16 autoimmune encephalitis. described since then.
In them, discreetly framed, there are three covers of
The Lancet Neurology
, a reference magazine in the specialty, dedicated to Dalmau's work.
And also a recognition that, among all those received, makes him especially excited: the invitation from the United States National Academy of Medicine to be part of the institution.
Only two other researchers who practice in Spain, the pharmacologist María José Alonso and the pathologist Elías Campo, share this distinction.
One of the encephalitis described by Dalmau's team, postherpetic autoimmune, is now the star of the documentary
Look at Fear
, included in CaixaForum+, the free
online
platform of the “la Caixa” Foundation.
Ask
.
Why did it take so long for medicine to understand autoimmune encephalitis?
Answer.
They are actually a large group of complex diseases.
We knew very little about them 20 years ago and today we understand them better.
But there are still some whose cause is not well known.
Q.
Why does the immune system attack the brain?
A.
In some cases, the reason is tumor proteins similar or identical to brain proteins.
These proteins trigger an immune response in which antibodies also attack those in the brain.
The tumor may be benign and so small that it has not been detected, but the process it sets in motion can be devastating.
There are also other factors that we are learning about, such as a certain genetic predisposition or some oncological treatments.
Q.
How does the patient experience the process?
A.
Any brain function may be altered.
Sometimes the manifestations are similar to psychiatric disorders, others cause motor, sensory, sleep alterations, epileptic attacks... What is notable is that each autoimmune encephalitis has a different symptom profile.
And they are very serious processes, which often require long admissions to the ICU and some are associated with high mortality if they are not treated.
Q.
Which people are most affected by these encephalitis?
A.
It depends on the type.
Some affect young women and children more, others affect people over 50 or 60 years old.
Q.
Your discovery is often cited as a paradigm shift.
Because?
A.
It was the spearhead that opened the door to new knowledge about many autoimmune encephalitis.
20 years ago, only one group was known.
They were related to cancer and usually affected people over 50 years of age.
But we saw that there were others, equally serious, but that the patients could be much younger, even adolescents or children.
Sometimes they did not have a tumor or it was benign... All of this had a great impact on neurology, pediatrics, psychiatry, intensive care... which were the specialties that faced these cases without knowing how.
The neurologist Josep Dalmau, at the Hospital Clínic of Barcelona. Albert Garcia
Q.
How did you achieve it?
A.
It was not a new method.
You start with clinical observation.
You see the symptoms and you begin, almost automatically, to mentally exclude illnesses.
In the end you are left without knowing exactly what is happening.
In this case she was a young woman of about 22 years old who had been in the ICU for almost two months.
And then, and experience helps here, you remember that you have seen three other almost identical paintings before.
All young women, with a small benign tumor, the same clinical profile...
Q.
And what does one say to oneself at that moment?
A.
Well, there is something you are missing.
And this is when the research process begins.
We took the matter to the laboratory because I was convinced that the process was immune-mediated, but all the initial antibody studies had been negative.
So we had to make a series of technical variations.
In the end we discovered that the four patients had antibodies in their cerebrospinal fluid that reacted exactly the same in the brain.
Q.
And then, what?
A.
You go back to the patient.
Once the antibody is discovered, you look at what proteins it went against.
This allows you to develop a very simple diagnostic test.
And for the next patient, you already know how to diagnose them quickly and treat them, which is the goal.
Q.
With what treatment?
A.
If you know that it is an antibody-mediated encephalitis, the strategy is useful.
It must be refined for each type of encephalitis, but the basic treatment is to eliminate the antibodies.
This can be done with techniques such as plasma exchange, a kind of washing of antibodies from the blood.
If this is not enough, you go to the base and eliminate the cells that produce the antibodies.
This is done with certain monoclonal antibody treatments.
And there are other more aggressive therapies.
You escalate according to severity and response to treatment.
Most encephalitis responds to this strategy.
Q.
Have you managed to relate each type of encephalitis to a specific cause?
A.
If it is for reasons not related to a tumor, it is less relevant to do so.
But if it is a tumor, yes, because the process can be repeated as long as the tumor remains and the immune system has been activated.
This is why it is key to look for the tumor and remove it.
Q.
But it shouldn't be very difficult to find it, right?
A.
Sometimes yes, because it can be very small.
We see this from queries we receive from other places in the world where perhaps they do not have as many diagnostic means.
Patients who have been correctly diagnosed and have improved with the indicated treatment.
But they relapse.
And we tell them to look for the tumor.
Let them take a good look at certain organs.
And in the end, many times, it was there.
Q.
Can you tell where the tumor is by the type of encephalitis?
A.
Often yes.
Some encephalitis is associated with tumors in certain areas.
Depending on the type of encephalitis and associated antibodies, we can say that they search primarily in the abdominal area, the ovaries or the lung.
Q.
How many types of encephalitis have been defined?
A.
Since 2007, there have been 16 of this type mediated by antibodies [those not related to types of cancer that affect older people].
But there is more that we have not managed to describe yet.
Almost every year we have about 50 patients with types of encephalitis that we can assume are related to an antibody that has not yet been characterized.
Achieving it with each one is difficult, it is practically a project each one.
They are like isolated cases that would be ideal to relate to others.
P.
What else is left to do?
A.
Our most recent studies try to understand why there are patients who take many months to recover and in other cases some alterations have become chronic.
These cases continue to come to us from all over the world.
Q.
And what do they do?
A.
We have returned to the clinic to better define the long-term condition of these patients.
We are characterizing these diseases in the chronic phase.
There is a very long recovery phase that we still don't fully understand.
If the patient had antibodies and you eliminate them, why does he still have the symptoms?
Here we have quite attractive studies, which no one has done until now and which we are carrying out with the help of the la Caixa Foundation.
We invite patients to come to Barcelona once they leave the hospital and have overcome the worst of the acute phase, but are still not well.
We do a thorough examination of them.
They are admitted one night and we do a sleep record and other studies such as an encephalogram and brain MRI.
We do this three times a year and we're seeing what happens.
Q.
It's a bit slow...
A.
Yes, we continue through Zoom and other applications from their homes.
We perform neuropsychological tests on them and, sometimes, we send them a type of headband that they can wear comfortably to sleep and that performs an electroencephalographic recording whose data reaches us via the Internet.
Q.
And what are they discovering?
A.
Amazing things, the brain never stops giving us surprises.
We have found that during the day, if you see the patient, they may appear fine.
The surprise is that, thanks to these records, we have seen that many suffer very important alterations in their sleep structure during the night.
They suffer epileptic seizures that we thought they had overcome and that probably contribute to cognitive and memory alterations...
Q.
And what can you do for them?
A.
This is another area in which there is still much to advance.
Some patients improve by restarting or extending treatment.
We are also studying how we can shorten the recovery period through remote cognitive rehabilitation.
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