This Spanish neurologist changed the lives of patients with brain firestorms

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In 2023, neurologist Josep Dalmau received an award from American Brain Foundation with special added value. I gave it to him young Canadian named Jayden which Dalmau visited in 2009, when he had just turned three.

The girl began to walk strangely and began to have convulsions. Doctors could not find a reason, but Jayden was getting worse, to the point that she did not recognize her family members and even showed aggression towards them.

“When his parents contacted me, Jayden had already been in hospital for several days. He suffered from a very serious autoimmune encephalitis with a huge range of symptoms. We treated her and she was able to recover,” explains Dalmau, who last April He gave the IX Tatyana Lecture at the Autonomous University of Madrid.

Now, at 18 years old, Jaden – Ambassador belonging Canadian NMDAR Encephalitis Foundation. She was lucky because just two years earlier, in 2007, Dalmau’s group, then at the Memorial Sloan-Kettering Cancer Center in New York, identified this new type of pathology.

The so-called NMDAR autoimmune encephalitis was the first of 18 known today. Twelve of them were described by this Spanish researcher’s teams during two decades of pioneering research.

“Its diagnosis can be very difficult as it can be confused with psychiatric or neurological diseases. However, most patients later develop neurological symptoms that distinguish them from illnesses of psychiatric origin,” explains Dalmau.

diagnostic tests and clinical guidelines which he developed are used all over the world. Despite this, diagnosis may be delayed even longer than it should be, with subsequent worsening of the disease.

Autoimmune encephalitis is a rare disease and affects 1 in 250,000–500,000 people, especially women. The average age is about 21 years, but they also occur in pediatric age. In fact, about 37% of patients are under the age of 18 when symptoms begin to appear.

This pathology causes inflammation of the brain, that is, any of the structures contained within the skull, such as the brain or cerebellum. They usually respond to immunotherapy. However, recovery is possible in 80% of cases, although it may take more than 18 months.

Paradigm change and better outlook

This encephalitis, caused by the brain’s own immune system attacking the brain, “causes a wide range of symptoms that can lead patients to intensive care with mechanical ventilation,” says Dalmau, who now works at the Barcelona Hospital Clinic and August Pi i Sunyer Biomedical Center. Research Institute (Idibaps).

Behavioral changes are a common symptom at all ages. To this should be added epileptic seizures and abnormal movements in children. In adolescents and young adults, psychosis and seizures are often the first symptom. And after 45 years, memory deficits also appear.

The immune system’s battle with neurons causes a “firestorm in the brain,” as described in the scientific literature, and is responsible for many symptoms. And the “confusion” that causes our defenses to attack such a vital structure as the brain should be looked for in the presence of a tumor (even if it is benign, like an ovarian teratoma) or in a previous infection with a virus such as herpes. .

Given the presence of these symptoms, “autoimmune encephalitis is now commonly suspected in hospital emergency departments,” Dalmau says. The diagnosis is confirmed by the detection of autoantibodies in the blood serum and cerebrospinal fluid.

The work of this expert, President of the Section of Autoimmune Neurology of the American Academy of Neurology, changed the landscape not only of these diseases, but also of neurology and psychiatry, as he played a fundamental role in unraveling their origins and thereby achieving effective treatments. It has also led to an improved prognosis for people suffering from them.

Make illness visible

Beyond its scientific impact, Dalmau’s work has changed the lives of people with this type of encephalitis and their families. Some cases have become internationally famous, such as that of New York Post journalist Suzanne Cahalan.

In 2009, at the age of 24, Cahalan suddenly entered a state of delirium and paranoia, which he recorded in his book after recovery. “Brain on Fire” best-seller Later they took me to the cinema. Her story had a happy ending, as the doctor on the medical team treating her suspected that her illness might be related to the cases described by Dalmau.

On the occasion of World Encephalitis Day last February, the Caixa Foundation, which finances the project in Dalmau, presented a documentary Face the Fear, supported by the Spanish Society of Neurology and the Spanish Society of Child Neurology, collects the testimony of a young woman from Alicante years after overcoming autoimmune encephalitis as a result of herpes virus infection.

Dalmau notes that these readings are “important because recognizing these changes and diagnosing them is critical because they are treatable.” However, the researcher insists on the role of early diagnosis, which is reflected in his last work published in March V Lancet Neurology. “Its delay may affect cognitive function,” he concludes.