Researchers from Erasmus MC followed 92 patients with the rare brain disease anti-NMDAR encephalitis. Mainly young women, but also men, get this encephalitis during the fertile period of their lives. Triggered by a herpes virus, known from cold sores, or an undiscovered tumor, but also for unknown reasons, the immune system sometimes attacks the brain. The diagnosis is not always made immediately, but the condition is treatable with immunosuppressive drugs.
26-year-old Betty, who wants to become an elementary school teacher, was in the middle of her exams when she began to suffer from severe headaches more than three years ago. She became confused, suffered from insomnia, and became aggressive. When she also started to have seizures, high fever, and breathing problems, she ended up first in a psychiatric institution and then in a coma in the Intensive Care Unit. Betty doesn’t remember much from that time. ‘I remember fainting and then going to my mother’s and sisters’ house. After that, it’s dark. Very strange. My life stood still.’
Hopeful
Juliette Brenner, a neurology trainee, came to Erasmus MC specifically to research this fascinating condition. She explains: ‘The hippocampus, where memory resides, and the frontal lobe, which has a role in planning, are often affected in these patients.’ Therefore, after their treatment, Betty and the other participants in her study underwent cognitive tests and filled out questionnaires about their symptoms. ‘It shows that there is a longer chance of recovery than we previously thought’, said the young researcher. Previously, researchers found indications that it was at least a year and a half.
Researchers Maarten Titulaer and Juliette Brenner.
Brenner and her colleagues now write in the scientific journal Neurology that the patients’ scores on cognitive tests slowly improved over time. The biggest steps in recovery are made in the first six months. After that, they see improvement up to three years after treatment. ‘First, the epileptic seizures disappear. And in the following years, we see that patients still show cognitive recovery. Especially when they go back to school or work. That’s hopeful’, said Brenner.
‘People around me also had to get to know me again’, said Betty.
Betty, now three and a half years after the diagnosis, recognizes all of this. ‘It’s actually going pretty well now and getting better and better. Sometimes I have trouble concentrating and am really forgetful. I also get tired quickly because I think a lot or search for the right words. But I believe in growth.’ She tells that her work in elementary school constantly challenges her cognitively. ‘All those math problems, it’s like I’m back in school myself. That has really helped me.’
The researchers see plenty of room for improvement. ‘Although more than eighty percent of patients recover after treatment and can live independently again after one to two years, over sixty percent still experience cognitive and social problems’, says neurologist and senior author Maarten Titulaer. He leads the international expertise center for autoimmune encephalitis in Rotterdam.
Rehabilitation Program
Recognizing subtle cognitive, social, or emotional symptoms is necessary, according to Titulaer. Currently, there is only one measuring instrument, the modified ranking scale (mRS), which is used in stroke patients. Thanks to all the information from Betty and the other patients, Brenner developed a new measuring instrument, the Patient Reported Outcome Scale for Encephalitis (PROSE), to record symptoms after encephalitis in daily care practice.
‘But a new measuring instrument alone is not enough’, says Brenner. ‘Rehabilitation care is now largely focused on physical recovery and insufficiently on cognitive and psychosocial well-being. More targeted rehabilitation programs can help patients better.’
After treatment, Betty had to reinvent herself. ‘I wondered: Who am I now? Who do I want to be, and what am I going to do with this experience? I am slower now. I used to be quick and sharp. I’m not that anymore. It takes time. People around me also had to get to know me again.’ It has given her meaning, though. She tells her story to help others. That a measuring instrument has now been created thanks to her cooperation moves her: ‘Wow!’
The research was made possible by Stichting Dioraphte and patient organizations Autoimmune Encephalitis Alliance, Encephalitis International, and ItsME.
Want to read more?
The impressive stories of patients with anti-NMDAR encephalitis in the media raise awareness of the disease, which was first identified in 2005. In 2016, young American journalist Susannah Cahalan wrote her memoirs in the book: ‘Brain on Fire’. In the Netherlands, there is much interest in the book ‘The Boy Without Yesterday’ and the podcast by Jur Deitmers (Dutch). On December 1, the three-part documentary series about him starts with Ivo Niehe and Josephine van der Erve on NPO1.
