“Brain on Fire” – Doctors Successfully Treat a Child Suffering From a Mysterious Disease

Brain Fire Disease Neuroscience Concept

The child suffered from anti-NMDR (N-methyl-D-aspartate receptor) encephalitis, a rare and difficult to diagnose brain defect.

A blood plasma exchange helped a 5-year-old with a rare autoimmune disorder get better.

The prognosis for sick children, who did not respond to conventional treatment, was bleak. Even so, a group of doctors at Rutgers University thought there might be hope for no further treatment, despite conventional wisdom.

What happened in the following few weeks in the fall of 2020, is described in a recently published case study European Medical JournalRemarkable and representative of a new method to effectively treat a rare disease, doctors said.

The study focuses on the medical case of a 5-year-old girl who suffered from anti-NMDR (N-methyl-D-aspartate receptor) encephalopathy, a rare and difficult to diagnose brain defect. Unresponsive to treatment, the child was transferred to a rehabilitation center and remained in a catatonic state for three months when a team of Rutgers physicians was called in to help.

Susannah Cahalan, author of the New York Post, has written a best-selling book on autoimmune diseases, which are thought to stem from both environmental and genetic causes. In her 2012 memoir, “Brain on Fire,” she recounted her medical ordeal with anti-NMDAR encephalitis and eventual recovery. The title of the book, as well as the subsequent Netflix film, derives from a term used by Cahalan’s treating physician to describe the catastrophic brain inflammation that eventually left the reporter in a trance-like state until he recovered.

“With autoimmune diseases, the body attacks a specific system that it mistakenly identifies as foreign,” said Vikram Vise, author of the case study and an associate professor of pediatrics and neurology and director of the Division of Child Neurology and Neurodevelopmental Disabilities at Rutgers Roberts. Bristol-Myers Squibb Children’s Hospital at Wood Johnson Medical School and Robert Wood Johnson University Hospital. “In NMDAR encephalitis, the body attacks the NMDA receptors in the brain. This causes a huge deficit, manifested by a combination of mental, cognitive and motor problems.” (NMDA receptors are brain structures that play an important role in learning and memory.)

Bies and two other Rutgers doctors were called into the case when the child’s mother sought a second opinion and contacted Bies, the family’s attending physician. The mother told the Rutgers team that the child was immobile and unresponsive after a rapid phase of mental and physical deterioration.

Generally, time is of the essence when treating autoimmune diseases, and the standard of care dictates that no treatment is effective if too much time passes, Visse said. Most of the time, any damage caused by the disease is irreversible.

Vissey admitted the child to the pediatric intensive care unit at Bristol-Myers Squibb Children’s Hospital at Robert Wood Johnson University Hospital and decided to try one more treatment.

“I said, ‘You know, it’s been a long time. But I think you still have to try these things,” recalls Bheesh.

The child was given a course of steroids, pooled antibodies and a long-term immunosuppressant. Bhish and his team decided to conduct a series of blood tests[{” attribute=””>plasma exchanges designed to reset the immune system by cleaning out all the inflammation in the bloodstream.

They saw progress almost instantly.

“Within one or two exchanges, the mom said, ‘Hey, I think something’s a little different,’” Bhise said. “I mean, no one knew this child better than her mom.”

As they continued with the treatment, ultimately with nearly a dozen more plasma exchanges, the child improved steadily until she had made a full recovery.

“I think the lesson that we’ve learned here is that you can still treat this disease after time has passed,” Bhise said. “You shouldn’t stop trying. This is important to know so that other folks in the field do not prematurely give up when they see children – and probably adults as well – with difficult-to-treat anti-NMDAR encephalitis.”

Reference: “Never Too Late to Treat NMDAR Encephalitis: A Paediatric Case Report and Review of Literature” by Yisha Cheng, Dalya Chefitz and Vikram Bhise, 9 August 2022, EMJ Neurology.
DOI: 10.33590/emjneurol/22-00096

Other Rutgers physicians who were members of the medical team and authors of the case study included Yisha Cheng, a resident physician in pediatric medicine and a 2020 graduate of Rutgers Robert Wood Johnson Medical School; and Dalya Chefitz, a physician in the department of pediatrics and director of the division of pediatric hospital medicine at The Bristol-Myers Squibb Children’s Hospital at Robert Wood Johnson University Hospital.


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