"The brain is on fire": the doctor successfully treats a child with a mysterious illness

The sick child did not respond to conventional treatment and had a poor prognosis
.
However, a team of doctors from Rutgers University believes that despite traditional wisdom against further treatment, there is hope
.

What happened in the next weeks of autumn 2020, described in a case study recently published in the European Medical Journal, is noteworthy and representative
of a new approach to effectively treating a strange disease, doctors say.

The study focused on the case of a 5-year-old girl who had anti-N-methyl-D-aspartate receptor encephalitis, a rare, difficult-to-diagnose form of brain dysfunction
.
After the treatment didn't work, the child was transferred to a rehabilitation center, and after three months in a catatonic state, a team of doctors from Rutgers University was called in to help
.

Susannah Cahalan, a writer for the New York Post, has written a best-selling book about autoimmune diseases that are thought to be caused by a combination of
environmental and genetic factors.
In her 2012 memoir, "Brain on Fire," she recounted her painful experience
of suffering from anti-NMDAR encephalitis and eventually recovering.
The book's name, and the Netflix movie that followed, comes from a term used by Kahalan's attending physician to describe Cahalan's catastrophic brain inflammation that eventually left the reporter in a trance until she was cured
.

"With autoimmune diseases, the body attacks specific systems that it mistakenly considers foreign," said Vikram Bhise, one of the authors of the case study and associate professor of pediatrics and neurology at Bristol-Myers Squibb Children's Hospital at Rutgers University and Bristol-Myers Squibb Children's Hospital
.
"In the case of anti-NMDAR encephalitis, the body attacks NMDA receptors
in the brain.
This leads to a range of mental, cognitive and motor problems
.
(NMDA receptors are brain structures
that play an important role in learning and memory.
) )

When the child's mother needed another opinion, the family physician contacted Bhise, and Bhise and two other Rutgers doctors were called into the case
.
The mother told the Rutgers team that after experiencing rapid mental and physical degradation, the child has been unable to move and unresponsive
.

In general, time is key to treating autoimmune diseases, Bhise said, and the standard of care states that if it is too long, no treatment is useful
.
Most often, any damage caused by the disease cannot be recovered
.

Bhise directed the child to be admitted to the pediatric intensive care unit at Bristol-Myers Squibb Children's Hospital at Robert WoodJohnson University Hospital and decided to try treatment again
.

"I said, 'You know, a lot of
time has passed.
But I think you're going to try these things anyway,'" Bhise recalled
.

The child received a course of steroids, collective antibodies, and long-term immunosuppressants
.
Bhise and his team decided to do a series of plasma swaps aimed at resetting the immune system by removing all inflammation from the
blood.

They saw progress
almost immediately.

"After one or two exchanges, the mother said, 'Hey, I think it's a little different,'" Bhise said
.
"I mean, no one knows this child
better than her mother.
"

They continued treatment, eventually performing nearly 12 more plasma exchanges, and the child's condition steadily improved until he fully recovered
.

"I think the lesson we learned from here is that you can still treat this disease
after time has passed," he said.
"You shouldn't stop trying
.
It's important to know this so that others in the field don't give up
prematurely when they see children — and possibly adults — with hard-to-treat anti-NMDAR encephalitis.
”

References:

“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