The child suffered from anti-NMDAR (N-methyl-D-aspartate receptor) encephalitis, a rare and difficult-to-diagnose brain dysfunction.
Plasmapheresis helped a 5-year-old with a rare autoimmune disease recover.
The prognosis for sick children who did not respond to conventional treatments was dismal. Nevertheless, a group of doctors at Rutgers University thought there was hope, despite conventional wisdom against pursuing further treatment.
What happened in the weeks following the fall of 2020 is described in a recently published case study. european medical journalsaid the doctor, is noteworthy and represents a new approach to effectively treat bizarre ailments.
This study focuses on the medical case of a 5-year-old girl with anti-NMDAR (N-methyl-D-aspartate receptor) encephalopathy, a rare and difficult-to-diagnose brain dysfunction. Unresponsive, the child was transferred to a rehabilitation center and was in a nervous state for three months when Dr. Rutgers’ team was called for help.
New York Post writer Susannah Cahalan has written a best-selling book on autoimmune diseases. Autoimmune diseases are believed to be caused by both environmental and genetic factors. In her 2012 memoir, Brain on Fire, she recounts her medical ordeal suffering from anti-NMDAR encephalitis and eventual recovery. The title of the book and her subsequent Netflix movie derive from the term Kahalan’s personal doctor used to describe the devastating inflammation of the brain.
“In autoimmune diseases, the body mistakenly identifies certain systems as foreign and attacks them,” says case study author and associate professor of pediatrics and neurology, Pediatric Neurology at Rutgers-Robert University. and Director of Neurodevelopmental Disorders Vikram Bisse. Wood Johnson Medical School and Bristol-Myers Squibb Children’s Hospital at Robert Wood Johnson University Hospital. “In anti-NMDAR encephalitis, the body attacks the NMDA receptors in the brain. (NMDA receptors are brain structures that play an important role in learning and memory.)
Bhise and two other Rutgers doctors were called to the case when the child’s mother sought a second opinion and the family physician contacted Bhise. The mother reported to Rutgers’ team that the child remained immobile and unresponsive after a rapid phase of mental and physical degeneration.
In general, time is of the essence in treating autoimmune diseases, and standard care stipulates that if too much time passes, the treatment is useless, Bhise said. It cannot be undone.
Bhise ordered that the child be admitted to the pediatric intensive care unit of Bristol-Myers Squibb Children’s Hospital at Robert Wood Johnson University Hospital to try alternative treatments.
“I said, ‘Look, it’s been a lot of time. But I think you still need to try these things.
The child had received a course of steroids, pooled antibodies, and long-term immunosuppressants. Bise and his team decided to administer a series of blood[{” 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.
