Neurology Today Conference Reporter: CNS Annual Meeting

Monday, October 9, 2017

Subset of Children with Anti-NMDA Receptor Encephalitis Have Persistent Impairments

BY JAMIE TALAN

KANSAS CITY, MO—Some children with anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis remain impaired even after months of intensive rehabilitation, according to an analysis of patients who were admitted to a rehabilitation hospital in New York state.

Reporting the results here at the Child Neurology Society annual meeting last week, Jay E. Selman, MD, chief of neurology at the Blythedale Children's Hospital in Valhalla, NY, said, "Most patients show functional improvement by discharge, but many have persistent neurological, cognitive, and behavioral problems."

Anti-NMDA receptor encephalitis is triggered by antibodies that attack NMDA type glutamate receptors at central neuronal synapses, Dr. Selman said. The antibodies disrupt NMDA-containing cells that control memory and behavior and other important brain functions. Patients can suffer psychosis, severe memory impairments, seizures, and abnormal movements.

Dr. Selman and his colleagues reviewed the medical records of 29 patients admitted to the rehabilitation facility since 2007. They looked at the duration of inpatient rehabilitation, medication use, severity of the illness, presence of teratomas that have been found to put female patients at risk, mortality, and changes in functional state on admission and discharge. Three of the patients are still at the center.

Dr. Selman said that the average age of the children was 14 years old, and the median length of stay for these patients was 5.5 months. Almost half were discharged from tertiary care into rehabilitation with tracheostomies, and two-thirds had feeding tubes. Functional assessments administered suggest that most were severely impaired upon arrival. One patient died.

The medical records showed that about a third of the female patients had ovarian teratomas. At present, no other risk factors have been identified. The course of treatment includes corticosteroids, IV immunoglobulin G, and plasmapheresis, Dr. Selman said, adding that the key to recovery is early diagnosis and treatment.

"This is the most common non-infectious form of encephalitis," Dr. Selman added. "The problem is getting neurologists to think about the diagnosis. We need to get these kids diagnosed early and treat aggressively. A subset of patients will require intensive in-patient rehabilitation. They are functioning but not like they were before."

Dr. Selman added that 56 percent of the patients were back at school within three months of discharge. After one year, three quarters were attending classes.

Commenting on the study, Michael D. Geschwind, MD, PhD, professor of neurology at the University of California, San Francisco (UCSF) School of Medicine, said the results were interesting, but he added: "The cases are biased towards the severest patients, as they are being discharged from in-patient services to an in-patient rehab. They already haven't recovered."

Dr. Geschwind, who was not involved with the study, said it would be important to know whether the age of the children had an impact on recovery, and whether those with teratomas did worse or better following treatment. "Most of the patients we see at UCSF are still continuing to recover after a year," he said.

LINK UP FOR RELATED INFORMATION:​

Dalmau J. NMDA receptor encephalitis and other antibody-mediated disorders of the synapse: The 2016 Cotzias Lecture. Neurology 2016;87(23):2471-2482.

Armangue T, Titulaer MJ, Málaga I, et al, for the Spanish Anti-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis Work Group. Pediatric anti-N-methyl-D-aspartate receptor encephalitis-clinical analysis and novel findings in a series of 20 patients. J Pediatrics 2013;162:850-856.