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Abstract Details

Pediatric anti-NMDA Receptor Encephalitis
Autoimmune Neurology
P6 - Poster Session 6 (12:00 PM-1:00 PM)
15-008

NMDAR encephalitis is a severe central nervous system inflammatory disease, often causing significant disability and potentially death. Over half of cases of NMDAR encephalitis occur in children, with more neurologic symptoms in younger children and more psychiatric symptoms in adolescents. Although most patients have substantial recovery, timely and appropriate immunotherapy is paramount. An association between HSE and NMDAR has been noted, with up to 30% of HSE patients subsequently developing NMDAR encephalitis. However, up to 30% of patients with HSE have anti-NMDAR antibodies without symptoms of NMDAR encephalitis, complicating the question of causality between these processes.

To evaluate clinical, diagnostic, management, and outcome factors in children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, with an additional subgroup analysis of those with history of herpes simplex encephalitis (HSE). 
Seventeen cases of NMDAR encephalitis at our institution were identified between 2012 and 2019, six of which also had a history of HSE. Demographic data, clinical characteristics, immunotherapy, and outcome data were collected on all patients. 

Median age was 13.5 years, 59% of patients were female, and 41% were African-American, 47% Caucasian, and 12% Hispanic. At disease nadir, 82% of all patients had behavioral symptoms, 71% had movement disorders, and 82% had seizures. Of HSE patients, 50% had behavioral symptoms, 67% had movement disorders, and 100% had seizures. Median modified Rankin Scale (mRS) at disease nadir was four for both groups (all patients and HSE patients). All patients received a median of three immunotherapies, as opposed to one for HSE patients. At most recent follow-up, median mRS for both groups was two.

Most patients with NMDAR encephalitis have a constellation of behavioral symptoms, movement disorders, and seizures. Most patients have significant disability at disease nadir, with substantial improvement over time. Outcomes of infants with HSE were reflective primarily of sequelae from HSE.

Authors/Disclosures
Lydia Robinson Marcus, MD (University of Alabama Medical Center, Child Neurology)
PRESENTER
Dr. Marcus has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Ragsdale LLC. The institution of Dr. Marcus has received research support from NIAID.
Jayne Ness, MD (UAB Dept of Pediatrics, Div Pediatric Neurology) An immediate family member of Dr. Ness has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Jan One. The institution of Dr. Ness has received research support from Chugai/Roche. The institution of Dr. Ness has received research support from National Multiple Sclerosis Society. The institution of an immediate family member of Dr. Ness has received research support from NIH. An immediate family member of Dr. Ness has received publishing royalties from a publication relating to health care.