[h=1]An infant born to a mother with anti-N-Methyl-D-Aspartate receptor (NMDAR) encephalitis (P3.208)[/h]
McGovern Medical School (UTHealth) Houston TX United States
Abstract
Objective: To describe a case of an infant born to a mother with anti-NMDAR encephalitis.
Background: Anti-NMDAR encephalitis is a synaptic autoimmune disorder that is mediated by antibodies against the NR1 subunit of the receptor.
1 Maternal-fetal transfer of NMDAR antibodies in pregnancy has been documented in both symptomatic and asymptomatic pregnant women.
2,
3 Animal models have demonstrated that NMDAR-specific auto antibodies(AAb) disrupt neocortical development and in a dose-dependent manner, these AAbs cause congenital brain injuries, resulting in long-term cognitive deficits.
4
Design/Methods: We present the case of a newborn infant with fatal outcome in the setting of elevated serum NMDAR antibodies.
Results: A term infant was born to a 32 year old female considered to be in remission from anti-NMDAR encephalitis associated with ovarian teratoma, status post oophorectomy. Mother presented with seizures and was found to be 37 weeks pregnant. C-section was performed due to fetal distress. Apgars were 1,2,5 and the infant was admitted to the neonatal intensive care unit. Due to clinical deterioration of the baby, extensive diagnostic workup was performed. Serum metabolic and infectious studies were negative. Cerebrospinal fluid (CSF) studies were unable to be obtained. Brain magnetic resonance imaging revealed diffuse cerebral edema with ischemic and hemorrhagic injury. Serum NMDAR antibody titers were elevated at 1:320. A course of intravenous immunoglobulin was given without clinical improvement. Care was withdrawn on day 20. Evaluation of the mother revealed elevated NMDAR antibodies in serum (1:80) and CSF and a new ovarian teratoma.
Conclusions: This case suggests that the presence of maternal NDMAR antibodies even in asymptomatic pregnant women can have devastating effects on the developing brain. Further discussion regarding pathophysiology of maternal-fetal NMDAR antibody transmission and the role of routine surveillance of pregnancy and antibody titers in women with history of anti-NMDAR encephalitis is necessary.
Study Supported by:
None
Disclosure: Dr. Chourasia has nothing to disclose. Dr. Watkins has nothing to disclose. Dr. Lankford has nothing to disclose. Dr. kamdar has nothing to disclose.
