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Pediatric anti-NMDA (N-methyl D-aspartate) receptor encephalitis.
Pediatr Neurol. 2014 May; 50(5):507-10.PN

Abstract

BACKGROUND

We report the clinical features and course of pediatric patients presenting with anti-N-methyl D-aspartate receptor (NMDA-R) encephalitis.

METHODS

Single-center 4-year observational study of pediatric encephalitis associated with NMDA-R antibodies in the serum and/or the cerebrospinal fluid.

RESULTS

Three girls with anti-NMDA-R encephalitis were identified. All presented with an acute hyperkinetic movement disorder and seizures, expressive aphasia, and emotional lability requiring inpatient treatment for 1-3 months. Imaging and electroencephalogram findings were nondiagnostic. None had an underlying tumor or ovarian teratoma. All received immune-modulatory therapy, including one or more of the following: high-dose methyl-prednisolone, plasma exchange, intravenous immunoglobulin or mycophenolate mofetil. Two of the three patients relapsed within 6 months of presentation and required retreatment with plasma exchange. All have remained in subsequent remission, with two of the three requiring second-line immunotherapy with rituximab.

CONCLUSIONS

Hyperkinetic movements in pediatric patients presenting with acute encephalopathy and prominent psychiatric symptoms should elicit a search for NMDA-R antibodies early in the evaluation. Relapses require aggressive immunomodulatory treatment for remission. This series highlights a unique positron emission tomography scan finding of hypermetabolism in one of the patients that correlated with her clinical symptoms. Recovery and rehabilitation can be prolonged, often taking years after the initial diagnosis. Early identification and treatment is likely to reduce relapses and limit morbidity associated with this potentially devastating but treatable encephalitis.

Authors+Show Affiliations

Alfred I. duPont Children's Hospital - Pediatric Neurology, Talleyville, Delaware.Mayo Clinic Florida - Jacksonville, Jacksonville, Florida; Nemours Children's Clinic - Jacksonville, Jacksonville, Florida.Mayo Clinic Florida - Jacksonville, Jacksonville, Florida; Nemours Children's Clinic - Jacksonville, Jacksonville, Florida.Mayo Clinic Florida - Jacksonville, Jacksonville, Florida; Nemours Children's Clinic - Jacksonville, Jacksonville, Florida. Electronic address: rsheth@nemours.org.

Pub Type(s)

Case Reports
Journal Article
Observational Study

Language

eng

PubMed ID

24656207

Citation

Salvucci, Alana, et al. "Pediatric anti-NMDA (N-methyl D-aspartate) Receptor Encephalitis." Pediatric Neurology, vol. 50, no. 5, 2014, pp. 507-10.
Salvucci A, Devine IM, Hammond D, et al. Pediatric anti-NMDA (N-methyl D-aspartate) receptor encephalitis. Pediatr Neurol. 2014;50(5):507-10.
Salvucci, A., Devine, I. M., Hammond, D., & Sheth, R. D. (2014). Pediatric anti-NMDA (N-methyl D-aspartate) receptor encephalitis. Pediatric Neurology, 50(5), 507-10. https://doi.org/10.1016/j.pediatrneurol.2014.01.012
Salvucci A, et al. Pediatric anti-NMDA (N-methyl D-aspartate) Receptor Encephalitis. Pediatr Neurol. 2014;50(5):507-10. PubMed PMID: 24656207.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Pediatric anti-NMDA (N-methyl D-aspartate) receptor encephalitis. AU - Salvucci,Alana, AU - Devine,Irisa M, AU - Hammond,David, AU - Sheth,Raj D, Y1 - 2014/01/06/ PY - 2013/10/29/received PY - 2013/12/13/revised PY - 2014/01/01/accepted PY - 2014/3/25/entrez PY - 2014/3/25/pubmed PY - 2014/12/15/medline KW - anti-NMDA-R KW - aphasia KW - autoimmune KW - dyskinesia KW - encephalitis KW - neuropsychiatric KW - pediatric SP - 507 EP - 10 JF - Pediatric neurology JO - Pediatr. Neurol. VL - 50 IS - 5 N2 - BACKGROUND: We report the clinical features and course of pediatric patients presenting with anti-N-methyl D-aspartate receptor (NMDA-R) encephalitis. METHODS: Single-center 4-year observational study of pediatric encephalitis associated with NMDA-R antibodies in the serum and/or the cerebrospinal fluid. RESULTS: Three girls with anti-NMDA-R encephalitis were identified. All presented with an acute hyperkinetic movement disorder and seizures, expressive aphasia, and emotional lability requiring inpatient treatment for 1-3 months. Imaging and electroencephalogram findings were nondiagnostic. None had an underlying tumor or ovarian teratoma. All received immune-modulatory therapy, including one or more of the following: high-dose methyl-prednisolone, plasma exchange, intravenous immunoglobulin or mycophenolate mofetil. Two of the three patients relapsed within 6 months of presentation and required retreatment with plasma exchange. All have remained in subsequent remission, with two of the three requiring second-line immunotherapy with rituximab. CONCLUSIONS: Hyperkinetic movements in pediatric patients presenting with acute encephalopathy and prominent psychiatric symptoms should elicit a search for NMDA-R antibodies early in the evaluation. Relapses require aggressive immunomodulatory treatment for remission. This series highlights a unique positron emission tomography scan finding of hypermetabolism in one of the patients that correlated with her clinical symptoms. Recovery and rehabilitation can be prolonged, often taking years after the initial diagnosis. Early identification and treatment is likely to reduce relapses and limit morbidity associated with this potentially devastating but treatable encephalitis. SN - 1873-5150 UR - https://www.unboundmedicine.com/medline/citation/24656207/Pediatric_anti_NMDA__N_methyl_D_aspartate__receptor_encephalitis_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0887-8994(14)00013-7 DB - PRIME DP - Unbound Medicine ER -