title: Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Rasmussen-like Syndrome: An Association?
abstract: N-methyl-D-aspartate (NMDA) receptor encephalitis is an immune-mediated condition that has a broad spectrum of manifestations, including seizures, coma, psychosis, and focal neurological deficits. Although usually a diffuse process, unihemispheric involvement mimicking early stages of Rasmussen encephalitis can occur. Rasmussen's encephalitis is a unique syndrome characterized by progressive hemiplegia, drug-resistant focal epilepsy, cognitive decline, and hemispheric brain atrophy contralateral to the hemiplegia.
  
  
  
  We describe a two-year-old girl with progressive right weakness and epilepsia partialis continua, concerning for early Rasmussen's encephalitis, who tested positive for anti-NMDA receptor antibodies. She experienced complete clinical recovery after immunotherapy. Anti-NMDA receptor antibodies were absent at three weeks and again at one year after the first treatment of intravenous immunoglobulin.
  
  
  
  There are few reports of Rasmussen-like encephalitis in individuals with anti-NMDA receptor antibody positivity. Thus the clinical significance of this association is yet to be determined. In addition, several other antibodies have been documented in individuals with Rasmussen encephalitis. The lack of a consistently reported antibody in Rasmussen encephalitis patients and the temporary nature of the anti-NMDA receptor antibody in our patient raise the following question: Is the presence of anti-NMDA receptor antibodies the cause of the symptoms or secondary to the pathogenic process?
authors.affiliation: Pediatric Neurology, Cohen Children's Medical Center, New Hyde Park, New York. Electronic address: Kgurchar12@northwell.edu.
authors.forename: Kevin
authors.initials: K
authors.lastname: Gurcharran
authors.affiliation: Pediatric Neurology, Cohen Children's Medical Center, New Hyde Park, New York.
authors.forename: Shefali
authors.initials: S
authors.lastname: Karkare
mesh_terms: D060426:Anti-N-Methyl-D-Aspartate Receptor Encephalitis / Q000150:complications* / Q000000981:diagnostic imaging / Q000276:immunology / Q000628:therapy; D001921:Brain / Q000000981:diagnostic imaging / Q000503:physiopathology; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004660:Encephalitis / Q000150:complications* / Q000000981:diagnostic imaging / Q000276:immunology / Q000628:therapy; D005260:Female; D006801:Humans; D007167:Immunotherapy
keywords: AMPA receptor; NMDA receptor; Rasmussen syndrome; autoimmune; encephalitis; glutamate; pediatrics