title: Recovery from severe frontotemporal dysfunction at 3years after N-methyl-d-aspartic acid (NMDA) receptor antibody encephalitis.
abstract: Encephalitis associated with antibodies against N-methyl-d-aspartic acid (NMDA) receptor is characterized by severe memory deficits, decreased consciousness, epileptic seizures and movement disorders and occurs most commonly in young women. Recovery is mostly good but little is known about the disease course in patients whose treatment has been delayed severely. We present a 16-year-old girl with a 36-month follow-up. A single course of methylprednisolone attenuated some symptoms but severe and incapacitating frontotemporal syndrome remained. Second-line treatment with rituximab was initiated 12months after the onset of symptoms. A surprising recovery occurred 18months after treatment and 30months after onset. Recovery in NMDA receptor antibody-associated encephalitis can be severely delayed and does not have to be linear. Whether delayed therapy contributed to recovery in this patient cannot be answered with certainty. Spontaneous recovery independent of therapy is possible, as it has been observed previously as late as 3years after onset. Although serum antibodies disappeared with recovery in this patient, previous cases have shown serum antibodies to be unreliable markers of disease activity. Second-line treatment, especially with substances as well tolerated as rituximab, should at least be considered in NMDA receptor encephalitis with persistent neuropsychiatric syndromes after first-line therapy.
authors: Department of Neurology, University Medical Center Eppendorf, Hamburg, Germany. f.leypoldt@uke.de
authors: Frank
authors: F
authors: Leypoldt
authors: Mathias
authors: M
authors: Gelderblom
authors: Daniel
authors: D
authors: Schöttle
authors: Sascha
authors: S
authors: Hoffmann
authors: Klaus-Peter
authors: KP
authors: Wandinger
mesh_terms: D000293:Adolescent; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001323:Autoantibodies / Q000032:analysis; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000276:immunology*; D001921:Brain / Q000473:pathology; D004660:Encephalitis / Q000188:drug therapy* / Q000276:immunology*; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D057174:Frontotemporal Lobar Degeneration / Q000188:drug therapy* / Q000276:immunology* / Q000523:psychology; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D008279:Magnetic Resonance Imaging; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*; D020127:Recovery of Function; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D007074:Immunoglobulin G; D016194:Receptors, N-Methyl-D-Aspartate; D000069283:Rituximab