Recovery from severe frontotemporal dysfunction at 3years after N-methyl-d-aspartic acid (NMDA) receptor antibody encephalitis.
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Recovery from severe frontotemporal dysfunction at 3years after N-methyl-d-aspartic acid (NMDA) receptor antibody encephalitis. / Leypoldt, Frank; Gelderblom, Mathias; Schöttle, Daniel; Hoffmann, Sascha; Wandinger, Klaus-Peter.
in: J CLIN NEUROSCI, Jahrgang 20, Nr. 4, 4, 2013, S. 611-613.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Recovery from severe frontotemporal dysfunction at 3years after N-methyl-d-aspartic acid (NMDA) receptor antibody encephalitis.
AU - Leypoldt, Frank
AU - Gelderblom, Mathias
AU - Schöttle, Daniel
AU - Hoffmann, Sascha
AU - Wandinger, Klaus-Peter
N1 - Copyright © 2012 Elsevier Ltd. All rights reserved.
PY - 2013
Y1 - 2013
N2 - 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.
AB - 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.
KW - Adolescent
KW - Antibodies, Monoclonal, Murine-Derived
KW - Autoantibodies
KW - Autoimmune Diseases
KW - Brain
KW - Encephalitis
KW - Female
KW - Fluorescent Antibody Technique, Indirect
KW - Frontotemporal Lobar Degeneration
KW - Humans
KW - Immunoglobulin G
KW - Magnetic Resonance Imaging
KW - Receptors, N-Methyl-D-Aspartate
KW - Recovery of Function
U2 - 10.1016/j.jocn.2012.03.036
DO - 10.1016/j.jocn.2012.03.036
M3 - SCORING: Journal article
C2 - 23313527
VL - 20
SP - 611
EP - 613
JO - J CLIN NEUROSCI
JF - J CLIN NEUROSCI
SN - 0967-5868
IS - 4
M1 - 4
ER -