Recovery from severe frontotemporal dysfunction at 3years after N-methyl-d-aspartic acid (NMDA) receptor antibody encephalitis.

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 -