Overlapping demyelinating syndromes and antiN-methyl-D-aspartate receptor encephalitis.
Ann Neurol
; 75(3): 411-28, 2014 Mar.
Article
em En
| MEDLINE
| ID: mdl-24700511
ABSTRACT
OBJECTIVE:
To report the clinical, radiological, and immunological association of demyelinating disorders with antiNmethyl- D-aspartate receptor (NMDAR) encephalitis.METHODS:
Clinical and radiological analysis was done of a cohort of 691 patients with anti-NMDAR encephalitis. Determination of antibodies to NMDAR, aquaporin-4 (AQP4), and myelin oligodendrocyte glycoprotein (MOG) was performed using brain immunohistochemistry and cell-based assays.RESULTS:
Twenty-three of 691 patients with anti-NMDAR encephalitis had prominent magnetic resonance imaging (MRI) and/or clinical features of demyelination. Group 1 included 12 patients in whom anti-NMDAR encephalitis was preceded or followed by independent episodes of neuromyelitis optica (NMO) spectrum disorder (5 cases, 4 anti-AQP4 positive) or brainstem or multifocal demyelinating syndromes (7 cases, all anti-MOG positive). Group 2 included 11 patients in whom anti-NMDAR encephalitis occurred simultaneously with MRI and symptoms compatible with demyelination (5 AQ4 positive, 2 MOG positive). Group 3 (136 controls) included 50 randomly selected patients with typical anti-NMDAR encephalitis, 56 with NMO, and 30 with multiple sclerosis; NMDAR antibodies were detected only in the 50 anti-NMDAR patients, MOG antibodies in 3 of 50 anti-NMDAR and 1 of 56 NMO patients, and AQP4 antibodies in 48 of 56 NMO and 1 of 50 anti-NMDAR patients (p<0.0001 for all comparisons with Groups 1 and 2). Most patients improved with immunotherapy, but compared with anti-NMDAR encephalitis the demyelinating episodes required more intensive therapy and resulted in more residual deficits. Only 1 of 23 NMDAR patients with signs of demyelination had ovarian teratoma compared with 18 of 50 anti-NMDAR controls (p50.011).INTERPRETATION:
Patients with anti-NMDAR encephalitis may develop concurrent or separate episodes of demyelinating disorders, and conversely patients with NMO or demyelinating disorders with atypical symptoms (eg, dyskinesias, psychosis) may have anti-NMDAR encephalitis.
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Doenças Desmielinizantes
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Encefalite Antirreceptor de N-Metil-D-Aspartato
Tipo de estudo:
Diagnostic_studies
Limite:
Adolescent
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Adult
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Animals
/
Child
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Child, preschool
/
Female
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Humans
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Infant
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Male
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Middle aged
Idioma:
En
Revista:
Ann Neurol
Ano de publicação:
2014
Tipo de documento:
Article