Overlapping demyelinating syndromes and anti-N-methyl-D-aspartate receptor encephalitis

Objective To report the clinical, radiological, and immunological association of demyelinating disorders with anti–N‐methyl‐D‐aspartate receptor (NMDAR) encephalitis. Methods Clinical and radiological analysis was done of a cohort of 691 patients with anti‐NMDAR encephalitis. Determination of antibo...

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Veröffentlicht in:Annals of neurology Jg. 75; H. 3; S. 411 - 428
Hauptverfasser: Titulaer, Maarten J., Höftberger, Romana, Iizuka, Takahiro, Leypoldt, Frank, McCracken, Lindsey, Cellucci, Tania, Benson, Leslie A., Shu, Huidy, Irioka, Takashi, Hirano, Makito, Singh, Gagandeep, Cobo Calvo, Alvaro, Kaida, Kenichi, Morales, Pamela S., Wirtz, Paul W., Yamamoto, Tomotaka, Reindl, Markus, Rosenfeld, Myrna R., Graus, Francesc, Saiz, Albert, Dalmau, Josep
Format: Journal Article
Sprache:Englisch
Veröffentlicht: United States Blackwell Publishing Ltd 01.03.2014
Wiley Subscription Services, Inc
Schlagworte:
Adolescent
Adult
Animals
Anti-N-Methyl-D-Aspartate Receptor Encephalitis - complications
Anti-N-Methyl-D-Aspartate Receptor Encephalitis - diagnosis
Anti-N-Methyl-D-Aspartate Receptor Encephalitis - immunology
Anti-N-Methyl-D-Aspartate Receptor Encephalitis - pathology
Aquaporin 4 - immunology
Autoantibodies - immunology
Brain - immunology
Brain - pathology
Child
Child, Preschool
Demyelinating Diseases - complications
Demyelinating Diseases - diagnosis
Demyelinating Diseases - immunology
Demyelinating Diseases - pathology
Encephalitis
Female
Humans
Infant
Male
Medical research
Middle Aged
Multiple sclerosis
Myelin-Oligodendrocyte Glycoprotein - immunology
Neuroimaging
Rats
Receptors, N-Methyl-D-Aspartate - immunology
ISSN:0364-5134, 1531-8249, 1531-8249
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Zusammenfassung:Objective To report the clinical, radiological, and immunological association of demyelinating disorders with anti–N‐methyl‐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 (p = 0.011). 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. Ann Neurol 2014;75:411–428
Bibliographie:ark:/67375/WNG-H16MZ2PQ-W
istex:1630F13FC6D25F04E867D9BA6EDFBCEFD0734E94
ArticleID:ANA24117
Members of the Clinical Investigators Group are listed in the Appendix on page 17.
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ISSN:0364-5134
1531-8249
1531-8249
DOI:10.1002/ana.24117