期刊
ANNALS OF NEUROLOGY
卷 75, 期 3, 页码 411-428出版社
WILEY
DOI: 10.1002/ana.24117
关键词
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资金
- Dutch Cancer Society
- ErasmusMC fellowship
- Fonds zur Forderung der wissenschaftlichen Forschung, Austria [J3230, W1206, I916]
- Forschungsforderungsfonds Hamburg Eppendorf Exzellenzjahr
- NIH National Institute of Neurologic Disorders and Stroke [RO1NS077851]
- McKnight Neuroscience of Brain Disorders award
- Fundacio la Marato TV3 [101530, 101610]
- Fondo de Investigaciones Sanitarias de la Seguridad Social, Spain [PI11/01780]
- A National MS Society
- Ministry of Education, Culture, Sports, Science, and Technology of Japan [25461297]
- Kinki University
- Austrian Science Fund (FWF) [I916, J3230] Funding Source: Austrian Science Fund (FWF)
- Grants-in-Aid for Scientific Research [25461297] Funding Source: KAKEN
- Austrian Science Fund (FWF) [I 916] Funding Source: researchfish
- ICREA Funding Source: Custom
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
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