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Case Reports
. 2022 Nov 16;22(1):434.
doi: 10.1186/s12883-022-02974-x.

Anti-NMDA receptor encephalitis and MOG-associated demyelination - a case report with long-term follow-up and a systematic review

Klaus Berek  1 , Astrid Grams  2 , Christian Uprimny  3 , Manuela Prieschl  1 , Melanie Ramberger  1 , Iris Unterberger  1 , Florian Deisenhammer  1 , Markus Reindl  1 , Harald Hegen  4
Affiliations
Case Reports

Anti-NMDA receptor encephalitis and MOG-associated demyelination - a case report with long-term follow-up and a systematic review

Klaus Berek et al. BMC Neurol. .

Abstract

Background: Overlap syndromes of anti-NMDA receptor encephalitis and MOG-mediated demyelination have been reported. In this case we provide a long-term longitudinal follow-up of clinical and imaging characteristics as well as of antibody dynamics.

Case presentation: We report a 32-year-old male patient who presented with psychosis, decreased consciousness and movement disorders and was tested positive for anti-NMDA receptor antibodies. Forty-four months after symptom onset and diagnosis of autoimmune encephalitis, he suffered from relapse. At this time, the patient developed anti-MOG and anti-Caspr2 antibodies. Treatment with plasmapheresis, steroids and rituximab eventually led to substantial clinical and radiological improvement. Anti-Caspr2 antibodies persisted, anti-NMDA receptor antibodies decreased, while anti-MOG antibodies turned negative again.

Conclusion: We provide long-term longitudinal follow-up of a patient with anti-NMDA receptor encephalitis who developed triple antibody positivity at the time of relapse. Antibody dynamics were associated with clinical disease course.

Keywords: Anti-N-Methyl-D-Aspartate; Case report; Demyelination; Encephalitis; Myelin Oligodendrocyte Glycoprotein; NMDA.

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Conflict of interest statement

Klaus Berek has participated in meetings sponsored by, received travel funding from or received honoraria for acting as an advisor/speaker for Roche, Biogen, TEVA and Sanofi.

Astrid Grams reports no conflicts of interest.

Christian Uprimny reports no conflicts of interest.

Manuela Prieschl reports no conflicts of interest.

Melanie Ramberger reports no conflicts of interest.

Iris Unterberger reports no conflicts of interest.

Florian Deisenhammer has participated in meetings sponsored by or received honoraria for acting as an advisor/speaker for Almirall, Alexion, Biogen, Celgene, Genzyme-Sanofi, Merck, Novartis Pharma, Roche, and TEVA ratiopharm. His institution has received research grants from Biogen and Genzyme Sanofi. He is section editor of the MSARD Journal (Multiple Sclerosis and Related Disorders) and review editor of Frontiers Neurology.

Markus Reindl is supported by research grants from the Austrian Science Fund (FWF project P32699), the Austrian Research Promotion Agency, Euroimmun and Roche; consulting fees and advisory board from Roche (to institution). He works at the Clinical Department of Medical University of Innsbruck which offers diagnostic testing for MOG and other autoantibodies.

Harald Hegen has participated in meetings sponsored by, received speaker honoraria or travel funding from Biogen, Celgene, Merck, Novartis, Sanofi-Genzyme, Siemens, Teva, and received honoraria for acting as consultant for Biogen, Celgene, Novartis and Teva.

Figures

Fig. 1
Fig. 1
Excerpts of MRI and PET scans performed. Legend: The cerebral MRI scan during the demyelinating event at month 44 revealed a T1 hypointense, Gd enhancing (A), T2 hyperintense (B) lesion in the left Gyrus frontalis, which shows hypermetabolism in the FET-PET scan (C: original FET-PET image, D: MRI fused FET-PET image; SUV max: 2.64). MRI showed regression of lesions over time until month 66 (E, G, I: contrast-enhanced T1-weighted imaging. F, H, J: T2-weighted imaging). Abbreviations: FET-PET/CT = O-(2[18F]fluoroethyl)-L-tyrosine—positron emission tomography / computer tomography; Gd = Gadolinium; M = Month; MRI = Magnetic resonance imaging
Fig. 2
Fig. 2
Overview of the main clinical characteristics, diagnostic results and therapeutic interventions. Legend: White “brains” indicate normal MRI findings and black “brains” indicate pathological MRI findings. White and black “brains” indicate radiological improvement. * indicates the single seizure which occurred in month 1. NMDAR IgG were detected by commercially available CBA (Euroimmun, Lübeck, Germany), titers are provided. Caspr2 IgG were detected by commercially available CBA (Euroimmun; positive at a titer of 1:10). MOG IgG were detected by an in-house CBA [24, 25], titers are provided. Abbreviations: CBA, cell-based assay PLEX = plasma exchange; IVMP = intravenous methylprednisolone; CSF = cerebrospinal fluid; WBC = white blood cells; OCB = oligoclonal bands; MRI = magnetic resonance tomography; FDG = 18F-fluoro-2-deoxy-D-glucose; FET = O-(2[18F]fluoroethyl)-L-tyrosine; PET = positron emission tomography; Caspr2 = contactin-associated protein-like 2; NMDAR = N-methyl-D-aspartate receptors; MOG = Myelin Oligodendrocyte Glycoprotein
See this image and copyright information in PMC

References

    1. Hegen H, Reindl M. Recent developments in MOG-IgG associated neurological disorders. Ther Adv Neurol Disord. 2020;13:1756286420945135. - PMC - PubMed
    1. Dalmau J, Armangue T, Planaguma J, Radosevic M, Mannara F, Leypoldt F, et al. An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models. Lancet Neurol. 2019;18(11):1045–1057. - PubMed
    1. Titulaer MJ, Höftberger R, Iizuka T, Leypoldt F, McCracken L, Cellucci T, et al. Overlapping demyelinating syndromes and anti–N-methyl-D-aspartate receptor encephalitis. Ann Neurol. 2014;75(3):411–428. - PMC - PubMed
    1. Aoe S, Kokudo Y, Takata T, Kobara H, Yamamoto M, Touge T, et al. Repeated anti-N-methyl-D-aspartate receptor encephalitis coexisting with anti-myelin oligodendrocyte glycoprotein antibody-associated diseases: a case report. Mult Scler Relat Disord. 2019;35:182–184. - PubMed
    1. Cao L, Ren L, Huang X. Clinical analysis of a patient simultaneously positive for antibodies of myelin oligodendrocyte glycoprotein and anti-N-methyl-D-aspartate receptor: a case report. Medicine (Baltimore) 2021;100(1):e24234. - PMC - PubMed

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