Abstract
FRONTIERS IN MEDICAL CASE REPORTS - Volume 6; Issue 1, (Jan-Feb, 2025)
Pages: 01-06
AQP4-IgG-Seropositive Patient Who Silently Carried Anti-NMDAR Antibodies Developed Encephalitis During A Relapse
Andrés María Villa, Carla Seimandi, Ana Mariel Finkelsteyn, Analisa Manin, Graciela Ramos
Category:Medical Case Reports
Abstract:
The association of autoimmune diseases is relatively common. Comorbidity anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis and Neuromyelitis Optica Spectrum Disorder (NMOSD) is an unusual and rare overlapping syndrome. We report the case of an AQP4-IgG-seropositive NMOSD patient who developed anti-NMDAR encephalitis during corticosteroid treatment for a relapse of Longitudinal Extensive Transverse Myelitis (LETM), with the presence of anti-NMDAR antibodies in the serum two years earlier. The patient showed significant improvement in both clinical syndromes with a good response to steroid and immunosuppressive therapy. Conclusion Early diagnosis and treatment of overlapping autoimmune syndromes, such as AQP4+IgG seropositive NMOSD with anti-NMDAR encephalitis, are key factors for ensuring early and better recovery outcomes.
Keywords: Encephalitis, Anti-N-Methyl-D-Aspartate Receptor, Neuromyelitis Optica Spectrum Disorders (NMOSD), Overlap Demyelinating Syndrome, AQP4, NMDA
References:
Irani SR. Autoimmune Encephalitis. Continuum (Minneap Minn) 2024; 30: 995-1020.
Nan D, Zhang Y, Han J, Jin T. Clinical features and management of coexisting anti-N-methyl-D-aspartate receptor encephalitis and myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis: a case report and review of the literature. Neurol Sci 2021; 42: 847-855.
Sechi E. NMOSD and MOGAD. Continuum (Minneap Minn) 2024; 30: 1052-1087.
Shahmohammadi S, Doosti R, Shahmohammadi A, Mohammadianinejad SE, Sahraian MA, Azimi AR, Harirchian MH, Asgari N, Naser Moghadasi A. Autoimmune diseases associated with Neuromyelitis Optica Spectrum Disorders: A literature review. Mult Scler Relat Disord 2019; 27: 350-363.
Sinani AA, Maawali SA, Alshekaili J, Kindi MA, Ramadhani KA, Khabouri JA, Nadeem A, Salti AA. Overlapping demyelinating syndrome (Neuromyelitis optica spectrum disorders NMOSD with anti-NMDA receptor encephalitis); A case report. Mult Scler Relat Disord 2020; 42: 102153.
Tao S, Zhang Y, Ye H, Guo D. AQP4-IgG-seropositive neuromyelitis optica spectrum disorder (NMOSD) coexisting with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis: A case report and literature review. Mult Scler Relat Disord 2019; 35: 185-192.
Villa AM, Manin A, Seimandi C, Guerrero DA, Ramos G. Distinct clinical patterns in AQP4-IgG- positive NMOSD patients vs. Seronegative: Insights from a single-center study in Argentina. Mult Scler Relat Disord 2024; 93: 106223.