Coexistent MOG, NMDAR, CASPR2 antibody positivity: Triumph over the triumvirate
Ajith Cherian1, K P Divya2, Sarath Chandra Shetty3
1Associate Professor, Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum , Kerala, PIN-695011, India.
Abstract:
Myelin oligodendrocyte glycoprotein (MOG) antibody (MOG -Ab) positive cases can have a focal encephalitis like presentation. Many reports of anti N-methyl-D-aspartate receptor (NMDAR) encephalitis have documented simultaneous coexistence of MOG-Ab which is explained by the coexpression of NMDA receptors and MOG on oligodendrocytic processes. Contactin-associated protein-like 2 (CASPR2) is expressed in central and peripheral nervous system (PNS) axons and there are no case reports of coexistent NMDAR, CASPR2 and MOG antibodies. We describe a young adult male who had neuropsychiatric, cognitive symptoms and long tract signs with a strong positivity for all three antibodies. Bilateral cingulate gyri involvement, especially with contrast enhancement may be an imaging clue suggesting coexistent MOG and NMDAR antibodies. Bilateral T2 hyperintensities affecting hippocampi, have been described in both CASPR2 and anti NMDAR encephalitis. Our patient had both, bilateral cingulate and hippocampal lesions, which may be an imaging clue favouring the presence of all three antibodies.
Insights
This study reports the first case of coexisting Myelin oligodendrocyte glycoprotein (MOG) antibody, N-methyl-D-aspartate receptor (NMDAR) antibody, and Contactin-associated protein-like 2 (CASPR2) antibody encephalitis. Imaging findings may suggest this rare triple-antibody autoimmune encephalitis.
Area of Science:
- Neuroimmunology
- Neurology
- Autoimmune Diseases
Background:
- Myelin oligodendrocyte glycoprotein (MOG) antibody disease can present as focal encephalitis.
- Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis frequently co-occurs with MOG antibodies due to receptor coexpression.
- Contactin-associated protein-like 2 (CASPR2) antibodies are associated with central and peripheral nervous system disorders.
Discussion:
- This report details a unique case of a young adult with neuropsychiatric, cognitive, and long tract symptoms.
- The patient exhibited strong positivity for MOG, NMDAR, and CASPR2 antibodies simultaneously.
- This presentation represents the first documented instance of coexistent antibodies against MOG, NMDAR, and CASPR2.
Key Insights:
- Bilateral cingulate gyrus involvement with contrast enhancement may indicate coexistent MOG and NMDAR antibodies.
- Bilateral hippocampal T2 hyperintensities are observed in both CASPR2 and anti-NMDAR encephalitis.
- The patient's combination of cingulate and hippocampal lesions suggests a potential imaging signature for triple-antibody positivity.
Outlook:
- Further research is needed to understand the clinical implications and diagnostic criteria for triple-antibody autoimmune encephalitis.
- Investigating the pathogenic mechanisms underlying coexistent MOG, NMDAR, and CASPR2 antibody encephalitis is crucial.
- Developing targeted therapeutic strategies for this rare autoimmune neurological condition is a future direction.
