Overlapping demyelinating syndrome and anti-N-methyl-d-aspartate receptor encephalitis with seizures

Abstract

Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis, the most recognized type of autoimmune encephalitis, manifests with rapid cognitive decline, psychosis, and seizures that develop in 78–86% of patients. Recently, anti-NMDAR encephalitis was reported in association with demyelinating diseases which are accompanied by a characteristic clinical phenotype, imaging abnormalities, and the presence of antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) in bodily fluids. The patient presented herein suffered from bilateral optic neuritis followed by recurrent encephalitis with focal seizures and demonstrated anti-NMDAR and MOG-IgGs in the cerebrospinal fluid and serum, respectively. Her symptoms responded to immunotherapy and antiseizure medication. The recognition of the novel syndrome of MOG antibody-associated demyelination (MOGAD), encompassing the overlapping anti-NMDAR encephalitis and other MOG-IgG associated disorders, is important for the successful management of these patients.

MOG-IgG1 and co-existence of neuronal autoantibodies

“…Seizures and cortical/leptomeningeal encephalitis has been described in MOGAD patients who are NMDA-R-IgG negative, 1,22 anti-NMDA-R-IgG encephalitis, 23 and GFAP-IgG meningoencephalitis, 24 thus these clinical and radiological findings are not specific for dual-positivity. Nonetheless, the frequency of these features in our dual-positive MOG-IgG1+/ NMDA-R-IgG+ cohort, similar to other reports, 18,25 suggests that clinicians should consider serological screening for MOG-IgG1 and NMDA-R-IgG in patients presenting with encephalopathy, seizures, and/or cortical/leptomeningeal encephalitis.…”

Clinical analysis of a patient simultaneously positive for antibodies of myelin oligodendrocyte glycoprotein and anti–N-methyl-D-aspartate receptor

“…Most reports of patients with co-occurrence of MOG-Ab and anti-NMDAR-Ab have shown that anti-NMDARe may develop initially, followed by MOG-AD [ 10 ] ; however, patients with the opposite occurrence [ 11 ] or repetitive onset of anti-NMDARe attacks with no MOG-AD attacks (positive MOG-Ab just being a bystander) have also been reported. When a patient is simultaneously positive for MOG-Ab and anti-NMDARe-Ab, the clinical presentation can be either sequential anti-NMDARe and MOG-AD attacks (or vice versa), [ 10 ] single attack, [ 12 – 14 ] or a mixed attack.…”

The Expanding Clinical Spectrum of Myelin Oligodendrocyte Glycoprotein (MOG) Antibody Associated Disease in Children and Adults

“…All patients with FLAMES responded to high dose steroids with resolution of FLAIR changes. Of note, a number of patients developed ON either before or after seizures (56,58,59). Thus, the emergence of seizures in the context of ON or focal brain inflammatory lesions should prompt testing for MOG-Ab (52).…”