MOG encephalomyelitis after vaccination against severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2): case report and comprehensive review of the literature

Abstract

Background In around 20% of cases, myelin oligodendrocyte glycoprotein (MOG) immunoglobulin (IgG)-associated encephalomyelitis (MOG-EM; also termed MOG antibody-associated disease, MOGAD) first occurs in a postinfectious or postvaccinal setting. Objective To report a case of MOG-EM with onset after vaccination with the Pfizer BioNTech COVID-19 mRNA vaccine BNT162b2 (Comirnaty®) and to provide a comprehensive review of the epidemiological, clinical, radiological, electrophysiological and laboratory features as well as treatment outcomes of all published patients with SARS-CoV-2 vaccination-associated new-onset MOG-EM. Methods Case report and review of the literature. Results In our patient, MOG-IgG-positive (serum 1:1000, mainly IgG1 and IgG2; CSF 1:2; MOG-specific antibody index < 4) unilateral optic neuritis (ON) occurred 10 days after booster vaccination with BNT162b2, which had been preceded by two immunizations with the vector-based Oxford AstraZeneca vaccine ChAdOx1-S/ChAdOx1-nCoV-19 (AZD1222). High-dose steroid treatment with oral tapering resulted in complete recovery. Overall, 20 cases of SARS-CoV2 vaccination-associated MOG-EM were analysed (median age at onset 43.5 years, range 28–68; female to male ratio = 1:1.2). All cases occurred in adults and almost all after immunization with ChAdOx1-S/ChAdOx1 nCoV-19 (median interval 13 days, range 7–32), mostly after the first dose. In 70% of patients, more than one CNS region (spinal cord, brainstem, supratentorial brain, optic nerve) was affected at onset, in contrast to a much lower rate in conventional MOG-EM in adults, in which isolated ON is predominant at onset and ADEM-like phenotypes are rare. The cerebrospinal fluid white cell count (WCC) exceeded 100 cells/μl in 5/14 (36%) patients with available data (median peak WCC 58 cells/μl in those with pleocytosis; range 6–720). Severe disease with tetraparesis, paraplegia, functional blindness, brainstem involvement and/or bladder/bowel dysfunction and a high lesion load was common, and treatment escalation with plasma exchange (N = 9) and/or prolonged IVMP therapy was required in 50% of cases. Complete or partial recovery was achieved in the majority of patients, but residual symptoms were significant in some. MOG-IgG remained detectable in 7/7 cases after 3 or 6 months. Conclusions MOG-EM with postvaccinal onset was mostly observed after vaccination with ChAdOx1-S/ChAdOx1 nCoV-19. Attack severity was often high at onset. Escalation of immunotherapy was frequently required. MOG-IgG persisted in the long term.

Clinical onset of CNS demyelinating disease after COVID-19 vaccination: denovo disease?

“…There was no pleocytosis and OCT showed no evidence of retinal neuroaxonal degeneration. Onset of MOGAD occurred in the context with ChAdOx1-S/nCoV-19 vaccination in most of the published cases ( Jarius et al, 2022 ) as well as in our MOGAD postvacc patient. The clinical presentation of our patient was also comparable to the already known MOGAD postvacc clinical and paraclinical features ( Mumoli et al, 2022 , Dams, Kraemer, and Becker, 2022 , Simone, Monti, and Amidei, 2021 , Francis, Palace, and Fugger, 2022 , Jarius et al, 2022 ).…”

“…Onset of MOGAD occurred in the context with ChAdOx1-S/nCoV-19 vaccination in most of the published cases ( Jarius et al, 2022 ) as well as in our MOGAD postvacc patient. The clinical presentation of our patient was also comparable to the already known MOGAD postvacc clinical and paraclinical features ( Mumoli et al, 2022 , Dams, Kraemer, and Becker, 2022 , Simone, Monti, and Amidei, 2021 , Francis, Palace, and Fugger, 2022 , Jarius et al, 2022 ). The clinical presentation in our MOGAD postvacc patient showed involvement of the cerebellar peduncle, a good clinical recovery after acute therapy, a stable MOG-IgG detection in the course and despite a resolution of inflammatory lesions in the follow-up MRI.…”

“…This is to answer the question of whether the onset characteristics of MS postvacc differ from MOGAD postvacc . Due to the rarity of this disease, we could describe the clinical characteristics at the time of clinical onset of MOGAD in only one patient (MOGAD postvacc ) and compared it with the MS postvacc cohort on the one hand, but also with other published MOGAD postvacc patients on the other hand ( Mumoli et al, 2022 , Dams, Kraemer, and Becker, 2022 , Simone, Monti, and Amidei, 2021 , Francis, Palace, and Fugger, 2022 , Jarius et al, 2022 ). Similar to other reported MOGAD postvacc patients our MOGAD postvacc patient showed at the time of the first clinical onset no evidence of preexisting chronic inflammatory CNS disease and no dissemination in time.…”

“…Different vaccines are currently available to prevent SARS-CoV-2 infection and serious disease progression, their safety and effectiveness have been repeatedly reported ( Pormohammad, Zarei, and Ghorbani, 2021 ). Central nervous system (CNS) demyelinating disorders (DD) after SARS-CoV-2 infection as well as following COVID-19 vaccination have been described ( Ismail and Salama, 2022 ), including onset of multiple sclerosis (MS) ( Havla et al, 2022 ) or cases of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) ( Mumoli et al, 2022 , Dams, Kraemer, and Becker, 2022 , Simone, Monti, and Amidei, 2021 , Francis, Palace, and Fugger, 2022 , Jarius et al, 2022 ). So far, no increased risk for relapse activity could be observed in MS patients following the first and second dose of COVID-19 vaccination ( Epstein, Xia, and Lee, 2022 ).…”

Neuro-ophthalmological Presentation of Optic Neuritis in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

“…In up to 20% of MOGAD patients, associations were found between a possible trigger and a first MOGAD event [3,8,[42][43][44][45]. Temporal associations have been reported with N-methyl-d-aspartate receptor encephalitis, infections, including herpes simplex virus, Borrelia, Epstein-Barr as well as, more recently, severe acute respiratory syndrome coronavirus type 2 (SARS-sCoV-2) [8,[42][43][44][45][46][47] and, albeit less frequently, with vaccinations (mostly with SARS-CoV-2 vaccination but also with diphtheria, tetanus, pertussis, polio, and influenza vaccination) [3,8,[42][43][44][45]. Recent research has shown that post-vaccination ON in the presence of MOG-IgG is particularly severe, with around 50% of affected patients experiencing severe and debilitating vision loss [45].…”