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Observational Study
. 2023 Dec 14;95(1):14-18.
doi: 10.1136/jnnp-2023-331137.

Cerebral enhancement in MOG antibody-associated disease

Paul Elsbernd #  1   2 , Laura Cacciaguerra #  3   4   5 , Karl N Krecke  6 , John J Chen  4   5   7 , David Gritsch  2   8 , A Sebastian Lopez-Chiriboga  9 , Elia Sechi  10 , Vyanka Redenbaugh  4   5 , Padraig P Morris  6 , Jonathan L Carter  2 , Dean M Wingerchuk  2 , Jan-Mendelt Tillema  4   11 , Cristina Valencia-Sanchez  2 , Smathorn Thakolwiboon  4 , Sean J Pittock  4   5   12 , Eoin P Flanagan  13   5   12
Affiliations
Observational Study

Cerebral enhancement in MOG antibody-associated disease

Paul Elsbernd et al. J Neurol Neurosurg Psychiatry. .

Abstract

Introduction: Limited data exist on brain MRI enhancement in myelin-oligodendrocyte-glycoprotein (MOG) antibody-associated disease (MOGAD) and differences from aquaporin-4-IgG-positive-neuromyelitis-optica-spectrum-disorder (AQP4+NMOSD), and multiple sclerosis (MS).

Methods: In this retrospective observational study, we identified 122 Mayo Clinic MOGAD patients (1 January 1996-1 July 2020) with cerebral attacks. We explored enhancement patterns using a discovery set (n=41). We assessed enhancement frequency and Expanded Disability Status Scale scores at nadir and follow-up in the remainder (n=81). Two raters assessed T1-weighted-postgadolinium MRIs (1.5T/3T) for enhancement patterns in MOGAD, AQP4+NMOSD (n=14) and MS (n=26). Inter-rater agreement was assessed. Leptomeningeal enhancement clinical correlates were analysed.

Results: Enhancement occurred in 59/81 (73%) MOGAD cerebral attacks but did not influence outcome. Enhancement was often patchy/heterogeneous in MOGAD (33/59 (56%)), AQP4+NMOSD (9/14 (64%); p=0.57) and MS (16/26 (62%); p=0.63). Leptomeningeal enhancement favoured MOGAD (27/59 (46%)) over AQP4+NMOSD (1/14 (7%); p=0.01) and MS (1/26 (4%); p<0.001) with headache, fever and seizures frequent clinical correlates. Ring enhancement favoured MS (8/26 (31%); p=0.006) over MOGAD (4/59 (7%)). Linear ependymal enhancement was unique to AQP4+NMOSD (2/14 (14%)) and persistent enhancement (>3 months) was rare (0%-8%) across all groups. Inter-rater agreement for enhancement patterns was moderate.

Conclusions: Enhancement is common with MOGAD cerebral attacks and often has a non-specific patchy appearance and rarely persists beyond 3 months. Leptomeningeal enhancement favours MOGAD over AQP4+NMOSD and MS.

Keywords: MRI; MULTIPLE SCLEROSIS; NEUROIMMUNOLOGY.

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Conflict of interest statement

Competing interests: PE has no conflicts of interests or financial disclosures to declare regarding this submission. LC received speaker and consultant honoraria from Biomedia, ACCMED, Roche, BMS Celgene and Sanofi. KNK and DG reports no disclosures. JJC has received consulting fees from Roche, UCB and Horizon. ASL-C has served on advisory boards for Genentech and Horizon Therapeutics. ES, VR and PPM reports no disclosures. JLC has received research support from Roche and Genentech for an MS clinical trial, and serves as chair of the DMSC for several migraine clinical trials. DMW has received consulting fees from Alexion, Roche, Genentech, Horizon Therapeutics, Imcyse, Bristol Myers Squibb and Reistone, serves on an attack adjudication committee for a MOGAD clinical trial funded by UCB Pharma and is co-editor in chief of the neurologist. J-MT is associate editor for Journal of Child Neurology. CV-S and ST no disclosures. SJP reports grants, personal fees and non-financial support from Alexion Pharmaceuticals; grants, personal fees, non-financial support and other support from MedImmune, Inc/Viela Bio.; personal fees for consulting from Genentech/Roche. He has a patent, Patent# 8889102 (application#12-678350, Neuromyelitis Optica Autoantibodies as a Marker for Neoplasia)—issued; a patent, Patent# 9891219B2 (application#12-573942, Methods for Treating Neuromyelitis Optica (NMO) by Administration of Eculizumab to an individual that is Aquaporin-4 (AQP4)-IgG Autoantibody positive)—issued. EPF has served on advisory boards for Alexion, Genentech, Horizon Therapeutics and UCB. He has received research support from UCB. He has received speaker honoraria from Pharmacy Times. He received royalties from UpToDate. EPF was a site primary investigator in a randomised clinical trial on Inebilizumab in neuromyelitis optica spectrum disorder run by Medimmune/Viela-Bio/Horizon Therapeutics. EPF has received funding from the NIH (R01NS113828). EPF is a member of the medical advisory board of the MOG project. EPF is an editorial board member of the Journal of the Neurological Sciences and Neuroimmunology Reports. A patent has been submitted on DACH1-IgG as a biomarker of paraneoplastic autoimmunity.

Figures

Figure 1.
Figure 1.. Comparison of enhancement in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), aquaporin-4-IgG positive neuromyelitis optica spectrum disorder (AQP4+NMOSD) and multiple sclerosis (MS).
(A)MOGAD: Axial T1-post-gadolinium images reveal right cerebral leptomeningeal enhancement (A.a, arrow) and cortical enhancement (A.a, arrowhead), left parasagittal cortical and white matter patchy enhancement (A.b, arrow), and pontine multifocal nodular enhancement (A.c, arrows). (B) AQP4+NMOSD. Axial T1-post gadolinium images reveal linear ependymal enhancement (B.a, arrow), bilateral midbrain and left supratentorial white matter patchy enhancement (B.b, arrows) and optic chiasm and right patchy occipital white matter enhancement (B.c, arrow). (C) MS: Axial T1-post gadolinium images reveal a variety of enhancement patterns including left frontal and left periventricular patchy/heterogeneous enhancing lesions, a right anterior frontal linear enhancing lesion and a right temporal punctate enhancing lesion (C.a, arrows), periventricular white matter open-ring enhancement (C.b, arrow) and dorsal punctate focus of enhancement (C.c, arrow).
See this image and copyright information in PMC

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