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Cerebral enhancement in MOG antibody-associated disease
  1. Paul Elsbernd1,2,
  2. Laura Cacciaguerra3,4,5,
  3. Karl N Krecke6,
  4. John J Chen4,5,7,
  5. David Gritsch2,8,
  6. A Sebastian Lopez-Chiriboga9,
  7. Elia Sechi10,
  8. Vyanka Redenbaugh4,5,
  9. Padraig P Morris6,
  10. Jonathan L Carter2,
  11. Dean M Wingerchuk2,
  12. Jan-Mendelt Tillema4,11,
  13. Cristina Valencia-Sanchez2,
  14. Smathorn Thakolwiboon4,
  15. Sean J Pittock4,5,12,
  16. Eoin P Flanagan4,5,12
  1. 1 Department of Neurology, Brooke Army Medical Center, Fort Sam Houston, Texas, USA
  2. 2 Department of Neurology, Mayo Clinic, Scottsdale, Arizona, USA
  3. 3 Department of Neurology, Vita-Salute San Raffaele University, Milano, Italy
  4. 4 Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
  5. 5 Center for Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, Rochester, Minnesota, USA
  6. 6 Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA
  7. 7 Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA
  8. 8 Department of Neurology, Mass General Brigham Inc, Boston, Massachusetts, USA
  9. 9 Department of Neurology, Mayo Clinic, Jacksonville, Forida, USA
  10. 10 Department of Medical, Surgical and Experimental Sciences, University of Sassari, Sassari, Italy
  11. 11 Department of pediatrics, Mayo Clinic, Rochester, Minnesota, USA
  12. 12 Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr Eoin P Flanagan, Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA; flanagan.eoin{at}mayo.edu

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.

  • MRI
  • NEUROIMMUNOLOGY
  • MULTIPLE SCLEROSIS

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Footnotes

  • Twitter @Lauz_Caccia, @chenmayo, @smathorn, @EoinFlanagan14

  • PE and LC contributed equally.

  • Presented at This paper was prepared partially by physicians while in the employment of the US federal government

  • Contributors PE: study concept, data analysis, interpretation of data, drafting/revising the manuscript. LC: data analysis (including the radiological evaluation of images), statistical analysis, interpretation of data, drafting/revising the manuscript. KNK: data analysis (including the radiological evaluation of images), interpretation of data, drafting/revising the manuscript. DG: drafting/revising the manuscript. JJC: patient recruitment, clinical assessment, drafting/revising the manuscript. ASL-C: patient recruitment, clinical assessment, drafting/revising the manuscript. ES: drafting/revising the manuscript. VR: data analysis, drafting/revising the manuscript. PPM: data analysis, drafting/revising the manuscript. JLC: drafting/revising the manuscript. DMW: drafting/revising the manuscript. J-MT: patient recruitment, clinical assessment, drafting/revising the manuscript. CV-S: drafting/revising the manuscript. ST: drafting/revising the manuscript. SJP: patient recruitment, clinical assessment, interpretation of data, drafting/revising the manuscript. EPF: study concept, patient recruitment, clinical assessment, data analysis (including the radiological evaluation of images), interpretation of data, drafting/revising the manuscript. All authors have approved the final version of the manuscript.

  • Funding This study was funded by an RO1 from the National Institute of Neurological Disorders and Stroke (R01NS113828).

  • Disclaimer The views expressed herein are those of the authors and do not reflect the official policy or position of Brooke Army Medical Center, the U.S. Army Medical Department, the US Army Office of the Surgeon General, the Department of the Army, the Department of the Air Force, the Department of Defense or the US government.

  • 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.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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