Clinical course, imaging, and pathological features of 45 adult and pediatric cases of myelin oligodendrocyte glycoprotein antibody-associated disease.

Abstract

BACKGROUND:

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently described neuroinflammatory demyelinating disease.

OBJECTIVE:

To better understand the clinical spectrum, risk factors and outcomes in MOGAD.

METHODS:

Retrospective cohort study including all subjects harboring anti-MOG antibodies identified in major academic hospitals across the province of Quebec.

RESULTS:

We identified 45 MOGAD cases. The minimal estimated point-prevalence was 0.52/100 000 in Quebec. Median age at presentation was 32 years (range 1-71) with equal sex ratio. Most frequent ethnic groups were Caucasians and Asians. The most frequent clinical manifestations at onset were optic neuritis (ON), affecting 56% of adults, and acute disseminated encephalomyelitis (ADEM), affecting 33% of children. First MRI was abnormal in 84% of cases. Most CSF samples showed pleocytosis without oligoclonal bands. Two brain biopsies revealed lipid-laden macrophages and reactive astrocytes. Despite steroids, only 38% had fully recovered at 4 weeks after onset. Half of pediatric and two thirds of adult-onset MOGAD subjects experienced relapses. At last follow-up, 69% showed residual deficits, which were moderate to severe in 17% of adults.

CONCLUSION:

MOGAD has heterogeneous disease course, and it is not a benign disease for a substantial proportion of adults. Best disease-modifying therapies remain to be determined.

• Authors: Hayet Boudjani, Giulia Fadda, Gabrielle Dufort, Jack Antel, Paul Giacomini, Myriam Levesque-Roy, Maryam Oskoui, Pierre Duquette, Alexandre Prat, Marc Girard, Rose-Marie Rebillard, Inge Meijer, Elana Pinchefsky, Cam-Tu Emilie Nguyen, Elsa Rossignol, Jacinthe Rouleau, Oliver Blanchard, Nicole Khairallah, Philippe Beauchemin, Anne-Marie Trudelle, Emmanuelle Lapointe, Alexander Saveriano, Catherine Larochelle
• Journal: Multiple sclerosis and related disorders (Mult Scler Relat Disord) Vol. 76 Pg. 104787 (Aug 2023) ISSN: 2211-0356 [Electronic] Netherlands
• PMID: 37320939 (Publication Type: Journal Article)
• Copyright: Crown Copyright © 2023. Published by Elsevier B.V. All rights reserved.

Chemical References

• Myelin-Oligodendrocyte Glycoprotein
• Autoantibodies

Topics

• Humans
• Myelin-Oligodendrocyte Glycoprotein
• Retrospective Studies
• Encephalomyelitis, Acute Disseminated (diagnostic imaging)
• Optic Neuritis
• Disease Progression
• Autoantibodies