There is often confusion among clinicians since both the terms NMOSD and MOGAD have been used in the literature to refer to the CNS demyelinating disorder associated with MOG-IgG. History and Definitions: The Concept of NMOSD and MOGAD Although not representing the focus of this work, AQP4-IgG+NMOSD and MS will be discussed as comparison groups to highlight differences and similarities with MOGAD. In this review article we will summarize our current understanding of MOGAD, the most recently characterized demyelinating CNS disorder, and provide guidance for diagnosis and management. Awareness of the specific features that define each demyelinating disorder is crucial for a correct diagnosis and timely initiation of an appropriate treatment ( 4). Consensual refinements in the clinical-MRI characterization of these three disease entities has increased diagnostic precision, and allowed identification of important differences in pathophysiology, treatment response, and outcomes. In summary, this article will review our rapidly evolving understanding of MOGAD diagnosis and management.ĭuring the last 15 years, the global concept of inflammatory demyelinating disorders of the central nervous system (CNS) has radically changed with the identification of specific autoantibody-associated conditions distinct from multiple sclerosis (MS), namely aquaporin-4 (AQP4)-IgG-positive neuromyelitis optica spectrum disorder (AQP4-IgG+NMOSD) and myelin oligodendrocyte glycoprotein (MOG)-IgG-associated disease (MOGAD) ( 1– 4). A variety of empiric steroid-sparing immunosuppressants can be considered and may be efficacious based on retrospective or prospective observational studies but prospective randomized placebo-controlled trials are needed to better guide treatment. Attack-prevention treatments also lack class-I data and empiric maintenance treatment is generally reserved for relapsing cases or patients with severe residual disability after the presenting attack. Although randomized controlled trials are lacking, MOGAD acute attacks appear to be very responsive to high dose steroids and plasma exchange may be considered in refractory cases. International consensus diagnostic criteria for MOGAD are currently being compiled and will assist in clinical diagnosis and be useful for enrolment in clinical trials. high) can influence the likelihood of MOGAD diagnosis. The type of cell-based assay used to evaluate for MOG-IgG (fixed vs. The detection of MOG-IgG in the serum (and sometimes CSF) confirms the diagnosis in patients with compatible clinical-MRI phenotypes, but false positive results are occasionally encountered, especially with indiscriminate testing of large unselected populations. Characteristic MRI features have been described that increase the diagnostic suspicion (e.g., perineural optic nerve enhancement, spinal cord H-sign, T2-lesion resolution over time) and help discriminate from MS and AQP4+NMOSD, despite some overlap. A relapsing course is observed in approximately 50% of patients. The clinical manifestations of MOGAD are heterogeneous, ranging from isolated optic neuritis or myelitis to multifocal CNS demyelination often in the form of acute disseminated encephalomyelitis (ADEM), or cortical encephalitis. Over the last decade, several studies have helped delineate the characteristic clinical-MRI phenotypes of the disease, allowing distinction from aquaporin-4 (AQP4)-IgG-positive neuromyelitis optica spectrum disorder (AQP4-IgG+NMOSD) and multiple sclerosis (MS).
Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is the most recently defined inflammatory demyelinating disease of the central nervous system (CNS).
6Department of Neurology and Neurosurgery, McGill University, Montreal, QC, Canada.5Neurology Unit, Department of Neurosciences, Biomedicine, and Movement Sciences, University of Verona, Verona, Italy.4Department of Ophthalmology, Mayo Clinic, Rochester, MN, United States.3Department of Neurology and Center for Multiple Sclerosis and Autoimmune Neurology Mayo Clinic, Rochester, MN, United States.2Neuroimaging Research Unit, Division of Neuroscience, IRCCS San Raffaele Scientific Institute and Vita-Salute San Raffaele University, Milan, Italy.1Neurology Unit, Department of Medical, Surgical and Experimental Sciences, University of Sassari, Sassari, Italy.Chen 3,4 Sara Mariotto 5 Giulia Fadda 6 Alessandro Dinoto 5 A. Elia Sechi 1 Laura Cacciaguerra 2,3 John J.
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