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Myelin Oligodendrocyte Glycoprotein MOG35-55 Induced Experimental Autoimmune Encephalomyelitis EAE in C57BL/6 Mice
Published on: April 15, 2014
Application of the ADEM definition to cerebral attacks of MOG antibody-associated disease
Sara J Hooshmand1, Nisa Vorasoot1,2,3, Xiaoyang Li1
1Departments of Neurology and Center for MS and Autoimmune Neurology, Mayo Clinic College of Medicine and Science, Rochester, MN, USA.
Background:
Acute disseminated encephalomyelitis (ADEM) is a demyelinating syndrome observed in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) but there are limited data on application of this definition to its cerebral attacks or in adults with this disease.
Methods:
We analyzed patients from the Mayo Clinic MOGAD cohort (n = 263) with cerebral attacks. We assessed whether children (<18 years-old) and adults (⩾18 years-old) fulfilled the 2013 ADEM definition.
Results:
We identified 89 patients (children, 34; adults, 55) and 55/89 (62%) were of female sex. Acute magnetic resonance imaging (MRI) abnormalities included: poorly demarcated T2 lesions (white matter, 85 (96%); deep gray matter, 41 (46%); T1-hypointense lesions (42 (47%)). Forty-two patients did not fulfill the ADEM definition due to: monofocal syndrome, 9; or no encephalopathy, 33. The remaining 47 patients (53%: children, 27/34 (79%); adults, 20/55 (36%)) fulfilled the ADEM definition, and 14 had preceding attacks, and 34 had subsequent attacks relative to ADEM episode. The features in children and adults with ADEM were broadly comparable.
Conclusion:
Applying the ADEM definition to MOGAD cerebral attacks highlighted that an update of the 2013 ADEM definition would be beneficial and could explicitly state its application to both adults and children and allow it to be applied to attacks at any time during the disease course.
Insights
The 2013 Acute Disseminated Encephalomyelitis (ADEM) definition needs updating. It should explicitly include adults and children with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) cerebral attacks, regardless of disease stage.
Area of Science:
- Neuroimmunology
- Demyelinating Diseases
Background:
- Acute Disseminated Encephalomyelitis (ADEM) is a demyelinating syndrome associated with Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD).
- Limited data exist on applying the ADEM definition to MOGAD cerebral attacks, especially in adults.
Purpose of the Study:
- To assess the applicability of the 2013 ADEM definition to cerebral attacks in a MOGAD cohort.
- To evaluate differences and similarities in ADEM features between pediatric and adult MOGAD patients.
Main Methods:
- Analysis of 263 patients from the Mayo Clinic MOGAD cohort with cerebral attacks.
- Assessment of pediatric (<18 years) and adult (≥18 years) patients against the 2013 ADEM criteria.
Main Results:
- 89 patients (34 children, 55 adults) with cerebral attacks were identified.
- 62% of patients were female. Common MRI findings included T2 lesions in white matter (96%) and deep gray matter (46%).
- 53% of patients met the ADEM criteria; 47% did not, primarily due to monofocal presentation or lack of encephalopathy. Features were comparable between children and adults.
Conclusions:
- The current 2013 ADEM definition requires updating for MOGAD cerebral attacks.
- The updated definition should explicitly include adults and children.
- It should also allow application to MOGAD attacks occurring at any point in the disease course.
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