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MOG encephalomyelitis: distinct clinical, MRI and CSF features in patients with longitudinal extensive transverse
Julia Loos1, Steffen Pfeuffer2, Katrin Pape1
1Department of Neurology, Focus Program Translational Neuroscience (FTN), Rhine Main Neuroscience Network (rmn2), University Medical Center of the Johannes Gutenberg-University of Mainz, Mainz, Germany.
Background:
Based on clinical, immunological and histopathological evidence, MOG-IgG-associated encephalomyelitis (MOG-EM) has emerged as a distinct disease entity different from multiple sclerosis (MS) and aquaporin-4-antibody-positive neuromyelitis optica spectrum disorder (NMOSD). MOG-EM is associated with a broader clinical phenotype including optic neuritis, myelitis, brainstem lesions and acute disseminated encephalomyelitis with a substantial clinical and radiological overlap to other demyelinating CNS disorders.
Objective:
To evaluate common clinical, MRI and CSF findings, as well as therapy responses in patients with longitudinal extensive transverse myelitis (LETM) as initial clinical presentation of MOG-EM.
Methods:
After excluding patients with a known diagnosis of MS, we identified 153 patients with myelitis of which 7 fulfilled the inclusion criteria and were investigated for MRI, CSF and clinical parameters.
Results:
Patients with LETM as first clinical presentation of MOG-EM display similar characteristics, namely a lack of gadolinium-enhancement in spinal cord MRI, marked pleocytosis, negative oligoclonal bands, a previous history of infections/vaccinations and response to antibody-depleting treatments for acute attacks and long-term treatment.
Conclusions:
We identify common pathological findings in patients with LETM as first clinical presentation of MOG-EM which distinguishes it from other forms of LETM and should lead to testing for MOG-IgG in these cases.
Insights
Myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis (MOG-EM) presenting as longitudinal extensive transverse myelitis (LETM) shows distinct MRI and CSF findings. Early MOG-IgG testing is recommended for accurate diagnosis and treatment.
Area of Science:
- Neurology
- Immunology
- Neuroinflammation
Background:
- Myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis (MOG-EM) is a distinct CNS demyelinating disorder.
- MOG-EM shares clinical and radiological features with multiple sclerosis (MS) and NMOSD.
- Understanding MOG-EM's unique presentation is crucial for differential diagnosis.
Purpose of the Study:
- To characterize clinical, MRI, and CSF findings in MOG-EM presenting as LETM.
- To assess treatment responses in MOG-EM patients with LETM.
- To differentiate MOG-EM from other myelitis etiologies.
Main Methods:
- Retrospective analysis of 153 myelitis patients.
- Inclusion of 7 patients with LETM as initial MOG-EM presentation.
- Evaluation of MRI, CSF, and clinical data.
Main Results:
- LETM in MOG-EM often lacks spinal cord gadolinium enhancement.
- Marked CSF pleocytosis and negative oligoclonal bands are common.
- Patients responded well to antibody-depleting therapies.
Conclusions:
- LETM as initial MOG-EM presentation has identifiable pathological features.
- These findings help distinguish MOG-EM from other LETM forms.
- MOG-IgG testing is advised for suspected cases.
