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Myelin Oligodendrocyte Glycoprotein MOG35-55 Induced Experimental Autoimmune Encephalomyelitis EAE in C57BL/6 Mice
Published on: April 15, 2014
Life-Threatening MOG Antibody-Associated Hemorrhagic ADEM With Elevated CSF IL-6
Akash Virupakshaiah1, Carson E Moseley1, Steven Elicegui1
1From the UCSF Weill Institute for Neurosciences (A.V., C.E.M., C.M.S., M.S., B.A.C., E.W., S.S.Z.), University of California, San Francisco; University of Nevada Reno School of Medicine (S.E.); Renown Health (L.M.G.), Reno, NV; Department of Radiology and Biomedical Imaging (E.G.); and Program in Immunology (S.S.Z.), University of California, San Francisco.
Abstract:
Acute disseminated encephalomyelitis (ADEM) is one characteristic manifestation of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). A previously healthy man presented with retro-orbital headache and urinary retention 14 days after Tdap vaccination. Brain and spine MRI suggested a CNS demyelinating process. Despite treatment with IV steroids, he deteriorated, manifesting hemiparesis and later impaired consciousness, requiring intubation. A repeat brain MRI demonstrated new bilateral supratentorial lesions associated with venous sinus thrombosis, hemorrhage, and midline shift. Anti-MOG antibody was present at a high titer. CSF IL-6 protein was >2,000 times above the upper limits of normal. He improved after plasma exchange, then began monthly treatment alone with anti-IL-6 receptor antibody, tocilizumab, and has remained stable. This case highlights how adult-onset MOGAD, like childhood ADEM, can rapidly become life-threatening. The markedly elevated CSF IL-6 observed here supports consideration for evaluating CSF cytokines more broadly in patients with acute MOGAD.
Insights
Adults can develop severe myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) after vaccination, presenting as acute disseminated encephalomyelitis (ADEM). This case shows elevated IL-6 levels and suggests tocilizumab may be effective.
Area of Science:
- Neurology
- Immunology
Background:
- Acute disseminated encephalomyelitis (ADEM) is a key presentation of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).
- Adult-onset MOGAD can be severe and life-threatening, mimicking childhood ADEM.
Purpose of the Study:
- To report a case of severe adult-onset MOGAD following Tdap vaccination.
- To highlight the role of elevated cerebrospinal fluid (CSF) IL-6 and the potential efficacy of IL-6 blockade.
Main Methods:
- Case report of a previously healthy adult male presenting with neurological symptoms post-vaccination.
- Diagnostic workup included MRI of brain and spine, anti-MOG antibody testing, and CSF analysis for cytokines.
- Treatment involved IV steroids, plasma exchange, and tocilizumab (anti-IL-6 receptor antibody).
Main Results:
- The patient developed severe neurological deficits, including impaired consciousness and hemiparesis, despite initial steroid treatment.
- MRI revealed extensive demyelination, venous sinus thrombosis, hemorrhage, and midline shift.
- High-titer anti-MOG antibodies and markedly elevated CSF IL-6 (>2,000x upper limit) were detected.
- Significant clinical improvement was observed after plasma exchange and subsequent tocilizumab therapy.
Conclusions:
- Adult-onset MOGAD can present aggressively, even after vaccination.
- Elevated CSF IL-6 is a notable finding in severe MOGAD, suggesting a role in pathogenesis.
- Targeting IL-6 with tocilizumab may be a viable therapeutic option for severe MOGAD.
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