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.

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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