Life-Threatening MOG Antibody-Associated Hemorrhagic ADEM With Elevated CSF IL-6

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.

Figure 1. Brain and Spinal Cord Imaging at the Time of Clinical Presentation

Brain MRI at presentation demonstrates small T2-FLAIR hyperintense lesions of the right frontal subcortical white matter and right lateral pons (A, C, arrows) with mild enhancement (B, arrow). Thoracic spine MRI demonstrates T2 hyperintensity at T2-3 (D, arrow) and enhancement involving the ventral cord at T2-3 and T6-7 (E, arrows).

Figure 2. Brain Imaging at the Time of Clinical Deterioration

Brain MRI at the time of clinical deterioration demonstrates new supratentorial T2-FLAIR hyperintense lesions involving the white matter and left globus pallidus (A, arrows) with extensive hemorrhage (B, arrows) and peripheral enhancement (C, arrows). Bilateral thalamic T2-FLAIR hyperintensity (A, arrowheads) with hemorrhage (B, arrowheads) and bilateral transverse sinus thrombosis (D, arrows). Internal cerebral vein thrombosis not shown.

Figure 3. Brain Imaging After Plasma Exchange (PLEX)

Brain MRI after treatment with 5 PLEX sessions demonstrates decrease in edema associated with supratentorial T2-FLAIR hyperintense lesions (A, arrow) with intrinsic T1 hyperintensity (B, arrow) and minimal peripheral enhancement (C, arrow). Improvement in previously seen transverse sinus thrombosis (D, arrows).