Refractory acute disseminated encephalomyelitis with anti-galactocerebroside antibody

Acute disseminated encephalomyelitis causes multifocal demyelination in the central nerve system. Although this disease generally responds well to steroid therapy, it is occasionally steroid-resistant, leading to poor outcomes. Serological markers of prognosis are currently unavailable. We measured anti-glycolipid antibodies in 25 consecutive patients with acute disseminated encephalomyelitis, and found that four patients were positive for anti-galactocerebroside antibodies. All four patients had a poor response to steroids. We summarize clinical information on these four patients and three similar patients reported previously. This is the first report to describe concomitant involvement of the central nerve system and peripheral nervous system in anti-galactocerebroside antibody-associated acute disseminated encephalomyelitis, consistent with the location of galactocerebroside, and to document a dramatic response to repeated intravenous immunoglobulin therapy after unsuccessful steroid treatment in one patient.

► Anti-glycolipid antibodies were measured in 25 consecutive patients with ADEM.
► Anti-galactcerebroside (Gal-C) antibodies were detected in four patients.
► Our four patients and three similar patients reported by others had steroid-resistance.
► One of our patients responded dramatically to repeated IVIg therapy.
► Anti-Gal-C antibodies can predict steroid-resistance or poor outcomes.