title: Refractory acute disseminated encephalomyelitis with anti-galactocerebroside antibody.
abstract: 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.
authors.affiliation: Department of Neurology, Sakai Hospital, Kinki University Faculty of Medicine, Osaka, Japan.
authors.forename: Makoto
authors.initials: M
authors.lastname: Samukawa
authors.forename: Makito
authors.initials: M
authors.lastname: Hirano
authors.forename: Jun
authors.initials: J
authors.lastname: Tsugawa
authors.forename: Hikaru
authors.initials: H
authors.lastname: Sakamoto
authors.forename: Emi
authors.initials: E
authors.lastname: Tabata
authors.forename: Kazuo
authors.initials: K
authors.lastname: Takada
authors.forename: Motoi
authors.initials: M
authors.lastname: Kuwahara
authors.forename: Seiko
authors.initials: S
authors.lastname: Suzuki
authors.forename: Mari
authors.initials: M
authors.lastname: Kitada
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Yamada
authors.forename: Hideo
authors.initials: H
authors.lastname: Hara
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Tsuboi
authors.forename: Yusaku
authors.initials: Y
authors.lastname: Nakamura
authors.forename: Susumu
authors.initials: S
authors.lastname: Kusunoki
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology; D004351:Drug Resistance / Q000276:immunology; D004673:Encephalomyelitis, Acute Disseminated / Q000097:blood / Q000188:drug therapy / Q000276:immunology*; D005260:Female; D005699:Galactosylceramides / Q000276:immunology*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011379:Prognosis
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D001324:Autoantigens; D005699:Galactosylceramides; D016756:Immunoglobulins, Intravenous; C002454:galactocerebroside