title: Bickerstaff's brainstem encephalitis associated with IgM antibodies to GM1b and GalNAc-GD1a.
abstract: This is the first report of a case of Bickerstaff's brainstem encephalitis (BBE) associated with IgM antibodies to GM1b and GalNAc-GD1a. Subsequent to Campylobacter jejuni enteritis, the patient rapidly developed consciousness disturbance and hyperreflexia in addition to external ophthalmoplegia and cerebellar-like ataxia. EEG showed transient 7 Hz monorhythmic theta activities, predominantly in the front-central area. He received high doses of immunoglobulin intravenously and had completely recovered 3 months later. High anti-GM1b and anti-GalNAc-GD1a IgM antibody titers present during the acute phase decreased with his clinical improvement. An absorption study showed the anti-GM1b and anti-GalNAc-GD1a IgM antibodies to be cross-reactive. Anti-GM1b and anti-GalNAc-GD1a antibodies have been detected in some patients who developed Guillain-Barré syndrome after C. jejuni enteritis, whereas the anti-GQ1b IgG antibody is associated with BBE. Infection by C. jejuni bearing a GM1b-like or GalNAc-GD1a-like lipooligosaccharide may trigger the production of anti-GalNAc-GD1a and anti-GM1b IgM antibodies. It is not clear why our patient developed BBE rather than Guillain-Barré syndrome. These antibodies may, however, prove useful serological markers for identifying BBE patients who do not have the anti-GQ1b IgG antibody.
authors.affiliation: Department of Pediatrics, Saga Medical School, Saga, Japan.
authors.forename: Muneaki
authors.initials: M
authors.lastname: Matsuo
authors.forename: Masaaki
authors.initials: M
authors.lastname: Odaka
authors.forename: Michiaki
authors.initials: M
authors.lastname: Koga
authors.forename: Katsunori
authors.initials: K
authors.lastname: Tsuchiya
authors.forename: Yuhei
authors.initials: Y
authors.lastname: Hamasaki
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Yuki
mesh_terms: D001259:Ataxia / Q000276:immunology / Q000503:physiopathology; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D001933:Brain Stem / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D002169:Campylobacter Infections / Q000150:complications / Q000276:immunology; D016123:Campylobacter jejuni / Q000276:immunology; D002648:Child; D003244:Consciousness Disorders / Q000276:immunology / Q000503:physiopathology; D004569:Electroencephalography; D004660:Encephalitis / Q000097:blood / Q000276:immunology* / Q000503:physiopathology; D005677:G(M1) Ganglioside / Q000031:analogs & derivatives* / Q000276:immunology*; D005732:Gangliosides / Q000276:immunology*; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood / Q000276:immunology*; D008297:Male; D020127:Recovery of Function; D012021:Reflex, Abnormal / Q000276:immunology
chemical_list: D001323:Autoantibodies; D005732:Gangliosides; D007075:Immunoglobulin M; D005677:G(M1) Ganglioside; C029624:ganglioside M1b; C055253:IV(4)-galactosyl-N-acetylganglioside GD1a