title: [High-dose intravenous immunoglobulin therapy in a child case of Bickerstaff's brainstem encephalitis].
abstract: We report an 11-year-old boy with Bickerstaff's brainstem encephalitis (BBE). He had gait disturbance, disturbed consciousness, and diplopia after upper respiratory tract infection. On admission, he showed multiple cranial nerve palsy, muscle weakness of arms, cerebeller ataxia and generalized areflexia. The cerebrospinal fluid on day 7 revealed albuminocytologic dissociation. IgG antibodies against GQ1b and GT1a were detected in the serum. Immunoglobulin was administered intravenously from day 11, and then his symptoms gradually diminished. When he was discharged on day 27, he had neither conscious disturbance nor limb weakness. There still were mild ophthalmoparesis and diminished deep tendon reflexes, but they disappeared by 10 months after the onset. Effective therapy for BBE has yet to be established. Our case had features of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome, such as an acute monophasic course, limb weakness with areflexia, albuminocytologic dissociation in the cerebrospinal fluid, detection of serum anti-ganglioside antibodies and efficacy of intravenous immunoglobulin, indicating that BBE and GBS are closely related. Our case suggested that intravenous immunoglobulin therapy, an established treatment for GBS, should be considered in some patients with BBE.
authors.affiliation: Division of Neurology, Saitama Children's Medical Center, Iwatsuki, Saitama. nobuyosi@is.icc.u-tokai.ac.jp
authors.forename: Nobuyoshi
authors.initials: N
authors.lastname: Sugiyama
authors.forename: Shin-ichiro
authors.initials: S
authors.lastname: Hamano
authors.forename: Michiaki
authors.initials: M
authors.lastname: Koga
mesh_terms: D001323:Autoantibodies / Q000097:blood; D001933:Brain Stem / Q000473:pathology; D002648:Child; D004660:Encephalitis / Q000276:immunology / Q000503:physiopathology / Q000628:therapy*; D005732:Gangliosides / Q000276:immunology; D020275:Guillain-Barre Syndrome / Q000503:physiopathology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D008297:Male; D019846:Miller Fisher Syndrome / Q000503:physiopathology
chemical_list: D001323:Autoantibodies; D005732:Gangliosides; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous; C016481:trisialoganglioside GT1; C039129:GQ1b ganglioside