title: Poststreptococcal acute disseminated encephalomyelitis with basal ganglia involvement and auto-reactive antibasal ganglia antibodies.
abstract: Antibasal ganglia antibodies (ABGA) are associated with Sydenham's chorea and pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections. We present 10 patients with acute disseminated encephalomyelitis (ADEM) associated with Group A beta hemolytic streptococcal infection. The clinical phenotype was novel, with 50% having a dystonic extrapyramidal movement disorder, and 70% a behavioral syndrome. None of the patients had rheumatic fever or Sydenham's chorea. Enzyme-linked immunosorbent assay, Western immunoblotting, and immunohistochemistry were used to detect ABGA. Neurological (n = 40) and streptococcal (n = 40) controls were used for comparison. Enzyme-linked immunosorbent assay results showed significantly elevated ABGA in the patients with poststreptococcal ADEM. Western immunoblotting demonstrated ABGA reactivity to three dominant protein bands of 60, 67, or 80 kDa; a finding not reproduced in controls. Fluorescent immunohistochemistry demonstrated specific binding to large striatal neurones, which was not seen in controls. Streptococcal serology was also significantly elevated in the poststreptococcal ADEM group compared with neurological controls. Magnetic resonance imaging studies showed hyperintense basal ganglia in 80% of patients with poststreptococcal ADEM, compared to 18% of patients with nonstreptococcal ADEM. These findings support a new subgroup of postinfectious autoimmune inflammatory disorders associated with Group A beta hemolytic streptococcus, abnormal basal ganglia imaging, and elevated ABGA.
authors.affiliation: Department of Neurology, Great Ormond Street Hospital National Health Service Trust and Institute of Child Health, University of London, UK
authors.forename: R C
authors.initials: RC
authors.lastname: Dale
authors.forename: A J
authors.initials: AJ
authors.lastname: Church
authors.forename: F
authors.initials: F
authors.lastname: Cardoso
authors.forename: E
authors.initials: E
authors.lastname: Goddard
authors.forename: T C
authors.initials: TC
authors.lastname: Cox
authors.forename: W K
authors.initials: WK
authors.lastname: Chong
authors.forename: A
authors.initials: A
authors.lastname: Williams
authors.forename: N J
authors.initials: NJ
authors.lastname: Klein
authors.forename: B G
authors.initials: BG
authors.lastname: Neville
authors.forename: E J
authors.initials: EJ
authors.lastname: Thompson
authors.forename: G
authors.initials: G
authors.lastname: Giovannoni
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000907:Antibodies, Bacterial / Q000097:blood; D001323:Autoantibodies / Q000097:blood*; D001480:Basal Ganglia Diseases / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology*; D015153:Blotting, Western; D002648:Child; D002675:Child, Preschool; D003342:Corpus Striatum / Q000276:immunology / Q000473:pathology; D004421:Dystonia / Q000209:etiology; D004673:Encephalomyelitis, Acute Disseminated / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology*; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D001523:Mental Disorders / Q000209:etiology; D009474:Neurons / Q000276:immunology / Q000473:pathology; D012698:Serologic Tests; D013290:Streptococcal Infections / Q000150:complications / Q000503:physiopathology*; D013297:Streptococcus pyogenes / Q000276:immunology / Q000302:isolation & purification
chemical_list: D000907:Antibodies, Bacterial; D001323:Autoantibodies