title: Post-streptococcal autoimmune dystonia with isolated bilateral striatal necrosis.
abstract: Infantile bilateral striatal necrosis (IBSN) is characterized by a dystonic movement disorder and basal ganglia imaging abnormalities. Acute IBSN often occurs after upper respiratory tract infections although no specific micro-organism which may cause IBSN has been identified. We present 2 children (1 year 2 months and 4 years) with acute IBSN after clinical pharyngitis. Both IBSN patients had serological evidence of recent beta-haemolytic streptococcal infection. Due to the association of post-streptococcal disorders with anti-basal ganglia antibodies (ABGA), we examined both patients for anti-neuronal antibodies. For comparison, 20 children with dystonia (9 females, 11 males; mean age 4 years 1 month), and 20 children with uncomplicated streptococcal infection (12 females, 8 males; mean age 5 years 9 months) were examined. Both IBSN patients had antibodies reactive against basal ganglia constituents of molecular weight 40 kDa. Immunohistochemistry showed antibody reactivity against large striatal neurons only. Other anti-neuronal antibodies were negative, supporting striatal specificity. All controls were negative for ABGA. Acute IBSN is part of the poststreptococcal autoimmune neuropsychiatric spectrum. An autoimmune aetiology should be considered in this phenotype, as immunomodulatory therapies may reduce morbidity and mortality.
authors.affiliation: Neurosciences Unit, Institute of Child Health, London, UK. r.dale@ion.ucl.ac.uk
authors.forename: Russell C
authors.initials: RC
authors.lastname: Dale
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Church
authors.forename: Sarah
authors.initials: S
authors.lastname: Benton
authors.forename: Robert A
authors.initials: RA
authors.lastname: Surtees
authors.forename: Andrew
authors.initials: A
authors.lastname: Lees
authors.forename: Edward J
authors.initials: EJ
authors.lastname: Thompson
authors.forename: Gavin
authors.initials: G
authors.lastname: Giovannoni
authors.forename: Brian G
authors.initials: BG
authors.lastname: Neville
mesh_terms: D000918:Antibody Specificity / Q000276:immunology; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology; D001480:Basal Ganglia Diseases / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology; D002675:Child, Preschool; D003342:Corpus Striatum / Q000276:immunology* / Q000473:pathology; D004292:Dominance, Cerebral / Q000502:physiology*; D004421:Dystonia / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009336:Necrosis; D009474:Neurons / Q000276:immunology / Q000473:pathology; D010612:Pharyngitis / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology; D013290:Streptococcal Infections / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology