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Post‐streptococcal autoimmune dystonia with isolated bilateral striatal necrosis
Author(s) -
Dale Russell C,
Church Andrew J,
Benton Sarah,
Surtees Robert A,
Lees Andrew,
Thompson Edward J,
Giovani Gavin,
Neville Brian G
Publication year - 2002
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/j.1469-8749.2002.tb00310.x
Subject(s) - basal ganglia , medicine , dystonia , etiology , autoantibody , antibody , serology , immunology , pathology , central nervous system , psychiatry
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 post‐streptococcal autoimmune neuropsychiatric spectrum. An autoimmune aetiology should be considered in this phenotype, as immunomodulatory therapies may reduce morbidity and mortality.