Post-streptococcal autoimmune dystonia with isolated bilateral striatal necrosis

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...

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Bibliographic Details
Published in:Developmental medicine and child neurology 2002-07, Vol.44 (7), p.485-489
Main Authors: Dale, Russell C, Church, Andrew J, Benton, Sarah, Surtees, Robert A, Lees, Andrew, Thompson, Edward J, Giovannoni, Gavin, Neville, Brian G
Format: Article
Language:English
Subjects:
Antibody Specificity - immunology
Autoimmune Diseases - diagnosis
Autoimmune Diseases - immunology
Autoimmune Diseases - pathology
Basal Ganglia Diseases - diagnosis
Basal Ganglia Diseases - immunology
Basal Ganglia Diseases - pathology
Child, Preschool
Corpus Striatum - immunology
Corpus Striatum - pathology
Dominance, Cerebral - physiology
Dystonia - diagnosis
Dystonia - immunology
Dystonia - pathology
Female
Females
Humans
Infant
Magnetic Resonance Imaging
Male
Males
Necrosis
Neurons - immunology
Neurons - pathology
Original Articles
Patients
Pharyngitis - diagnosis
Pharyngitis - immunology
Pharyngitis - pathology
Streptococcal Infections - diagnosis
Streptococcal Infections - immunology
Streptococcal Infections - pathology
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Summary: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.
ISSN:0012-1622
1469-8749
DOI:10.1017/S0012162201002390