Antibody binding to neuronal surface in Sydenham chorea, but not in PANDAS or Tourette syndrome
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Abstract
Objective: To test the hypothesis that Sydenham chorea (SC) immunoglobulin G (IgG) autoantibodies bind to specific neuronal surface proteins, whereas IgG from patients with pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS) or Tourette syndrome (TS) do not bind to neuronal surface proteins.
Methods: We used live differentiated SH-SY5Y cells, which have neuronal and dopaminergic characteristics. Using flow cytometry, we measured serum IgG cell surface binding in patients with SC (n = 11), PANDAS (n = 12), and TS (n = 11), and compared the findings to healthy controls (n = 11) and other neurologic controls (n = 11). In order to determine the specificity of binding to neuronal antigens, we also used a non-neuronal cell line, HEK 293.
Results: The mean IgG cell surface binding was significantly higher in the SC group compared to all other groups (p < 0.001). By contrast, there was no difference between the PANDAS or TS groups and the controls. Using the non-neuronal HEK-293 cells, there was no significant difference in IgG cell surface binding between any groups.
Conclusions: Serum autoantibodies that bind to neuronal cell surface antigens are present in SC, but not in PANDAS or TS. These findings strengthen the hypothesis that SC is due to a pathogenic autoantibody, but weaken the autoantibody hypothesis in PANDAS and TS.
Footnotes
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Study funding: Supported by the American Tourette Syndrome Association. R.D. and F.B. received funding from the University of Sydney postdoctoral fellowship scheme.
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Supplemental data at www.neurology.org
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- ASO
- anti-streptolysin-O
- DSM-IV
- Diagnostic and Statistical Manual of Mental Disorders, 4th edition
- HC
- healthy children
- IgG
- immunoglobulin G
- MFI
- mean fluorescence intensity
- OCD
- obsessive-compulsive disorder
- OND
- other neurologic disease
- PANDAS
- pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection
- PMA
- phorbol-12-myristate 13-acetate
- RA
- retinoic acid
- SC
- Sydenham chorea
- TH
- tyrosine hydroxylase
- TS
- Tourette syndrome.
- Received July 25, 2010.
- Accepted November 23, 2010.
- Copyright © 2011 by AAN Enterprises, Inc.
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