期刊
FUTURE NEUROLOGY
卷 11, 期 1, 页码 63-76出版社
FUTURE MEDICINE LTD
DOI: 10.2217/fnl.16.4
关键词
autoantibodies; autoimmunity; blood-brain barrier; gene set analysis; MHC; PANDAS; pathway-based genomic studies; Streptococcus pyogenes; Sydenham's chorea; T(h)17 cell
资金
- NHLBI NIH HHS [R01 HL116995] Funding Source: Medline
- NIMH NIH HHS [R56 MH109987] Funding Source: Medline
Streptococcus pyogenes infections have been associated with two autoimmune diseases of the CNS: Sydenham's chorea (SC) and pediatric autoimmune neuropsychiatric disorders associated with streptococcus infections (PANDAS). Despite the high frequency of pharyngeal streptococcus infections among children, only a small fraction develops SC or PANDAS. This suggests that several factors in combination are necessary to trigger autoimmune complications: specific S. pyogenes strains that induce a strong immune response toward the host nervous system; genetic susceptibility that predispose children toward an autoimmune response involving movement or tic symptoms; and multiple infections of the throat or tonsils that lead to a robust T(h)17 cellular and humoral immune response when untreated. In this review, we summarize the evidence for each factor and propose that all must be met for the requisite neurovascular pathology and behavioral deficits found in SC/PANDAS.
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