Autoimmune neuropsychiatric disorders associated with streptococcal infection: Sydenham chorea, PANDAS, and PANDAS variants.
Streptococcal infection in children is usually benign and self-limited. In a small percentage of children, prominent neurologic and/or psychiatric sequelae can occur. Sydenham chorea is the best defined and best recognized. PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection) is a well-defined syndrome in which tics (motor and/or vocal) and/or obsessive-compulsive disorder consistently exacerbate in temporal correlation to a group A beta-hemolytic streptococcal infection. PANDAS constitutes a subset of children with tics, Tourette syndrome, and obsessive-compulsive disorder. In addition to strictly defined PANDAS, we and others have recognized several PANDAS variants, including adult-onset variant, a dystonic variant, a myoclonic variant, and a "chronic" PANDAS variant. The nosology and classification of these entities are rapidly evolving. The recognition that some pediatric neurobehavioral syndromes have infectious and/or immunologic triggers points to important new avenues of disease treatment. In this review, we summarize this complex and rapidly evolving area of clinical research.
Pavone
Piero
P
Department of Pediatrics, Division of Clinical Pediatrics, University of Catania, Viale Andrea Doria 6, 95125 Catania, Italy. ppavone@mbox.unict.it
Parano
Enrico
E
Rizzo
Renata
R
Trifiletti
Rosario R
RR
D000293:Adolescent; D000328:Adult; D020274:Autoimmune Diseases of the Nervous System / Q000145:classification / Q000175:diagnosis* / Q000276:immunology / Q000382:microbiology; D002648:Child; D002675:Child, Preschool; D002819:Chorea / Q000175:diagnosis* / Q000276:immunology / Q000382:microbiology; D006801:Humans; D007223:Infant; D009771:Obsessive-Compulsive Disorder / Q000175:diagnosis* / Q000276:immunology / Q000382:microbiology; D011135:Polysaccharides, Bacterial / Q000276:immunology; D013290:Streptococcal Infections / Q000150:complications* / Q000276:immunology; D013297:Streptococcus pyogenes; D013577:Syndrome; D013981:Tic Disorders / Q000175:diagnosis* / Q000276:immunology / Q000382:microbiology
D011135:Polysaccharides, Bacterial; C036112:streptococcal polysaccharide group A