PANDAS: current status and directions for research.
The recognition of the five criteria for PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) by Swedo et al established a homogenous subgroup of children with childhood onset obsessive-compulsive disorder (OCD) and/or tic disorders. The five clinical characteristics that define the PANDAS subgroup are the presence of OCD and/or tic disorder, prepubertal age of onset, abrupt onset and relapsing-remitting symptom course, association with neurological abnormalities during exacerbations (adventitious movements or motoric hyperactivity), and a temporal association between symptom exacerbations and a Group-A beta-hemolytic streptococcal (GAS) infection. These five criteria have been used for the purpose of systematic research on the phenomenology and unique therapies for the PANDAS subgroup as well as studies of the pathophysiology of post-streptococcal OCD and tic disorders. The etiology of OCD and tics in the PANDAS subgroup is unknown, but is theorized to occur as a result of post-streptococcal autoimmunity in a manner similar to that of Sydenham's chorea. The working hypothesis for the pathophysiology begins with a GAS infection in a susceptible host that incites the production of antibodies to GAS that crossreact with the cellular components of the basal ganglia, particularly in the caudate nucleus and putamen. The obsessions, compulsions, tics, and other neuropsychiatric symptoms seen in these children are postulated to arise from an interaction of these antibodies with neurons of the basal ganglia.
Snider
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LA
Pediatrics & Developmental Neuropsychiatry Branch, Department of Health and Human Services, National Institute of Mental Health, Bethesda, MD 20892, USA. SniderL@intra.nimh.nih.gov
Swedo
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SE
D019072:Antibiotic Prophylaxis; D001327:Autoimmune Diseases / Q000453:epidemiology / Q000209:etiology / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D017963:Azithromycin / Q000008:administration & dosage / Q000627:therapeutic use; D001479:Basal Ganglia / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D002819:Chorea / Q000453:epidemiology / Q000209:etiology; D004311:Double-Blind Method; D005544:Forecasting; D005625:Frontal Lobe / Q000503:physiopathology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007223:Infant; D008959:Models, Neurological; D009474:Neurons / Q000276:immunology; D009771:Obsessive-Compulsive Disorder / Q000453:epidemiology / Q000209:etiology / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D010406:Penicillins / Q000008:administration & dosage / Q000627:therapeutic use; D010951:Plasma Exchange; D016032:Randomized Controlled Trials as Topic; D012106:Research; D012213:Rheumatic Fever / Q000150:complications; D013290:Streptococcal Infections / Q000150:complications*; D013297:Streptococcus pyogenes / Q000472:pathogenicity*; D013981:Tic Disorders / Q000453:epidemiology / Q000209:etiology / Q000276:immunology / Q000503:physiopathology / Q000628:therapy
D016756:Immunoglobulins, Intravenous; D010406:Penicillins; D017963:Azithromycin