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Topical Review: Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infection: Sydenham Chorea, PANDAS, and PANDAS Variants

Department of Pediatrics, University of Catania, Catania, Italy, ppavone{at}mbox.unict.it.

Enrico Parano, MD

Institute of Neurological Science, The National Research Council of Italy - National Research Council, Catania, Italy

Renata Rizzo, MD

Institute of Neurological Science, The National Research Council of Italy - National Research Council, Catania, Italy

Rosario R. Trifiletti, MD, PhD

Department of Neurology, UMDNJ-New Jersey Medical School, Newark, NJ

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 ß-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. (J Child Neurol 2006;21:727—736; DOI 10.2310/7010.2006.00182).

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