On defining Sydenham’s chorea: where do we draw the line?

• Published in: Biological Psychiatry 2000-05, Vol.47 (10), p.851-857
• Main Authors: Murphy, Tanya K, Goodman, Wayne K, Ayoub, Elia M, Voeller, Kytja K
• Format: Article
• Language: English
• Subjects: Arthritis - diagnosis; Arthritis - microbiology; Autoimmune Diseases; Biological and medical sciences; Child; Child, Preschool; Chorea - diagnosis; Diagnosis, Differential; Humans; Medical sciences; Mental Disorders - diagnosis; Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis; Myocarditis - diagnosis; Myocarditis - microbiology; Neurology; Obsessive-Compulsive Disorder - diagnosis; obsessive–compulsive disorder; PANDAS; Rheumatic Fever - diagnosis; Streptococcal Infections; Sydenham’s chorea; Tourette Syndrome - diagnosis; Tourette’s syndrome
• ISSN: 0006-3223; 1873-2402
• DOI: 10.1016/S0006-3223(00)00843-X

Sydenham’s chorea (SC) is a major manifestation of rheumatic fever characterized by an array of neuropsychiatric symptoms that vary in severity, timing, and character. Some of the same symptoms are seen in Tourette’s syndrome and childhood-onset obsessive–compulsive disorder. Genetic vulnerability appears to play a role in all three conditions. The term PANDAS (pediatric autoimmune neuropsychiatric disorder associated with streptococcus) has been introduced to describe a putative subset of obsessive–compulsive disorder and Tourette’s syndrome that bears some resemblance to Sydenham’s chorea. This article discusses whether PANDAS should be subsumed under Sydenham’s chorea, thus expanding the diagnostic boundaries of Sydenham’s chorea to include primarily neuropsychiatric presentations now classified as cases of obsessive–compulsive disorder or Tourette’s syndrome. We conclude that PANDAS is a useful construct, but that it would be premature to view it as a subset of Sydenham’s chorea—whether defined narrowly or broadly.