A. Osterc Koprivšek
Enota za pediatrično nevrologijo, Klinika za pediatrijo Univerzitetni klinični center Maribor, Maribor, Slovenija

R. Rehar
Območna enota za varstvo otrok in mladine, Zdravstveni dom dr. Adolfa Drolca Maribor, Maribor, Slovenija

M. Kovač
Območna enota za varstvo otrok in mladine, Zdravstveni dom dr. Adolfa Drolca Maribor, Maribor, Slovenija

Abstract

Sydenham’s chorea (SH), the most common form of acute chorea in children, is the neurological manifestation of acute rheumatic fever (ARF). The onset of chorea is typically several weeks to months after the group A beta-hemolytic Streptococcus infection. Children between ages 8 and 9 are the most commonly affected, with female predominance of 2:1. The pathophysiological mechanism of SH is the process of molecular mimicry between the streptococcal and host antigens; cross-reactive antibodies target mainly the basal ganglia. The diagnosis of SH is made clinically, involves an application of the Jones criteria and the exclusion of other identifiable causes of acute chorea. Treatment consists of antibiotic therapy of group A beta-hemolytic Streptococcus infection, prophylactic antibiotic treatment to prevent recurrence and symptomatic treatment of chorea in the case of moderate to severe form of chorea or significant impairment in everyday activities. Given that SH is an autoimmune disorder, immunomodulatory treatment have been utilized; steroids being most frequently used. In the article we present a case of a girl with multiple recurrences of SH.

Key words: chorea, rheumatic fever, group A beta-hemolytic Streptococcus, autoimmune process, basal ganglia, hypotonia.