Sydenham’s Chorea

Sydenham’s chorea (SC) is a major neurological manifestation of acute rheumatic fever (ARF) caused by group A β-haemolytic streptococci (GABHS). It occurs mainly in children between 5-15 years, with a female predominance, occurring at a ratio of approximately 2:1 (Oosterveer Overweg-Pladsoen & Roos 2010). Typically occurring 2-6 months after infection with GABHS, SC is characterized by uncoordinated, involuntary movements, muscle weakness and hypotonia. Psychiatric symptoms, including depression, anxiety, attention deficit hyperactivity disorder (ADHD) and obsessive compulsive disorder (OCD) have also been linked to SC (Teixeira Maia & Cardoso 2007). Current research indicates that the symptoms of SC are the result of basal ganglia dysfunction, caused by autoimmune mechanisms elicited by GABHS infection (Cardoso 2010). The following essay will describe SC and its effects on the nervous system. Links between basal ganglia dysfunction and SC manifestations will be discussed. SC is a neurologic manifestation of ARF hypothesised to trigger an autoimmune response leading to movement abnormalities and neuropsychiatric disturbances. The initial illness is usually characterized by pharyngitis, followed by a symptom free period of between 1-5 weeks (Cardoso 2010). The patient then develops ARF, an inflammatory disease that affects multiple tissues and organs including the joints, skin, heart, and brain. The disease usually develops in children between 5-15 years, although adult-onset disease has been described. (Gordon 2009). SC typically occurs between 2-6 months after initial illness. The disease often begins acutely, with sudden onset, however in some instances symptoms begin gradually, progressing over a period of time (Cardoso 2010). Mostly described as a benign disorder that remits after a few months, current research indicates that in up to half the patients, SC remained active 2 years after an acute episode. Recurrences have also been observed (Gordon