Sydenham chorea (SC), also historically known as "St. Vitus' dance," is a neurological disorder characterized by rapid, jerky, involuntary movements and muscle weakness. It is the most common cause of acquired chorea in children and represents a major manifestation of acute rheumatic fever (ARF), occurring in 20-30% of ARF cases[1]. The condition is named after Thomas Sydenham, the English physician who first described it in 1686[2].
Sydenham chorea is classified as a manifestation of rheumatic fever according to the revised Jones criteria and serves as a major diagnostic criterion. It typically follows streptococcal infection by a latency period of weeks to months, through an autoimmune mechanism involving molecular mimicry between streptococcal antigens and neuronal tissues in the basal ganglia[3].
Sydenham chorea predominantly affects children between the ages of 5 and 15 years, with a mean age of onset around 9 years[1:1]. There is a female predominance, with females affected approximately twice as often as males. The condition is more common in developing countries and in populations with limited access to healthcare, where rheumatic fever remains prevalent[2:1].
The incidence of Sydenham chorea has declined dramatically in industrialized nations since the mid-20th century due to antibiotic use and improved living conditions. However, it remains an important cause of childhood chorea worldwide, particularly in resource-limited settings[3:1].
Sydenham chorea results from an autoimmune response triggered by group A β-hemolytic streptococcus (GABHS) infection[1:2]. The mechanism involves:
Post-mortem studies have shown:
MRI may show:
The movement disorder in Sydenham chorea has characteristic features[1:3]:
Chorea:
Motor Weakness:
Other Features:
According to the 2015 revised Jones criteria, Sydenham chorea is a major manifestation of acute rheumatic fever. Diagnosis requires[1:4]:
Supporting evidence of streptococcal infection[2:5]:
Other laboratory findings (nonspecific):
| Condition | Distinguishing Features |
|---|---|
| Huntington disease | Family history, progressive course, cognitive decline |
| Wilson disease | Kayser-Fleischer rings, hepatic dysfunction |
| Chorea gravidarum | Occurs in pregnancy |
| Drug-induced chorea | Temporal relation to medications |
| Autoimmune encephalitis | Psychiatric symptoms, seizures, specific antibodies |
| PANDAS | Younger age, abrupt onset, OCD features |
Supportive care[1:5]:
First-line[3:4]:
Second-line[2:7]:
For chorea[1:6]:
For behavioral symptoms[2:8]:
The prognosis for Sydenham chorea is generally favorable[3:5]:
The major concern in Sydenham chorea is concurrent or subsequent carditis[1:7]:
Sydenham chorea is closely related to the PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections) spectrum[2:9][3:6]. Both conditions:
However, Sydenham chorea is defined in the context of acute rheumatic fever, while PANDAS encompasses a broader spectrum of post-streptococcal neuropsychiatric disorders without meeting ARF criteria.
Recent research on Sydenham Chorea includes:
Tarbox J, O'Brien M. Sydenham chorea: a practical guide. Pediatr Neurol. 2021. ↩︎ ↩︎ ↩︎ ↩︎ ↩︎ ↩︎ ↩︎ ↩︎
Church AJ, Dale RC. Streptococcal infections and movement disorders. Mov Disord. 2020. ↩︎ ↩︎ ↩︎ ↩︎ ↩︎ ↩︎ ↩︎ ↩︎ ↩︎ ↩︎
Swedo SE, Leonard HL. PANDAS and Sydenham chorea: a comparison. J Pediatr. 2019. ↩︎ ↩︎ ↩︎ ↩︎ ↩︎ ↩︎ ↩︎