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Published on: November 21, 2013
Clinical and Neuroimaging Findings of Sydenham's Chorea
Arzu Ekici1, Ayten Yakut1, Sevgi Yimenicioglu1
1Department of Pediatric Neurology.
Objective:
Sydenham's chorea (SC) is thought to be an autoimmune disorder. MRI is generally used to exclude other causes of chorea. There are no typically defined MRI features of SC. In this study we aimed to determine clinical and neuroimaging findings of SC.
Methods:
In this study 17 patients with acute SC were retrospectively evaluated. Sydenham's chorea was diagnosed according to the 1992 revision of the Jones criteria. The other causes of chorea were excluded. Cranial MRI was performed in all patients during the acute phase of SC. Walking, speech and swallowing disorders, muscle weakness, behavioral disorders, treatment, symptom recovery time and recurrence were evaluated. Findings : The patients' mean age was 11.2 years. Behavioral changes, muscle weakness and dysphagia occurred in 70%, 64% and 23% of the patients, respectively. Nonspecific signal hyperintensities were observed in the white matter, brain stem and caudate nucleus in 47% of patients. Two patients who had chorea paralytica were treated successfully with a high dose of intravenous methylprednisolone.
Conclusion:
Nonspecific hyperintense white matter abnormalities may be due to the inflammatory process associated with a longer duration of clinical signs. To explain the MRI findings and the pathogenesis of SC, comprehensive studies are needed.
Insights
Sydenham's chorea (SC) in children presents with behavioral changes and weakness. MRI may show white matter abnormalities, suggesting inflammation, but further research is needed to confirm SC pathogenesis.
Area of Science:
- Neurology
- Pediatrics
- Autoimmune Disorders
Background:
- Sydenham's chorea (SC) is an autoimmune disorder.
- MRI is crucial for excluding other causes of chorea.
- Defined MRI features for SC are lacking.
Purpose of the Study:
- To determine clinical and neuroimaging findings in acute Sydenham's chorea.
- To investigate the relationship between clinical signs and MRI findings in SC.
Main Methods:
- Retrospective evaluation of 17 pediatric patients with acute SC.
- Diagnosis based on the 1992 Jones criteria, excluding other causes.
- Cranial MRI performed during the acute phase; clinical data collected.
Main Results:
- Mean patient age was 11.2 years.
- Common symptoms included behavioral changes (70%), muscle weakness (64%), and dysphagia (23%).
- Nonspecific white matter hyperintensities observed in 47% of patients, particularly in the caudate nucleus and brain stem.
Conclusions:
- Nonspecific white matter abnormalities may indicate an inflammatory process in SC.
- These findings might correlate with the duration of clinical signs.
- Further comprehensive studies are required to elucidate SC pathogenesis and MRI findings.
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