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Cerebral blood flow abnormalities in children with Sydenham's chorea: a SPECT study
Nimet Kabakus1, Tansel Ansal Balci, Abdullah Kurt
1Department of Pediatrics, Firat University, Faculty of Medicine, Firat University, Faculty of Medicine, Elazig, Turkey. nimetkabakus@yahoo.com
Insights
Sydenhams chorea patients showed brain perfusion abnormalities on SPECT scans. Abnormalities in the basal ganglia and frontal regions correlated with poor treatment response in children with Sydenhams chorea.
Area of Science:
- Neurology
- Pediatrics
- Nuclear Medicine
Background:
- Sydenhams chorea is a neurological disorder characterized by involuntary movements.
- Previous functional imaging studies suggest potential brain perfusion abnormalities in affected patients.
Purpose of the Study:
- To investigate cerebral perfusion patterns in children with Sydenhams chorea using Tc-99m HMPAO SPECT.
- To correlate perfusion findings with clinical presentation and treatment response.
Main Methods:
- Utilized Tc-99m hexamethylpropylenamine oxime (HMPAO) single-photon emission computed tomography (SPECT) for brain perfusion assessment.
- Included magnetic resonance imaging (MRI) and cranial Doppler studies for all participants.
- Analyzed perfusion patterns in cortical/subcortical structures.
Main Results:
- Brain MRI and Doppler studies were normal in all children.
- SPECT revealed cerebral perfusion abnormalities in six patients.
- Two patients with hypoperfusion in the right frontal region and basal ganglia showed poor treatment response.
- Generalized chorea was observed in patients with perfusion abnormalities, while hemichorea was seen in those with normal perfusion.
Conclusions:
- Tc-99m HMPAO SPECT can detect cerebral perfusion abnormalities in Sydenhams chorea.
- Specific perfusion patterns, particularly in the basal ganglia and frontal regions, may predict treatment outcomes.
- Cerebral perfusion patterns correlate with the clinical presentation (generalized chorea vs. hemichorea).
Abstract:
Functional imaging studies in patients with Sydenhams chorea have suggested brain perfusional abnormalities. In this study, we aimed to investigate the cerebral perfusion patterns of the cortical/subcortical structures by using Tc-99m hexamethylpropylenamine oxime single photon emission computed tomography in children with Sydenhams chorea, accompanied with magnetic resonance imaging and cranial Doppler studies. Brain MRI and Doppler studies of the all cases were normal. In the brain SPECT study, six patients were determined to have cerebral perfusion abnormalities. Although six patients responded well to the therapy, two girls who had hypoperfusion in the right frontal region as well as hypo- / hyperperfusion in the basal ganglia did not respond to therapy. While the chorea form of the patients who have cerebral perfusional abnormality was generalized, the clinical picture of the patients with normal cerebral perfusion was in the form of hemichorea.
