 |
|
Serial Brain SPECT Images in a Case of Sydenham Chorea
Phil Hyu Lee, MD;
Hyo Suk Nam, MD;
Kyung Yul Lee, MD;
Byung In Lee, MD;
Jong Doo Lee, MD
Arch Neurol. 1999;56:237-240.
Background The pathophysiological nature of Sydenham chorea (SC) has been presumed to be an autoimmune-mediated inflammatory process. Positron emission tomography in SC has revealed a striatal hypermetabolism that might explain the transient neuronal dysfunction. However, any focal hyperperfusion in the striatum or its related structures has not been demonstrated in previous single photon emission computed tomographic (SPECT) imaging studies, which raised a concern about the pathogenesis of the striatal hypermetabolism.
Objective To investigate the cerebral perfusion patterns of the subcortical structures by using serial technetium Tc 99m–ethyl cysteinate dimer SPECT in a case of SC, which may provide a clue for the pathophysiological mechanisms.
Design A case report and serial SPECT studies.
Case Presentation A girl aged 4 years 3 months showed severe generalized choreic movements with concomitant signs of acute pharyngitis. Results of a laboratory study taken 7 days after the onset of chorea showed elevated antistreptolysin O titer, C-reactive protein levels, and erythrocyte sedimentation rate. Other laboratory data, throat culture, echocardiography, brain magnetic resonance imaging, and electroencephalography did not reveal any abnormalities. Five days after treatment with haloperidol and penicillin, the chorea began to improve slowly, and completely resolved in 2 months.
Results Three serial SPECT images and semiquantitative analysis of cerebral perfusion were obtained. Cerebral perfusion in the striatum and thalamus was markedly increased bilaterally during the stage of active chorea and then returned nearly to its baseline level during the convalescent phase. These cerebral perfusion patterns were concordant with semiquantitative analysis.
Conclusions Hyperperfusion in both the striatum and thalamus in our patient may reflect the subcortical inflammatory processes in SC. The unequivocal SPECT findings in our patient are difficult to reconcile with the negative findings of previous SPECT studies but may suggest the heterogeneity of the perfusion patterns in SC.
From the Department of Neurology (Drs P. H. Lee, Nam, K. Lee, and B. I. Lee) and the Division of Nuclear Medicine, Department of Diagnostic Radiology (Dr J. D. Lee), Yonsei University College of Medicine, Seoul, Korea.
RELATED ARTICLE
Archives of Neurology Reader's Choice: Continuing Medical Education
Arch Neurol. 1999;56(2):254-255.
FULL TEXT
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
Sydenham's Chorea: A Clinical Follow-Up of 65 Patients
Demiroren et al.
J Child Neurol 2007;22:550-554.
ABSTRACT
Topical Review: Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infection: Sydenham Chorea, PANDAS, and PANDAS Variants
Pavone et al.
J Child Neurol 2006;21:727-736.
ABSTRACT
Functional Brain Imaging in Sydenham's Chorea
Demiroren
J Child Neurol 2006;21:544-545.
Functional Brain Imaging in Sydenham's Chorea and Streptococcal Tic Disorders
Citak et al.
J Child Neurol 2004;19:387-390.
ABSTRACT
Dementia-Like Presentation of Striatal Hypermetabolic State With Antistriatal Antibodies Responsive to Steroids
Leger et al.
Arch Neurol 2004;61:754-757.
ABSTRACT
| FULL TEXT
Autoimmunity and the basal ganglia: new insights into old diseases
Dale
QJM 2003;96:183-191.
ABSTRACT
| FULL TEXT
|
