Serial brain SPECT images in a case of Sydenham chorea

Phil Hyu Lee, Hyo Suk Nam, Kyung Yul Lee, Byung In Lee, Jong Doo Lee

Research output: Contribution to journalArticle

40 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)237-240
Number of pages4
JournalArchives of Neurology
Volume56
Issue number2
DOIs
Publication statusPublished - 1999 Feb 1

Fingerprint

Chorea
Photons
Brain
Perfusion
Corpus Striatum
Thalamus
Antistreptolysin
Pharyngitis
Blood Sedimentation
Technetium
Haloperidol
Pharynx
Penicillins
Positron-Emission Tomography
C-Reactive Protein
Echocardiography
Electroencephalography
Magnetic Resonance Imaging
Striatum

All Science Journal Classification (ASJC) codes

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

Cite this

Lee, Phil Hyu ; Nam, Hyo Suk ; Lee, Kyung Yul ; Lee, Byung In ; Lee, Jong Doo. / Serial brain SPECT images in a case of Sydenham chorea. In: Archives of Neurology. 1999 ; Vol. 56, No. 2. pp. 237-240.
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abstract = "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.",
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Serial brain SPECT images in a case of Sydenham chorea. / Lee, Phil Hyu; Nam, Hyo Suk; Lee, Kyung Yul; Lee, Byung In; Lee, Jong Doo.

In: Archives of Neurology, Vol. 56, No. 2, 01.02.1999, p. 237-240.

Research output: Contribution to journalArticle

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T1 - Serial brain SPECT images in a case of Sydenham chorea

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