A 76-year-old woman presented with asymmetric chorea (Video 1) that has evolved within the past 6 weeks before admission. The cMRI showed no brain lesions apart from slight cerebral small vessel disease (Figure, A and B). The 18F-FDG-PET revealed an increased striatal metabolism (Figure, C–F) that has been reported in young patients with Sydenham chorea after Streptococcus infections.1,2 Additional laboratory tests yield an elevated anti–streptolysin O (ASO) titer with 1352 IU/mL (ref < 200 IU/mL). Suspecting a Sydenham chorea, we initiated a treatment with the antibiotic clarithromycin combined with immunomodulatory treatment (methylprednisolone and intravenous immunoglobulins) leading to significant improvement of the movement disorder (Video 1).
(A and B) FLAIR sequences of MRI. Apart from slight signs of cerebral small vessel disease and moderate brain atrophy, the images were unremarkable. (C–E) 18F-FDG-PET scan expressing hypermetabolism in the striatum of both hemispheres. (F and G) MRI superimposed with PET images.
New-onset Sydenham chorea rarely occurs in old age. 18F-FDG-PET can contribute to diagnosis by showing increased glucose uptake in the striatum. Affected older patients may significantly benefit from drug treatment with antibiotics and immunomodulators (additional information is listed in the Supplement [eAppendix 1, links.lww.com/WNL/D36]).
Author Contributions
A. Stuetzer: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; analysis or interpretation of data. C. Strasilla: major role in the acquisition of data; analysis or interpretation of data. F.C. Robiller: major role in the acquisition of data; analysis or interpretation of data. A. Kunze: drafting/revision of the manuscript for content, including medical writing for content; study concept or design; analysis or interpretation of data.
Study Funding
The authors report no targeted funding.
Disclosure
The authors report no relevant disclosures. Go to Neurology.org/N for full disclosures.
Footnotes
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Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
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The Article Processing Charge was funded by NIH, Cleveland Clinic, Genzyme.
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Submitted and externally peer reviewed. The handling editor was Editor-in-Chief José Merino, MD, MPhil, FAAN.
- Received March 13, 2023.
- Accepted in final form June 20, 2023.
- Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
