Corticosteroid Treatment Associated With Fast, Improved Outcomes in Patients With Sydenham Chorea

In a study on treatments and outcomes in patients with Sydenham chorea (SC), corticosteroids were associated with faster resolution, while antibiotics, corticosteroids, and sodium valproate were associated with a monophasic disease course.

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This meta-analysis is published in JAMA Network Open.¹

“Herein we present a comprehensive evidence synthesis of published SC cases with individual patient data (IPD), with the aims of describing the clinical features and management of SC and of identifying associations between early clinical and treatment factors and disease course and outcome,” wrote the researchers of the study.

SC is an autoimmune neuropsychiatric disorder that affects children, and is characterized by clumsy movements, muscle weakness, stumbling or falling down, not being able to speak clearly, having a hard time focusing or writing, and having a hard time keeping emotions stable, according to the NIH.²

Databases and registers of clinical trials were searched from inception in 1945 to November 1, 2022, with 3 extracted outcomes: chorea duration at the first SC episode; relapsing disease course, defined as the occurrence of at least 1 relapse at any time; and monophasic disease course, defined as no relapse after a minimum of 24 months of follow-up.

Additionally, the researchers evaluated for poor functional outcome, defined as a modified Rankin Scale (mRS) score of 2 to 6 or persisting chorea or psychiatric or behavioral symptoms at the final follow-up of 6 or more months after the last SC episode. Good functional outcome was defined as an mRS score of 0 to 1 and no chorea or psychiatric or behavioral symptoms at any time leading to the final follow-up.

A total of 1479 patients from 307 articles were included in the analysis. The median (IQR) age at onset was 10 (8-13) years, and 68.8% of patients were female patients.

Overall, immunotherapy was associated with shorter chorea duration (HR, 1.51; 95% CI, 1.05-2.19; P = .03). Additionally, patients who received 1 or more months of corticosteroids had a median chorea duration of 1.2 months (95% CI, 1.2-2.0) compared with 2.8 months (95% CI, 2.0-3.0) among patients who received none (P = .004).

Furthermore, treatments associated with monophasic disease course were antibiotics (OR, 0.28; 95% CI, 0.09-0.85; P = .02), corticosteroids (OR, 0.32; 95% CI, 0.15-0.67; P = .003), and sodium valproate (OR, 0.33; 95% CI, 0.15-0.71; P = .004).

Patients with at least 1 month of corticosteroid treatment also had significantly lower odds of relapsing disease (OR, 0.10; 95% CI, 0.04-0.25; P < .001). However, no treatment factor was associated with good functional outcome.

The researchers acknowledged some limitations to their study, including itbeing retrospective in nature; restricted to database studies in English, French, Spanish, Portuguese, and Italian, underrepresented low and lower-middle-income countries; and the inclusion of case reports may have been susceptible to diagnostic error.

Yet, despite these limitations, the researchers believe the study findings support the use of immunotherapy, particularly corticosteroids, to reduce the duration of chorea at the first episode of SC.

“Most patients achieved a good final functional outcome; however, specific treatment factors associated with this outcome remain unknown,” wrote the researchers. “This synthesis should help direct future research questions and is forming the base for an ongoing international effort with Delphi methodology to provide consensus-based recommendations for the management of SC.”

References

1. Eyre M, Thomas T, Ferrarin E, et al. Treatments and outcomes among patients with sydenham chorea: A meta-analysis. JAMA Netw Open. 2024;7(4):e246792. doi:10.1001/jamanetworkopen.2024.6792

2. Sydenham Chorea. National Institute of Neurological Disorders and Stroke. Accessed May 16, 2024. https://www.ninds.nih.gov/health-information/disorders/sydenham-chorea#disorders-r1.