Sydenham's chorea is characterized by uncoordinated movements, emotional instability, and hypotonia. It can occur up to several months after group A beta-hemolytic Streptococcus infection. A diagnosis of Sydenham's chorea in a patient with acute chorea involves an application of the Jones criteria and the exclusion of other causes of chorea. In patients with an atypical history or hemichorea, cranial magnetic resonance imaging is indicated to exclude other cerebral pathologies. A pathogenesis has not been elucidated, and therapy has not been investigated in placebo-controlled trials. Antibiotic treatment and a 2-week or 3-week schedule of antibiotic prophylaxis are recommended. If the chorea is severe, valproate or carbamazepine can be effective. In more severely affected patients, dopamine receptor blocking agents or corticosteroids can be used.
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http://dx.doi.org/10.1016/j.pediatrneurol.2009.11.015 | DOI Listing |
Acta Paediatr
January 2025
Department of Health and Wellbeing, University of Glasgow, Glasgow, UK.
Aim: Sydenham chorea (SC) is a globally significant, post-streptococcal, childhood neuropsychiatric condition that is rare in western Europe. This retrospective single-centre study focused on children with neuropsychiatric features of SC.
Methods: Participants were recruited from neuropsychiatry referrals to a regional paediatric neurology department in Glasgow, Scotland, from 2009 to 2012.
Transl Neurodegener
January 2025
State Key Laboratory of Bioactive Molecules and Druggability Assessment, Guangdong Key Laboratory of Non-Human Primate Research, Key Laboratory of CNS Regeneration (Ministry of Education), Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China.
Amyotrophic lateral sclerosis (ALS) and Huntington's disease (HD) are diverse in clinical presentation and are caused by complex and multiple factors, including genetic mutations and environmental factors. Numerous therapeutic approaches have been developed based on the genetic causes and potential mechanisms of ALS and HD. Currently, available treatments for various neurodegenerative diseases can alleviate symptoms but do not provide a definitive cure.
View Article and Find Full Text PDFBMJ Case Rep
January 2025
Internal Medicine, Yale School of Medicine, New Haven, Connecticut, USA
An African American man in his early 40s with progressive gait impairment and chronic cognitive impairment initially presented to the emergency department after statements of self-harm and was hospitalised. Examination revealed notable neurological abnormalities including impaired memory recall, oral dyskinesia/choreiform movements, dystonia of the right upper extremity with drift, hyper-reflexia and spastic gait. On further evaluation, including neurology and genetics consultation and workup, a clinical diagnosis of the neurodegenerative disorder Huntington's disease (HD) was made.
View Article and Find Full Text PDFCNS Drugs
January 2025
Innovative Medicines and Global Clinical Development, Teva Branded Pharmaceutical Products R&D, Inc., West Chester, PA, USA.
Background: Huntington disease (HD) is a progressive neurodegenerative disease that causes psychiatric and neurological symptoms, including involuntary and irregular muscle movements (chorea). Chorea can disrupt activities of daily living, pose safety issues, and may lead to social withdrawal. The vesicular monoamine transporter 2 inhibitors tetrabenazine, deutetrabenazine, and valbenazine are approved treatments that can reduce chorea.
View Article and Find Full Text PDFNat Commun
January 2025
Center for Biomolecular and Cellular Structure, Institute for Basic Science (IBS), Daejeon, Republic of Korea.
Toxic protein aggregates are associated with various neurodegenerative diseases, including Huntington's disease (HD). Since no current treatment delays the progression of HD, we develop a mechanistic approach to prevent mutant huntingtin (mHttex1) aggregation. Here, we engineer the ATP-independent cytosolic chaperone PEX19, which targets peroxisomal membrane proteins to peroxisomes, to remove mHttex1 aggregates.
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