Background: Sydenham's chorea is associated with dysfunction of fronto-striatal circuits induced by cross-reactive antibodies to group A β-hemolytic streptococcus. High susceptibility of extrapyramidal effects of neuroleptics in patients with Sydenham's chorea suggests underlying nigro-striatal dysfunction.
Objective: To study the presence of parkinsonism in patients with a history of Sydenham's Chorea.
Methods: We used the UFMG Sydenham's Chorea Rating Scale (USCRS) and the Unified Parkinson's Disease Rating Scale (UPDRS) part III, respectively, to determine the presence of chorea and parkinsonian symptoms and signs in 25 adults with a history of previous Sydenham's Chorea currently without chorea or use of anti-choreic drugs.
Results: Bradykinesia was found in 64% of subjects. There was a statistically significant correlation between bradykinesia and hemichorea (-0.412; p = 0.036) and bradykinesia and generalized chorea (0.412; p = 0.036). There was no correlation between bradykinesia and use of anti-choreic drugs.
Conclusions: Bradykinesia is common in patients with Sydenham's Chorea in remission. This finding suggests an immune-mediated dysfunction of the nigro-striatal system.
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http://dx.doi.org/10.1016/j.parkreldis.2011.11.002 | DOI Listing |
J Neurol
January 2025
Department of Neurobiology and Behavior, University of California Irvine, Irvine, CA, USA.
Fluid biomarkers play important roles in many aspects of neurodegenerative diseases, such as Huntington's disease (HD). However, a main question relates to how well levels of biomarkers measured in CSF are correlated with those measured in peripheral fluids, such as blood or saliva. In this study, we quantified levels of four neurodegenerative disease-related proteins, neurofilament light (NfL), total tau (t-tau), glial fibrillary acidic protein (GFAP) and YKL-40 in matched CSF, plasma and saliva samples from Huntingtin (HTT) gene-positive individuals (n = 21) using electrochemiluminescence assays.
View Article and Find Full Text PDFJ Neurol
January 2025
Turner Institute for Brain and Mental Health, School of Psychological Sciences, Faculty of Medicine, Nursing and Health Sciences, Monash University, 18 Innovation Walk, Clayton, Victoria, 3800, Australia.
Background: Huntington's disease (HD) is a rare neurodegenerative disease that causes progressive cognitive, physical, and psychiatric symptoms. Computerised cognitive training (CCT) is a novel intervention that aims to improve and maintain cognitive functions through repeated practice. The effects of CCT have yet to be established in HD.
View Article and Find Full Text PDFActa 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.
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