Background: Neuropsychiatric comorbidities are frequent in Sydenham chorea. However, cognitive impairment in Sydenham chorea has not been sufficiently described. The objective of this study was to evaluate expressive and receptive language deficits in adolescents with Sydenham chorea.
Methods: Twenty patients with Sydenham chorea were compared with 20 patients with rheumatic fever without chorea and 20 healthy controls. Participants were matched for age and gender. Participants were assessed with verbal fluency tasks (phonemic and semantic) and with verbal comprehension tasks (Token Test). Patients with Sydenham chorea were also assessed with the Universidade Federal de Minas Gerais Sydenham Chorea Rating Scale.
Results: Performance in verbal fluency and in verbal comprehension tasks differed significantly (P < 0.01) among the three groups. Patients with Sydenham chorea performed significantly worse than healthy control group in phonemic and semantic verbal fluency tasks as well as in the Token Test. The group with rheumatic fever also performed worse than healthy controls in phonemic verbal fluency. Severity of motor signs in Sydenham chorea inversely correlated with performance in phonemic verbal fluency (words beginning with letter S, and total sum of words beginning with letters F, A, and S).
Conclusions: Adolescents with Sydenham chorea show difficulties in verbal fluency and in verbal comprehension. Patients with rheumatic fever also have some degree of language impairment. Future studies must investigate language impairment in difference stages of Sydenham chorea (acute, persistent, and remission) and putative biological markers.
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http://dx.doi.org/10.1016/j.pediatrneurol.2015.06.022 | DOI Listing |
Neurology
January 2025
APHP- Salpêtrière Hospital, DMU BioGem, CNRS, INSERM, Paris Brain Institute, Sorbonne University.
Background And Objectives: Brain energy deficiency occurs at the early stage of Huntington disease (HD). Triheptanoin, a drug that targets the Krebs cycle, can restore a normal brain energetic profile in patients with HD. In this study, we aimed at assessing its efficacy on clinical and neuroimaging structural measures in HD.
View Article and Find Full Text PDFCells
January 2025
Department of Neurosurgery, University of Florida, Gainesville, FL 32608, USA.
Huntington's disease (HD) is an inherited neurodegenerative disease characterized by uncontrolled movements, emotional disturbances, and progressive cognitive impairment. It is estimated to affect 4.3 to 10.
View Article and Find Full Text PDFSci Rep
January 2025
Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands.
Rare diseases may affect the quality of life of patients and be life-threatening. Therapeutic opportunities are often limited, in part because of the lack of understanding of the molecular mechanisms underlying these diseases. This can be ascribed to the low prevalence of rare diseases and therefore the lower sample sizes available for research.
View Article and Find Full Text PDFNeurodegener Dis Manag
January 2025
Turner Institute for Brain & Mental Health, School of Psychological Sciences, Faculty of Medicine, Nursing & Health Sciences, 18 Innovation Walk, Monash University, Clayton VIC 3800, Australia.
Huntington's disease (HD) causes progressive cognitive decline, with no available treatments. Computerized cognitive training (CCT) has shown efficacy in other populations, but its effects in HD are largely unknown. This pilot study will explore the effects and neural mechanisms of CCT in HD.
View Article and Find Full Text PDFMol Med
January 2025
Department of Neurology, Shengjing Hospital of China Medical University, Shenyang, China.
Background: Mitochondrial dysfunction and neuronal damage are major sign of cytopathology in Huntington's disease (HD), a neurodegenerative disease. Ubiquitin specific peptidase 11 (USP11) is a deubiquitinating enzyme involved in various physiological processes through regulating protein degradation. However, its specific role in HD is unclear.
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