Objectives: In a previous short-term study, rivastigmine has shown a mild effect in ameliorating cognitive impairment and slowing motor deterioration in patients affected by Huntington disease (HD). The aim of the present study was to assess the long-term efficacy of rivastigmine on motor and cognitive impairment in HD patients.
Methods: This was an open-label, controlled study with blinded rates: 11 HD patients were evaluated after 2 years under 6 mg rivastigmine treatment versus 6 patients sorted as controls. In basal conditions and after 2 years' follow-up, patients were submitted to the Mini-Mental State Examination, Marsden and Quinn Chorea Severity Scale, Total Functional Capacity score, Abnormal Involuntary Movement Scale, and the motor and functional section of the Unified Huntington Disease Rating Scale.
Results: Patients treated with rivastigmine showed a significant improvement of global motor performances and chorea in comparison with the control group, with a trend toward a reduction of functional disability and cognitive impairment.
Conclusions: On the basis of the long-term follow-up of HD patients, rivastigmine exerted a significant improvement of motor performances with a positive trend on cognitive and functional scales. The results of this study suggest long-term efficacy of rivastigmine in HD, which needs to be confirmed in larger series.
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http://dx.doi.org/10.1097/01.wnf.0000240945.44370.f0 | DOI Listing |
Neurochem Res
January 2025
Diagnostic Radiology Department, National Cancer Institute, Misrata, Libya.
Huntington's disease (HD) is a progressive neurodegenerative disease resulting from a mutation in the huntingtin (HTT) gene and characterized by progressive motor dysfunction, cognitive decline, and psychiatric disturbances. Currently, no disease-modifying treatments are available. Recent research has developed therapeutic agents that may have the potential to directly target the disease pathology, such as gene silencing or clearing the mutant protein.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
University of Exeter, Exeter, Devon, United Kingdom.
Background: Huntington's disease (HD) is an autosomal dominant condition causing severe neurodegeneration in the striatum and the entorhinal cortex (EC). An epigenome wide association study of DNA methylation in HD by our group, identified potential hypomethylation at the PTGDS gene in the striatum. We aimed to validate this result through pyrosequencing, examining the locus in fine detail, and to assess the signal specificity by profiling multiple neurodegenerative diseases.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
Sapienza University of Rome, Rome, Italy.
Background: Huntington's disease (HD) is a progressive neurodegenerative disorder phenotypically manifested by motor, cognitive and psychiatric symptoms (Novak and Tabrizi, 2011). These patients are also characterized by vigilance abnormalities. This has been demonstrated by electrophysiological measures (Wiegand et al.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
Sapienza University of Rome, Rome, Italy.
Background: Parkinson's disease and Huntington's disease are both neurodegenerative conditions involving the basal ganglia area of the brain. Both conditions can cause symptoms that affect movement. Cognitive decline or dementia can also occur in both.
View Article and Find Full Text PDFUnlabelled: Neurodegenerative disorders, including Alzheimer's disease and AD-related dementias (AD/ADRD), pose significant challenges to health care systems globally, particularly in Africa. With the advances in medical technology and research capabilities, especially in next-generation sequencing and imaging, vast amounts of data have been generated from AD/ADRD research. Given that the greatest increase in AD/ADRD prevalence is expected to occur in Africa, it is critical to establish comprehensive bioinformatics training programs to help African scientists leverage existing data and collect additional information to untangle AD/ADRD heterogeneity in African populations.
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