Category Ranking
98%
Total Visits
921
Avg Visit Duration
2 minutes
Citations
20
Article Abstract
Objective: To identify an improved measure of clinical progression in early Huntington disease (HD) using data from prospective observational cohort studies and placebo group data from randomized double-blind clinical trials.
Methods: We studied Unified Huntington Disease Rating Scale (UHDRS) and non-UHDRS clinical measures and brain measures of progressive atrophy in 1,668 individuals with early HD followed up prospectively for up to 30 to 36 months of longitudinal clinical follow-up.
Results: The results demonstrated that a composite measure of motor, cognitive, and global functional decline best characterized clinical progression and was most strongly associated with brain measures of progressive corticostriatal atrophy.
Conclusions: Use of a composite motor, cognitive, and global functional clinical outcome measure in HD provides an improved measure of clinical progression more related to measures of progressive brain atrophy and provides an opportunity for enhanced clinical trial efficiency relative to currently used individual motor, cognitive, and functional outcome measures.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5729794 | PMC |
| http://dx.doi.org/10.1212/WNL.0000000000004743 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
A Brazilian Minamata disease? Neurologists must be aware of mercury exposure and intoxication.
Arq Neuropsiquiatr
September 2025
Universidade Federal do Pará, Instituto de Ciências Biológicas, Belém PR, Brazil.
Mercury intoxication poses a significant challenge and growing threat to public health, particularly in the Amazon region. Despite a known history of neurological damage, as evidenced by Japan's Minamata disease, mercury intoxication remains underdiagnosed in Brazil. This review underscores the need for increased clinical awareness among neurologists, as mercury exposure has been linked to over 250 neurological symptoms, including cognitive impairment, cerebellar ataxia, peripheral neuropathy, and psychiatric disturbances.
View Article and Find Full Text PDFPrevalence and magnitude of perceived dual-task difficulties in people with multiple sclerosis: an international study.
Arch Phys Med Rehabil
September 2025
REVAL Rehabilitation Research Center, Faculty of Rehabilitation Sciences, Hasselt University, Hasselt, Belgium; UMSC, Hasselt-Pelt, Belgium. Electronic address:
Objective: To investigate the prevalence and magnitude of dual-task (DT) difficulties and the discriminative ability of three questionnaires evaluating perceived DT difficulties: the Dual-Tasking Questionnaire (DTQ), Dual-Task Screening-List (DTSL), and Dual-Task-Impact on Daily-life Activities Questionnaire (DIDA-Q).
Design: Multicenter, cross-sectional study SETTING: Persons with multiple sclerosis (pwMS) and healthy controls (HC) were recruited from 7 multiple sclerosis centers across 6 countries (Belgium, Chile, Italy, Israel, Spain, and Turkey).
Participants: A total of 540 participants: 175 with mild disability (mean EDSS: 2.
Mobility impairment and fall concern predict wayfinding ability in persons with multiple sclerosis.
Mult Scler Relat Disord
September 2025
Department of Psychology, Wayne State University, Detroit, MI, 48202, USA; Institute of Gerontology, Wayne State University, Detroit, MI, 48202, USA; Translational Neuroscience Program, Wayne State University, Detroit, MI, 48201, USA. Electronic address:
The ability to navigate through one's environment is crucial for maintaining independence in daily life and depends on complex cognitive and motor functions that are vulnerable to decline in persons with Multiple Sclerosis (MS). While previous research suggests a role for mobility in the physical act of navigation, it remains unclear to what extent mobility impairment and perceptions of mobility constraints may modify wayfinding and the recall of environment details in support of successful navigation. Therefore, this study examined the relations among clinical mobility function, concern about falling, and recall of environment details in a clinical sample of MS.
View Article and Find Full Text PDFEvolution of Motor and Nonmotor Characteristics in an Idiopathic/Isolated REM Sleep Behavior Disorder Cohort.
Neurology
October 2025
Montreal Neurological Institute-Hospital, McGill University, Montreal, Canada.
Background And Objectives: Years before diagnosis of Parkinson disease (PD), dementia with Lewy bodies (DLB), or multiple system atrophy (MSA), mild prodromal manifestations can be detected. Longitudinal follow-up of people with prodromal synucleinopathy, particularly idiopathic/isolated REM sleep behavior disorder (iRBD), enables in-depth clinical phenotyping of early disease, which could facilitate stratification for clinical trials, provide the definition of appropriate end points, or predict phenoconversion more precisely. The aim of this study was to update and expand on previous studies assessing clinical evolution from iRBD to clinically diagnosed disease, up to 14 years before diagnosis.
View Article and Find Full Text PDFTLR-4/Notch1/NF-κB pathway modulation by dapagliflozin: a novel mechanism for neuroprotection in hepatic encephalopathy.
Metab Brain Dis
September 2025
Department of Pharmacology and Toxicology, Faculty of Pharmacy, Cairo University, Cairo, Egypt.
Acute or chronic liver damage can result in Hepatic Encephalopathy (HE), a potentially fatal neuropsychiatric condition that leads to cerebral and neurological alterations. Dapagliflozin (DAPA), an orally active Sodium/Glucose cotransporter 2 inhibitor with long duration of action. The study aim was to evaluate the potential protective impact of DAPA against HE caused by Thioacetamide (TAA) in rats.
View Article and Find Full Text PDF