Related Experiment Video
Updated: May 1, 2026
08:27
Single Synapse Indicators of Glutamate Release and Uptake in Acute Brain Slices from Normal and Huntington Mice
Published on: March 11, 2020
5.7K
Movement sequencing in Huntington disease.
Nellie Georgiou-Karistianis1, Jeffrey D Long, Spencer G Lourens
1School of Psychological Sciences, Monash University , Wellington Road, Clayton, Victoria , Australia.
Summary
Movement sequencing declines in prodromal Huntington's disease (HD). Individuals closer to diagnosis showed slower movement execution, suggesting compensatory strategies may impact motor control.
Area of Science:
- Neuroscience
- Movement Disorders
- Neurodegenerative Diseases
Background:
- Huntington's disease (HD) is a progressive neurodegenerative disorder.
- Prodromal HD is characterized by subtle motor and cognitive changes before clinical diagnosis.
- Understanding early motor deficits is crucial for timely intervention.
Purpose of the Study:
- To investigate longitudinal changes in movement sequencing in individuals with prodromal Huntington's disease (HD).
- To assess how proximity to HD diagnosis, indicated by the CAG-Age Product (CAP) score, influences motor sequencing.
- To analyze the impact of advance cueing on response initiation and execution in prodromal HD.
Main Methods:
- Longitudinal study of 795 prodromal HD participants and 225 controls over seven annual visits.
- Participants stratified into low, medium, and high CAP score groups.
- Cued movement sequence task with varying levels of advance information to assess initiation and execution.
Main Results:
- Prodromal HD participants exhibited longer initiation and movement times compared to controls at baseline.
- A disease gradient was observed, with higher CAP scores correlating with more pronounced motor deficits.
- The high CAP group showed increased movement time and faster, yet ultimately slower, initiation over time.
Conclusions:
- Prodromal HD progression is associated with altered movement sequencing.
- Individuals nearing diagnosis may employ compensatory strategies, leading to faster initiation but slower execution.
- This suggests a decline in the ability to translate planned movements into efficient motor actions in advancing prodromal HD.
More Related Videos
Related Concept Videos
01:21Huntington Disease l: Introduction
110
Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show...
110
01:24Parkinson Disease ll: Pathophysiology
28
Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...
28
01:24Parkinson Disease l: Introduction
28
Parkinson’s disease is a chronic, progressive neurodegenerative disorder that primarily affects movement. It is characterized by motor symptoms such as resting tremors, muscle rigidity, bradykinesia (slowness of movement), and postural instability. Patients may notice hand tremors at rest, stiffness during movement, or a shuffling gait. In addition to motor features, non-motor symptoms include sleep disturbances, mood and behavioral changes, constipation, and cognitive impairment, all of...
28
01:15Parkinson's Disease: Overview
2.2K
Neurodegenerative disorders are progressive diseases that cause irreversible damage and loss to neurons in specific brain areas. Examples of these disorders include Parkinson's disease, Alzheimer's disease, Multiple Sclerosis (MS), and Amyotrophic Lateral Sclerosis (ALS). These disorders share characteristics such as proteinopathies, selective neuronal vulnerability, and a complex interplay between genetic and environmental factors. The primary therapeutic goal for these conditions is...
2.2K
01:23Alzheimer Disease ll: Pathophysiology
35
Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and...
35
01:29Alzheimer Disease l: Introduction
21
Alzheimer disease is a chronic, progressive, and irreversible neurodegenerative disorder and the most common cause of dementia in older adults. It leads to gradual neuronal loss, causing cognitive decline, behavioral changes, and loss of functional independence.Risk Factors and EtiologyThe disease is multifactorial. Age is the strongest risk factor, with prevalence doubling every 5 years after age 65. Genetic factors include mutations in genes such as APP, PSEN1, and PSEN2, which are associated...
21