Huntington's disease (HD) is a neurodegenerative genetic disorder that affects muscle coordination and leads to cognitive decline and psychiatric problems. It typically becomes noticeable in mid-adult life. About 6% of cases start before the age of 21 years with an akinetic-rigid syndrome; they progress faster and vary slightly. The variant is classified as juvenile, akinetic-rigid or Westphal variant HD. HD is the most common genetic cause of abnormal involuntary writhing movements called chorea, which is why the disease used to be called Huntington's chorea. HD affects the whole brain, but certain areas are more vulnerable than others. The most prominent early effects are in a part of the basal ganglia called the neostriatum, which is composed of the caudate nucleus and putamen.
PODCAST: Huntington’s Disease Research and Progress
Huntington’s disease is a rare genetic condition that affects nearly 30,000 people in the United States. However, doctors believe there are about 150,000 people with the genetic mutation that causes Huntington’s but have not begun to show clinical symptoms. Dr. Jody Corey-Bloom, a leading researcher at the Huntington’s Disease Center of Excellence at UC San Diego Health System, updates listeners on the latest research and gives people guidance on participating in clinical trials. She offers resources for how patients can learn about different trials and gives listeners a brief update about the latest treatment options available for the disease.
ABSTRACT: Cognitive Reserve and Brain Reserve in Prodromal Huntington's Disease
Huntington disease (HD) is associated with decline in cognition and progressive morphological changes in brain structures. Cognitive reserve may represent a mechanism by which disease-related decline may be delayed or slowed. The current study examined the relationship between cognitive reserve and longitudinal change in cognitive functioning and brain volumes among prodromal (gene expansion-positive) HD individuals. Participants were genetically confirmed individuals with prodromal HD enrolled in the PREDICT-HD study. Cognitive reserve was computed as the composite of performance on a lexical task estimating premorbid intellectual level, occupational status, and years of education. Linear mixed effects regression (LMER) was used to examine longitudinal changes on four cognitive measures and three brain volumes over approximately 6 years. Higher cognitive reserve was significantly associated with a slower rate of change on one cognitive measure (Trail Making Test, Part B) and slower rate of volume loss in two brain structures (caudate, putamen) for those estimated to be closest to motor disease onset. This relationship was not observed among those estimated to be further from motor disease onset. Our findings demonstrate a relationship between cognitive reserve and both a measure of executive functioning and integrity of certain brain structures in prodromal HD individuals. (JINS, 2013, 19, 1–12)
FURTHER READING:
Structural MRI in Huntington's disease and recommendations for its potential use in clinical trials
Longitudinal evaluation of cognitive disorder in Huntington's disease
Diffusion weighted imaging of prefrontal cortex in prodromal huntington's disease
PODCAST: Huntington’s Disease Research and Progress
Huntington’s disease is a rare genetic condition that affects nearly 30,000 people in the United States. However, doctors believe there are about 150,000 people with the genetic mutation that causes Huntington’s but have not begun to show clinical symptoms. Dr. Jody Corey-Bloom, a leading researcher at the Huntington’s Disease Center of Excellence at UC San Diego Health System, updates listeners on the latest research and gives people guidance on participating in clinical trials. She offers resources for how patients can learn about different trials and gives listeners a brief update about the latest treatment options available for the disease.
ABSTRACT: Cognitive Reserve and Brain Reserve in Prodromal Huntington's Disease
Huntington disease (HD) is associated with decline in cognition and progressive morphological changes in brain structures. Cognitive reserve may represent a mechanism by which disease-related decline may be delayed or slowed. The current study examined the relationship between cognitive reserve and longitudinal change in cognitive functioning and brain volumes among prodromal (gene expansion-positive) HD individuals. Participants were genetically confirmed individuals with prodromal HD enrolled in the PREDICT-HD study. Cognitive reserve was computed as the composite of performance on a lexical task estimating premorbid intellectual level, occupational status, and years of education. Linear mixed effects regression (LMER) was used to examine longitudinal changes on four cognitive measures and three brain volumes over approximately 6 years. Higher cognitive reserve was significantly associated with a slower rate of change on one cognitive measure (Trail Making Test, Part B) and slower rate of volume loss in two brain structures (caudate, putamen) for those estimated to be closest to motor disease onset. This relationship was not observed among those estimated to be further from motor disease onset. Our findings demonstrate a relationship between cognitive reserve and both a measure of executive functioning and integrity of certain brain structures in prodromal HD individuals. (JINS, 2013, 19, 1–12)
FURTHER READING:
Structural MRI in Huntington's disease and recommendations for its potential use in clinical trials
Longitudinal evaluation of cognitive disorder in Huntington's disease
Diffusion weighted imaging of prefrontal cortex in prodromal huntington's disease
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