Increased CBF velocity during word fluency in Huntington's disease patients

A W Deckel1, D Cohen

  • 1Dept. Psychiatry, Huntington's Disease Treatment Center, University of Connecticut Medical School, Farmington, USA. Deckel@Psychiatry.uchc.edu

Insights

Huntington's disease (HD) patients show altered cerebral blood flow (CBF) during cognitive tasks. Speech production increased CBF velocity in HD, unlike other tasks, indicating a fundamental regulation disturbance.

Area of Science:

  • Neuroscience
  • Neurology
  • Cerebrovascular Physiology

Background:

  • Huntington's disease (HD) is a neurodegenerative disorder affecting cognitive and motor functions.
  • Previous research indicates abnormal cerebral blood flow (CBF) in HD patients during certain tasks.
  • Understanding CBF regulation is crucial for managing HD progression.

Purpose of the Study:

  • To investigate the impact of word fluency and reading tasks on cerebral blood flow velocity in HD patients.
  • To differentiate the effects of cognitive versus motor components of word fluency on CBF.
  • To explore potential correlations between CBF changes and disease severity in HD.

Main Methods:

  • Functional transcranial Doppler ultrasonography (fTCD) was used to measure cerebral flow velocity in the anterior (ACA) and middle (MCA) cerebral arteries.
  • Thirteen healthy controls and nine gene-positive HD patients participated.
  • Tasks included aloud word fluency, silent word fluency, and a reading test to isolate motor and cognitive demands.

Main Results:

  • HD patients exhibited increased CBF velocity in the ACA during aloud word fluency compared to controls.
  • Silent word fluency led to decreased CBF velocity in HD patients relative to controls, suggesting motor involvement.
  • CBF velocity changes correlated with word fluency scores and could predict group assignment (control, mild HD, moderate HD).

Conclusions:

  • CBF velocity in HD is dysregulated during both cognitive and motor tasks.
  • Speech production uniquely increases CBF velocity in HD patients, contrasting with findings from motor tasks.
  • These results suggest a fundamental disturbance in CBF regulation in Huntington's disease, potentially involving pathways like nitric oxide.

Related Concept Videos

Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

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 reduced penetrance,...
Parkinson Disease ll: Pathophysiology01:24

Parkinson Disease ll: Pathophysiology

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...
Parkinson's Disease: Overview01:15

Parkinson's Disease: Overview

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 to...
Alzheimer Disease ll: Pathophysiology01:23

Alzheimer Disease ll: Pathophysiology

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 microglia. Abnormal...
Parkinson Disease l: Introduction01:24

Parkinson Disease l: Introduction

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 which...
Parkinson's Disease: Treatment01:24

Parkinson's Disease: Treatment

Neurodegenerative disorders, such as Parkinson's Disease (PD), involve the gradual and irreversible destruction of neurons in particular brain areas. These disorders exhibit standard features like proteinopathies, selective vulnerability of some neurons, and an interaction of intrinsic properties, genetics, and environmental influences in neural injury.
Parkinson's Disease is primarily a result of the loss of dopaminergic neurons in the substantia nigra pars compacta. The cornerstone of its...