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Related Experiment Videos

When does Huntington's disease begin?

J R Campodonico1, E Aylward, A M Codori

  • 1Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD 21287-7218, USA.

Journal of the International Neuropsychological Society : JINS
|September 24, 1998
PubMed
Summary
This summary is machine-generated.

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Individuals with the Huntington

Area of Science:

  • Neuroscience
  • Genetics
  • Neurology

Background:

  • Basal ganglia atrophy has been observed in asymptomatic individuals carrying the Huntington's disease (HD) genetic mutation.
  • The striatum, including the caudate and putamen, is a key area affected in HD.

Purpose of the Study:

  • To investigate the association between reduced striatal volume and cognitive/neurologic changes in healthy adults with the Huntington's disease (IT-15) mutation.

Main Methods:

  • Magnetic Resonance Imaging (MRI) was used to assess caudate and putamen volume.
  • Cognitive and neurologic functioning were evaluated in 13 healthy adults with the IT-15 mutation.

Main Results:

  • Reduced striatal volume correlated with increased neurologic (motor) impairment.

Related Experiment Videos

  • Smaller striatal volume was associated with slower mental processing speed and poorer verbal learning.
  • These changes were observed in individuals without clinical HD diagnosis.

    • Conclusions:

    • Striatal volume reduction may indicate the earliest signs of Huntington's disease (HD) in genetically positive, asymptomatic individuals.
    • These findings suggest subclinical neurodegenerative changes occur before overt clinical symptoms.