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Related Experiment Videos

Huntington's disease progression. PET and clinical observations.

T C Andrews1, R A Weeks, N Turjanski

  • 1MRC Cyclotron Unit, Hammersmith Hospital, Institute of Neurology, London, UK. thom@cu.rpms.ac.uk

Brain : a Journal of Neurology
|December 3, 1999
PubMed
Summary

Positron emission tomography (PET) reveals dopamine D1 and D2 receptor loss in Huntington's disease (HD) mutation carriers. This method identifies asymptomatic individuals with active progression, crucial for neuroprotective therapy trials.

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Area of Science:

  • Neuroscience
  • Radiology
  • Genetics

Background:

  • Huntington's disease (HD) is a progressive neurodegenerative disorder.
  • Dopamine receptor dysfunction is implicated in HD pathogenesis.
  • Early identification of disease progression is vital for therapeutic interventions.

Purpose of the Study:

  • To quantify the rate of striatal dopamine D1 and D2 receptor binding loss in asymptomatic and symptomatic HD mutation carriers using serial PET imaging.
  • To correlate receptor binding changes with clinical assessments in HD.
  • To evaluate the utility of PET imaging in identifying actively progressing asymptomatic HD mutation carriers for clinical trials.

Main Methods:

  • Serial Positron Emission Tomography (PET) scans using [(11)C]SCH 23390 (for D1 receptors) and [11C]raclopride (for D2 receptors) were performed.

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  • Participants included asymptomatic HD mutation carriers, symptomatic HD patients, mutation-negative controls, and at-risk individuals.
  • Clinical assessments utilized the Unified Huntington's Disease Rating Scale (UHDRS).

    • Main Results:

    • Asymptomatic HD mutation carriers showed a mean annual loss of striatal D1 binding of 2.0% and D2 binding of 4.0%.
    • Actively progressing asymptomatic carriers exhibited higher annual loss rates (D1: 4.5%, D2: 6.5%).
    • Symptomatic HD patients showed annual binding losses of 5.0% for D1 and 3.0% for D2.
    • Striatal D1 and D2 binding loss was significantly greater in known HD mutation carriers compared to controls (P < 0.05).
    • Motor function and functional capacity scores correlated significantly with D1 and D2 binding levels.

    Conclusions:

    • Serial PET imaging of striatal dopamine D1 and D2 receptors can quantify disease progression in HD.
    • This PET methodology can identify asymptomatic HD mutation carriers with active progression, suitable for neuroprotective therapy trials.
    • Disease progression rates measured by PET are critical for future clinical trials of restorative treatments in HD.