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Related Experiment Video

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Diffusion Tensor Magnetic Resonance Imaging in the Analysis of Neurodegenerative Diseases
09:33

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Published on: July 28, 2013

Upper and extra-motoneuron involvement in early motoneuron disease: a diffusion tensor imaging study.

Maaike M van der Graaff1, Caroline A Sage, Matthan W A Caan

  • 1Academic Medical Centre, Department of Neurology (room H2-234), PO Box 22660, 1100 DD Amsterdam, The Netherlands. m.m.vandergraaff@amc.uva.nl

Brain : a Journal of Neurology
|March 3, 2011
PubMed
Summary
This summary is machine-generated.

Motoneuron disease phenotypes show early white matter degeneration beyond motor pathways. This suggests they are part of a single multisystem neurodegenerative disease spectrum, with voxel-based analysis proving sensitive for tracking changes.

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Area of Science:

  • Neuroscience
  • Neurology
  • Radiology

Background:

  • Motoneuron disease (MND) encompasses amyotrophic lateral sclerosis (ALS), primary lateral sclerosis (PLS), and progressive muscular atrophy (PMA).
  • Clinical diagnoses of MND phenotypes may not fully capture the extent of neurodegeneration.
  • Understanding white matter involvement is crucial for delineating MND phenotypes.

Purpose of the Study:

  • To assess upper and extra-motoneuron white matter involvement across MND phenotypes shortly after diagnosis.
  • To compare diffusion tensor imaging (DTI) data between MND cohorts and healthy controls.
  • To evaluate longitudinal changes in white matter integrity over six months.

Main Methods:

  • Controlled study comparing DTI data from 12 patients per MND phenotype (ALS, PLS, PMA) and healthy controls.
  • Utilized diffusion tensor tractography (DTT) of the corticospinal tract and whole-brain voxel-based analysis (VBA).
  • Acquired follow-up DTI data after 6 months to assess changes in fractional anisotropy (FA).

Main Results:

  • VBA revealed varying extents of white matter involvement across MND phenotypes, primarily in similar anatomical locations.
  • Fractional anisotropy reductions were modest in PMA and most extensive in PLS.
  • Longitudinal VBA showed progressive extra-motor white matter degeneration in ALS and PMA.

Conclusions:

  • Early upper and extra-motoneuron involvement in all MND phenotypes suggests a shared multisystem neurodegenerative disease spectrum.
  • Voxel-based analysis is more sensitive than DTT for detecting longitudinal white matter changes in early-stage MND.
  • VBA is valuable for evaluating and monitoring motor and extra-motor white matter pathology in MND.