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Related Experiment Videos

Amyotrophic lateral sclerosis: objective upper motor neuron markers.

Petra Kaufmann1, Hiroshi Mitsumoto

  • 1Neurological Institute, 9th Floor, 710 West 168th Street, New York, NY 10032, USA. pk88@columbia.edu

Current Neurology and Neuroscience Reports
|March 20, 2002
PubMed
Summary

Diagnosing amyotrophic lateral sclerosis (ALS) relies on clinical signs. This review explores advancements in identifying objective markers for upper motor neuron involvement, crucial for new ALS therapies.

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Area of Science:

  • Neuroscience
  • Neurology
  • Biochemistry

Background:

  • Amyotrophic lateral sclerosis (ALS) diagnosis is clinical, combining upper and lower motor neuron signs.
  • Objective markers are needed for diagnosis and clinical trials due to emerging ALS therapies.
  • Currently, no widely accepted marker exists for upper motor neuron involvement in ALS.

Purpose of the Study:

  • To review progress in establishing valid diagnostic and natural history markers for upper motor neuron involvement in ALS.
  • To highlight the need for objective measures beyond clinical examination.
  • To discuss the role of advanced neuroimaging and neurophysiological techniques.

Main Methods:

  • Review of magnetic resonance (MR) imaging techniques including MR spectroscopy and diffusion tensor imaging.

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  • Evaluation of metabolic brain changes using positron emission tomography.
  • Assessment of neurophysiological integrity via transcranial magnetic stimulation.

    • Main Results:

    • MR imaging shows changes in the corticospinal tract but lacks sensitivity and specificity.
    • MR spectroscopy demonstrates a progressive decrease in N-acetylaspartate, indicating neuronal loss.
    • Diffusion tensor imaging reveals diffusivity changes in the corticospinal tract in ALS patients.
    • Positron emission tomography assesses metabolic brain alterations.
    • Transcranial magnetic stimulation evaluates upper motor neuron neurophysiological integrity.

    Conclusions:

    • Significant progress has been made in identifying potential markers for upper motor neuron involvement in ALS.
    • Advanced neuroimaging and neurophysiological techniques show promise for objective ALS assessment.
    • Further research is needed to validate these markers for clinical use and therapeutic trials.