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Related Experiment Videos

Cortical versus spinal dysfunction in amyotrophic lateral sclerosis.

Shahram Attarian1, Jean-Pierre Vedel, Jean Pouget

  • 1Department of Neurology and Neuromuscular Diseases, CHU La Timone, 264 rue Saint-Pierre, 13385 Marseille, France. sattarian@@ap-hm.fr

Muscle & Nerve
|March 1, 2006
PubMed
Summary
This summary is machine-generated.

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Researchers investigated motor neuron dysfunction in amyotrophic lateral sclerosis (ALS) and Kennedy's disease. Findings suggest corticospinal axon sprouting in surviving motor neurons in ALS, linking transcranial magnetic stimulation (TMS) responses to motor unit function.

Area of Science:

  • Neuroscience
  • Neurology
  • Motor Neuron Diseases

Background:

  • Amyotrophic lateral sclerosis (ALS) and Kennedy's disease involve motor neuron degeneration.
  • The relationship between cortical and spinal motor neuron dysfunction in ALS remains unclear.
  • Understanding motor unit properties provides insight into disease mechanisms.

Purpose of the Study:

  • To correlate transcranial magnetic stimulation (TMS) responses with single motor unit (MU) electromechanical properties and firing patterns in ALS and Kennedy's disease.
  • To explore potential mechanisms of motor neuron dysfunction, including axon sprouting.
  • To establish relationships between TMS responsiveness and MU functional state.

Main Methods:

  • Single motor unit (MU) recordings were performed in nine ALS patients, three Kennedy's disease patients, and 15 healthy controls.

Related Experiment Videos

  • Electromechanical properties and firing patterns of MUs were analyzed.
  • Responses of MUs to transcranial magnetic stimulation (TMS) were assessed and correlated with MU characteristics.

    • Main Results:

    • In Kennedy's disease, MUs showed enlargement, good electromechanical coupling, and variable discharge, with excitatory responses increasing with MU size.
    • In ALS, a subset of MUs exhibited characteristics similar to Kennedy's disease (enlargement, good coupling).
    • Another subset of ALS MUs displayed poor electromechanical coupling and high firing rates, while others showed moderate alterations with inhibitory responses.

    Conclusions:

    • Results suggest corticospinal axon sprouting may occur on surviving motoneurons in both ALS and Kennedy's disease.
    • A direct relationship exists between motor unit (MU) responsiveness to TMS and their functional state in these motor neuron diseases.
    • This study enhances understanding of motor neuron pathophysiology in ALS and related disorders.