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Evaluation of Motor Impairment in C. elegans Models of Amyotrophic Lateral Sclerosis
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Regional motor cortex dysfunction in amyotrophic lateral sclerosis.

Parvathi Menon1,2, Con Yiannikas2,3, Matthew C Kiernan2,4

  • 1Westmead Hospital, Sydney, New South Wales, Australia.

Annals of Clinical and Translational Neurology
|August 13, 2019
PubMed
Summary
This summary is machine-generated.

Amyotrophic lateral sclerosis (ALS) shows varied cortical dysfunction across body regions. Cortical hyperexcitability is more prominent in upper limbs, while inexcitability is seen in lower limbs and bulbar regions, indicating heterogeneous pathology.

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Area of Science:

  • Neuroscience
  • Neurology

Background:

  • Amyotrophic lateral sclerosis (ALS) pathophysiology requires deeper understanding.
  • Cortical dysfunction is implicated in ALS, with prior studies focusing on hand representation.

Purpose of the Study:

  • To investigate cortical dysfunction across hand, leg, and bulbar representations in ALS patients.
  • To correlate these cortical changes with clinical features of ALS.

Main Methods:

  • Cross-sectional study involving 60 sporadic ALS patients and 28 healthy controls.
  • Threshold tracking transcranial magnetic stimulation (TMS) used to record motor evoked potentials (MEPs).
  • MEPs recorded from abductor pollicis brevis (hand), tibialis anterior (leg), and trapezius (bulbar) muscle regions.

Main Results:

  • Cortical dysfunction was observed across all three body regions in ALS patients compared to controls.
  • Cortical hyperexcitability (reduced short interval intracortical inhibition) was evident in all regions (hand, leg, bulbar).
  • Cortical inexcitability was more prominent in the leg and bulbar regions.

Conclusions:

  • Cortical dysfunction in ALS exhibits heterogeneity, varying by body region.
  • Cortical hyperexcitability was more pronounced in upper limbs, while inexcitability was more evident in lower limbs and bulbar regions.
  • Findings suggest diverse cortical pathophysiological processes in ALS.