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Related Experiment Videos

The split hand in ALS has a cortical basis.

M Weber1, A Eisen, H Stewart

  • 1The Neuromuscular Diseases Unit, Vancouver Hospital and the University of British Columbia, 855 West 12th Avenue, Vancouver, Canada V5Z 1M9. maus@interchange.ubc.ca

Journal of the Neurological Sciences
|November 25, 2000
PubMed
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Amyotrophic lateral sclerosis (ALS) preferentially impairs corticomotoneuronal input to the thenar complex, affecting thumb and index finger movement. This selective vulnerability explains early hand dysfunction in ALS patients.

Area of Science:

  • Neuroscience
  • Neurology
  • Motor Neuron Disease Research

Background:

  • Hand dysfunction, particularly loss of fine motor control of the thumb and index finger, is an early symptom in Amyotrophic Lateral Sclerosis (ALS).
  • The thenar complex, crucial for these movements, shows more pronounced wasting than the hypothenar complex in ALS patients, despite both being innervated by the same spinal cord segments.
  • This differential vulnerability suggests a selective impairment in the neural pathways controlling the thenar muscles.

Purpose of the Study:

  • To investigate whether corticomotoneuronal (descending motor pathway) input to the thenar spinal cord pool is disproportionately affected compared to the hypothenar pool in ALS.
  • To elucidate the relationship between corticomotoneuronal integrity and hand dysfunction in ALS.

Main Methods:

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  • Studied 18 ALS patients and 11 healthy controls.
  • Recorded compound muscle action potentials (CMAPs) via peripheral nerve stimulation and motor evoked potentials (MEPs) via transcranial magnetic stimulation.
  • Analyzed MEP/CMAP ratios for both thenar and hypothenar muscle complexes to assess corticomotoneuronal input strength.

Main Results:

  • Healthy subjects exhibited significantly higher MEP/CMAP ratios for the thenar complex, indicating greater corticomotoneuronal input.
  • ALS patients showed a significant reduction in the MEP/CMAP ratio for the thenar complex compared to controls.
  • No significant difference in MEP/CMAP ratios was observed for the hypothenar complex between ALS patients and controls.

Conclusions:

  • Corticomotoneuronal input to the thenar complex is preferentially impaired in Amyotrophic Lateral Sclerosis.
  • The selective vulnerability of corticomotoneuronal pathways to the thenar motor pool is a key determinant of hand dysfunction and muscle wasting in ALS.