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Neurophysiological features of primary lateral sclerosis.

Mamede de Carvalho1, Matthew C Kiernan2, Seth L Pullman3

  • 1Instituto de Fisiologia, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Department of Neurosciences and Mental Health, Hospital de Santa Maria, Centro Hospitalar Universitário de Lisboa Norte, Lisbon, Portugal.

Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration
|February 19, 2021
PubMed
Summary
This summary is machine-generated.

Primary lateral sclerosis (PLS) is a motor neuron disease. Transcranial magnetic stimulation (TMS) helps differentiate PLS from ALS by assessing upper motor neuron dysfunction, offering a better prognosis for PLS patients.

Keywords:
Amyotrophic lateral sclerosiselectromyographyneurophysiologyprimary lateral sclerosis

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Area of Science:

  • Neurology
  • Neuroscience
  • Motor Neuron Diseases

Background:

  • Primary lateral sclerosis (PLS) is a rare motor neuron disease.
  • PLS presents with upper motor neuron (UMN) signs like spasticity, without significant lower motor neuron (LMN) degeneration.
  • Distinguishing PLS from amyotrophic lateral sclerosis (ALS) is crucial due to differing prognoses and management.

Purpose of the Study:

  • To evaluate the utility of neurophysiological techniques, particularly transcranial magnetic stimulation (TMS), in differentiating PLS from ALS.
  • To highlight the role of TMS in assessing UMN dysfunction in PLS.

Main Methods:

  • Electromyography (EMG) to exclude significant LMN involvement.
  • Transcranial magnetic stimulation (TMS) to assess UMN dysfunction, measuring cortical excitability and central conduction times.
  • Interpretation of neurophysiological findings in conjunction with clinical presentation.

Main Results:

  • Electromyography is key to rule out LMN degeneration, though minor neurogenic changes can occur in PLS.
  • TMS reveals characteristic UMN dysfunction in PLS, including high cortical threshold and delayed central conduction times.
  • TMS is sensitive for detecting cortical dysfunction and may aid in monitoring disease progression.

Conclusions:

  • TMS is a valuable tool for objective assessment of UMN dysfunction in PLS and aids in differentiating it from ALS.
  • Clinical correlation with TMS findings is essential for accurate diagnosis and distinguishing between PLS and ALS.
  • Further research with larger cohorts is needed to clarify the role of other neurophysiological techniques in PLS vs. ALS differentiation.