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Related Experiment Video

Updated: Jul 8, 2025

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Current challenges in primary lateral sclerosis diagnosis.

Miguel Oliveira Santos1,2, Michael Swash1,3, Mamede de Carvalho1,2

  • 1Institute of Physiology, Instituto de Medicina Molecular João Lobo Antunes, Centro de Estudos Egas Moniz, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal.

Expert Review of Neurotherapeutics
|December 14, 2023
PubMed
Summary

Primary Lateral Sclerosis (PLS) is a rare motor neuron disorder. Research highlights diagnostic challenges and explores neurophysiology, imaging, genetics, and biomarkers for better understanding and treatment.

Keywords:
Amyotrophic lateral sclerosisdifferential diagnosiselectromyographygeneticsprimary lateral sclerosis

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Area of Science:

  • Neurology
  • Neuroscience
  • Rare Diseases

Background:

  • Primary Lateral Sclerosis (PLS) is a rare, adult-onset, slowly progressive motor neuron disorder.
  • Clinical diagnosis relies on upper motor neuron (UMN) dysfunction, which can be challenging to differentiate early on.
  • Current diagnostic criteria are disease duration-dependent, posing limitations.

Purpose of the Study:

  • To review and discuss current aspects of Primary Lateral Sclerosis (PLS).
  • To cover diagnostic criteria, differential diagnosis, and the role of various investigative tools.
  • To address symptomatic treatment and future research directions.

Main Methods:

  • Comprehensive literature search of MEDLINE and Scopus databases.
  • Inclusion of relevant articles based on quality, significance, and expert appraisal.
  • Review of diagnostic criteria, differential diagnoses, neurophysiology, neuroimaging, genetics, and biomarkers.

Main Results:

  • The review details the limitations of current PLS diagnostic criteria.
  • It discusses pitfalls in differential diagnosis from other UMN disorders.
  • The roles of neurophysiology, neuroimaging, genetics, and molecular biomarkers are examined.

Conclusions:

  • Further investigation into novel molecular biomarkers for PLS is essential.
  • Multicenter clinical trials are needed but require international collaboration due to patient rarity.
  • A deeper understanding of PLS pathophysiology is critical for developing targeted therapies.