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Updated: Jul 4, 2025

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
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Primary Lateral Sclerosis: An Overview.

Veria Vacchiano1, Luigi Bonan2, Rocco Liguori1,2

  • 1IRCCS, Istituto delle Scienze Neurologiche di Bologna, UOC Clinica Neurologica, 40139 Bologna, Italy.

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|January 26, 2024
PubMed
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Primary lateral sclerosis (PLS) selectively affects upper motor neurons, causing progressive spasticity. This review highlights diagnostic challenges and knowledge gaps for this rare neurodegenerative disease.

Keywords:
ALShereditary spastic paraplegiamotor neuron diseaseprimary lateral sclerosisreview

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Area of Science:

  • Neurology
  • Neurodegenerative Diseases

Background:

  • Primary lateral sclerosis (PLS) is a rare neurodegenerative disorder characterized by selective upper motor neuron (UMN) deterioration.
  • It leads to progressive motor disability, primarily muscle spasticity in the lower extremities and bulbar muscles.
  • While often sporadic, familial cases and potential genetic links exist, and its place in the motor neuron disease spectrum remains debated.

Purpose of the Study:

  • To provide a comprehensive analysis of Primary Lateral Sclerosis (PLS).
  • To highlight controversies in differential diagnoses and current diagnostic criteria.
  • To discuss existing knowledge gaps in diagnosis and therapy for PLS.

Main Methods:

  • Comprehensive literature review.
  • Analysis of neuropathological, clinical, neuroimaging, and neurophysiological features.
  • Examination of differential diagnoses and diagnostic criteria.

Main Results:

  • PLS selectively impacts UMNs, causing progressive spasticity.
  • Diagnostic imprecision and overlap with ALS and HSP hinder therapeutic trials.
  • Lack of specific biomarkers impedes accurate clinical diagnosis.

Conclusions:

  • PLS is a distinct rare neurodegenerative entity with progressive motor disability.
  • Significant knowledge gaps exist in diagnosing and treating PLS.
  • Further research is needed for specific biomarkers and targeted therapies.