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Primary lateral sclerosis: consensus diagnostic criteria.

Martin R Turner1, Richard J Barohn2, Philippe Corcia3

  • 1Nuffield Department of Clinical Neurosciences, Oxford University, Oxford, UK martin.turner@ndcn.ox.ac.uk.

Journal of Neurology, Neurosurgery, and Psychiatry
|February 8, 2020
PubMed
Summary
This summary is machine-generated.

Primary lateral sclerosis (PLS) is a motor neuron disorder. Updated criteria aim to speed diagnosis, aiding research into new treatments for this progressive neurodegenerative condition.

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Area of Science:

  • Neurology
  • Neurodegenerative Diseases

Background:

  • Primary lateral sclerosis (PLS) is a rare adult motor system neurodegenerative disorder.
  • PLS is characterized by a slowly progressive upper motor neuron syndrome.
  • Distinguishing early PLS from amyotrophic lateral sclerosis (ALS) presents a diagnostic challenge.

Purpose of the Study:

  • To present updated consensus diagnostic criteria for PLS.
  • To reduce diagnostic delays in PLS.
  • To optimize therapeutic trial design and accelerate the development of disease-modifying therapies for PLS.

Main Methods:

  • Clinical diagnosis based on exclusion of mimics.
  • Review and update of existing diagnostic criteria for PLS.
  • Consideration of emerging technologies for motor neuron assessment.

Main Results:

  • Current diagnostic criteria may impede timely diagnosis and therapeutic development.
  • Updated criteria aim to shorten the time from symptom onset to formal PLS diagnosis.
  • Revised criteria are intended to facilitate the design of clinical trials for PLS.

Conclusions:

  • Updated consensus criteria are proposed to address diagnostic challenges in PLS.
  • Reducing diagnostic delay is crucial for advancing PLS research and treatment.
  • These revised criteria aim to catalyze the development of effective therapies for PLS.