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Diffusion Tensor Magnetic Resonance Imaging in the Analysis of Neurodegenerative Diseases
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Neuroimaging in primary lateral sclerosis.

Erik P Pioro1, Martin R Turner2, Peter Bede3

  • 1Section of ALS & Related Disorders, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA.

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

Primary lateral sclerosis (PLS) and its relationship to amyotrophic lateral sclerosis (ALS) are increasingly studied using neuroimaging. Magnetic resonance imaging (MRI), MRS, and PET reveal central nervous system (CNS) changes in PLS, aiding diagnosis and therapy evaluation.

Keywords:
ImagingMRIPETspectroscopy

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Area of Science:

  • Neuroimaging
  • Neurology
  • Radiology

Background:

  • Primary lateral sclerosis (PLS) is rare and difficult to diagnose early, leading to less research compared to amyotrophic lateral sclerosis (ALS).
  • Recent years have seen increased interest in PLS pathogenesis and its connection to ALS, driving more central nervous system (CNS) imaging studies.
  • Neuroimaging techniques are crucial for understanding the upper motor neuron (UMN) degeneration characteristic of PLS.

Purpose of the Study:

  • To review current neuroimaging studies (MRI, MRS, PET) analyzing CNS changes in PLS.
  • To explore the relationship between PLS and ALS through the lens of neuroimaging findings.
  • To highlight the potential of neuroimaging in diagnosing PLS and evaluating new therapies.

Main Methods:

  • Review of relevant literature applying magnetic resonance imaging (MRI), magnetic resonance spectroscopy (MRS), and positron emission tomography (PET) in PLS.
  • Analysis of studies evaluating brain and spinal cord changes in PLS patients.
  • Examination of findings related to cortical and subcortical white matter abnormalities, and metabolic/cellular changes.

Main Results:

  • Structural and functional MRI and MRS studies have identified abnormalities in the cortex and subcortical white matter tracts in PLS.
  • PET imaging has revealed metabolic and cell-specific changes within the brain of PLS patients.
  • Neuroimaging confirms that neurodegeneration in PLS is primarily confined to UMN structures and pathways.

Conclusions:

  • Neuroimaging plays a vital role in understanding PLS pathogenesis and its distinction from ALS.
  • Future research should focus on novel imaging sequences and radiotracers to enhance diagnostic capabilities and explore the PLS-ALS continuum.
  • Advanced neuroimaging holds promise for assessing the efficacy of future therapeutic interventions for PLS.