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Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis
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Subcortical structures in amyotrophic lateral sclerosis.

Henk-Jan Westeneng1, Esther Verstraete1, Renée Walhout1

  • 1Department of Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, The Netherlands.

Neurobiology of Aging
|October 5, 2014
PubMed
Summary

Amyotrophic lateral sclerosis (ALS) patients exhibit progressive neurodegeneration in deep gray matter and hippocampal subfields, alongside ventricular enlargement. These structural brain changes correlate with disease severity, impacting motor function.

Keywords:
Amyotrophic lateral sclerosisBasal gangliaHippocampal subfieldsLongitudinalMagnetic resonance imaging

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Area of Science:

  • Neuroimaging
  • Neurology
  • Radiology

Background:

  • Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons.
  • Understanding subcortical and ventricular changes in ALS is crucial for disease monitoring and therapeutic development.

Purpose of the Study:

  • To investigate deep gray matter, hippocampal subfield, and ventricular volume changes in ALS patients using MRI.
  • To assess the longitudinal progression of these changes and their correlation with clinical parameters.

Main Methods:

  • High-resolution 3T MRI scans were acquired from 112 ALS patients and 60 healthy controls.
  • Volumetric and shape analyses were performed on subcortical structures and ventricles.
  • Longitudinal changes were assessed in a subset of patients, and correlations with ALSFRS-R scores were analyzed.

Main Results:

  • ALS patients showed reduced hippocampal volumes and enlarged inferior lateral ventricles compared to controls.
  • Longitudinal analysis revealed progressive volume loss in specific hippocampal subfields (cornu ammonis, presubiculum) and significant ventricular enlargement.
  • Larger ventricular volumes correlated with lower ALSFRS-R scores, indicating greater disease severity.

Conclusions:

  • ALS is characterized by progressive neurodegeneration in deep gray matter and hippocampal subfields, accompanied by ventricular enlargement.
  • These structural brain alterations are linked to clinical progression and disease severity in ALS patients.