Abstract

BackgroundAmyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by loss of upper and lower motor neurons. There is a need for an imaging biomarker to track disease progression. Previously, magnetic resonance imaging (MRI) has shown loss of grey and white matter in the brain of patients with ALS compared to controls. We performed serial diffusion tractography imaging (DTI) study of patients with ALS looking for changes over time.MethodsOn all subjects (n = 15), we performed three MRI studies at 6 month intervals. DTI changes were assessed with tract-based spatial statistics (TBSS) and region of interest (ROI) studies. Cortic-spinal tract (CST) was selected for our ROI at the upper level; the posterior limb of internal capsule (PLIC), and a lower level in the pons.ResultsThere was no significant change in DTI measures over 12 months of observation. Better correlation of manual and atlas-based ROI methods was found in the posterior limb of the internal capsule than the pons.ConclusionWhile previous DTI studies showed significant differences between ALS subjects and controls, within individual subjects there is little evidence of progression over 12 months. This suggests that DTI is not a suitable biomarker to assess disease progression in ALS.

Highlights

  • Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by loss of upper and lower motor neurons

  • In this study we have focused on the Cortic-spinal tract (CST), because degeneration of the CST is a cardinal feature of the pathology in ALS

  • The subjects had a disease duration (DD), that was calculated from the date of onset to the first magnetic resonance imaging (MRI) scan, and ranged from 3 to 118 months

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Summary

Introduction

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by loss of upper and lower motor neurons. There is a need for an imaging biomarker to track disease progression. We performed serial diffusion tractography imaging (DTI) study of patients with ALS looking for changes over time. Amyotrophic lateral sclerosis (ALS) is characterized by the progressive loss of lower motor neurons (LMN) from the spinal cord and brain stem and upper motor neurons (UMN) from the motor and premotor cortex. There is a need for non-invasive biomarkers as a means of monitoring progression of patients with ALS, for prognosis and for use in clinical trials. An imaging biomarker is desirable because it can show changes in brain structure that can be related to the pathology of disease

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