Susceptibility-weighted imaging provides insight into white matter damage in amyotrophic lateral sclerosis.

Tino Prell,Florian Tietz,Jürgen R Reichenbach,Benjamin Ilse,Martin Bokemeyer,Andreas Deistung,Susanne Penzlin,Otto W Witte,Viktor Hartung,Ferdinand Schweser,Julian Grosskreutz

doi:10.1371/journal.pone.0131114

Abstract

BackgroundAmyotrophic lateral sclerosis (ALS) is a fatal, progressive neurodegenerative disorder, characterised by widespread white matter damage. There is growing evidence that disturbances in iron metabolism contribute to white matter alterations.Materials & MethodsWe analysed the data of susceptibility-weighted imaging (SWI) of white matter in a cohort of 27 patients with ALS and 30 healthy age-matched controls.ResultsSignal alterations were found on SWI in the corpus callosum; along the corticospinal tract (subcortical motor cortex, posterior limb of the internal capsule and brainstem levels) and in the subgyral regions of frontal, parietal, temporal, occipital and limbic lobes. Alterations of white matter in the corpus callosum correlated with disease severity as assessed by the revised ALS functional rating scale.ConclusionSWI is capable of indicating iron and myelin disturbances in white matter of ALS patients. The SWI patterns observed in this study suggest that widespread alterations due to iron disturbances occur in patients with ALS and correlate with disease severity.

Highlights

Amyotrophic lateral sclerosis (ALS) is the most common adult-onset motor neuron disease and is characterised by progressive failure of the upper and lower motor neurons
Signal alterations were found on susceptibility-weighted imaging (SWI) in the corpus callosum; along the corticospinal tract and in the subgyral regions of frontal, parietal, temporal, occipital and limbic lobes
Alterations of white matter in the corpus callosum correlated with disease severity as assessed by the revised ALS functional rating scale

Summary

Introduction

Amyotrophic lateral sclerosis (ALS) is the most common adult-onset motor neuron disease and is characterised by progressive failure of the upper and lower motor neurons. Similar to other neurodegenerative disorders like Parkinson or Alzheimers disease, magnetic resonance imaging (MRI) markers, indicative of excess iron levels, have been reported in the brains of patients with ALS as well as in animal models of ALS [2,3,4,5]. Since extensive white matter alterations are frequently observed in patients with ALS [6, 7], it seems reasonable to hypothesise a link between white matter damage and disturbances in iron levels. Amyotrophic lateral sclerosis (ALS) is a fatal, progressive neurodegenerative disorder, characterised by widespread white matter damage. There is growing evidence that disturbances in iron metabolism contribute to white matter alterations

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