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.
Materials & methods:
We 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.
Signal 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.
SWI 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.
Research Support, Non-U.S. Gov't
Amyotrophic Lateral Sclerosis / pathology*
Brain / pathology*
Magnetic Resonance Imaging
White Matter / pathology*
Tino Prell received funding from IZKF grant from University Hospital Jena.Viktor Hartung reports no disclosures. Florian Tietz reports no disclosures. Susanne Penzlin reports no disclosures. Benjamin Ilse reports no disclosures. Ferdinand Schweser received funding from seed grants awarded by the International Organization for Magnetic Resonance in Medicine (ISMRM) and by the Friedrich Schiller University Jena. Andreas Deistung received funding from a seed grant awarded by the Interdisciplinary Center for Clinical Research (IZKF) in Jena, Germany. Martin Bokemeyer reports no disclosures. Jürgen R. Reichenbach received funding from the German Research Foundation (DFG, 2011–2015). Otto W. Witte received funding from the German Research Foundation (DFG, 2012–2015), Thuringian Ministry for Education and Research (2009–2012), Federal Ministry for Research and Technology, (2009–2015) (JenAge), Federal Ministry for Research and Technology (2009–15) (Bernstein Fokus), European Union (2006–10). Julian Grosskreutz received funding from a BMBF (the Bundesministerium für Bildung und Forschung) grant (ERMCC-NDEG) in the framework of the ERANET–NEURON program of the European Union. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.