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Central white matter degeneration in bulbar- and limb-onset amyotrophic lateral sclerosis

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Abstract

Previous studies using diffusion tensor imaging (DTI) have examined for differences between bulbar- and limb-onset amyotrophic lateral sclerosis (ALS). Findings between studies have been markedly inconsistent, though possibly as a consequence of poor matching for confounding variables. To address this problem, this study contrasted the DTI profiles of limb-onset (ALS-L) and bulbar-onset (ALS-B) in groups that were tightly matched for the potential confounding effects of power, age, cognitive impairment and motor dysfunction. 14 ALS-L and 14 ALS-B patients were selected from a large prospective study so as to be matched on clinical and demographic features. All subjects, including 29 controls, underwent neuropsychological and neurological assessment. Tract-based spatial statistics and region of interest techniques were used to analyse fractional anisotropy (FA), mean diffusivity (MD), radial diffusivity (RD) and axial diffusivity (λ 1). Extensive bilateral FA and RD changes along the corticospinal tract were found in ALS-B compared to controls, p (corrected) <0.05; a similar distribution was seen for ALS-L at a less stringent statistical threshold. ROI analyses also showed more significant changes in ALS-B than ALS-L when each was compared to controls; for FA, MD and RD the changes reached statistical significance in the direct contrast between the two patient groups. With careful matching for confounding factors, the results suggest that ALS-B is associated with greater central white matter degeneration than ALS-L, possibly contributing to the known worse prognosis of ALS-B. The study, however, found no evidence that the spatial distribution of white matter degeneration differs between these groups.

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Conflicts of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

Ethical standard

The study was approved by the local ethics committee of Otto-von-Guericke University.

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Authors and Affiliations

  1. German Center for Neurodegenerative Diseases (DZNE), Leipziger Strasse 44, 39120, Magdeburg, Germany

    Arturo Cardenas-Blanco, Judith Machts, Julio Acosta-Cabronero, Susanne Abdulla, Hans-Jochen Heinze, Stefan Vielhaber & Peter J. Nestor

  2. Department of Neurology, Otto-von-Guericke University, Leipziger Strasse 44, 39120, Magdeburg, Germany

    Joern Kaufmann, Susanne Abdulla, Hans-Jochen Heinze & Stefan Vielhaber

  3. Department of Neurology, Hannover Medical School, Carl-Neuberg-Strasse 1, 30625, Hannover, Germany

    Susanne Abdulla, Katja Kollewe, Susanne Petri & Reinhard Dengler

  4. Leibniz Institute for Neurobiology, Brenneckestrasse 6, 39118, Magdeburg, Germany

    Hans-Jochen Heinze

Authors
  1. Arturo Cardenas-Blanco
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  2. Judith Machts
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  3. Julio Acosta-Cabronero
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  4. Joern Kaufmann
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  5. Susanne Abdulla
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  6. Katja Kollewe
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  7. Susanne Petri
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  8. Hans-Jochen Heinze
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  9. Reinhard Dengler
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  10. Stefan Vielhaber
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  11. Peter J. Nestor
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Corresponding author

Correspondence to Arturo Cardenas-Blanco.

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Cardenas-Blanco, A., Machts, J., Acosta-Cabronero, J. et al. Central white matter degeneration in bulbar- and limb-onset amyotrophic lateral sclerosis. J Neurol 261, 1961–1967 (2014). https://doi.org/10.1007/s00415-014-7434-4

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  • DOI: https://doi.org/10.1007/s00415-014-7434-4

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