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Focal thinning of the motor cortex mirrors clinical features of amyotrophic lateral sclerosis and their phenotypes: a neuroimaging study

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Abstract

Amyotrophic lateral sclerosis (ALS) is characterised by degeneration of upper (UMN) and lower motor neurons (LMN).We aimed to relate clinical variables to cortical thinning of the primary motor cortex (PMC). The PMC was defined as the region of interest in high-resolution structural MRI scans. We related vertex-wise measures of cortical thinning to UMN involvement, bulbar/limb onset, the total ALS functional rating scale (ALSFRS-R), and its bulbar and upper limb subscore. In total, 93 ALS patients were recruited (60 with classical ALS; 17 with dominant UMN, e.g., primary lateral sclerosis; 16 with pure LMN variant, e.g., progressive muscular atrophy, flail arm or leg syndrome) and compared to 67 age and gender matched healthy controls. The UMN signs in the bulbar regions were associated with bilateral thinning within the bulbar segment on the motor cortex, and UMN signs in spinal regions were associated with thinning in the limb segment of the motor cortex. The site of disease onset (bulbar/lower limb) exhibited the most pronounced thinning in the corresponding part of the motor cortex. According to our analysis, dominant UMN patients demonstrated the most distinct thinning followed by classical ALS patients. Pure LMN variants did not differ from healthy controls. The bulbar subscore of the ALSFRS-R correlated with thinning of the left inferior PMC. Focal morphological changes within the PMC correspond to clinically measured impairments and depend on disease phenotype. Measuring cortical thickness may potentially offer an objective in vivo marker to quantify disease pathology.

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Acknowledgments

We are thankful to Maria Veit for assistance in data acquisition.

Conflicts of interest

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

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Authors and Affiliations

  1. DZNE German Center for Neurodegenerative Diseases, Gehlsheimer Str. 20, 18147, Rostock, Germany

    Christina Schuster, Elisabeth Kasper, Stefan Teipel & Johannes Prudlo

  2. DZNE German Center for Neurodegenerative Diseases, Leipziger Str. 44, 39120, Magdeburg, Germany

    Judith Machts & Stefan Vielhaber

  3. Department of Neurology, Otto-von-Guericke-University, Leipziger Str. 44, 39120, Magdeburg, Germany

    Daniel Bittner, Jörn Kaufmann & Stefan Vielhaber

  4. Department of Neurology, University of Rostock, Gehlsheimer Str. 20, 18147, Rostock, Germany

    Reiner Benecke & Johannes Prudlo

  5. Department of Psychosomatic Medicine, University of Rostock, Gehlsheimer Str. 20, 18147, Rostock, Germany

    Stefan Teipel

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  1. Christina Schuster
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  2. Elisabeth Kasper
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Correspondence to Johannes Prudlo.

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Schuster, C., Kasper, E., Machts, J. et al. Focal thinning of the motor cortex mirrors clinical features of amyotrophic lateral sclerosis and their phenotypes: a neuroimaging study. J Neurol 260, 2856–2864 (2013). https://doi.org/10.1007/s00415-013-7083-z

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  • DOI: https://doi.org/10.1007/s00415-013-7083-z

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