FV 9 Diffusion tensor imaging in pure lower motor neuron disease. Issue 10 (October 2017)

• Record Type: Journal Article
• Authors: Kassubek, J.; Rosenbohm, A.; Müller, H.P.; Hübers, A.; Ludolph, A.C.
• Is Part Of: Clinical neurophysiology. Volume 128:Issue 10(2017:Oct.)
• Page Start: e310
• Publication Date: 2017-10
• Subjects: Neurophysiology -- Periodicals; Electroencephalography -- Periodicals; Electromyography -- Periodicals; Neurology -- Periodicals; 612.8
• DOI: 10.1016/j.clinph.2017.06.051
• Languages: English
• ISSNs: 1388-2457

Abstract

Abstract : Introduction: Criteria for assessing upper motor neuron pathology in lower motor neuron disease (LMND) still remain a major issue in clinical diagnosis. This study was designed to investigate patients with the clinical diagnosis of adult pure LMND by use of whole-brain based diffusion tensor imaging (DTI) in order to delineate alterations of corticoefferent pathways in vivo. Methods: Comparison of fractional anisotropy (FA) maps was performed by whole brain-based spatial statistics for 37 LMND patients vs. 53 matched controls to detect white matter structural alterations. LMND patients were clinically differentiated in fast and slow progressors. Tract specific alterations were investigated by fiber tracking techniques according to the staging hypothesis for amyotrophic lateral sclerosis (ALS). Results: The analysis of white matter structural connectivity demonstrated widespread and characteristic patterns of alterations in patients with LMND, predominantly along the corticospinal tract (CST), with multiple clusters of regional FA reductions in the motor system at p < 0.05 (corrected for multiple comparisons). Fast progressing LMND showed substantial CST involvement, while slow progressors showed less CST alterations. In the tract-specific analysis according to the ALS-staging pattern (Kassubek et al., 2014 ), fast progressing LMND showed significant alterations of ALS-related tract systems (Müller et al., xxxx ) beyond the CST compared to slow progressors and controls. Conclusion: In clinically pure LMND patients, the involvement of corticoefferent tracts was demonstrated, in particular along the CST, supporting the hypothesis that LMND is a phenotypical variant of ALS. This finding suggests to treat these patients like ALS, including the opportunity to participate in clinical trials.