Propagation patterns in motor neuron diseases: Individual and phenotype-associated disease-burden trajectories across the UMN-LMN spectrum of MNDs.

Autor: Tahedl M; Computational Neuroimaging Group, Biomedical Sciences Institute, Trinity College Dublin, Ireland; Department of Psychiatry and Psychotherapy and Institute for Psychology, University of Regensburg, 93053 Regensburg, Germany., Li Hi Shing S; Computational Neuroimaging Group, Biomedical Sciences Institute, Trinity College Dublin, Ireland., Finegan E; Computational Neuroimaging Group, Biomedical Sciences Institute, Trinity College Dublin, Ireland., Chipika RH; Computational Neuroimaging Group, Biomedical Sciences Institute, Trinity College Dublin, Ireland., Lope J; Computational Neuroimaging Group, Biomedical Sciences Institute, Trinity College Dublin, Ireland., Hardiman O; Computational Neuroimaging Group, Biomedical Sciences Institute, Trinity College Dublin, Ireland., Bede P; Computational Neuroimaging Group, Biomedical Sciences Institute, Trinity College Dublin, Ireland; Pitié-Salpêtrière University Hospital, Sorbonne University, Paris, France. Electronic address: bedep@tcd.ie.
Jazyk: angličtina
Zdroj: Neurobiology of aging [Neurobiol Aging] 2022 Jan; Vol. 109, pp. 78-87. Date of Electronic Publication: 2021 Oct 08.
DOI: 10.1016/j.neurobiolaging.2021.04.031
Abstrakt: Motor neuron diseases encompass a divergent group of conditions with considerable differences in clinical manifestations, survival, and genetic vulnerability. One of the key aspects of clinical heterogeneity is the preferential involvement of upper (UMN) and lower motor neurons (LMN). While longitudinal imaging patters are relatively well characterized in ALS, progressive cortical changes in UMN,- and LMN-predominant conditions are seldom evaluated. Accordingly, the objective of this study is the juxtaposition of longitudinal trajectories in 3 motor neuron phenotypes; a UMN-predominant syndrome (PLS), a mixed UMN-LMN condition (ALS), and a lower motor neuron condition (poliomyelitis survivors). A standardized imaging protocol was implemented in a prospective, multi-timepoint longitudinal study with a uniform follow-up interval of 4 months. Forty-five poliomyelitis survivors, 61 patients with amyotrophic lateral sclerosis (ALS), and 23 patients with primary lateral sclerosis (PLS) were included. Cortical thickness alterations were evaluated in a dual analysis pipeline, using standard cortical thickness analyses, and a z-score-based individualized approach. Our results indicate that PLS patients exhibit rapidly progressive cortical thinning primarily in motor regions; ALS patients show cortical atrophy in both motor and extra-motor regions, while poliomyelitis survivors exhibit cortical thickness gains in a number of cerebral regions. Our findings suggest that dynamic cortical changes in motor neuron diseases may depend on relative UMN and/or LMN involvement, and increased cortical thickness in LMN-predominant conditions may represent compensatory, adaptive processes.
(Copyright © 2021. Published by Elsevier Inc.)
Databáze: MEDLINE
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