The ability to resiliently cope with neuropathological lesions is a key scientific concern. Accordingly, this study aims to investigate whether motor reserve (MR), likely to be boosted by exercise engagement in a lifetime, affects motor symptom severity, cognitive functioning, and functional brain networks in spinocerebellar ataxia type 2 (SCA2)-a cerebellar neurodegenerative disease. The MR of 12 SCA2 patients was assessed using the Motor Reserve Index Questionnaire (MRIq), developed ad hoc for estimating lifespan MR. The International Cooperative Ataxia Rating Scale was used to assess clinical motor features, and neuropsychological tests were used to evaluate cognitive functioning. Patients underwent an MRI examination, and network-based statistics (NBS) analysis was carried out to detect patterns of functional connectivity (FC). Significant correlations were found between MRIq measures and the severity of motor symptoms, educational and intellectual levels, executive function, and processing speed. NBS analysis revealed a higher FC within subnetworks consisting of specific cerebellar and cerebral areas. FC patterns were positively correlated with MRIq measures, likely indicating the identification of an MR network. The identified network might reflect a biomarker likely to underlie MR, influenced by education and cognitive functioning, and impacting the severity of motor symptoms.
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http://dx.doi.org/10.3390/biomedicines10092166 | DOI Listing |
Int J Mol Sci
December 2024
Department of Neurosciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH 44195, USA.
Spinal cord injury (SCI) results in functional deficits below the injured spinal level. The descending serotonergic system in the spinal cord is critically involved in the control of motor and autonomic functions. Specifically, SCI damages the projections of serotonergic fibers, which leads to reduced serotonin inputs and increased amounts of spinal serotonergic receptors.
View Article and Find Full Text PDFJ Neural Eng
January 2025
Department of Physical Medicine and Rehabilitation, MetroHealth Medical Center, 4229 Pearl Road, Suite N4-13, Cleveland, Ohio, 44109-1998, UNITED STATES.
Ipsilateral motor evoked potentials (iMEPs) are believed to represent cortically evoked excitability of uncrossed brainstem-mediated pathways. In the event of extensive injury to (crossed) corticospinal pathways, which can occur following a stroke, uncrossed ipsilateral pathways may serve as an alternate resource to support the recovery of the paretic limb. However, iMEPs, even in neurally intact people, can be small, infrequent, and noisy, so discerning them in stroke survivors is very challenging.
View Article and Find Full Text PDFDev Med Child Neurol
January 2025
Child Health Research Centre, Faculty of Medicine, The University of Queensland, Brisbane, Queensland, Australia.
Aim: To examine the diagnostic accuracy of Early structural and diffusion-weighted magnetic resonance imaging (MRI) (acquired at < 36 weeks postmenstrual age) to detect cerebral palsy (CP) or other adverse motor outcomes at or beyond 3 years corrected age in infants born preterm.
Method: In this systematic review and meta-analysis, we searched the CINAHL, Embase, PubMed, and Web of Science databases for studies without language restrictions and a prospectively registered protocol up to October 2023. We extracted the study details, associations presented, and meta-analyses conducted with pooled sensitivity and specificity.
Eur J Neurol
January 2025
Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milan, Italy.
Background: This study aimed to determine whether educational attainment-a common proxy of cognitive reserve (CR)-influences the association between motor and cognitive/behavioural outcomes in a large cohort of ALS patients without dementia.
Methods: N = 726 ALS patients without FTD were assessed for motor (ALSFRS-R), cognitive (Edinburgh Cognitive and Behavioural ALS Screen, ECAS) and behavioural outcomes (ECAS-Carer Interview, ECAS-CI). CR was operationalized via educational attainment (in years).
bioRxiv
December 2024
Department of Neurosciences, School of Medicine, Case Western Reserve University, Cleveland, OH 44106, United States.
Pathogenic coding mutations are prevalent in human neuronal transcription factors (TFs) but how they disrupt development is poorly understood. Lmx1b is a master transcriptional regulator of postmitotic neurons that give rise to mature serotonin (5-HT) neurons; over two hundred pathogenic heterozygous mutations have been discovered in human yet their impact on brain development has not been investigated. Here, we developed mouse models with different DNA-binding missense mutations.
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