Physiological Biomarkers of Upper Motor Neuron Dysfunction in ALS DOI Creative Commons
Aicee Dawn Calma, Mehdi van den Bos, Nathan Pavey

et al.

Brain Sciences, Journal Year: 2024, Volume and Issue: 14(8), P. 760 - 760

Published: July 29, 2024

Upper motor neuron (UMN) dysfunction is an important feature of amyotrophic lateral sclerosis (ALS) for the diagnosis and understanding pathogenesis. The identification UMN signs forms basis ALS diagnosis, although may be difficult to discern, especially in setting severe muscle weakness. Transcranial magnetic stimulation (TMS) techniques have yielded objective physiological biomarkers ALS, enabling interrogation cortical subcortical neuronal networks with diagnostic, pathophysiological, prognostic implications. provided pertinent pathogenic insights novel diagnostic biomarkers. Cortical hyperexcitability, as heralded by a reduction short interval intracortical inhibition (SICI) increase facilitation (SICF), has been associated lower degeneration, patterns disease evolution, well development specific clinical features including split hand phenomenon. Reduction SICI also emerged potential aid ALS. More recently, distinct inhibitory facilitatory interneuronal circuits identified, which shown contribute triple technique (TST) was enhance utility conventional TMS measures detecting dysfunction. Resting-state EEG neurophysiological developed directly interrogating that potentially useful present review discusses encompassing interrogate functional integrity corticomotoneuronal system, focusing on pathogenic, utility.

Language: Английский

Transcranial static magnetic stimulation for amyotrophic lateral sclerosis: a bicentric, randomised, double-blind placebo-controlled phase 2 trial DOI Creative Commons
Vincenzo Di Lazzaro, Federico Ranieri, Alberto Doretti

et al.

The Lancet Regional Health - Europe, Journal Year: 2024, Volume and Issue: 45, P. 101019 - 101019

Published: July 30, 2024

Enhanced glutamatergic transmission leading to motor neuron death is considered the major pathophysiological mechanism of amyotrophic lateral sclerosis (ALS). Motor cortex excitability can be suppressed by transcranial static magnetic stimulation (tSMS), thus tSMS evaluated as a potential treatment for ALS. The aim present study was investigate efficacy and safety in

Language: Английский

Citations

6
Schwann Cells in Neuromuscular Disorders: A Spotlight on Amyotrophic Lateral Sclerosis DOI Creative Commons
Kathryn R. Moss, Smita Saxena

Cells, Journal Year: 2025, Volume and Issue: 14(1), P. 47 - 47

Published: Jan. 3, 2025

Amyotrophic Lateral Sclerosis (ALS) is a complex neurodegenerative disease primarily affecting motor neurons, leading to progressive muscle atrophy and paralysis. This review explores the role of Schwann cells in ALS pathogenesis, highlighting their influence on progression through mechanisms involving demyelination, neuroinflammation, impaired synaptic function. While have been traditionally viewed as peripheral supportive cells, especially neuron disease, recent evidence indicates that they play significant by impacting survival plasticity, influencing inflammatory responses, altering myelination processes. Furthermore, advancements understanding cell pathology combined with lessons learned from studying Charcot–Marie–Tooth Type 1 (CMT1) suggest potential therapeutic strategies targeting these may support nerve repair slow progression. Overall, this aims provide comprehensive insights into classification, physiology, function, underscoring critical pathological contributions suggests new avenues for targeted interventions aimed at modulating function ALS.

Language: Английский

Citations

0
High-Fat Diet—Shared Environmental Risk Factor for Amyotrophic Lateral Sclerosis and Multiple Sclerosis DOI Open Access
Thomas Gabriel Schreiner,

L Iacob,

Cristina Croitoru

et al.

Sclerosis, Journal Year: 2025, Volume and Issue: 3(1), P. 1 - 1

Published: Jan. 12, 2025

Background: Amyotrophic lateral sclerosis (ALS) and multiple (MS) are, in essence, neurodegenerative disorders with significant individual, social, economic burdens worldwide. Despite having different clinical onset evolution, the two diseases share common risk factors underlying pathophysiological mechanisms. Environmental are particularly interesting, considering available effective counter strategies. High-fat diets remain a element that negatively impacts evolution of several disorders, including ALS MS. Focusing on changeable disease-related aspects is increasingly appealing context lack an treatment. Methods: This review aims to offer updated overview influence high-fat modulating progression MS, based search three relevant online databases. Results: In first part, shared mechanisms MS shown, differences between highlighted. Subsequently, most research this topic conducted animal models humans presented, bringing additional proof critical role neurodegeneration. Finally, current knowledge, authors potential therapeutic approaches future directions better control nutrition patients, hoping increase survival quality life. Conclusions: impact

Language: Английский

Citations

0
Microstructural Changes in the Corpus Callosum in Neurodegenerative Diseases DOI Open Access
Emad Ali Albadawi

Cureus, Journal Year: 2024, Volume and Issue: unknown

Published: Aug. 21, 2024

The corpus callosum, the largest white matter structure in brain, plays a crucial role interhemispheric communication and cognitive function. This review examines microstructural changes observed callosum across various neurodegenerative diseases, including Alzheimer's disease, Parkinson's Huntington's amyotrophic lateral sclerosis (ALS). New neuroimaging studies, mainly those that use diffusion tensor imaging (DTI) advanced tractography methods, were put together to show how have happened organization of connections between them. Some most common ways breaks down are discussed, less fractional anisotropy, higher mean diffusivity, atrophy certain regions. relationship these decline, motor dysfunction, disease progression is explored. Additionally, we consider potential as biomarker for early detection monitoring. Studies people with disorders lower anisotropy diffusivity often specific disease. These happen before gray linked symptoms, which suggests could be used an sign neurodegeneration. also highlights implications findings understanding mechanisms developing therapeutic strategies. Future directions, application techniques longitudinal discussed elucidate degeneration processes. underscores importance pathophysiology diseases its target interventions.

Language: Английский

Citations

1
Aberrant evoked calcium signaling and nAChR cluster morphology in a SOD1 D90A hiPSC-derived neuromuscular model DOI Creative Commons

Nathalie Couturier,

Sarah Janice Hörner, Elina Nürnberg

et al.

Frontiers in Cell and Developmental Biology, Journal Year: 2024, Volume and Issue: 12

Published: June 20, 2024

Familial amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disorder that due to mutations in one of several target genes, including SOD1 . So far, clinical records, rodent studies, and vitro models have yielded arguments for either primary motor neuron disease, or pleiotropic pathogenesis ALS. While mouse lack the human origin, using induced pluripotent stem cells (hiPSC) been recently developed addressing ALS pathogenesis. In spite improvements regarding generation muscle from hiPSC, degree maturation resulting these protocols has remained limited. To fill shortcomings, we here present new protocol an enhanced myotube differentiation hiPSC with option further upon coculture hiPSC-derived neurons. The described model first yield combination key myogenic features are consistent sarcomeric organization association complex nAChR clusters myotubes derived control hiPSC. this model, carrying D90A mutation had reduced expression markers, sarcomeres, morphologically different clusters, altered nAChR-dependent Ca 2+ response compared myotubes. Notably, trophic support provided by neurons cluster differences between summary, novel yields evidence both muscle-intrinsic nerve-dependent aspects dysfunction -based

Language: Английский

Citations

0
Adipose mesenchymal stem cells-derived extracellular vesicles exert their preferential action in damaged central sites of SOD1 mice rather than peripherally DOI Creative Commons
Ermanna Turano, Federica Virla, Ilaria Scambi

et al.

European Journal of Histochemistry, Journal Year: 2024, Volume and Issue: 68(3)

Published: July 4, 2024

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder involving motor neuron (MN) loss in the cortex, brainstem and spinal cord leading to progressive paralysis death. Due pathogenetic complexity, there are no effective therapies available. In this context use of mesenchymal stem cells their vesicular counterpart an emerging therapeutic strategy counteract neurodegeneration. The extracellular vesicles derived from adipose (ASC-EVs) recapitulate ameliorate neuroprotective effect and, thanks small dimensions, makes suitable develop novel approaches for diseases as ALS. Here we investigate regimen ASC-EVs injection SOD1(G93A) mice, most widely used murine model Repeated intranasal administrations high doses were able performance injected mice at early stage disease produce significant improvement end-stage lumbar MNs rescue. Moreover, preserve structure neuromuscular junction without counteracting muscle atrophy. results indicate that administration acts central nervous system sites rather than peripheral level mice. These considerations allow us identify future applications involve different targets simultaneously maximize clinical neuropathological outcomes ALS vivo models.

Language: Английский

Citations

0
Physiological Biomarkers of Upper Motor Neuron Dysfunction in ALS DOI Creative Commons
Aicee Dawn Calma, Mehdi van den Bos, Nathan Pavey

et al.

Brain Sciences, Journal Year: 2024, Volume and Issue: 14(8), P. 760 - 760

Published: July 29, 2024

Upper motor neuron (UMN) dysfunction is an important feature of amyotrophic lateral sclerosis (ALS) for the diagnosis and understanding pathogenesis. The identification UMN signs forms basis ALS diagnosis, although may be difficult to discern, especially in setting severe muscle weakness. Transcranial magnetic stimulation (TMS) techniques have yielded objective physiological biomarkers ALS, enabling interrogation cortical subcortical neuronal networks with diagnostic, pathophysiological, prognostic implications. provided pertinent pathogenic insights novel diagnostic biomarkers. Cortical hyperexcitability, as heralded by a reduction short interval intracortical inhibition (SICI) increase facilitation (SICF), has been associated lower degeneration, patterns disease evolution, well development specific clinical features including split hand phenomenon. Reduction SICI also emerged potential aid ALS. More recently, distinct inhibitory facilitatory interneuronal circuits identified, which shown contribute triple technique (TST) was enhance utility conventional TMS measures detecting dysfunction. Resting-state EEG neurophysiological developed directly interrogating that potentially useful present review discusses encompassing interrogate functional integrity corticomotoneuronal system, focusing on pathogenic, utility.

Language: Английский

Citations

0