Version 1
: Received: 31 October 2024 / Approved: 31 October 2024 / Online: 1 November 2024 (18:28:25 CET)
How to cite:
Donaghy, R.; Pioro, E. P. Neurophysiologic Innovations in ALS: Enhancing Diagnosis, Monitoring, and Treatment Evaluation. Preprints2024, 2024110009. https://doi.org/10.20944/preprints202411.0009.v1
Donaghy, R.; Pioro, E. P. Neurophysiologic Innovations in ALS: Enhancing Diagnosis, Monitoring, and Treatment Evaluation. Preprints 2024, 2024110009. https://doi.org/10.20944/preprints202411.0009.v1
Donaghy, R.; Pioro, E. P. Neurophysiologic Innovations in ALS: Enhancing Diagnosis, Monitoring, and Treatment Evaluation. Preprints2024, 2024110009. https://doi.org/10.20944/preprints202411.0009.v1
APA Style
Donaghy, R., & Pioro, E. P. (2024). Neurophysiologic Innovations in ALS: Enhancing Diagnosis, Monitoring, and Treatment Evaluation. Preprints. https://doi.org/10.20944/preprints202411.0009.v1
Chicago/Turabian Style
Donaghy, R. and Erik P Pioro. 2024 "Neurophysiologic Innovations in ALS: Enhancing Diagnosis, Monitoring, and Treatment Evaluation" Preprints. https://doi.org/10.20944/preprints202411.0009.v1
Abstract
Amyotrophic lateral sclerosis (ALS) is a progressive disease of both upper motor neurons (UMNs) and lower motor neurons (LMNs) leading invariably to a decline in motor function. The clinical exam is foundational to the diagnosis of the disease and ordinal severity scales are used to track its progression. However, the lack of objective biomarkers of disease classification and progression delay clinical trial enrollment and muddle inclusion criteria. Ultimately, biomarker evidence of therapeutic target engagement will support and perhaps supplant more traditional clinical trial outcome measures. Electrophysiology tools of nerve conduction study and electromyography have already been established as diagnostic biomarkers of LMN degeneration in ALS, with the more recent introduction of motor unit number estimation (including MScanFit), motor unit number index, and electrical impedance myography. Dysfunction of UMN and non-motor brain areas is being increasingly assessed with transcranial magnetic stimulation, high-density electroencephalography, and magnetoencephalography. Although most of these techniques are used to explore the underlying disease mechanisms of ALS in research settings primarily, they have the potential on a broader scale to noninvasively identify disease subtypes, predict progression rates, and assess physiologic engagement of experimental therapies.
Keywords
Amyotrophic lateral sclerosis (ALS); Motor unit number estimation (MUNE); Transcranial magnetic stimulation (TMS); Biomarker; Clinical trial
Subject
Medicine and Pharmacology, Neuroscience and Neurology
Copyright:
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
