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Revisiting distinct nerve excitability patterns in patients with amyotrophic lateral sclerosis.


ABSTRACT: Amyotrophic lateral sclerosis is a devastating neurodegenerative disease, characterized by loss of central and peripheral motor neurons. Although the disease is clinically and genetically heterogeneous, axonal hyperexcitability is a commonly observed feature that has been suggested to reflect an early pathophysiological step linked to the neurodegenerative cascade. Therefore, it is important to clarify the mechanisms causing axonal hyperexcitability and how these relate to the clinical characteristics of patients. Measures derived directly from a nerve excitability recording are frequently used as study end points, although their biophysical basis is difficult to deduce. Mathematical models can aid in the interpretation but are reliable only when applied to group-averaged recordings. Conse

SUBMITTER: Stikvoort Garcia DJL 

PROVIDER: S-EPMC11491535 | biostudies-literature | 2024 Aug

REPOSITORIES: biostudies-literature