Cortical hyperexcitability: Diagnostic and pathogenic biomarker of ALS

Steve Vucic; Nathan Pavey; Mouna Haidar; Bradley J Turner; Matthew C Kiernan

doi:10.1016/j.neulet.2021.136039

Cortical hyperexcitability: Diagnostic and pathogenic biomarker of ALS

Neurosci Lett. 2021 Aug 10:759:136039. doi: 10.1016/j.neulet.2021.136039. Epub 2021 Jun 10.

Authors

Steve Vucic¹, Nathan Pavey², Mouna Haidar³, Bradley J Turner³, Matthew C Kiernan⁴

Affiliations

¹ Western Clinical School, University of Sydney, Sydney, Australia. Electronic address: steve.vucic@sydney.edu.au.
² Western Clinical School, University of Sydney, Sydney, Australia.
³ Florey Institute of Neuroscieace and Mental Health, University of Melbourne, Parkville, Victoria, Australia.
⁴ Brain and Mind Centre, University of Sydney and Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, Sydney, Australia.

PMID: 34118310
DOI: 10.1016/j.neulet.2021.136039

Abstract

Cortical hyperexcitability is an early and intrinsic feature of both sporadic and familial forms of amyotrophic lateral sclerosis (ALS).. Importantly, cortical hyperexcitability appears to be associated with motor neuron degeneration, possibly via an anterograde glutamate-mediated excitotoxic process, thereby forming a pathogenic basis for ALS. The presence of cortical hyperexcitability in ALS patients may be readily determined by transcranial magnetic stimulation (TMS), a neurophysiological tool that provides a non-invasive and painless method for assessing cortical function. Utilising the threshold tracking TMS technique, cortical hyperexcitability has been established as a robust diagnostic biomarker that distinguished ALS from mimicking disorders at early stages of the disease process. The present review discusses the pathophysiological and diagnostic utility of cortical hyperexcitability in ALS.

Keywords: Amyotrophic lateral sclerosis; Cortical hyperexcitability; Threshold tracking TMS.

Publication types

Research Support, Non-U.S. Gov't
Review

MeSH terms

Amyotrophic Lateral Sclerosis / diagnosis*
Amyotrophic Lateral Sclerosis / physiopathology*
Cerebral Cortex / physiopathology*
Humans
Transcranial Magnetic Stimulation / methods*