Regional Motor Cortex Dysfunction in Amyotrophic Lateral Sclerosis

Overview

Journal Ann Clin Transl Neurol

Specialty Neurology

Date 2019 Aug 13

PMID 31402622

Citations 13

Authors

Parvathi Menon

Con Yiannikas

Matthew C Kiernan

Steve Vucic

Affiliations

Soon will be listed here.

Abstract

Objective: The pathophysiological processes underlying amyotrophic lateral sclerosis (ALS) need to be better understood, although cortical dysfunction has been implicated. Previous transcranial magnetic stimulation (TMS) studies have assessed cortical dysfunction from the hand. The aim of the present study was to determine whether cortical dysfunction was evident across representations of three body regions, and to relate these changes to clinical features of ALS.

Methods: In this cross-sectional study, threshold tracking TMS was undertaken in 60 sporadic ALS patients, with motor evoked potential (MEP) responses recorded over the hand (abductor pollicis brevis), lower limb (tibialis anterior), and bulbar (trapezius) regions. The cross-sectional findings were compared to 28 age- and gender-matched controls.

Results: Cortical dysfunction was evident across the representation of the three body regions, although the degree and nature of the dysfunction varied. Cortical hyperexcitability, as heralded by reduced short interval intracortical inhibition (SICI), was evident in all cortical regions (hand, P < 0.01; leg, P < 0.05; bulbar, P < 0.05) in ALS patients when compared with healthy control subjects. Importantly, features of cortical hyperexcitability seemed more prominent in clinically affected body regions and correlated with functional disability and muscle weakness. Cortical inexcitability was more prominent in the leg (P < 0.001) and bulbar regions (P < 0.01) when compared with controls.

Interpretation: The nature of cortical dysfunction varied across the body regions in ALS, with cortical hyperexcitability being more prominent in the upper limbs while cortical inexcitability was more evident in the lower limbs and bulbar regions. The findings suggest a heterogeneity of cortical pathophysiological processes in ALS.

Citing Articles

Amyotrophic lateral sclerosis established as a multistep process across phenotypes.

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Physiological Biomarkers of Upper Motor Neuron Dysfunction in ALS.

Calma A, van den Bos M, Pavey N, Santos Silva C, Menon P, Vucic S Brain Sci. 2024; 14(8).

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Abnormalities of cortical stimulation strength-duration time constant in amyotrophic lateral sclerosis.

Pavey N, Menon P, Peterchev A, Kiernan M, Vucic S Clin Neurophysiol. 2024; 164:161-167.

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Can Repetitive Transcranial Magnetic Stimulation (rTMS) Promote Neurogenesis and Axonogenesis in Subacute Human Ischemic Stroke?.

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Reduction in short interval intracortical inhibition from the early stage reflects the pathophysiology in amyotrophic lateral sclerosis: A meta-analysis study.

Higashihara M, Pavey N, Menon P, van den Bos M, Shibuya K, Kuwabara S Eur J Neurol. 2024; 31(7):e16281.

PMID: 38504632 PMC: 11235657. DOI: 10.1111/ene.16281.

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