Transcranial magnetic stimulation and amyotrophic lateral sclerosis: pathophysiological insights
TLDR
A central origin of ALS is supported by TMS studies, with an anterograde transsynaptic mechanism implicated in ALS pathogenesis and a potential diagnostic utility of TMS techniques in ALS is suggested.Abstract:
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder of the motor neurons in the motor cortex, brainstem and spinal cord. A combination of upper and lower motor neuron dysfunction comprises the clinical ALS phenotype. Although the ALS phenotype was first observed by Charcot over 100 years ago, the site of ALS onset and the pathophysiological mechanisms underlying the development of motor neuron degeneration remain to be elucidated. Transcranial magnetic stimulation (TMS) enables non-invasive assessment of the functional integrity of the motor cortex and its corticomotoneuronal projections. To date, TMS studies have established motor cortical and corticospinal dysfunction in ALS, with cortical hyperexcitability being an early feature in sporadic forms of ALS and preceding the clinical onset of familial ALS. Taken together, a central origin of ALS is supported by TMS studies, with an anterograde transsynaptic mechanism implicated in ALS pathogenesis. Of further relevance, TMS techniques reliably distinguish ALS from mimic disorders, despite a compatible peripheral disease burden, thereby suggesting a potential diagnostic utility of TMS in ALS. This review will focus on the mechanisms underlying the generation of TMS measures used in assessment of cortical excitability, the contribution of TMS in enhancing the understanding of ALS pathophysiology and the potential diagnostic utility of TMS techniques in ALS.read more
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Non-invasive electrical and magnetic stimulation of the brain, spinal cord, roots and peripheral nerves: Basic principles and procedures for routine clinical and research application: An updated report from an I.F.C.N. Committee
Paolo Maria Rossini,David Burke,Robert Chen,L.G. Cohen,Zafiris J. Daskalakis,R. Di Iorio,V. Di Lazzaro,Florinda Ferreri,Florinda Ferreri,Paul B. Fitzgerald,Mark S. George,Mark Hallett,Jean-Pascal Lefaucheur,Berthold Langguth,Hideyuki Matsumoto,Carlo Miniussi,Michael A. Nitsche,Alvaro Pascual-Leone,Walter Paulus,Simone Rossi,John C. Rothwell,Hartwig R. Siebner,Yoshikazu Ugawa,Vincent Walsh,Ulf Ziemann +24 more
TL;DR: These guidelines provide an up-date of previous IFCN report on “Non-invasive electrical and magnetic stimulation of the brain, spinal cord and roots: basic principles and procedures for routine clinical application” and include some recent extensions and developments.
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The connectomics of brain disorders
TL;DR: This work considers how brain-network topology shapes neural responses to damage, highlighting key maladaptive processes and the resources and processes that enable adaptation, and shows how knowledge of network topology allows for predictive models of the spread and functional consequences of brain disease.
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Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies.
TL;DR: Dysfunction of molecular pathways, including glutamate-mediated excitotoxicity, has been identified in sporadic and familial ALS, indicating the existence of a common pathogenic pathway and novel therapeutic approaches have been suggested, providing hope for feasible treatment of ALS.
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Pathophysiological and diagnostic implications of cortical dysfunction in ALS
TL;DR: Changes in cortical function that develop in ALS could prove useful as diagnostic biomarkers, potentially enhancing the diagnosis of ALS at an early stage of the disease process and providing potential for more-effective management of patients with ALS.
Journal ArticleDOI
Sensitivity and specificity of threshold tracking transcranial magnetic stimulation for diagnosis of amyotrophic lateral sclerosis: a prospective study
Parvathi Menon,Nimeshan Geevasinga,Con Yiannikas,James Howells,Matthew C. Kiernan,Steve Vucic +5 more
TL;DR: The threshold tracking TMS technique reliably distinguishes ALS from non-ALS disorders and, if these findings are replicated in larger studies, could represent a useful diagnostic investigation when combined with the Awaji criteria to prove upper motor neuron dysfunction at early stages of ALS.
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TL;DR: The chromosome 9p21 amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD) locus contains one of the last major unidentified autosomal-dominant genes underlying these common neurodegenerative diseases, and a large hexanucleotide repeat expansion in the first intron of C9ORF72 is shown.
Journal ArticleDOI
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Journal ArticleDOI
Non-invasive electrical and magnetic stimulation of the brain, spinal cord and roots: basic principles and procedures for routine clinical application. Report of an IFCN committee
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