Cortical hyperexcitability: Diagnostic and pathogenic biomarker of ALS

Interneuronal networks mediate cortical inhibition and facilitation

(2020) Mana Higashihara et al.   CLINICAL NEUROPHYSIOLOGY

Advanced neuroimaging approaches in amyotrophic lateral sclerosis: refining the clinical diagnosis

(2020) Jan Kassubek et al.   Expert Review of Neurotherapeutics

ALS is a multistep process in South Korean, Japanese, and Australian patients

(2020) Steve Vucic et al.   NEUROLOGY

Motor Neuron Generation from iPSCs from Identical Twins Discordant for Amyotrophic Lateral Sclerosis

(2020) Emily R. Seminary et al.   Cells

A proposal for new diagnostic criteria for ALS

(2020) Jeremy M. Shefner et al.   CLINICAL NEUROPHYSIOLOGY

Going the Extra (Synaptic) Mile: Excitotoxicity as the Road Toward Neurodegenerative Diseases

(2020) Adam Armada-Moreira et al.   Frontiers in Cellular Neuroscience

Hexanucleotide Repeat Expansions in c9FTD/ALS and SCA36 Confer Selective Patterns of Neurodegeneration In Vivo

(2020) Tiffany W. Todd et al.   Cell Reports

ALS skin fibroblasts reveal oxidative stress and ERK1/2-mediated cytoplasmic localization of TDP-43

(2020) Nicla Romano et al.   CELLULAR SIGNALLING

The Italian multicenter experience with edaravone in amyotrophic lateral sclerosis

(2020) Christian Lunetta et al.   JOURNAL OF NEUROLOGY

A novel hypothesis on metal dyshomeostasis and mitochondrial dysfunction in amyotrophic lateral sclerosis: Potential pathogenetic mechanism and therapeutic implications

(2020) Yutaka Nakagawa et al.   EUROPEAN JOURNAL OF PHARMACOLOGY

Amyotrophic lateral sclerosis diagnostic index

(2019) Nimeshan Geevasinga et al.   NEUROLOGY

Pathophysiology and Diagnosis of ALS: Insights from Advances in Neurophysiological Techniques

(2019) Mehdi A. J. van den Bos et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Amyotrophic lateral sclerosis as a multi-step process: an Australia population study

(2019) Steve Vucic et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Absence of hyperexcitability of spinal motoneurons in patients with ALS

(2019) Véronique Marchand‐Pauvert et al.   JOURNAL OF PHYSIOLOGY-LONDON

Hyperexcitability in Cultured Cortical Neuron Networks from the G93A-SOD1 Amyotrophic Lateral Sclerosis Model Mouse and its Molecular Correlates

(2019) Stefania Marcuzzo et al.   NEUROSCIENCE

Chronic nigral neuromodulation aggravates behavioral deficits and synaptic changes in an α-synuclein based rat model for Parkinson’s disease

(2019) Teresa Torre-Muruzabal et al.   Acta Neuropathologica Communications

Early focality and spread of cortical dysfunction in amyotrophic lateral sclerosis: A regional study across the motor cortices

(2019) Thanuja Dharmadasa et al.   CLINICAL NEUROPHYSIOLOGY

Short-interval intracortical inhibition: Comparison between conventional and threshold-tracking techniques

(2018) Gintaute Samusyte et al.   Brain Stimulation

The multistep hypothesis of ALS revisited

(2018) Adriano Chiò et al.   NEUROLOGY

Imbalance of cortical facilitatory and inhibitory circuits underlies hyperexcitability in ALS

(2018) Mehdi A.J. Van den Bos et al.   NEUROLOGY

Cortical hyperexcitability and disease spread in amyotrophic lateral sclerosis

(2017) P. Menon et al.   EUROPEAN JOURNAL OF NEUROLOGY

Brain functional connectome abnormalities in amyotrophic lateral sclerosis are associated with disability and cortical hyperexcitability

(2017) N. Geevasinga et al.   EUROPEAN JOURNAL OF NEUROLOGY

Cortical influences drive amyotrophic lateral sclerosis

(2017) Andrew Eisen et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial

(2017) Koji Abe et al.   LANCET NEUROLOGY

Current Advances and Limitations in Modeling ALS/FTD in a Dish Using Induced Pluripotent Stem Cells

(2017) Wenting Guo et al.   Frontiers in Neuroscience

Virtual brain biopsies in amyotrophic lateral sclerosis: Diagnostic classification based on in vivo pathological patterns

(2017) Peter Bede et al.   NeuroImage-Clinical

Awaji criteria improves the diagnostic sensitivity in amyotrophic lateral sclerosis: A systematic review using individual patient data

(2016) Nimeshan Geevasinga et al.   CLINICAL NEUROPHYSIOLOGY

The frontotemporal dementia-motor neuron disease continuum

(2016) James R Burrell et al.   LANCET

Hyperactive somatostatin interneurons contribute to excitotoxicity in neurodegenerative disorders

(2016) Wen Zhang et al.   NATURE NEUROSCIENCE

Pathophysiological and diagnostic implications of cortical dysfunction in ALS

(2016) Nimeshan Geevasinga et al.   Nature Reviews Neurology

Motor cortical function determines prognosis in sporadic ALS

(2016) Kazumoto Shibuya et al.   NEUROLOGY

A randomized trial of mexiletine in ALS

(2016) Michael D. Weiss et al.   NEUROLOGY

4-Aminopyridine Induced Activity Rescues Hypoexcitable Motor Neurons from Amyotrophic Lateral Sclerosis Patient-Derived Induced Pluripotent Stem Cells

(2016) Maximilian Naujock et al.   STEM CELLS

Cortical contributions to the flail leg syndrome: Pathophysiological insights

(2016) Parvathi Menon et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

DREADDs (Designer Receptors Exclusively Activated by Designer Drugs): Chemogenetic Tools with Therapeutic Utility

(2015) Daniel J. Urban et al.   Annual Review of Pharmacology and Toxicology

Altered Functionality, Morphology, and Vesicular Glutamate Transporter Expression of Cortical Motor Neurons from a Presymptomatic Mouse Model of Amyotrophic Lateral Sclerosis

(2015) L. Saba et al.   CEREBRAL CORTEX

Cortical hyperexcitability precedes lower motor neuron dysfunction in ALS

(2015) Parvathi Menon et al.   CLINICAL NEUROPHYSIOLOGY

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

(2015) P.M. Rossini et al.   CLINICAL NEUROPHYSIOLOGY

What Does Imaging Reveal About the Pathology of Amyotrophic Lateral Sclerosis?

(2015) Martin R. Turner et al.   Current Neurology and Neuroscience Reports

Sensitivity and specificity of threshold tracking transcranial magnetic stimulation for diagnosis of amyotrophic lateral sclerosis: a prospective study

(2015) Parvathi Menon et al.   LANCET NEUROLOGY

Amyotrophic lateral sclerosis: Current perspectives from basic research to the clinic

(2015) Renzo Mancuso et al.   PROGRESS IN NEUROBIOLOGY

Human endogenous retrovirus-K contributes to motor neuron disease

(2015) Wenxue Li et al.   Science Translational Medicine

Human iPSC-derived motoneurons harbouring TARDBP or C9ORF72 ALS mutations are dysfunctional despite maintaining viability

(2015) Anna-Claire Devlin et al.   Nature Communications

Cortical Function in Asymptomatic Carriers and Patients WithC9orf72Amyotrophic Lateral Sclerosis

(2015) Nimeshan Geevasinga et al.   JAMA Neurology

Axonal Ion Channel Dysfunction inC9orf72Familial Amyotrophic Lateral Sclerosis

(2015) Nimeshan Geevasinga et al.   JAMA Neurology

Flecainide in Amyotrophic Lateral Sclerosis as a Neuroprotective Strategy (FANS): A Randomized Placebo-Controlled Trial

(2015) Susanna B. Park et al.   EBioMedicine

A Model of TMS-induced I-waves in Motor Cortex

(2014) Cătălin V. Rusu et al.   Brain Stimulation

Analysis of amyotrophic lateral sclerosis as a multistep process: a population-based modelling study

(2014) Ammar Al-Chalabi et al.   LANCET NEUROLOGY

Modeling pain in vitro using nociceptor neurons reprogrammed from fibroblasts

(2014) Brian J Wainger et al.   NATURE NEUROSCIENCE

Cortical Dysfunction Underlies the Development of the Split-Hand in Amyotrophic Lateral Sclerosis

(2014) Parvathi Menon et al.   PLoS One

Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies

(2014) Steve Vucic et al.   TRENDS IN NEUROSCIENCES

Intrinsic Membrane Hyperexcitability of Amyotrophic Lateral Sclerosis Patient-Derived Motor Neurons

(2014) Brian J. Wainger et al.   Cell Reports

Diagnostic timelines and delays in diagnosing amyotrophic lateral sclerosis (ALS)

(2014) Sabrina Paganoni et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Study of motor asymmetry in ALS indicates an effect of limb dominance on onset and spread of weakness, and an important role for upper motor neurons

(2014) Matthew S. Devine et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Early intrinsic hyperexcitability does not contribute to motoneuron degeneration in amyotrophic lateral sclerosis

(2014) Félix Leroy et al.   eLife

Riluzole exerts central and peripheral modulating effects in amyotrophic lateral sclerosis

(2013) Steve Vucic et al.   BRAIN

ALS pathophysiology: Insights from the split-hand phenomenon

(2013) Parvathi Menon et al.   CLINICAL NEUROPHYSIOLOGY

Altered astrocytic expression of TDP-43 does not influence motor neuron survival

(2013) Amanda M. Haidet-Phillips et al.   EXPERIMENTAL NEUROLOGY

Pathophysiological insights into ALS with C9ORF72 expansions

(2013) K. L. Williams et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Split hand syndrome in amyotrophic lateral sclerosis: different excitability changes in the thenar and hypothenar motor axons

(2013) K. Shibuya et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Neuroprotection through Excitability and mTOR Required in ALS Motoneurons to Delay Disease and Extend Survival

(2013) Smita Saxena et al.   NEURON

Awaji Criteria for the Diagnosis of Amyotrophic Lateral Sclerosis

(2012) João Costa et al.   ARCHIVES OF NEUROLOGY

Role of the N-methyl-d-aspartate receptors complex in amyotrophic lateral sclerosis

(2012) Alida Spalloni et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Neuroimaging in amyotrophic lateral sclerosis

(2012) Martin R Turner et al.   Biomarkers in Medicine

Transcranial Direct Current Stimulation Effects on the Excitability of Corticospinal Axons of the Human Cerebral Cortex

(2012) Vincenzo Di Lazzaro et al.   Brain Stimulation

Excitotoxicity: Bridge to various triggers in neurodegenerative disorders

(2012) Ankita Mehta et al.   EUROPEAN JOURNAL OF PHARMACOLOGY

Why are upper motor neuron signs difficult to elicit in amyotrophic lateral sclerosis?: Figure 1

(2012) Michael Swash   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Transcranial magnetic stimulation and amyotrophic lateral sclerosis: pathophysiological insights

(2012) Steve Vucic et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study

(2012) Elisa Majounie et al.   LANCET NEUROLOGY

AAV2 mediated retrograde transduction of corticospinal motor neurons reveals initial and selective apical dendrite degeneration in ALS

(2012) Javier H. Jara et al.   NEUROBIOLOGY OF DISEASE

Split-hand plus sign in ALS: Differential involvement of the flexor pollicis longus and intrinsic hand muscles

(2012) Parvathi Menon et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS

(2011) Safa Al-Sarraj et al.   ACTA NEUROPATHOLOGICA

Cortical excitability distinguishes ALS from mimic disorders

(2011) Steve Vucic et al.   CLINICAL NEUROPHYSIOLOGY

Links between Electrophysiological and Molecular Pathology of Amyotrophic Lateral Sclerosis

(2011) K. A. Quinlan   INTEGRATIVE AND COMPARATIVE BIOLOGY

Amyotrophic lateral sclerosis

(2011) Matthew C Kiernan et al.   LANCET

Fasciculation potentials in amyotrophic lateral sclerosis and the diagnostic yield of the Awaji algorithm

(2011) Mana Higashihara et al.   MUSCLE & NERVE

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

Concordance between site of onset and limb dominance in amyotrophic lateral sclerosis

(2010) M. R. Turner et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import

(2010) Q. Li et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Deficits in axonal transport precede ALS symptoms in vivo

(2010) L. G. Bilsland et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cortical excitability in hereditary motor neuronopathy with pyramidal signs: comparison with ALS

(2009) S Vucic et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Upregulation of persistent sodium conductances in familial ALS

(2009) S. Vucic et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Progressive Changes in Synaptic Inputs to Motoneurons in Adult Sacral Spinal Cord of a Mouse Model of Amyotrophic Lateral Sclerosis

(2009) M. Jiang et al.   JOURNAL OF NEUROSCIENCE

ALS motor phenotype heterogeneity, focality, and spread: Deconstructing motor neuron degeneration

(2009) J. M. Ravits et al.   NEUROLOGY

Miro1 Is a Calcium Sensor for Glutamate Receptor-Dependent Localization of Mitochondria at Synapses

(2009) Andrew F. MacAskill et al.   NEURON

Descending Pathways in Motor Control

(2008) Roger N. Lemon   Annual Review of Neuroscience

Cortical hyperexcitability may precede the onset of familial amyotrophic lateral sclerosis

(2008) Steve Vucic et al.   BRAIN

Cortical excitability testing distinguishes Kennedy’s disease from amyotrophic lateral sclerosis

(2008) Steve Vucic et al.   CLINICAL NEUROPHYSIOLOGY

Electrodiagnostic criteria for diagnosis of ALS

(2008) Mamede de Carvalho et al.   CLINICAL NEUROPHYSIOLOGY

Increased persistent sodium current determines cortical hyperexcitability in a genetic model of amyotrophic lateral sclerosis

(2008) Massimo Pieri et al.   EXPERIMENTAL NEUROLOGY

Morphological differences between wild-type and transgenic superoxide dismutase 1 lumbar motoneurons in postnatal mice

(2008) Julien Amendola et al.   JOURNAL OF COMPARATIVE NEUROLOGY

Biomarkers in amyotrophic lateral sclerosis

(2008) Martin R Turner et al.   LANCET NEUROLOGY