Neurophysiological and imaging biomarkers of lower motor neuron dysfunction in motor neuron diseases/amyotrophic lateral sclerosis: IFCN handbook chapter

[1]  S. Rutkove,et al.  Combining electromyographic and electrical impedance data sets through machine learning: A study in D2‐mdx and wild‐type mice , 2023, Muscle & nerve.

[2]  L. H. van den Berg,et al.  Diagnostic accuracy of nerve excitability and compound muscle action potential scan derived biomarkers in amyotrophic lateral sclerosis , 2023, European journal of neurology.

[3]  J. M. Matamala,et al.  Strength-duration properties and excitability of motor and sensory axons across different target thresholds. , 2023, Journal of neurophysiology.

[4]  C. Krarup,et al.  Estimating motor unit numbers from a CMAP scan: Repeatability study on three muscles at 15 centres , 2023, Clinical Neurophysiology.

[5]  P. Rossini,et al.  Clinical diagnostic utility of transcranial magnetic stimulation in neurological disorders. Updated report of an IFCN committee , 2023, Clinical Neurophysiology.

[6]  E. Hodson-Tole,et al.  Fasciculation electromechanical latency is prolonged in amyotrophic lateral sclerosis , 2022, Clinical Neurophysiology.

[7]  A. Mirea,et al.  Clinical and Electrophysiological Changes in Pediatric Spinal Muscular Atrophy after 2 Years of Nusinersen Treatment , 2022, Pharmaceutics.

[8]  O. Witte,et al.  Motor unit number index (MUNIX) in the D50 disease progression model reflects disease accumulation independently of disease aggressiveness in ALS , 2022, Scientific Reports.

[9]  R. Iniesta,et al.  A shortened surface electromyography recording is sufficient to facilitate home fasciculation assessment , 2022, Muscle & nerve.

[10]  E. Chroni,et al.  Quantitative muscle ultrasound assessment using automatic thresholding methods in amyotrophic lateral sclerosis , 2022, Clinical Neurophysiology.

[11]  A. Holobar,et al.  Detecting motor unit abnormalities in amyotrophic lateral sclerosis using high-density surface EMG , 2022, Clinical Neurophysiology.

[12]  B. Cengiz,et al.  Feasibility and reliability of MScanFit motor unit number estimation in peroneus longus muscle , 2022, Muscle & nerve.

[13]  S. Rutkove,et al.  Using machine learning algorithms to enhance the diagnostic performance of electrical impedance myography , 2022, Muscle & nerve.

[14]  B. Beland,et al.  Gold Coast criteria expand clinical trial eligibility in amyotrophic lateral sclerosis , 2022, Muscle & nerve.

[15]  P. van Damme,et al.  Characterising ALS disease progression according to El Escorial and Gold Coast criteria , 2022, Journal of Neurology, Neurosurgery, and Psychiatry.

[16]  Y. Izumi,et al.  Early diagnosis of amyotrophic lateral sclerosis based on fasciculations in muscle ultrasonography: A machine learning approach , 2022, Clinical Neurophysiology.

[17]  S. Rutkove,et al.  Design and pilot testing of a 26‐gauge impedance‐electromyography needle in wild‐type and ALS mice , 2022, Muscle & nerve.

[18]  E. Stålberg,et al.  Analysis of the compound muscle action potential scan: Step index (STEPIX) and amplitude index (AMPIX) , 2022, Clinical Neurophysiology.

[19]  H. Tankisi,et al.  Nerve excitability as a biomarker for amyotrophic lateral sclerosis: a systematic review and meta-analysis. , 2022, medRxiv.

[20]  D. Burke.  Neurophysiological testing in the diagnosis of amyotrophic lateral sclerosis , 2022, Neurological Sciences and Neurophysiology.

[21]  Gary L. Pattee,et al.  A multidimensional facial surface EMG analysis for objective assessment of bulbar involvement in amyotrophic lateral sclerosis , 2021, Clinical Neurophysiology.

[22]  C. Krarup,et al.  Assessing inter-rater reproducibility in MScanFit MUNE in a 6-subject, 12-rater “Round Robin” setup , 2021, Neurophysiologie Clinique.

[23]  Y. Guan,et al.  Fasciculation differences between ALS and non-ALS patients: an ultrasound study , 2021, BMC Neurology.

[24]  E. Stålberg,et al.  Reinnervation as measured by the motor unit size index is associated with preservation of muscle strength in amyotrophic lateral sclerosis, but not all muscles reinnervate , 2021, Muscle & nerve.

[25]  H. Tankisi,et al.  Advancing disease monitoring of amyotrophic lateral sclerosis with the compound muscle action potential scan , 2021, Clinical Neurophysiology.

[26]  D. Maintz,et al.  MRI DTI and PDFF as Biomarkers for Lower Motor Neuron Degeneration in ALS , 2021, Frontiers in Neuroscience.

[27]  N. Shahrizaila,et al.  A model incorporating ultrasound to predict the probability of fast disease progression in amyotrophic lateral sclerosis , 2021, Clinical Neurophysiology.

[28]  G. Fink,et al.  Motor unit number estimation in adult patients with spinal muscular atrophy treated with nusinersen , 2021, European journal of neurology.

[29]  T. Jenkins,et al.  Muscle MRI in motor neuron diseases: a systematic review , 2021, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[30]  A. L. Gomes,et al.  Surface electromyography for testing motor dysfunction in amyotrophic lateral sclerosis , 2021, Neurophysiologie Clinique.

[31]  W. Arnold,et al.  Safety, Tolerability, and Effect of Nusinersen in Non-ambulatory Adults With Spinal Muscular Atrophy , 2021, Frontiers in Neurology.

[32]  Hyeuknam Kwon,et al.  Modeling and Reproducibility of Twin Concentric Electrical Impedance Myography , 2021, IEEE Transactions on Biomedical Engineering.

[33]  E. Zanoteli,et al.  Motor unit number index (MUNIX) in children and adults with 5q-spinal muscular atrophy: Variability and clinical correlations , 2021, Neuromuscular Disorders.

[34]  J. Shefner,et al.  Diagnostic Utility of Gold Coast Criteria in Amyotrophic Lateral Sclerosis , 2021, Annals of neurology.

[35]  A. Blamire,et al.  The muscle twitch profile assessed with motor unit magnetic resonance imaging , 2021, NMR in biomedicine.

[36]  H. Reichmann,et al.  One nerve suffices: A clinically guided nerve ultrasound protocol for the differentiation of multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis (ALS) , 2020, Journal of Neurology.

[37]  Stephen A. Goutman,et al.  Effects of mexiletine on hyperexcitability in sporadic amyotrophic lateral sclerosis: Preliminary findings from a small phase II randomized controlled trial , 2020, Muscle & nerve.

[38]  R. Iniesta,et al.  Fasciculation analysis reveals a novel parameter that correlates with predicted survival in amyotrophic lateral sclerosis , 2020, Muscle & nerve.

[39]  Sylvia Baedorf Kassis,et al.  Effect of Ezogabine on Cortical and Spinal Motor Neuron Excitability in Amyotrophic Lateral Sclerosis: A Randomized Clinical Trial. , 2020, JAMA neurology.

[40]  Sevki Sahin,et al.  Use of CMAP, MScan fit-MUNE, and MUNIX in understanding neurodegeneration pattern of ALS and detection of early motor neuron loss in daily practice , 2020, Neuroscience Letters.

[41]  D. Kariyawasam,et al.  Motor unit changes in children with symptomatic spinal muscular atrophy treated with nusinersen , 2020, Journal of Neurology, Neurosurgery, and Psychiatry.

[42]  E. Bertini,et al.  Clinical and radiological profile of patients with spinal muscular atrophy type 4 , 2020, European journal of neurology.

[43]  N. van Alfen,et al.  Muscle ultrasound: Present state and future opportunities , 2020, Muscle & nerve.

[44]  T. Mizuno,et al.  Difference in distribution of fasciculations between multifocal motor neuropathy and amyotrophic lateral sclerosis , 2020, Clinical Neurophysiology.

[45]  S. Rutkove,et al.  Using Electrical Impedance Myography as a Biomarker of Muscle Deconditioning in Rats Exposed to Micro- and Partial-Gravity Analogs , 2020, Frontiers in Physiology.

[46]  C. Liang,et al.  Neuropathy in sporadic inclusion body myositis: A multi-modality neurophysiological study , 2020, Clinical Neurophysiology.

[47]  L. H. van den Berg,et al.  Excitability of motor and sensory axons in multifocal motor neuropathy , 2020, Clinical Neurophysiology.

[48]  N. van Alfen,et al.  Application of muscle ultrasound for the evaluation of patients with amyotrophic lateral sclerosis: An observational cross‐sectional study , 2020, Muscle & nerve.

[49]  B. Levin,et al.  Lack of effect on ambulation of dalfampridine-ER (4-AP) treatment in adult SMA patients , 2020, Neuromuscular Disorders.

[50]  S. Rutkove,et al.  Quantitative ultrasound of the tongue: Echo intensity is a potential biomarker of bulbar dysfunction in amyotrophic lateral sclerosis , 2020, Clinical Neurophysiology.

[51]  J. Lefaucheur,et al.  Motor unit number index as an individual biomarker: Reference limits of intra-individual variability over time in healthy subjects , 2020, Clinical Neurophysiology.

[52]  Visar Berisha,et al.  Improved ALS clinical trials through frequent at‐home self‐assessment: a proof of concept study , 2020, Annals of clinical and translational neurology.

[53]  D. Burke,et al.  A proposal for new diagnostic criteria for ALS , 2020, Clinical Neurophysiology.

[54]  A. Boon,et al.  AANEM – IFCN glossary of terms in neuromuscular electrodiagnostic medicine and ultrasound , 2020, Clinical Neurophysiology.

[55]  M. Swash,et al.  Diaphragmatic CMAP amplitude from phrenic nerve stimulation predicts functional decline in ALS , 2020, Journal of Neurology.

[56]  L. Barrios,et al.  Intramuscular Injection of Bone Marrow Stem Cells in Amyotrophic Lateral Sclerosis Patients: A Randomized Clinical Trial , 2020, Frontiers in Neuroscience.

[57]  Chang Xueli,et al.  Muscle ultrasonography in the diagnosis of amyotrophic lateral sclerosis , 2020, Neurological research.

[58]  P. Nestor,et al.  Sonographic and 3T-MRI-based evaluation of the tongue in ALS , 2020, NeuroImage: Clinical.

[59]  A. Blamire,et al.  Non-invasive imaging of single human motor units , 2020, Clinical Neurophysiology.

[60]  S. Vucic,et al.  Reproducibility of motor unit number index and MScanFit motor unit number estimation across intrinsic hand muscles , 2020, Muscle & nerve.

[61]  D. Burke,et al.  Occasional essay: Upper motor neuron syndrome in amyotrophic lateral sclerosis , 2020, Journal of Neurology, Neurosurgery, and Psychiatry.

[62]  Toshio Shimizu,et al.  Relationship between EMG-detected and ultrasound-detected fasciculations in amyotrophic lateral sclerosis: A prospective cohort study , 2020, Clinical Neurophysiology.

[63]  D. Burke,et al.  Measurement of axonal excitability: Consensus guidelines , 2020, Clinical Neurophysiology.

[64]  K. Mills,et al.  The evolving role of surface electromyography in amyotrophic lateral sclerosis: A systematic review , 2019, Clinical Neurophysiology.

[65]  H. Idrisoğlu,et al.  Repeater F-waves in amyotrophic lateral sclerosis: Electrophysiologic indicators of upper or lower motor neuron involvement? , 2019, Clinical Neurophysiology.

[66]  R. Iniesta,et al.  Preprocessing surface EMG data removes voluntary muscle activity and enhances SPiQE fasciculation analysis , 2019, Clinical Neurophysiology.

[67]  I. Wilkinson,et al.  Longitudinal multi-modal muscle-based biomarker assessment in motor neuron disease , 2019, Journal of Neurology.

[68]  H. Shang,et al.  Neurophysiological index is associated with the survival of patients with amyotrophic lateral sclerosis , 2019, Clinical Neurophysiology.

[69]  Elif Kocasoy Orhan,et al.  Parameters derived from compound muscle action potential scan for discriminating amyotrophic lateral sclerosis‐related denervation , 2019, Muscle & nerve.

[70]  Z. Simmons,et al.  ULTRASOUND IN THE DIAGNOSIS AND MONITORING OF AMYOTROPHIC LATERAL SCLEROSIS: A REVIEW , 2019, Muscle & nerve.

[71]  A. Al-Chalabi,et al.  Safety and tolerability of Triumeq in amyotrophic lateral sclerosis: the Lighthouse trial , 2019, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[72]  M. Swash,et al.  Sensory modulation of fasciculation discharge frequency , 2019, Muscle & nerve.

[73]  J. Pollard,et al.  Conduction block in immune‐mediated neuropathy: paranodopathy versus axonopathy , 2019, European journal of neurology.

[74]  J. Shefner,et al.  Nusinersen in later-onset spinal muscular atrophy , 2019, Neurology.

[75]  J. Bashford,et al.  SPiQE: An automated analytical tool for detecting and characterising fasciculations in amyotrophic lateral sclerosis , 2019, Clinical Neurophysiology.

[76]  A. Blamire,et al.  Functional magnetic resonance imaging of human motor unit fasciculation in amyotrophic lateral sclerosis , 2019, Annals of neurology.

[77]  M. Swash,et al.  Diagnostic criteria for amyotrophic lateral sclerosis: A multicentre study of inter-rater variation and sensitivity , 2019, Clinical Neurophysiology.

[78]  Markus Weber,et al.  Motor Unit Number Index (MUNIX) of hand muscles is a disease biomarker for adult spinal muscular atrophy , 2019, Clinical Neurophysiology.

[79]  L. Greensmith,et al.  Skeletal muscle MRI differentiates SBMA and ALS and correlates with disease severity , 2018, Neurology.

[80]  H. Tankisi,et al.  Following disease progression in motor neuron disorders with 3 motor unit number estimation methods , 2018, Muscle & nerve.

[81]  V. Marchand-Pauvert,et al.  The motor unit number index (MUNIX) profile of patients with adult spinal muscular atrophy , 2018, Clinical Neurophysiology.

[82]  D. Burke,et al.  In vivo evidence for reduced ion channel expression in motor axons of patients with amyotrophic lateral sclerosis , 2018, The Journal of physiology.

[83]  J. M. Matamala,et al.  Comparison of cross‐sectional areas and distal‐proximal nerve ratios in amyotrophic lateral sclerosis , 2018, Muscle & nerve.

[84]  B. Sanchez,et al.  Electrical Impedance Methods in Neuromuscular Assessment: An Overview. , 2018, Cold Spring Harbor perspectives in medicine.

[85]  M. Kiernan,et al.  Fasciculation intensity and disease progression in amyotrophic lateral sclerosis , 2018, Clinical Neurophysiology.

[86]  M. Benatar,et al.  Reducing sample size requirements for future ALS clinical trials with a dedicated electrical impedance myography system , 2018, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[87]  K. Kapur,et al.  Estimating Myofiber Size With Electrical Impedance Myography: a Study In Amyotrophic Lateral Sclerosis MICE , 2018, Muscle & nerve.

[88]  J. Glass,et al.  Implementing Motor Unit Number Index (MUNIX) in a large clinical trial: Real world experience from 27 centres , 2018, Clinical Neurophysiology.

[89]  M. Swash,et al.  Motor unit number estimation (MUNE): Where are we now? , 2018, Clinical Neurophysiology.

[90]  D. Burke,et al.  Excitability of sensory axons in amyotrophic lateral sclerosis , 2018, Clinical Neurophysiology.

[91]  M. Cudkowicz,et al.  Mexiletine for muscle cramps in amyotrophic lateral sclerosis: A randomized, double‐blind crossover trial , 2018, Muscle & nerve.

[92]  L. H. van den Berg,et al.  Acute Effects of Riluzole and Retigabine on Axonal Excitability in Patients With Amyotrophic Lateral Sclerosis: A Randomized, Double‐Blind, Placebo‐Controlled, Crossover Trial , 2018, Clinical pharmacology and therapeutics.

[93]  Ping Zhou,et al.  A Novel Validation Approach for High-Density Surface EMG Decomposition in Motor Neuron Disease , 2018, IEEE Transactions on Neural Systems and Rehabilitation Engineering.

[94]  Ping Zhou,et al.  Assessing Hand Muscle Structural Modifications in Chronic Stroke , 2018, Front. Neurol..

[95]  F. Schick,et al.  Spontaneous mechanical and electrical activities of human calf musculature at rest assessed by repetitive single‐shot diffusion‐weighted MRI and simultaneous surface electromyography , 2018, Magnetic resonance in medicine.

[96]  J. M. Matamala,et al.  Ectopic impulse generation in peripheral nerve hyperexcitability syndromes and amyotrophic lateral sclerosis , 2018, Clinical Neurophysiology.

[97]  Y. Kim,et al.  Split hand muscle echo intensity index as a reliable imaging marker for differential diagnosis of amyotrophic lateral sclerosis , 2018, Journal of Neurology, Neurosurgery, and Psychiatry.

[98]  Jonghye Woo,et al.  Magnetic resonance imaging based anatomical assessment of tongue impairment due to amyotrophic lateral sclerosis: A preliminary study. , 2018, The Journal of the Acoustical Society of America.

[99]  E. Stålberg,et al.  Motor unit number index: Guidelines for recording signals and their analysis , 2018, Muscle & nerve.

[100]  Agnieszka Sabisz,et al.  Understanding the Physiopathology Behind Axial and Radial Diffusivity Changes—What Do We Know? , 2018, Front. Neurol..

[101]  L. Zinman,et al.  Motor unit number index and neurophysiological index as candidate biomarkers of presymptomatic motor neuron loss in amyotrophic lateral sclerosis , 2018, Muscle & nerve.

[102]  P. Nestor,et al.  Peripheral nerve atrophy together with higher cerebrospinal fluid progranulin indicate axonal damage in amyotrophic lateral sclerosis , 2018, Muscle & nerve.

[103]  J. M. Matamala,et al.  Detection of fasciculations in amyotrophic lateral sclerosis: The optimal ultrasound scan time , 2017, Muscle & nerve.

[104]  A. Fuglsang-Frederiksen,et al.  Fasciculations in nerve and muscle disorders – A prospective study of muscle ultrasound compared to electromyography , 2017, Clinical Neurophysiology.

[105]  I. Wilkinson,et al.  Imaging muscle as a potential biomarker of denervation in motor neuron disease , 2017, Journal of Neurology, Neurosurgery, and Psychiatry.

[106]  L. Korngut,et al.  Decremental responses in patients with motor neuron disease , 2017, Brain and behavior.

[107]  A. Fuglsang-Frederiksen,et al.  Reproducibility, and sensitivity to motor unit loss in amyotrophic lateral sclerosis, of a novel MUNE method: MScanFit MUNE , 2017, Clinical Neurophysiology.

[108]  F. Medina-Mirapeix,et al.  Muscular Echovariation: A New Biomarker in Amyotrophic Lateral Sclerosis. , 2017, Ultrasound in medicine & biology.

[109]  J. Bae,et al.  Early discrimination of sensorimotor Guillain‐Barré syndrome into demyelinating or axonal subtype by automated nerve excitability testing , 2017, Journal of the peripheral nervous system : JPNS.

[110]  K. Shiga,et al.  A muscle ultrasound score in the diagnosis of amyotrophic lateral sclerosis , 2017, Clinical Neurophysiology.

[111]  F. Medina-Mirapeix,et al.  Quantitative Muscle Ultrasonography Using Textural Analysis in Amyotrophic Lateral Sclerosis , 2017, Ultrasonic imaging.

[112]  J. Lagopoulos,et al.  Peripheral nerve diffusion tensor imaging as a measure of disease progression in ALS , 2017, Journal of Neurology.

[113]  E. Stålberg,et al.  Motor Unit Number Index (MUNIX) detects motor neuron loss in pre-symptomatic muscles in Amyotrophic Lateral Sclerosis , 2017, Clinical Neurophysiology.

[114]  Yang Liu,et al.  Transplantation of autologous peripheral blood mononuclear cells in the subarachnoid space for amyotrophic lateral sclerosis: a safety analysis of 14 patients , 2017, Neural regeneration research.

[115]  J. M. Matamala,et al.  Dynamic muscle ultrasound identifies upper motor neuron involvement in amyotrophic lateral sclerosis , 2017, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[116]  M. Swash,et al.  Phrenic nerve stimulation is more sensitive than ultrasound measurement of diaphragm thickness in assessing early ALS progression , 2017, Neurophysiologie Clinique/Clinical Neurophysiology.

[117]  J. Hendrikse,et al.  Comparative study of peripheral nerve Mri and ultrasound in multifocal motor neuropathy and amyotrophic lateral sclerosis , 2016, Muscle & nerve.

[118]  Benjamin Sanchez,et al.  Electrical Impedance Myography and Its Applications in Neuromuscular Disorders , 2016, Neurotherapeutics.

[119]  L. Defebvre,et al.  The value of magnetic resonance imaging as a biomarker for amyotrophic lateral sclerosis: a systematic review , 2016, BMC Neurology.

[120]  C. Yiannikas,et al.  Diagnostic criteria in amyotrophic lateral sclerosis , 2016, Neurology.

[121]  Jia Li,et al.  Impedance Alterations in Healthy and Diseased Mice During Electrically Induced Muscle Contraction , 2016, IEEE Transactions on Biomedical Engineering.

[122]  E. Clini,et al.  Ultrasound assessment of diaphragmatic function in patients with amyotrophic lateral sclerosis , 2016, Respirology.

[123]  C. Krarup,et al.  Estimating motor unit numbers from a CMAP scan , 2016, Muscle & nerve.

[124]  H. Heinze,et al.  Quantifying disease progression in amyotrophic lateral sclerosis using peripheral nerve sonography , 2016, Muscle & nerve.

[125]  M. Swash,et al.  Fasciculation discharge frequency in amyotrophic lateral sclerosis and related disorders , 2016, Clinical Neurophysiology.

[126]  M. Filippi,et al.  MR Imaging of Brachial Plexus and Limb-Girdle Muscles in Patients with Amyotrophic Lateral Sclerosis. , 2016, Radiology.

[127]  G. Sobue,et al.  Amyotrophic lateral sclerosis and motor neuron syndromes in Asia , 2016, Journal of Neurology, Neurosurgery & Psychiatry.

[128]  David Burke,et al.  Clinical uses of H reflexes of upper and lower limb muscles , 2016, Clinical neurophysiology practice.

[129]  B. Mohammadi,et al.  Lower motor neuron involvement in ALS assessed by motor unit number index (MUNIX): Long-term changes and reproducibility , 2016, Clinical Neurophysiology.

[130]  Ian Loram,et al.  Ultrasound-Based Detection of Fasciculations in Healthy and Diseased Muscles , 2016, IEEE Transactions on Biomedical Engineering.

[131]  J. Blok,et al.  Electrically evoked multiplet discharges are associated with more marked clinical deterioration in motor neuron disease , 2016, Muscle & nerve.

[132]  J. Blok,et al.  Identifying fasciculation potentials in motor neuron disease: A matter of probability , 2016, Muscle & nerve.

[133]  M. Oda,et al.  Tongue thickness evaluation using ultrasonography can predict swallowing function in amyotrophic lateral sclerosis patients , 2016, Clinical Neurophysiology.

[134]  M. Swash,et al.  Ultrasound for assessment of diaphragm in ALS , 2016, Clinical Neurophysiology.

[135]  N. Graziane,et al.  Electrophysiological Analysis of Synaptic Transmission , 2015, Neuromethods.

[136]  M. Kiernan,et al.  Flecainide in Amyotrophic Lateral Sclerosis as a Neuroprotective Strategy (FANS): A Randomized Placebo-Controlled Trial , 2015, EBioMedicine.

[137]  Susanne Hanner,et al.  Increased HCN channel driven inward rectification in benign cramp fasciculation syndrome. , 2015, Brain : a journal of neurology.

[138]  N. Staff,et al.  Magnetic resonance imaging abnormalities of peripheral nerve and muscle are common in amyotrophic lateral sclerosis and share features with multifocal motor neuropathy , 2015, Muscle & nerve.

[139]  F. Schick,et al.  Addressing spontaneous signal voids in repetitive single‐shot DWI of musculature: spatial and temporal patterns in the calves of healthy volunteers and consideration of unintended muscle activities as underlying mechanism , 2015, NMR in biomedicine.

[140]  Ping Zhou,et al.  Innervation zones of fasciculating motor units: observations by a linear electrode array , 2015, Front. Hum. Neurosci..

[141]  Toshio Shimizu,et al.  A single blind randomized controlled clinical trial of mexiletine in amyotrophic lateral sclerosis: Efficacy and safety of sodium channel blocker phase II trial , 2015, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[142]  Christian Burkhardt,et al.  Tracking motor neuron loss in a set of six muscles in amyotrophic lateral sclerosis using the Motor Unit Number Index (MUNIX): a 15-month longitudinal multicentre trial , 2015, Journal of Neurology, Neurosurgery & Psychiatry.

[143]  H. Heinze,et al.  Peripheral nerve ultrasound in amyotrophic lateral sclerosis phenotypes , 2015, Muscle & nerve.

[144]  D. Burke,et al.  Dissociated lower limb muscle involvement in amyotrophic lateral sclerosis , 2015, Journal of Neurology.

[145]  H. Axer,et al.  Muscle ultrasonography as an additional diagnostic tool for the diagnosis of amyotrophic lateral sclerosis , 2015, Clinical Neurophysiology.

[146]  E. Stålberg,et al.  Cumulative Motor Index: An Index to Study Progression of Amyotrophic Lateral Sclerosis , 2015, Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society.

[147]  H. Axer,et al.  Nerve ultrasound for differentiation between amyotrophic lateral sclerosis and multifocal motor neuropathy , 2015, Journal of Neurology.

[148]  J. Blok,et al.  Diagnostic accuracy of electrically elicited multiplet discharges in patients with motor neuron disease , 2014, Journal of Neurology, Neurosurgery & Psychiatry.

[149]  H. Krämer,et al.  Excellent interrater agreement for the differentiation of fasciculations and artefacts – A dynamic myosonography study , 2014, Clinical Neurophysiology.

[150]  Jia Li,et al.  The effect of profound dehydration on electrical impedance of mouseskeletal muscle , 2014, 2014 36th Annual International Conference of the IEEE Engineering in Medicine and Biology Society.

[151]  M. Kiernan,et al.  Axonal dysfunction with voltage gated potassium channel complex antibodies , 2014, Experimental Neurology.

[152]  R. Kaji,et al.  Thinning of cervical nerve roots and peripheral nerves in ALS as measured by sonography , 2014, Clinical Neurophysiology.

[153]  M. Kiernan,et al.  Early identification of 'acute-onset' chronic inflammatory demyelinating polyneuropathy. , 2014, Brain : a journal of neurology.

[154]  W. Rymer,et al.  Sensitivity of fasciculation potential detection is dramatically reduced by spatial filtering of surface electromyography , 2014, Clinical Neurophysiology.

[155]  D. Burke,et al.  Axonal excitability in X-linked dominant Charcot Marie Tooth disease , 2014, Clinical Neurophysiology.

[156]  Ping Zhou,et al.  Analysis of surface EMG baseline for detection of hidden muscle activity , 2014, Journal of neural engineering.

[157]  J. Blok,et al.  CMAP scan discontinuities: Automated detection and relation to motor unit loss , 2014, Clinical Neurophysiology.

[158]  W. Rymer,et al.  A Clinically Applicable Approach for Detecting Spontaneous Action Potential Spikes in Amyotrophic Lateral Sclerosis With a Linear Electrode Array , 2014, Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society.

[159]  Anthony N Pettitt,et al.  Bayesian Experimental Design for Models with Intractable Likelihoods , 2013, Biometrics.

[160]  R. Kaji,et al.  Increased variability of axonal excitability in amyotrophic lateral sclerosis , 2013, Clinical Neurophysiology.

[161]  Iain D. Wilkinson,et al.  A prospective pilot study measuring muscle volumetric change in amyotrophic lateral sclerosis , 2013, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[162]  S. Rutkove,et al.  Electrophysiologic Biomarkers for Assessing Disease Progression and the Effect of Riluzole in SOD1 G93A ALS Mice , 2013, PloS one.

[163]  M. Kiernan,et al.  Riluzole exerts central and peripheral modulating effects in amyotrophic lateral sclerosis. , 2013, Brain : a journal of neurology.

[164]  D. Burke,et al.  Pathophysiology of HNPP explored using axonal excitability , 2013, Journal of Neurology, Neurosurgery & Psychiatry.

[165]  M. Swash,et al.  Fasciculation potentials and earliest changes in motor unit physiology in ALS , 2013, Journal of Neurology, Neurosurgery & Psychiatry.

[166]  C. Yiannikas,et al.  Split-hand index for the diagnosis of amyotrophic lateral sclerosis , 2013, Clinical Neurophysiology.

[167]  Hideto Miwa,et al.  Ultrasonography of the diaphragm in amyotrophic lateral sclerosis: Clinical significance in assessment of respiratory functions , 2013, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[168]  Diaphragm ultrasonography in the management of patients with amyotrophic lateral sclerosis , 2013, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[169]  Mamede de Carvalho,et al.  Phrenic nerve studies predict survival in amyotrophic lateral sclerosis , 2012, Clinical Neurophysiology.

[170]  W. Rymer,et al.  Duration of observation required in detecting fasciculation potentials in amyotrophic lateral sclerosis using high-density surface EMG , 2012, Journal of NeuroEngineering and Rehabilitation.

[171]  J. Blok,et al.  Multiplet discharges after electrical stimulation: New evidence for distal excitability changes in motor neuron disease , 2012, Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases.

[172]  S. Pillen,et al.  Muscle ultrasonography: A diagnostic tool for amyotrophic lateral sclerosis , 2012, Clinical Neurophysiology.

[173]  Yasunori Sato,et al.  Motor axonal excitability properties are strong predictors for survival in amyotrophic lateral sclerosis , 2012, Journal of Neurology, Neurosurgery & Psychiatry.

[174]  Michael Benatar,et al.  Electrical impedance myography as a biomarker to assess ALS progression , 2012, Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases.

[175]  M. Swash.  Why are upper motor neuron signs difficult to elicit in amyotrophic lateral sclerosis? , 2012, Journal of Neurology, Neurosurgery & Psychiatry.

[176]  Dick F. Stegeman,et al.  Fasciculations and their F-response revisited: High-density surface EMG in ALS and benign fasciculations , 2012, Clinical Neurophysiology.

[177]  S. Kuwabara,et al.  Ultrasonographic detection of fasciculations markedly increases diagnostic sensitivity of ALS , 2011, Neurology.

[178]  M. Kiernan,et al.  Dysfunction of axonal membrane conductances in adolescents and young adults with spinal muscular atrophy , 2011, Brain : a journal of neurology.

[179]  F. Walker,et al.  Peripheral nerve and muscle ultrasound in amyotrophic lateral sclerosis , 2011, Muscle & nerve.

[180]  Mamede de Carvalho,et al.  Motor Unit Number Index (MUNIX): A novel neurophysiological marker for neuromuscular disorders; test–retest reliability in healthy volunteers , 2011, Clinical Neurophysiology.

[181]  M. Kiernan,et al.  Modulatory effects on axonal function after intravenous immunoglobulin therapy in chronic inflammatory demyelinating polyneuropathy. , 2011, Archives of neurology.

[182]  M. Schroth,et al.  SMA CARNI-VAL TRIAL PART II: A Prospective, Single-Armed Trial of L-Carnitine and Valproic Acid in Ambulatory Children with Spinal Muscular Atrophy , 2011, PloS one.

[183]  S. Pillen,et al.  Detection of fibrillations using muscle ultrasound: Diagnostic accuracy and identification of pitfalls , 2011, Muscle & nerve.

[184]  F. Nomura,et al.  Altered axonal excitability properties in juvenile muscular atrophy of distal upper extremity (Hirayama disease) , 2011, Clinical Neurophysiology.

[185]  Seward B Rutkove,et al.  Characterizing spinal muscular atrophy with electrical impedance myography , 2010, Muscle & nerve.

[186]  Sanjeev D Nandedkar,et al.  Motor unit number index (MUNIX): principle, method, and findings in healthy subjects and in patients with motor neuron disease , 2010, Muscle & nerve.

[187]  Kyung Seok Park,et al.  Reproducibility of the motor unit number index (MUNIX) in normal controls and amyotrophic lateral sclerosis patients , 2010, Muscle & nerve.

[188]  M. Schroth,et al.  Compound muscle action potential and motor function in children with spinal muscular atrophy , 2010, Muscle & nerve.

[189]  A. Peltier,et al.  Muscle ultrasound quantifies the rate of reduction of muscle thickness in amyotrophic lateral sclerosis , 2010, Muscle & nerve.

[190]  R. Kaji,et al.  Peripheral Nerve Excitability Measures at Different Target Levels: The Effects of Aging and Diabetic Neuropathy , 2010, Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society.

[191]  M. de Carvalho,et al.  A randomized, placebo-controlled trial of memantine for functional disability in amyotrophic lateral sclerosis , 2010, Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases.

[192]  Erik Stålberg,et al.  Motor unit number index (MUNIX): A novel neurophysiological technique to follow disease progression in amyotrophic lateral sclerosis , 2010, Muscle & nerve.

[193]  D. Burke,et al.  Properties of low‐threshold motor axons in the human median nerve , 2010, The Journal of physiology.

[194]  M. Gunetti,et al.  Mesenchymal stem cell transplantation in amyotrophic lateral sclerosis: A Phase I clinical trial , 2010, Experimental Neurology.

[195]  S. Pillen,et al.  Muscle ultrasonography to predict survival in amyotrophic lateral sclerosis , 2010, Journal of Neurology, Neurosurgery & Psychiatry.

[196]  A. Reginelli,et al.  Amyotrophic lateral sclerosis: sonographic evaluation of dysphagia , 2010, La radiologia medica.

[197]  V. Drory,et al.  Recombinant human granulocyte-colony stimulating factor administration for treating amyotrophic lateral sclerosis: A pilot study , 2010, Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases.

[198]  M. Kiernan,et al.  Axonal ion channels from bench to bedside: A translational neuroscience perspective , 2009, Progress in Neurobiology.

[199]  M. Kiernan,et al.  Upregulation of persistent sodium conductances in familial ALS , 2009, Journal of Neurology, Neurosurgery & Psychiatry.

[200]  S. Pillen,et al.  Quantitative gray‐scale analysis in skeletal muscle ultrasound: A comparison study of two ultrasound devices , 2009, Muscle & nerve.

[201]  M. Schroth,et al.  Phase II Open Label Study of Valproic Acid in Spinal Muscular Atrophy , 2009, PloS one.

[202]  Baziel G M van Engelen,et al.  Skeletal muscle ultrasound: correlation between fibrous tissue and echo intensity. , 2009, Ultrasound in medicine & biology.

[203]  B. Mädler,et al.  Pilot study of granulocyte colony stimulating factor (G‐CSF)‐mobilized peripheral blood stem cells in amyotrophic lateral sclerosis (ALS) , 2008, Muscle & nerve.

[204]  N. Venkatanarasimha,et al.  MRI appearance of muscle denervation , 2008, Skeletal Radiology.

[205]  S. Pillen,et al.  Quantitative muscle ultrasonography in amyotrophic lateral sclerosis. , 2008, Ultrasound in medicine & biology.

[206]  M. Swash,et al.  Electrodiagnostic criteria for diagnosis of ALS , 2008, Clinical Neurophysiology.

[207]  Dick F. Stegeman,et al.  Firing pattern of fasciculations in ALS , 2008, Neurology.

[208]  Farrah J Mateen,et al.  Strength, physical activity, and fasciculations in patients with ALS , 2008, Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases.

[209]  M. Kiernan,et al.  Pathophysiologic insights into motor axonal function in Kennedy disease , 2007, Neurology.

[210]  Joleen H Blok,et al.  The electrophysiological muscle scan , 2007, Muscle & nerve.

[211]  Anthony N Pettitt,et al.  Bayesian statistical MUNE method , 2007, Muscle & nerve.

[212]  Sanjeev D Nandedkar,et al.  Contribution of reference electrode to the compound muscle action potential , 2007, Muscle & nerve.

[213]  Gea Drost,et al.  Fasciculation Potentials in High-Density Surface EMG , 2007, Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society.

[214]  M. Kiernan,et al.  Abnormalities in cortical and peripheral excitability in flail arm variant amyotrophic lateral sclerosis , 2007, Journal of Neurology, Neurosurgery & Psychiatry.

[215]  Anthony N Pettitt,et al.  Motor Unit Number Estimation—A Bayesian Approach , 2006, Biometrics.

[216]  S. Kuwabara,et al.  Increased nodal persistent Na+ currents in human neuropathy and motor neuron disease estimated by latent addition , 2006, Clinical Neurophysiology.

[217]  H. Bostock,et al.  Distal excitability changes in motor axons in amyotrophic lateral sclerosis , 2006, Clinical Neurophysiology.

[218]  Matthew C. Kiernan,et al.  Axonal excitability properties in amyotrophic lateral sclerosis , 2006, Clinical Neurophysiology.

[219]  Kazuaki Kanai,et al.  Altered axonal excitability properties in amyotrophic lateral sclerosis: impaired potassium channel function related to disease stage. , 2006, Brain : a journal of neurology.

[220]  Michael Swash,et al.  Quantitating progression in ALS , 2005, Neurology.

[221]  Mark B Bromberg,et al.  Natural history of denervation in SMA: Relation to age, SMN2 copy number, and function , 2005, Annals of neurology.

[222]  M. Swash,et al.  Neurophysiological measures in amyotrophic lateral sclerosis: Markers of progression in clinical trials , 2005, Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases.

[223]  D. Stegeman,et al.  Statistical motor unit number estimation assuming a binomial distribution , 2005 .

[224]  Sanjeev D. Nandedkar,et al.  Motor unit number index (MUNIX) , 2004, IEEE Transactions on Biomedical Engineering.

[225]  M. Swash,et al.  The Neurophysiological Index in ALS , 2004, Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases.

[226]  Hugh Bostock,et al.  Variations in excitability of single human motor axons, related to stochastic properties of nodal sodium channels , 2004, The Journal of physiology.

[227]  C. Wessig,et al.  Muscle magnetic resonance imaging of denervation and reinnervation: correlation with electrophysiology and histology , 2004, Experimental Neurology.

[228]  R. Kaji,et al.  Threshold electrotonus in chronic inflammatory demyelinating polyneuropathy: Correlation with clinical profiles , 2004, Muscle & nerve.

[229]  M. Bromberg,et al.  Counting motor units in chronic motor neuropathies , 2003, Experimental Neurology.

[230]  Catherine Lomen-Hoerth,et al.  Statistical motor unit number estimation: From theory to practice , 2003, Muscle & nerve.

[231]  S. Kuwabara,et al.  Muscle cramp in Machado-Joseph disease: altered motor axonal excitability properties and mexiletine treatment. , 2003, Brain : a journal of neurology.

[232]  Jasper R Daube,et al.  Effect of changing data collection parameters on statistical motor unit number estimates , 2003, Muscle & nerve.

[233]  D. Burke,et al.  Differences in membrane properties of axonal and demyelinating Guillain‐Barré syndromes , 2002, Annals of neurology.

[234]  M. Bromberg,et al.  Electrophysiologic endpoint measures in a multicenter ALS drug trial , 2001, Journal of the Neurological Sciences.

[235]  D. Burke,et al.  Multiple measures of axonal excitability: A new approach in clinical testing , 2000, Muscle & nerve.

[236]  A. Wilbourn,et al.  The “split hand syndrome” , 2000, Muscle & nerve.

[237]  J. Shefner,et al.  Reducing intersubject variability in motor unit number estimation , 1999, Muscle & nerve.

[238]  G. Barker,et al.  Diffusion tensor imaging of lesions and normal-appearing white matter in multiple sclerosis , 1999, Neurology.

[239]  D. Burke,et al.  Ischemic resistance of cutaneous afferents and motor axons in patients with amyotrophic lateral sclerosis , 1998, Muscle & nerve.

[240]  M. Swash,et al.  Riluzole has no acute effect on motor unit parameters in ALS , 1998, Journal of the Neurological Sciences.

[241]  J. Reisch,et al.  Magnetic resonance imaging of muscle in amyotrophic lateral sclerosis , 1998, Neurology.

[242]  D. Burke,et al.  Strength-duration properties of sensory and motor axons in amyotrophic lateral sclerosis. , 1998, Brain : a journal of neurology.

[243]  D. Burke,et al.  Threshold tracking techniques in the study of human peripheral nerve , 1998, Muscle & nerve.

[244]  J R Daube,et al.  Estimating the Number of Motor Units in a Muscle , 1995, Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society.

[245]  H Bostock,et al.  Axonal ion channel dysfunction in amyotrophic lateral sclerosis. , 1995, Brain : a journal of neurology.

[246]  D W Stashuk,et al.  Motor unit number estimates based on the automated analysis of F‐responses , 1994, Muscle & nerve.

[247]  B. Brooks,et al.  El escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis , 1994, Journal of the Neurological Sciences.

[248]  M J Zwarts,et al.  Muscle fiber conduction velocity in amyotrophic lateral sclerosis and traumatic lesions of the plexus brachialis. , 1993, Electroencephalography and clinical neurophysiology.

[249]  W. Brown,et al.  The estimated numbers and relative sizes of thenar motor units as selected by multiple point stimulation in young and older adults , 1993, Muscle & nerve.

[250]  E. Stålberg,et al.  A pilot trial of dextromethorphan in amyotrophic lateral sclerosis. , 1993, Journal of neurology, neurosurgery, and psychiatry.

[251]  R S Howard,et al.  Surface EMG in the recording of fasciculations , 1992, Muscle & nerve.

[252]  A. Pestronk,et al.  Nerve conduction studies in amyotrophic lateral sclerosis , 1992, Muscle & nerve.

[253]  Y. Mano,et al.  [MRI findings of the tongue in neurodegenerative diseases with bulbar sign]. , 1990, Rinsho shinkeigaku = Clinical neurology.

[254]  W. Brown,et al.  Methods for estimating numbers of motor units in biceps‐brachialis muscles and losses of motor units with aging , 1988, Muscle & nerve.

[255]  G. Wieneke,et al.  Estimation of the number of motor units based on macro-EMG. , 1988, Journal of neurology, neurosurgery, and psychiatry.

[256]  L. Bour,et al.  The flexor carpi radialis H-reflex in polyneuropathy: relations to conduction velocities of the median nerve and the soleus H-reflex latency. , 1987, Journal of neurology, neurosurgery, and psychiatry.

[257]  H. Mitsumoto,et al.  Amyotrophic lateral sclerosis , 1986, Neurology.

[258]  E Stålberg,et al.  Macro EMG, a new recording technique. , 1980, Journal of neurology, neurosurgery, and psychiatry.

[259]  M. Hallett.  Ballistic elbow flexion movements in patients with amyotrophic lateral sclerosis. , 1979, Journal of neurology, neurosurgery, and psychiatry.

[260]  R. Stein,et al.  Contractile and electrical properties of human motor units in neuropathies and motor neurone disease , 1974, Journal of neurology, neurosurgery, and psychiatry.

[261]  R. Stein,et al.  Pattern of recruiting human motor units in neuropathies and motor neurone disease , 1974, Journal of neurology, neurosurgery, and psychiatry.

[262]  R. Willison,et al.  The distribution and frequency of spontaneous fasciculations in motor neurone disease. , 1973, Journal of the neurological sciences.

[263]  A. McComas,et al.  The physiology of single human nerve fibres , 1972 .

[264]  M J Campbell,et al.  Electrophysiological estimation of the number of motor units within a human muscle , 1971, Journal of neurology, neurosurgery, and psychiatry.

[265]  F. Medina-Mirapeix,et al.  Monitoring Progression of Amyotrophic Lateral Sclerosis Using Ultrasound Morpho-Textural Muscle Biomarkers: A Pilot Study. , 2018, Ultrasound in medicine & biology.

[266]  Ping Zhou,et al.  A Novel Framework Based on FastICA for High Density Surface EMG Decomposition , 2016, IEEE Transactions on Neural Systems and Rehabilitation Engineering.

[267]  R. Kaji,et al.  Sonographic evaluation of cervical nerve roots in ALS and its clinical subtypes. , 2016, The journal of medical investigation : JMI.

[268]  L. Singh,et al.  Therapy with Bone Marrow-Derived Autologous Adult Stem Cells in Quadriparesis due to Motor Neuron Disease. , 2016, Journal of stem cells.

[269]  M. Kiernan,et al.  Axonal ion channel dysfunction in c9orf72 familial amyotrophic lateral sclerosis. , 2015, JAMA neurology.

[270]  A. Storch,et al.  Nerve ultrasound in the differentiation of multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis with predominant lower motor neuron disease (ALS/LMND) , 2015, Journal of Neurology.

[271]  Ping Zhou,et al.  Machine Learning for Supporting Diagnosis of Amyotrophic Lateral Sclerosis Using Surface Electromyogram , 2014, IEEE Transactions on Neural Systems and Rehabilitation Engineering.

[272]  G. Sobue,et al.  Nerve excitability properties in Charcot-Marie-Tooth disease type 1A. , 2004, Brain : a journal of neurology.

[273]  J. Rothwell,et al.  Latent addition in motor and sensory fibres of human peripheral nerve. , 1997, The Journal of physiology.

[274]  P M Weeks,et al.  Fascicular topography of the median nerve: a computer based study to identify branching patterns. , 1991, The Journal of hand surgery.

[275]  JoVE Video Dataset , 2022 .

[276]  I. Schofield,et al.  Whole-body fasciculation detection in Amyotrophic Lateral Sclerosis (ALS) using motor unit MRI (MUMRI) , 2022, ISMRM Annual Meeting.