Matthew C Kiernan

Summary

Affiliation: University of New South Wales
Country: Australia

Publications

  1. ncbi request reprint Novel threshold tracking techniques suggest that cortical hyperexcitability is an early feature of motor neuron disease
    Steve Vucic
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Prince of Wales Hospital, Randwick, Sydney, Australia
    Brain 129:2436-46. 2006
  2. ncbi request reprint Axonal excitability properties in amyotrophic lateral sclerosis
    Steve Vucic
    Prince of Wales Medical Research Institute, Prince of Wales Clinical School, University of New South Wales, and Prince of Wales Hospital, Randwick, Sydney, NSW 2031, Australia
    Clin Neurophysiol 117:1458-66. 2006
  3. ncbi request reprint Riche-Cannieu anastomosis as an inherited trait
    Robert A Boland
    Spinal Injuries Research Centre, Prince of Wales Medical Research Institute, Barker Street, Randwick, Sydney, NSW 2031, Australia
    Clin Neurophysiol 118:770-5. 2007
  4. doi request reprint Physiology and pathophysiology of myelinated nerve fibers
    Matthew C Kiernan
    Prince of Wales Clinical School, University of New South Wales, and Neuroscience Research Australia, Sydney, Australia Electronic address
    Handb Clin Neurol 115:43-53. 2013
  5. doi request reprint Progressive axonal dysfunction and clinical impairment in amyotrophic lateral sclerosis
    Benjamin C Cheah
    Neuroscience Research Australia, Sydney, New South Wales, Australia
    Clin Neurophysiol 123:2460-7. 2012
  6. ncbi request reprint Acute tetrodotoxin-induced neurotoxicity after ingestion of puffer fish
    Matthew C Kiernan
    Prince of Wales Medical Research Institute, University of New South Wales, Barker Street, Randwick, Sydney NSW 2031, Australia
    Ann Neurol 57:339-48. 2005
  7. ncbi request reprint Mutation in the Na+ channel subunit SCN1B produces paradoxical changes in peripheral nerve excitability
    Matthew C Kiernan
    Prince of Wales Medical Research Institute, Prince of Wales Clinical School, University of New South Wales, Sydney, Australia
    Brain 128:1841-6. 2005
  8. ncbi request reprint The pathophysiology of oxaliplatin-induced neurotoxicity
    Matthew C Kiernan
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Sydney, NSW, Australia
    Curr Med Chem 13:2901-7. 2006
  9. doi request reprint Adaptation of motor function after spinal cord injury: novel insights into spinal shock
    Robert A Boland
    Spinal Injuries Research Centre, Neuroscience Research Australia, Sydney, NSW 2031, Australia
    Brain 134:495-505. 2011
  10. pmc Differences in activity-dependent hyperpolarization in human sensory and motor axons
    Matthew C Kiernan
    Prince of Wales Medical Research Institute, University of New South Wales and Institute of Neurological Sciences, Prince of Wales Hospital, Sydney, Australia
    J Physiol 558:341-9. 2004

Collaborators

Detail Information

Publications106 found, 100 shown here

  1. ncbi request reprint Novel threshold tracking techniques suggest that cortical hyperexcitability is an early feature of motor neuron disease
    Steve Vucic
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Prince of Wales Hospital, Randwick, Sydney, Australia
    Brain 129:2436-46. 2006
    ..Simultaneous assessment of central and peripheral nerve excitability has established the presence of co-existent upper and lower motor neuron dysfunction, with cortical hyperexcitability an early feature in MND...
  2. ncbi request reprint Axonal excitability properties in amyotrophic lateral sclerosis
    Steve Vucic
    Prince of Wales Medical Research Institute, Prince of Wales Clinical School, University of New South Wales, and Prince of Wales Hospital, Randwick, Sydney, NSW 2031, Australia
    Clin Neurophysiol 117:1458-66. 2006
    ..To investigate axolemmal ion channel function in patients diagnosed with sporadic amyotrophic lateral sclerosis (ALS)...
  3. ncbi request reprint Riche-Cannieu anastomosis as an inherited trait
    Robert A Boland
    Spinal Injuries Research Centre, Prince of Wales Medical Research Institute, Barker Street, Randwick, Sydney, NSW 2031, Australia
    Clin Neurophysiol 118:770-5. 2007
    ..To explore the basis for the Riche-Cannieu anastomosis (RCA) and specifically whether this anomaly is an hereditary characteristic...
  4. doi request reprint Physiology and pathophysiology of myelinated nerve fibers
    Matthew C Kiernan
    Prince of Wales Clinical School, University of New South Wales, and Neuroscience Research Australia, Sydney, Australia Electronic address
    Handb Clin Neurol 115:43-53. 2013
    ....
  5. doi request reprint Progressive axonal dysfunction and clinical impairment in amyotrophic lateral sclerosis
    Benjamin C Cheah
    Neuroscience Research Australia, Sydney, New South Wales, Australia
    Clin Neurophysiol 123:2460-7. 2012
    ..To elucidate longitudinal changes in axonal function in amyotrophic lateral sclerosis (ALS) patients, and to relate such changes with motor unit loss and functional impairment...
  6. ncbi request reprint Acute tetrodotoxin-induced neurotoxicity after ingestion of puffer fish
    Matthew C Kiernan
    Prince of Wales Medical Research Institute, University of New South Wales, Barker Street, Randwick, Sydney NSW 2031, Australia
    Ann Neurol 57:339-48. 2005
    ..It demonstrates the ability of noninvasive nerve excitability studies to detect Na(+) channel blockade in vivo and also the utility of mathematical modeling to aid interpretation of altered excitability properties in disease...
  7. ncbi request reprint Mutation in the Na+ channel subunit SCN1B produces paradoxical changes in peripheral nerve excitability
    Matthew C Kiernan
    Prince of Wales Medical Research Institute, Prince of Wales Clinical School, University of New South Wales, Sydney, Australia
    Brain 128:1841-6. 2005
    ....
  8. ncbi request reprint The pathophysiology of oxaliplatin-induced neurotoxicity
    Matthew C Kiernan
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Sydney, NSW, Australia
    Curr Med Chem 13:2901-7. 2006
    ..Specific emphasis will be placed on oxaliplatin, a platinum-based chemotherapy effective for colorectal cancer that exhibits dose-limiting neurotoxicity...
  9. doi request reprint Adaptation of motor function after spinal cord injury: novel insights into spinal shock
    Robert A Boland
    Spinal Injuries Research Centre, Neuroscience Research Australia, Sydney, NSW 2031, Australia
    Brain 134:495-505. 2011
    ..Contrary to prevailing opinion, these data demonstrate that significant changes in peripheral motor axonal excitability occur early during spinal shock, with subsequent further deterioration in axonal function, before recovery ensues...
  10. pmc Differences in activity-dependent hyperpolarization in human sensory and motor axons
    Matthew C Kiernan
    Prince of Wales Medical Research Institute, University of New South Wales and Institute of Neurological Sciences, Prince of Wales Hospital, Sydney, Australia
    J Physiol 558:341-9. 2004
    ....
  11. ncbi request reprint Mitochondrial dysfunction and rod-like lesions associated with administration of beta2 adrenoceptor agonist formoterol
    Matthew C Kiernan
    Prince of Wales Medical Research Institute, University of New South Wales, Sydney, NSW 2031, Australia
    Neuromuscul Disord 14:375-7. 2004
    ..Clinical and biochemical improvement occurred following withdrawal of formoterol. Possible mechanisms involved in the development of myopathy are explored...
  12. ncbi request reprint Characteristics of late Na(+) current in adult rat small sensory neurons
    M C Kiernan
    Sobell Department of Neurophysiology, Institute of Neurology, Queen Square, WC1N 3BG, London, UK
    Neuroscience 119:653-60. 2003
    ..In contrast, a single population of high-threshold TTX-r channels may account for both transient and late TTX-r currents...
  13. doi request reprint Amyotrophic lateral sclerosis
    Matthew C Kiernan
    Neuroscience Research Australia and Prince of Wales Clinical School, University of New South Wales, Sydney, Australia
    Lancet 377:942-55. 2011
    ....
  14. doi request reprint Oxaliplatin-induced neurotoxicity: changes in axonal excitability precede development of neuropathy
    Susanna B Park
    Prince of Wales Medical Research Institute, Barker Street, Randwick, Sydney, NSW 2031, Australia
    Brain 132:2712-23. 2009
    ..05) than those left with mild or moderate neurotoxicity, suggesting that assessment of sensory excitability parameters may provide a sensitive biomarker of severity for oxaliplatin-induced neurotoxicity...
  15. ncbi request reprint Dexpramipexole, the R(+) enantiomer of pramipexole, for the potential treatment of amyotrophic lateral sclerosis
    Benjamin C Cheah
    Neuroscience Research Australia and Multi disciplinary Amyotrophic Lateral Sclerosis Clinic, Prince of Wales Clinical School, University of New South Wales, Barker Street, Randwick, Sydney, NSW 2031, Australia
    IDrugs 13:911-20. 2010
    ..This report summarizes the chemical and pharmacological properties of dexpramipexole and describes the potential utility of the drug in the pharmaceutical development pipeline...
  16. pmc Threshold behaviour of human axons explored using subthreshold perturbations to membrane potential
    David Burke
    Institute of Clinical Neurosciences, Royal Prince Alfred Hospital and University of Sydney, Sydney, Australia
    J Physiol 587:491-504. 2009
    ..By extrapolation, we suggest that, when human axons discharge, nodal slow K+ currents will be activated sufficiently early to contribute to the early changes in excitability following the action potential...
  17. ncbi request reprint Inflections in threshold electrotonus to depolarizing currents in sensory axons
    David Burke
    Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, University of Sydney, Sydney, NSW 2006, Australia
    Muscle Nerve 36:849-52. 2007
    ..When large, the notch can distort subsequent phases of threshold electrotonus and could complicate quantitative measurements and modeling studies...
  18. ncbi request reprint Ischaemia induces paradoxical changes in axonal excitability in end-stage kidney disease
    Arun V Krishnan
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Randwick, Sydney, Australia
    Brain 129:1585-92. 2006
    ..Blockade of persistent Na+ conductances by H+ would also explain the reduction in strength-duration time constant observed during ischaemia...
  19. doi request reprint Evidence for a causal relationship between hyperkalaemia and axonal dysfunction in end-stage kidney disease
    Ria Arnold
    Translational Neuroscience Facility, School of Medical Sciences, University of New South Wales, Sydney, Australia
    Clin Neurophysiol 125:179-85. 2014
    ..Potassium (K(+)) has been implicated as a factor in the development of uraemic neuropathy. This study was undertaken to investigate whether hyperkalaemia plays a causal role in axonal dysfunction in end-stage kidney disease (ESKD)...
  20. pmc Transcranial magnetic stimulation and amyotrophic lateral sclerosis: pathophysiological insights
    Steve Vucic
    Sydney Medical School Westmead, University of Sydney, Sydney, New South Wales, Australia
    J Neurol Neurosurg Psychiatry 84:1161-70. 2013
    ....
  21. doi request reprint Changes in human sensory axonal excitability induced by an ischaemic insult
    S E Han
    Prince of Wales Clinical School and Prince of Wales Medical Research Institute, The University of New South Wales, Barker St, Randwick, NSW 2031, Australia
    Clin Neurophysiol 119:2054-63. 2008
    ..To identify the sensitivity and the patterns of change in sensory excitability that accompany an ischaemic insult...
  22. doi request reprint Pathophysiological insights into ALS with C9ORF72 expansions
    Kelly L Williams
    Australian School of Advanced Medicine, Macquarie University, Sydney, New South Wales, Australia
    J Neurol Neurosurg Psychiatry 84:931-5. 2013
    ..We sought to examine clinical and neurophysiological features of familial and sporadic ALS with C9ORF72 expansions...
  23. pmc Fatigue and activity dependent changes in axonal excitability in amyotrophic lateral sclerosis
    Steve Vucic
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Randwick, Australia
    J Neurol Neurosurg Psychiatry 78:1202-8. 2007
    ..The aim of the present study was to further delineate the mechanisms underlying increased fatigability in ALS by measuring activity dependent changes in axonal excitability following a maximum voluntary contraction (MVC)...
  24. ncbi request reprint Neuropathy, axonal Na+/K+ pump function and activity-dependent excitability changes in end-stage kidney disease
    Arun V Krishnan
    Institute of Neurological Sciences, Prince of Wales Hospital, Randwick, Sydney, NSW, Australia
    Clin Neurophysiol 117:992-9. 2006
    ..To investigate the mechanisms underlying peripheral neuropathy and to provide insights into axonal Na(+)/K(+) pump function in patients with end-stage kidney disease (ESKD)...
  25. doi request reprint Riluzole exerts central and peripheral modulating effects in amyotrophic lateral sclerosis
    Steve Vucic
    Neuroscience Research Australia, Barker Street, Randwick, Sydney, NSW 2031, Australia
    Brain 136:1361-70. 2013
    ..Taken together, these findings suggest that the neuroprotective effects of riluzole in amyotrophic lateral sclerosis are complex, with evidence of independent effects across both compartments of the nervous system...
  26. pmc Dysfunction of axonal membrane conductances in adolescents and young adults with spinal muscular atrophy
    Michelle A Farrar
    Neuroscience Research Australia, Randwick, Sydney, NSW, Australia
    Brain 134:3185-97. 2011
    ....
  27. doi request reprint Corticomotoneuronal integrity and adaptation in spinal muscular atrophy
    Michelle A Farrar
    Neuroscience Research Australia, Randwick, Sydney, NSW 2031, Australia
    Arch Neurol 69:467-73. 2012
    ..To gain further insight into disease pathophysiologic process and potential adaptations through investigating whether cortical dysfunction or plasticity is a feature of spinal muscle atrophy (SMA)...
  28. ncbi request reprint Pathophysiologic insights into motor axonal function in Kennedy disease
    Steve Vucic
    Prince of Wales Medical Research Institute, University of New South Wales, Sydney, Australia
    Neurology 69:1828-35. 2007
    ..Consequently, the present study applied axonal excitability techniques to investigate the pathophysiologic mechanisms associated with KD...
  29. doi request reprint Plasticity of lower limb motor axons after cervical cord injury
    Robert A Boland
    Spinal Injuries Research Centre, Prince of Wales Medical Research Institute, Randwick, NSW, Australia
    Clin Neurophysiol 120:204-9. 2009
    ..To assess changes in peripheral motor excitability after traumatic spinal cord injury (SCI)...
  30. ncbi request reprint Altered motor nerve excitability in end-stage kidney disease
    Arun V Krishnan
    Institute of Neurological Sciences, Prince of Wales Hospital, Randwick, Sydney, Australia
    Brain 128:2164-74. 2005
    ..The correlation between serum K+ and excitability measures indicates that hyperkalaemia is primarily responsible for uraemic depolarization, and a likely contributing factor to the development of neuropathy...
  31. doi request reprint Utilizing natural activity to dissect the pathophysiology of acute oxaliplatin-induced neuropathy
    Susanna B Park
    Prince of Wales Clinical School, Barker Street, Randwick, Sydney, NSW 2063, Australia
    Exp Neurol 227:120-7. 2011
    ..Findings from the present series suggest that oxaliplatin affects nerve excitability through voltage-dependent mechanisms, with specific effects mediated through axonal Na(+) channel inactivation...
  32. doi request reprint Acute abnormalities of sensory nerve function associated with oxaliplatin-induced neurotoxicity
    Susanna B Park
    Prince of Wales Medical Research Institute, Barker St, Randwick, Sydney, New South Wales 2031
    J Clin Oncol 27:1243-9. 2009
    ..Because the mechanism of oxaliplatin-induced neurotoxicity remains unclear, the present study investigated the potential of axonal excitability techniques in identifying pathophysiologic mechanisms and early markers of nerve dysfunction...
  33. pmc Evolution of peripheral nerve function in humans: novel insights from motor nerve excitability
    Michelle A Farrar
    Neuroscience Research Australia, Barker St, Randwick, Sydney, NSW 2031, Australia
    J Physiol 591:273-86. 2013
    ..Significantly, these findings bring the dynamics of axonal development to the clinical domain and serve to further illuminate pathophysiological mechanisms that occur during development...
  34. ncbi request reprint Axonal changes in spinal cord injured patients distal to the site of injury
    Cindy Shin Yi Lin
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Sydney, Australia
    Brain 130:985-94. 2007
    ..Changes in axonal structure and ion channel function, but perhaps more critically decentralization and consequent inactivity, are likely to underlie the complex changes observed in axonal excitability in SCI patients...
  35. doi request reprint Ischaemic sensitivity of axons in carpal tunnel syndrome
    S Eric Han
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Barker Street, Randwick NSW, Australia
    J Peripher Nerv Syst 14:190-200. 2009
    ....
  36. doi request reprint Neurophysiological index as a biomarker for ALS progression: validity of mixed effects models
    Benjamin C Cheah
    Neuroscience Research Australia, Sydney, New South Wales, Australia
    Amyotroph Lateral Scler 12:33-8. 2011
    ..29, p = 0.03). Linear mixed effects models were appropriate for detailing the longitudinal changes in NI. The present findings support incorporation of NI as an outcome measure for ALS clinical trials conducted over short time periods...
  37. doi request reprint FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysis
    Ian P Blair
    Northcott Neuroscience Laboratory, ANZAC Research Institute, Concord Hospital, Sydney, Australia
    J Neurol Neurosurg Psychiatry 81:639-45. 2010
    ..The present studies sought to define the clinical, post-mortem and neurophysiological phenotypes in ALS families with FUS mutations and to determine the frequency of FUS mutations in familial and sporadic ALS...
  38. pmc Changes in human sensory axonal excitability induced by focal nerve compression
    S Eric Han
    Prince of Wales Medical Research Institute, Barker Street, Randwick, Sydney, NSW 2031 Australia
    J Physiol 588:1737-45. 2010
    ....
  39. doi request reprint Regional differences in ulnar nerve excitability may predispose to the development of entrapment neuropathy
    Arun V Krishnan
    Translational Neuroscience Facility, School of Medical Sciences, University of New South Wales, Australia
    Clin Neurophysiol 122:194-8. 2011
    ....
  40. pmc Corticomotoneuronal function and hyperexcitability in acquired neuromyotonia
    Steve Vucic
    Neuroscience Research Australia, Barker Street, Randwick, Sydney, NSW 2031, Australia
    Brain 133:2727-33. 2010
    ....
  41. ncbi request reprint Evidence for axonal membrane hyperpolarization in multifocal motor neuropathy with conduction block
    Matthew C Kiernan
    Sobell Department of Neurophysiology, Institute of Neurology, National Hospital for Neurology and Neurosurgery, London, UK
    Brain 125:664-75. 2002
    ..It is suggested that the distal hyperpolarization is probably linked to focal depolarization and that the clinical features of MMN are consistent with a depolarizing/hyperpolarizing lesion...
  42. doi request reprint Neuropsychiatric changes precede classic motor symptoms in ALS and do not affect survival
    Eneida Mioshi
    From Neuroscience Research Australia E M, J C, S H, E R, E D, M H, J R H, M C K, Sydney School of Medical Sciences E M, E D, M H, J R H, University of New South Wales, Sydney University of Sydney M C K, Sydney, Australia and University of Chile P L, Santiago
    Neurology 82:149-55. 2014
    ..To investigate patient susceptibility to neuropsychiatric symptoms in the context of progression of more classic motor symptoms in amyotrophic lateral sclerosis (ALS) and to examine the impact of neuropsychiatric symptoms on survival...
  43. pmc Effects of hemodiafiltration and high flux hemodialysis on nerve excitability in end-stage kidney disease
    Ria Arnold
    Translational Neuroscience Facility, University of New South Wales, Sydney, New South Wales, Australia
    PLoS ONE 8:e59055. 2013
    ..While high flux hemodialysis (HFHD) and hemodiafiltration (HDF) have become the preferred options for extracorporeal dialysis therapy, the effects of these treatments on nerve excitability have not yet been examined...
  44. doi request reprint Longitudinal plasticity across the neural axis in acute stroke
    William Huynh
    Neuroscience Research Australia, Randwick, New South Wales, Australia
    Neurorehabil Neural Repair 27:219-29. 2013
    ....
  45. doi request reprint Nerve compression, membrane excitability, and symptoms of carpal tunnel syndrome
    S Eric Han
    Neuroscience Research Australia and Prince of Wales Clinical School, University of New South Wales, Barker Street, Randwick, New South Wales 2031, Australia
    Muscle Nerve 44:402-9. 2011
    ..In this study we investigated the changes in axonal excitability and the generation of neurological symptoms in response to focal nerve compression (FNC) of the median nerve in carpal tunnel syndrome (CTS)...
  46. doi request reprint Axonal ion channels from bench to bedside: a translational neuroscience perspective
    Arun V Krishnan
    Translational Neuroscience Facility, University of New South Wales, Randwick, Sydney, NSW, Australia
    Prog Neurobiol 89:288-313. 2009
    ....
  47. ncbi request reprint Oxaliplatin-induced neurotoxicity and the development of neuropathy
    Arun V Krishnan
    Institute of Neurological Sciences, Prince of Wales Hospital, Sydney, NSW, Australia
    Muscle Nerve 32:51-60. 2005
    ..Differences in nerve excitability measures, particularly refractoriness, support in vitro studies indicating involvement of voltage-gated transient Na+-channel dysfunction in the development of oxaliplatin-induced neurotoxicity...
  48. doi request reprint Oxaliplatin-induced lhermitte's phenomenon as a manifestation of severe generalized neurotoxicity
    Susanna B Park
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, Sydney, NSW, Australia
    Oncology 77:342-8. 2009
    ..Limited cases have been described previously and the pathophysiology underlying Lhermitte's phenomenon in oxaliplatin-treated patients has not been established...
  49. pmc Dose effects of oxaliplatin on persistent and transient Na+ conductances and the development of neurotoxicity
    Susanna B Park
    Prince of Wales Clinical School, University of New South Wales, Sydney, New South Wales, Australia
    PLoS ONE 6:e18469. 2011
    ..Given that a Na(+) channelopathy has been proposed as the mechanism underlying acute oxaliplatin-induced neuropathy, the present study aimed to determine specific mechanisms of Na(+) channel dysfunction...
  50. ncbi request reprint Uremic neuropathy: clinical features and new pathophysiological insights
    Arun V Krishnan
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Barker Street, Randwick, Sydney, NSW 2031, Australia
    Muscle Nerve 35:273-90. 2007
    ..These recent findings suggest that maintenance of serum K(+) within normal limits between periods of dialysis, rather than simple avoidance of hyperkalemia, is likely to reduce the incidence and severity of uremic neuropathy...
  51. doi request reprint Axonal function in a family with episodic ataxia type 2 due to a novel mutation
    Arun V Krishnan
    Prince of Wales Medical Research Institute, Barker Street, Randwick Sydney, NSW 2031, Australia
    J Neurol 255:750-5. 2008
    ..These findings raise the possibility that excitability testing may prove a convenient screening test for patients with this suspected channelopathy...
  52. doi request reprint Nerve function and dysfunction in acute intermittent porphyria
    Cindy S Y Lin
    Prince of Wales Clinical School, Prince of Wales Medical Research Institute, School of Medical Sciences, University of New South Wales, Sydney, New South Wales, Australia
    Brain 131:2510-9. 2008
    ..It is proposed that porphyrin neurotoxicity causes a subclinical reduction in I(H) in AIPWN axons, whereas porphyric neuropathy may develop when reduced activity of the Na(+)/K(+) pump results in membrane depolarization...
  53. doi request reprint Activity-dependent excitability changes suggest Na+/K+ pump dysfunction in diabetic neuropathy
    Arun V Krishnan
    Prince of Wales Medical Research Institute, Prince of Wales Clinical School, University of New South Wales, Randwick, Sydney, NSW, Australia
    Brain 131:1209-16. 2008
    ..Alteration in Na(+)/K(+) pump function, coupled with reductions in nodal Na(+) currents, may be sufficient to trigger conduction failure in DN patients and are likely to contribute to the clinical symptoms of weakness and fatigue...
  54. doi request reprint Cortical atrophy in ALS is critically associated with neuropsychiatric and cognitive changes
    Eneida Mioshi
    Neuroscience Research Australia, Sydney, Australia
    Neurology 80:1117-23. 2013
    ..To characterize the patterns of brain atrophy in patients with amyotrophic lateral sclerosis (ALS) with and without cognitive and neuropsychiatric symptoms, in comparison to controls and patients with ALS-frontotemporal dementia (FTD)...
  55. doi request reprint Impact of oxaliplatin-induced neuropathy: a patient perspective
    Barbara K Bennett
    NSW Cancer Survivors Centre, University of New South Wales, Sydney, NSW, Australia
    Support Care Cancer 20:2959-67. 2012
    ..The improvement in survival for patients with early-stage colorectal cancer treated with oxaliplatin has highlighted the need for valid and reliable assessment of peripheral neuropathy...
  56. ncbi request reprint Oxaliplatin and axonal Na+ channel function in vivo
    Arun V Krishnan
    Institute of Neurological Sciences, Prince of Wales Hospital, Sydney, New South Wales, Australia
    Clin Cancer Res 12:4481-4. 2006
    ..The aim of the study was to investigate the pathophysiology of oxaliplatin-induced neurotoxicity using clinical nerve excitability techniques that provide information about axonal ion channel function...
  57. pmc Effects of axonal ion channel dysfunction on quality of life in type 2 diabetes
    Natalie C G Kwai
    School of Medical Sciences, University of New South Wales, Kensington, Australia
    Diabetes Care 36:1272-7. 2013
    ..The aim of this study was to determine the effects of axonal ion channel dysfunction on neuropathy-specific quality-of-life (QoL) measures in DN...
  58. doi request reprint Split-hand index for the diagnosis of amyotrophic lateral sclerosis
    Parvathi Menon
    Department of Neurology, Westmead Hospital, Sydney, Australia
    Clin Neurophysiol 124:410-6. 2013
    ..Preferential wasting of the thenar group of muscles, the split hand sign, appears to be a specific feature of ALS. The present study developed a novel split-hand index (SI) and assessed its diagnostic utility in ALS...
  59. pmc Abnormalities in cortical and peripheral excitability in flail arm variant amyotrophic lateral sclerosis
    Steve Vucic
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Australia
    J Neurol Neurosurg Psychiatry 78:849-52. 2007
    ..Consequently, the present study applied novel central and peripheral nerve excitability techniques to further explore disease pathophysiology in flail arm syndrome...
  60. doi request reprint Impaired energy-dependent processes underlie acute lead neuropathy
    Arun V Krishnan
    Translational Neuroscience Facility, School of Medical Sciences, University of New South Wales, Sydney, New South Wales 2052, Australia
    Muscle Nerve 46:957-61. 2012
    ..Two weeks later, he developed fasciculations, weakness, and muscle wasting. Nerve conduction studies demonstrated a motor neuropathy. A high serum lead level was identified, leading to a diagnosis of acute lead neuropathy...
  61. pmc Grey and white matter changes across the amyotrophic lateral sclerosis-frontotemporal dementia continuum
    Patricia Lillo
    Neuroscience Research Australia, Sydney, Australia
    PLoS ONE 7:e43993. 2012
    ..Nevertheless, frontal and temporal atrophy as well as corticospinal tract degeneration emerged as marker for subtype classification, which will inform future diagnosis and target disease management across the continuum...
  62. ncbi request reprint Excitability differences in lower-limb motor axons during and after ischemia
    Arun V Krishnan
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Barker Street, Randwick, Sydney, NSW 2031, Australia
    Muscle Nerve 31:205-13. 2005
    ..The greater changes in threshold in EDB during and after ischemia suggest an increased susceptibility of more distal axons to ischemia and are likely to contribute to the length-dependent development of neuropathy...
  63. doi request reprint Activity-dependent conduction failure: molecular insights
    Susanna B Park
    Prince of Wales Clinical School, University of New South Wales, Sydney, Australia
    J Peripher Nerv Syst 16:159-68. 2011
    ....
  64. ncbi request reprint Neurophysiological methodologies: diagnosis of peripheral nerve disease and assessment of pharmacological agents
    Benjamin C Cheah
    Prince of Wales Medical Research Institute, Barker Street, Randwick, NSW 2031, Australia
    Curr Opin Investig Drugs 11:72-9. 2010
    ....
  65. doi request reprint The 10-metre gait speed as a functional biomarker in amyotrophic lateral sclerosis
    Shafqat Inam
    Prince of Wales Medical Research Institute, Barker Street, Randwick, Sydney, NSW 2031, Australia
    Amyotroph Lateral Scler 11:558-61. 2010
    ..The 10-metre gait speed correlated with the total ALSFRS-R score (R = 0.6) and the gross motor subscore (R = 0.8, p <0.001). As such, the 10-metre gait speed may serve as a robust marker of disability and disease progression in ALS...
  66. doi request reprint Cortical hyperexcitability may precede the onset of familial amyotrophic lateral sclerosis
    Steve Vucic
    Prince of Wales Medical Research Institute, University of New SouthWales, Sydney, NSW 2031, Australia
    Brain 131:1540-50. 2008
    ....
  67. doi request reprint Assessment of nerve excitability in toxic and metabolic neuropathies
    Arun V Krishnan
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Randwick, Sydney, Australia
    J Peripher Nerv Syst 13:7-26. 2008
    ....
  68. ncbi request reprint Nerve excitability changes in chronic renal failure indicate membrane depolarization due to hyperkalaemia
    Matthew C Kiernan
    Sobell Department of Neurophysiology, Institute of Neurology, Queen Square, London, UK
    Brain 125:1366-78. 2002
    ..We conclude that nerves are depolarized in many CRF patients and that the depolarization is primarily due to hyperkalaemia...
  69. doi request reprint Early, progressive, and sustained dysfunction of sensory axons underlies paclitaxel-induced neuropathy
    Susanna B Park
    Prince of Wales Clinical School, University of New South Wales, Sydney, New South Wales, Australia
    Muscle Nerve 43:367-74. 2011
    ..Importantly, significant axonal dysfunction within the first month of treatment predated symptom onset, suggesting a window for neuroprotective therapies...
  70. pmc Long-term neuropathy after oxaliplatin treatment: challenging the dictum of reversibility
    Susanna B Park
    Prince of Wales Clinical School, University of New South Wales, Sydney, Australia
    Oncologist 16:708-16. 2011
    ..However, the long-term neurological sequelae have not been adequately described. The present study aimed to describe the natural history of oxaliplatin-induced neuropathy, using subjective and objective assessments...
  71. doi request reprint Defining the mechanisms that underlie cortical hyperexcitability in amyotrophic lateral sclerosis
    Steve Vucic
    Department of Neurology, Westmead Hospital and Western Clinical School, University of Sydney, Sydney, New South Wales, Australia
    Exp Neurol 220:177-82. 2009
    ..Neuroprotective strategies aimed at preserving the integrity of intracortical inhibitory circuits, in addition to antagonizing excitatory cortical circuits, may provide novel therapeutic targets in ALS...
  72. ncbi request reprint Focal and generalized peripheral nerve dysfunction in spinal cord-injured patients
    Joseph H Nogajski
    Department of Clinical Neurophysiology, Prince of Wales Hospital, Sydney, Australia
    J Clin Neurophysiol 23:273-9. 2006
    ..Maintenance of peripheral nerve function is a critical issue in all acute SCI and rehabilitation units, particularly in the context of spinal cord neuronal regeneration projects...
  73. doi request reprint Modulatory effects on axonal function after intravenous immunoglobulin therapy in chronic inflammatory demyelinating polyneuropathy
    Cindy Shin Yi Lin
    Faculty of Medicine, School of Medical Science, University of New South Wales, Sydney, New South Wales, Australia
    Arch Neurol 68:862-9. 2011
    ..To investigate the immediate and longitudinal mechanisms of action of intravenous immunoglobulin (IVIg) on axonal function in chronic inflammatory demyelinating polyneuropathy (CIDP)...
  74. ncbi request reprint Axonal function and activity-dependent excitability changes in myotonic dystrophy
    Arun V Krishnan
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Barker Street, Randwick, Sydney, NSW 2031, Australia
    Muscle Nerve 33:627-36. 2006
    ..The excitability changes and prolonged recovery of threshold following contraction are likely to contribute to symptoms of fatigue and weakness in MD patients...
  75. doi request reprint Maladaptation of cortical circuits underlies fatigue and weakness in ALS
    Steve Vucic
    Sydney Medical School Westmead, University of Sydney, Sydney, Australia
    Amyotroph Lateral Scler 12:414-20. 2011
    ..59, p < 0.05). In conclusion, maladaptation of cortical processes related to degeneration of inhibitory GABAergic intracortical circuits, is a feature of ALS that significantly correlates with development of fatigue and weakness...
  76. doi request reprint Motor neuron dysfunction in frontotemporal dementia
    James R Burrell
    Neuroscience Research Australia, Cnr Barker St and Easy St, Randwick, Sydney, NSW 2031, Australia
    Brain 134:2582-94. 2011
    ..05). Motor system dysfunction in frontotemporal dementia may result from pathological involvement of the primary motor cortex, with secondary degeneration of lower motor neurons in the brainstem and anterior horn of the spinal cord...
  77. ncbi request reprint Normal axonal ion channel function in large peripheral nerve fibers following chronic ciguatera sensitization
    Steve Vucic
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Barker Street, Randwick, Sydney, NSW 2031, Australia
    Muscle Nerve 37:403-5. 2008
    ..Excitability parameters were all within normal limits, thereby arguing against dysfunction of axonal membrane ion channels in large-diameter fibers in ciguatera sensitization...
  78. ncbi request reprint Hypokalemic weakness in hyperaldosteronism: activity-dependent conduction block
    Arun V Krishnan
    The Institute of Neurological Sciences, Prince of Wales Hospital, University of New South Wales, Randwick, Sydney, NSW, Australia
    Neurology 65:1309-12. 2005
    ..Excitability abnormalities resolved with K+ replacement. Activity-dependent conduction block induced by normal activity may contribute to weakness and paralysis developing with hypokalemia...
  79. doi request reprint Upregulation of persistent sodium conductances in familial ALS
    Steve Vucic
    Department of Neurology, Westmead Hospital and Western Clinical School, University of Sydney, Sydney, Australia
    J Neurol Neurosurg Psychiatry 81:222-7. 2010
    ..Consequently, novel threshold tracking techniques were used to assess whether upregulation of persistent Na(+) conductances was a feature linked to axonal degeneration in FALS...
  80. doi request reprint Cortical excitability testing distinguishes Kennedy's disease from amyotrophic lateral sclerosis
    Steve Vucic
    Prince of Wales Medical Research Institute, Barker Street, Randwick, Sydney, NSW 2031, Australia
    Clin Neurophysiol 119:1088-96. 2008
    ....
  81. ncbi request reprint Assessment of cortical excitability using threshold tracking techniques
    Steve Vucic
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Barker Street, Randwick, Sydney, NSW 2031, Australia
    Muscle Nerve 33:477-86. 2006
    ..Simultaneous assessment of upper and lower motor neuronal function with threshold tracking techniques may help to determine the site of disease onset and patterns of progression in neurodegenerative diseases...
  82. doi request reprint Fatigue in multiple sclerosis: mechanisms and management
    Steve Vucic
    Department of Neurology, Westmead Hospital and Western Clinical School, University of Sydney, Sydney, NSW, Australia
    Clin Neurophysiol 121:809-17. 2010
    ....
  83. ncbi request reprint Has potassium been prematurely discarded as a contributing factor to the development of uraemic neuropathy?
    Hugh Bostock
    Sobell Department of Neurophysiology, Institute of Neurology, National Hospital for Neurology and Neurosurgery, London, UK
    Nephrol Dial Transplant 19:1054-7. 2004
  84. doi request reprint Conduction block and impaired axonal function in tick paralysis
    Arun V Krishnan
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Barker Street, Randwick, Sydney, NSW, Australia
    Muscle Nerve 40:358-62. 2009
    ..The alteration in refractoriness is similar to that noted in disorders involving the terminal portion of the motor nerve. The changes raise the possibility that TP may cause weakness through impairment of distal neural transmission...
  85. doi request reprint Acute, reversible axonal energy failure during stroke-like episodes in MELAS
    Michelle A Farrar
    Neuroscience Research Australia, Barker Street, Randwick, Sydney, New South Wales 2031, Australia
    Pediatrics 126:e734-9. 2010
    ..Novel axonal excitability techniques have established acute, reversible ischemic-like depolarization that may serve as a surrogate marker of central events that develop during stroke-like episodes in MELAS...
  86. ncbi request reprint Nerve excitability properties in lower-limb motor axons: evidence for a length-dependent gradient
    Arun V Krishnan
    Institute of Neurological Sciences, Prince of Wales Hospital and Prince of Wales Medical Research Institute, University of New South Wales, Barker Street, Randwick, Sydney, NSW 2031, Australia
    Muscle Nerve 29:645-55. 2004
    ....
  87. ncbi request reprint Axonal excitability properties in hemifacial spasm
    Arun V Krishnan
    Institute of Neurological Sciences, Prince of Wales Hospital, University of New South Wales, Randwick, Sydney, New South Wales, Australia
    Mov Disord 22:1293-8. 2007
    ..It is concluded that the impulse generator underlying HFS must consequently be sited more proximally and does not cause a generalized disturbance of motor axon excitability...
  88. doi request reprint Assessing the accuracy of a combination of clinical tests for identifying carpal tunnel syndrome
    Robert A Boland
    Spinal Injuries Research Centre, Prince of Wales Medical Research Institute, Barker Street, Randwick 2031, New South Wales, Australia Faculty of Health Sciences, Discipline of Physiotherapy, University of Sydney, Sydney, NSW, Australia
    J Clin Neurosci 16:929-33. 2009
    ..60; 0.91). These data indicate that assessment of thenar sensation does not improve the diagnostic accuracy of CTS. However, a positive Phalen's test is more likely to be associated with NCS changes that are consistent with CTS...
  89. ncbi request reprint Altered nerve excitability properties in established diabetic neuropathy
    Arun V Krishnan
    Institute of Neurological Sciences, Prince of Wales Hospital, Sydney, NSW, Australia
    Brain 128:1178-87. 2005
    ....
  90. doi request reprint Neurological complications of chronic kidney disease
    Arun V Krishnan
    Translational Neuroscience Facility, School of Medical Sciences, University of New South Wales, Sydney, NSW, Australia
    Nat Rev Neurol 5:542-51. 2009
    ..The present Review dissects the pathophysiology of neurological complications related to CKD and highlights the spectrum of therapies currently available...
  91. ncbi request reprint Reversible myeloradiculopathy due to Mycoplasma pneumoniae
    Joanna Hsing
    Institute of Neurological Sciences, Prince of Wales Hospital, Sydney, Australia
    J Clin Neurosci 14:61-4. 2007
    ..The patient was treated with pulse intravenous methylprednisone and underwent complete recovery over a 4-week period...
  92. doi request reprint Guillain-Barré syndrome: an update
    Steve Vucic
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Randwick, New South Wales, Australia
    J Clin Neurosci 16:733-41. 2009
    ..This review provides an update on the epidemiology, clinical features, diagnosis, pathogenesis and treatment of GBS...
  93. doi request reprint Changes in axonal excitability and burst pattern behaviour in synkinesis
    Ann M Bacsi
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Barker Street, Randwick, Sydney, New South Wales, 2031, Australia
    J Clin Neurosci 15:1288-90. 2008
    ..Analysis of the discharge patterns in the present case suggests that synkinesis reflects aberrant regeneration with effects mediated by properties of axonal excitability that determine the burst pattern characteristics...
  94. ncbi request reprint Pathophysiology of neurodegeneration in familial amyotrophic lateral sclerosis
    Steve Vucic
    Prince of Wales Medical Research Institute and Prince of Wales Clinical School, University of New South Wales, Sydney, Australia
    Curr Mol Med 9:255-72. 2009
    ..This review provides an overview of the genetics of ALS, and describes recent advances in the understanding of the pathophysiological mechanisms underlying neurodegeneration...
  95. ncbi request reprint Assessment of disease progression in motor neuron disease
    Jennica M C Winhammar
    Department of Neurology and Multidisciplinary Motor Neurone Disease Clinic, Royal North Shore Hospital, NSW, Australia
    Lancet Neurol 4:229-38. 2005
    ..Here, we critically appraise developments in each of these areas and discuss the potential of such measures to be included in the future assessment of disease progression in patients with MND...
  96. doi request reprint Acute cortical blindness due to posterior reversible encephalopathy
    Jenny Nguyen-Lam
    Prince of Wales Clinical School, Prince of Wales Medical Research Institute, University of New South Wales Institute of Neurological Sciences, Prince of Wales Hospital, Randwick, New South Wales, Australia
    J Clin Neurosci 15:1182-5. 2008
    ..The resultant failure of compensatory vasoconstriction to prevent hyperperfusion causes fluid to extravasate into the occipital lobes, which in the present case resulted in cortical blindness...
  97. ncbi request reprint Neurotoxic marine poisoning
    Geoffrey K Isbister
    Tropical Toxicology Unit, Menzies School of Health Research, Charles Darwin University, NT, and Department of Clinical Toxicology and Pharmacology, Newcastle Mater Misericordiae Hospital, NSW, Australia
    Lancet Neurol 4:219-28. 2005
    ..Because there are no antidotes, supportive care, including mechanical ventilation in patients with severe paralysis, is the mainstay of treatment...
  98. doi request reprint Neuromuscular disease in the dialysis patient: an update for the nephrologist
    Arun V Krishnan
    Prince of Wales Medical Research Institute, Barker Street, Randwick, Sydney, NSW 2031, Australia
    Semin Dial 22:267-78. 2009
    ..Treatment with membrane-stabilizing agents, such as amitryptiline and gabapentin, are highly effective in patients with painful neuropathy...
  99. doi request reprint Cortical excitability in hereditary motor neuronopathy with pyramidal signs: comparison with ALS
    S Vucic
    Department of Neurology, Westmead Hospital, Western Clinical School, University of Sydney, Sydney, Australia
    J Neurol Neurosurg Psychiatry 81:97-100. 2010
    ....
  100. ncbi request reprint Riluzole, neuroprotection and amyotrophic lateral sclerosis
    B C Cheah
    Prince of Wales Medical Research Institute, Barker Street, Randwick, NSW 2031, Australia
    Curr Med Chem 17:1942-199. 2010
    ..Finally, the hypothesis that Na+ conductances may be involved in the processes of neuronal and axonal degeneration in ALS will be explored...
  101. ncbi request reprint Back to the future: excitability studies take on a new clinical role
    Matthew C Kiernan
    Clin Neurophysiol 117:712-3. 2006