Research Topics
Species | Matthew C KiernanSummaryAffiliation: University of New South Wales Country: Australia Publications
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Detail Information
Publications
Novel threshold tracking techniques suggest that cortical hyperexcitability is an early feature of motor neuron diseaseSteve 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...- Axonal excitability properties in amyotrophic lateral sclerosisSteve 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..Axonal excitability studies may provide insight into mechanisms responsible for motor neuron loss in ALS... - Riche-Cannieu anastomosis as an inherited traitRobert 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... 
Evolution of peripheral nerve function in humans: novel insights from motor nerve excitabilityMichelle A Farrar
M C Kiernan 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...- Progressive axonal dysfunction and clinical impairment in amyotrophic lateral sclerosisBenjamin C Cheah
Neuroscience Research Australia, Sydney, New South Wales, Australia Prince of Wales Clinical School, University of New South Wales, Sydney, New South Wales, Australia Electronic address
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... - Mitochondrial dysfunction and rod-like lesions associated with administration of beta2 adrenoceptor agonist formoterolMatthew 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... - Amyotrophic lateral sclerosisMatthew C Kiernan
Neuroscience Research Australia and Prince of Wales Clinical School, University of New South Wales, Sydney, Australia
Lancet 377:942-55. 2011.... - Adaptation of motor function after spinal cord injury: novel insights into spinal shockRobert 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... - The pathophysiology of oxaliplatin-induced neurotoxicityMatthew 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... - Mutation in the Na+ channel subunit SCN1B produces paradoxical changes in peripheral nerve excitabilityMatthew 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.... - Oxaliplatin-induced neurotoxicity: changes in axonal excitability precede development of neuropathySusanna 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... - Acute tetrodotoxin-induced neurotoxicity after ingestion of puffer fishMatthew 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... 
Differences in activity-dependent hyperpolarization in human sensory and motor axonsMatthew 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....- Characteristics of late Na(+) current in adult rat small sensory neuronsM 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... - Dexpramipexole, the R(+) enantiomer of pramipexole, for the potential treatment of amyotrophic lateral sclerosisBenjamin 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... - Inflections in threshold electrotonus to depolarizing currents in sensory axonsDavid 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... - Ischaemia induces paradoxical changes in axonal excitability in end-stage kidney diseaseArun 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... - Threshold behaviour of human axons explored using subthreshold perturbations to membrane potentialDavid 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... - Changes in human sensory axonal excitability induced by an ischaemic insultS 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... - Neuropathy, axonal Na+/K+ pump function and activity-dependent excitability changes in end-stage kidney diseaseArun V Krishnan
Institute of Neurological Sciences, Prince of Wales Hospital, Randwick, Sydney, NSW, Australia
Clin Neurophysiol 117:992-9. 2006..Alteration in Na(+)/K(+) pump function does not appear to be a contributing factor to the development of neuropathy in ESKD patients... - Fatigue and activity dependent changes in axonal excitability in amyotrophic lateral sclerosisSteve 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)... - Dysfunction of axonal membrane conductances in adolescents and young adults with spinal muscular atrophyMichelle A Farrar
Neuroscience Research Australia, Randwick, Sydney, NSW, Australia
Brain 134:3185-97. 2011.... - Corticomotoneuronal integrity and adaptation in spinal muscular atrophyMichelle 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)... - Acute abnormalities of sensory nerve function associated with oxaliplatin-induced neurotoxicitySusanna 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... - Plasticity of lower limb motor axons after cervical cord injuryRobert 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)... - Pathophysiologic insights into motor axonal function in Kennedy diseaseSteve 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... - Utilizing natural activity to dissect the pathophysiology of acute oxaliplatin-induced neuropathySusanna 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... - Altered motor nerve excitability in end-stage kidney diseaseArun 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... - FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysisIan 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... - Ischaemic sensitivity of axons in carpal tunnel syndromeS 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.... - Axonal changes in spinal cord injured patients distal to the site of injuryCindy 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... - Changes in human sensory axonal excitability induced by focal nerve compressionS Eric Han
Prince of Wales Medical Research Institute, Barker Street, Randwick, Sydney, NSW 2031 Australia
J Physiol 588:1737-45. 2010.... - Corticomotoneuronal function and hyperexcitability in acquired neuromyotoniaSteve Vucic
Neuroscience Research Australia, Barker Street, Randwick, Sydney, NSW 2031, Australia
Brain 133:2727-33. 2010.... - Regional differences in ulnar nerve excitability may predispose to the development of entrapment neuropathyArun V Krishnan
Translational Neuroscience Facility, School of Medical Sciences, University of New South Wales, Australia
Clin Neurophysiol 122:194-8. 2011.... - Neurophysiological index as a biomarker for ALS progression: validity of mixed effects modelsBenjamin 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... - Activity-dependent excitability changes suggest Na+/K+ pump dysfunction in diabetic neuropathyArun 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... - Axonal ion channels from bench to bedside: a translational neuroscience perspectiveArun V Krishnan
Translational Neuroscience Facility, University of New South Wales, Randwick, Sydney, NSW, Australia
Prog Neurobiol 89:288-313. 2009.... - Nerve compression, membrane excitability, and symptoms of carpal tunnel syndromeS 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)... - Axonal function in a family with episodic ataxia type 2 due to a novel mutationArun 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... - Oxaliplatin-induced lhermitte's phenomenon as a manifestation of severe generalized neurotoxicitySusanna 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... - Dose effects of oxaliplatin on persistent and transient Na+ conductances and the development of neurotoxicitySusanna 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... - Uremic neuropathy: clinical features and new pathophysiological insightsArun 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... - Oxaliplatin-induced neurotoxicity and the development of neuropathyArun 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... - Oxaliplatin and axonal Na+ channel function in vivoArun V Krishnan
Institute of Neurological Sciences, Prince of Wales Hospital, Sydney, New South Wales, Australia
Clin Cancer Res 12:4481-4. 2006..Clinical nerve excitability techniques may prove beneficial in monitoring for early signs of neurotoxicity and in the assessment of future prophylactic therapies... - Excitability differences in lower-limb motor axons during and after ischemiaArun 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... - Activity-dependent conduction failure: molecular insightsSusanna B Park
Prince of Wales Clinical School, University of New South Wales, Sydney, Australia
J Peripher Nerv Syst 16:159-68. 2011.... - The 10-metre gait speed as a functional biomarker in amyotrophic lateral sclerosisShafqat 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... - Modulatory effects on axonal function after intravenous immunoglobulin therapy in chronic inflammatory demyelinating polyneuropathyCindy 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)... - Defining the mechanisms that underlie cortical hyperexcitability in amyotrophic lateral sclerosisSteve 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... - Long-term neuropathy after oxaliplatin treatment: challenging the dictum of reversibilitySusanna 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... - Early, progressive, and sustained dysfunction of sensory axons underlies paclitaxel-induced neuropathySusanna 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... - Neurophysiological methodologies: diagnosis of peripheral nerve disease and assessment of pharmacological agentsBenjamin C Cheah
Prince of Wales Medical Research Institute, Barker Street, Randwick, NSW 2031, Australia
Curr Opin Investig Drugs 11:72-9. 2010.... - Focal and generalized peripheral nerve dysfunction in spinal cord-injured patientsJoseph 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... - Assessment of nerve excitability in toxic and metabolic neuropathiesArun 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.... - Cortical hyperexcitability may precede the onset of familial amyotrophic lateral sclerosisSteve Vucic
Prince of Wales Medical Research Institute, University of New SouthWales, Sydney, NSW 2031, Australia
Brain 131:1540-50. 2008.... - Evidence for axonal membrane hyperpolarization in multifocal motor neuropathy with conduction blockMatthew 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... - Axonal function and activity-dependent excitability changes in myotonic dystrophyArun 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... - Nerve excitability changes in chronic renal failure indicate membrane depolarization due to hyperkalaemiaMatthew 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... - Nerve function and dysfunction in acute intermittent porphyriaCindy 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... - Maladaptation of cortical circuits underlies fatigue and weakness in ALSSteve 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... - Normal axonal ion channel function in large peripheral nerve fibers following chronic ciguatera sensitizationSteve 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... - Motor neuron dysfunction in frontotemporal dementiaJames 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... - Abnormalities in cortical and peripheral excitability in flail arm variant amyotrophic lateral sclerosisSteve 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... - Hypokalemic weakness in hyperaldosteronism: activity-dependent conduction blockArun 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... - Fatigue in multiple sclerosis: mechanisms and managementSteve Vucic
Department of Neurology, Westmead Hospital and Western Clinical School, University of Sydney, Sydney, NSW, Australia
Clin Neurophysiol 121:809-17. 2010.... - Assessment of cortical excitability using threshold tracking techniquesSteve 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... - Cortical excitability testing distinguishes Kennedy's disease from amyotrophic lateral sclerosisSteve Vucic
Prince of Wales Medical Research Institute, Barker Street, Randwick, Sydney, NSW 2031, Australia
Clin Neurophysiol 119:1088-96. 2008.... - Upregulation of persistent sodium conductances in familial ALSSteve 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... - 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 - Conduction block and impaired axonal function in tick paralysisArun 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... - Acute, reversible axonal energy failure during stroke-like episodes in MELASMichelle 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... - Assessing the accuracy of a combination of clinical tests for identifying carpal tunnel syndromeRobert 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... - Altered nerve excitability properties in established diabetic neuropathyArun V Krishnan
Institute of Neurological Sciences, Prince of Wales Hospital, Sydney, NSW, Australia
Brain 128:1178-87. 2005.... - Axonal excitability properties in hemifacial spasmArun 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... - Nerve excitability properties in lower-limb motor axons: evidence for a length-dependent gradientArun 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.... - Neurological complications of chronic kidney diseaseArun 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... - Reversible myeloradiculopathy due to Mycoplasma pneumoniaeJoanna 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... - Guillain-Barré syndrome: an updateSteve 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... - Changes in axonal excitability and burst pattern behaviour in synkinesisAnn 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... - Pathophysiology of neurodegeneration in familial amyotrophic lateral sclerosisSteve 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... - Assessment of disease progression in motor neuron diseaseJennica 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... - Acute cortical blindness due to posterior reversible encephalopathyJenny 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... - Neurotoxic marine poisoningGeoffrey 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... - Neuromuscular disease in the dialysis patient: an update for the nephrologistArun 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... - Cortical excitability in hereditary motor neuronopathy with pyramidal signs: comparison with ALSS Vucic
Department of Neurology, Westmead Hospital, Western Clinical School, University of Sydney, Sydney, Australia
J Neurol Neurosurg Psychiatry 81:97-100. 2010.... - Riluzole, neuroprotection and amyotrophic lateral sclerosisB 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... - Puffer fish poisoning: a potentially life-threatening conditionGeoffrey K Isbister
Discipline of Clinical Pharmacology, University of Newcastle, Newcastle Mater Misericordiae Hospital, Waratah, NSW
Med J Aust 177:650-3. 2002..Symptoms of poisoning may include paralysis, respiratory failure, numbness, paraesthesia, nausea and ataxia. Health professionals should be aware of the condition so as to institute early and appropriate management... - The pain with platinum: oxaliplatin and neuropathyMatthew C Kiernan
Eur J Cancer 43:2631-3. 2007 - Activity-induced weakness in recessive myotonia congenita with a novel (696+1G>A) mutationOwen M McKay
Faculty of Science, Australian National University, Canberra, Australia
Clin Neurophysiol 117:2064-8. 2006..To investigate the cause of the transient weakness that occurs in recessive myotonia congenita (RMC) following sustained muscle contraction... - Back to the future: excitability studies take on a new clinical roleMatthew C Kiernan
Clin Neurophysiol 117:712-3. 2006 - Paraspinal muscles and amyotrophic lateral sclerosis - getting to the core?Matthew C Kiernan
Clin Neurophysiol 119:1457-8. 2008 - Cytoplasmic body myopathy masquerading as motor neuron diseaseArun V Krishnan
Multidisciplinary Motor Neuron Disease Service, Institute of Neurological Sciences, Prince of Wales Hospital, High Street, Randwick, Sydney, NSW 2031, Australia
Muscle Nerve 30:667-72. 2004..The case highlights a further diagnostic possibility in the assessment of patients with "possible" motor neuron disease. The clinical features of CBM are briefly reviewed...