J M Shefner

Summary

Affiliation: SUNY Upstate Medical University
Country: USA

Publications

  1. ncbi The use of statistical MUNE in a multicenter clinical trial
    J M Shefner
    Department of Neurology, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, New York 13210, USA
    Muscle Nerve 30:463-9. 2004
  2. ncbi Effect of neurophilin ligands on motor units in mice with SOD1 ALS mutations
    J M Shefner
    Department of Neurology, Upstate Medical University, Syracuse, NY 13210, USA
    Neurology 57:1857-61. 2001
  3. ncbi Revised statistical motor unit number estimation in the Celecoxib/ALS trial
    J M Shefner
    Department of Neurology, SUNY Upstate Medical University, Syracuse, New York 13210, USA
    Muscle Nerve 35:228-34. 2007
  4. ncbi Multipoint incremental motor unit number estimation as an outcome measure in ALS
    J M Shefner
    Department of Neurology, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210, USA
    Neurology 77:235-41. 2011
  5. ncbi Motor unit number estimation in human neurological diseases and animal models
    J M Shefner
    Department of Neurology, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210, USA
    Clin Neurophysiol 112:955-64. 2001
  6. ncbi A clinical trial of creatine in ALS
    J M Shefner
    Department of Neurology, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, NY 13104, USA
    Neurology 63:1656-61. 2004
  7. ncbi Functional outcome measures as clinical trial endpoints in ALS
    B J Traynor
    Neurology Clinical Trials Unit, Department of Neurology, Massachusetts General Hospital, Boston, MA, USA
    Neurology 63:1933-5. 2004
  8. ncbi Mice lacking cytosolic copper/zinc superoxide dismutase display a distinctive motor axonopathy
    J M Shefner
    Department of Neurology, State University of New York Health Science Center at Syracuse, 13210, USA
    Neurology 53:1239-46. 1999
  9. ncbi The use of non-invasive positive pressure ventilation (NIPPV) in ALS patients. A need for improved determination of intervention timing
    E D Sivak
    State University of New York, Upstate Medical University, Syracuse 13210, USA
    Amyotroph Lateral Scler Other Motor Neuron Disord 2:139-45. 2001
  10. ncbi A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis
    M E Cudkowicz
    Neurology Clinical Trials Unit, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
    Neurology 61:456-64. 2003

Detail Information

Publications13

  1. ncbi The use of statistical MUNE in a multicenter clinical trial
    J M Shefner
    Department of Neurology, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, New York 13210, USA
    Muscle Nerve 30:463-9. 2004
    ..Thus, MUNE can be used reliably as an outcome measure in multicenter clinical trials; specific remedies are suggested for the difficulties encountered in this study...
  2. ncbi Effect of neurophilin ligands on motor units in mice with SOD1 ALS mutations
    J M Shefner
    Department of Neurology, Upstate Medical University, Syracuse, NY 13210, USA
    Neurology 57:1857-61. 2001
    ..Mice with trangenes that express mutations in the gene for cytosolic copper/zinc superoxide dismutase (SOD1) develop motor neuron degeneration resembling human ALS. Neurophilin ligands are small molecules that promote neurite outgrowth...
  3. ncbi Revised statistical motor unit number estimation in the Celecoxib/ALS trial
    J M Shefner
    Department of Neurology, SUNY Upstate Medical University, Syracuse, New York 13210, USA
    Muscle Nerve 35:228-34. 2007
    ..Thus, we conclude that the statistical method is not an appropriate measure of motor unit number in any disease associated with motor unit instability...
  4. ncbi Multipoint incremental motor unit number estimation as an outcome measure in ALS
    J M Shefner
    Department of Neurology, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210, USA
    Neurology 77:235-41. 2011
    ..Motor unit number estimation (MUNE) is a potentially attractive tool. MUNE methods previously employed in multicenter trials exhibited excessive variability and were prone to artifact...
  5. ncbi Motor unit number estimation in human neurological diseases and animal models
    J M Shefner
    Department of Neurology, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210, USA
    Clin Neurophysiol 112:955-64. 2001
    ..Despite the lack of a perfect method for performing MUNE, it has great clinical value in the assessment of progressive motor axon loss. Further refinements in the method will likely increase its utility in the future...
  6. ncbi A clinical trial of creatine in ALS
    J M Shefner
    Department of Neurology, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, NY 13104, USA
    Neurology 63:1656-61. 2004
    ..Creatine stabilizes the mitochondrial transition pore, and is important in mitochondrial ATP production. In a transgenic mouse model of ALS, administration of creatine prolongs survival and preserves motor function and motor neurons...
  7. ncbi Functional outcome measures as clinical trial endpoints in ALS
    B J Traynor
    Neurology Clinical Trials Unit, Department of Neurology, Massachusetts General Hospital, Boston, MA, USA
    Neurology 63:1933-5. 2004
    ..Rates of FVC% and ALFRS decline, but not of MVIC-arm or MVIC-grip, were independent predictors of survival...
  8. ncbi Mice lacking cytosolic copper/zinc superoxide dismutase display a distinctive motor axonopathy
    J M Shefner
    Department of Neurology, State University of New York Health Science Center at Syracuse, 13210, USA
    Neurology 53:1239-46. 1999
    ..To characterize the motor neuron dysfunction in two models by performing physiologic and morphometric studies...
  9. ncbi The use of non-invasive positive pressure ventilation (NIPPV) in ALS patients. A need for improved determination of intervention timing
    E D Sivak
    State University of New York, Upstate Medical University, Syracuse 13210, USA
    Amyotroph Lateral Scler Other Motor Neuron Disord 2:139-45. 2001
    ....
  10. ncbi A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis
    M E Cudkowicz
    Neurology Clinical Trials Unit, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
    Neurology 61:456-64. 2003
    ..To determine if long-term topiramate therapy is safe and slows disease progression in patients with ALS...
  11. ncbi Percutaneous endoscopic gastrostomy (PEG) in patients with ALS and bulbar dysfunction
    H Mitsumoto
    Elenor and Lou Gehrig MDA ALS Research Center, The Neurologic Institute, Columbia University, New York 10032, USA
    Amyotroph Lateral Scler Other Motor Neuron Disord 4:177-85. 2003
    ..To compare characteristics of ALS patients with and without percutaneous endoscopic gastrostomy (PEG)...
  12. ncbi ALS surrogate markers. MUNE
    C L Gooch
    Columbia College of Physicians and Surgeons, Columbia Presbyterian Medical Center, New York, NY 10032, USA
    Amyotroph Lateral Scler Other Motor Neuron Disord 5:104-7. 2004
    ..As MUNE methods continue to be refined and disseminated, they are proving to be useful and unique tools for the study of motor neuron disease...
  13. ncbi Lead exposure as a risk factor for amyotrophic lateral sclerosis
    F Kamel
    National Institute of Environmental Health Sciences, National Institutes of Health, Department of Health and Human Services, Research Triangle Park, NC 27709, USA
    Neurodegener Dis 2:195-201. 2005
    ..Most previous case-control studies found an association of ALS with self-reported occupational exposure to lead, with increased risks of 2- to >4-fold. However, these results may have been affected by recall bias...