Jeremy M Shefner

Summary

Affiliation: SUNY Upstate Medical University
Country: USA

Publications

  1. doi request reprint Safety, tolerability and pharmacodynamics of a skeletal muscle activator in amyotrophic lateral sclerosis
    Jeremy Shefner
    Department of Neurology, Upstate Medical University, Syracuse, NY 13210 2306, USA
    Amyotroph Lateral Scler 13:430-8. 2012
  2. ncbi request reprint 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
  3. ncbi request reprint Motor unit number estimation predicts disease onset and survival in a transgenic mouse model of amyotrophic lateral sclerosis
    Jeremy M Shefner
    Department of Neurology, Upstate Medical University, Syracuse, New York 13210, USA
    Muscle Nerve 34:603-7. 2006
  4. ncbi request reprint 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
  5. ncbi request reprint Motor unit number estimation
    Jeremy M Shefner
    Clinical Neurophysiology Laboratory, MDA ALS Research and Treatment Center, Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210, USA
    Phys Med Rehabil Clin N Am 14:243-60. 2003
  6. doi request reprint Designing clinical trials in amyotrophic lateral sclerosis
    Jeremy M Shefner
    Department of Neurology, State University of New York Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210, USA
    Phys Med Rehabil Clin N Am 19:495-508, ix. 2008
  7. doi request reprint Commentary on 'a novel, efficient, randomized selection trial comparing combinations of drug therapy for ALS'
    Jeremy M Shefner
    Department of Neurology, SUNY Upstate Medical University, Syracuse, New York 13210, USA
    Amyotroph Lateral Scler 9:254-6. 2008
  8. ncbi request reprint Statistical motor unit number estimation and ALS trials: the effect of motor unit instability
    Jeremy M Shefner
    Department of Neurology, SUNY Upstate Medical University, Syracuse, NY 13210, USA
    Suppl Clin Neurophysiol 60:135-41. 2009
  9. ncbi request reprint Recent MUNE studies in animal models of motor neuron disease
    Jeremy M Shefner
    Department of Neurology, SUNY Upstate Medical University, Syracuse, NY 13210, USA
    Suppl Clin Neurophysiol 60:203-8. 2009
  10. pmc 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

Collaborators

Detail Information

Publications37

  1. doi request reprint Safety, tolerability and pharmacodynamics of a skeletal muscle activator in amyotrophic lateral sclerosis
    Jeremy Shefner
    Department of Neurology, Upstate Medical University, Syracuse, NY 13210 2306, USA
    Amyotroph Lateral Scler 13:430-8. 2012
    ..Measures of endurance appear to be improved in a dose-related fashion, and both patients and investigators perceived a global benefit. Further study of this agent is warranted...
  2. ncbi request reprint 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...
  3. ncbi request reprint Motor unit number estimation predicts disease onset and survival in a transgenic mouse model of amyotrophic lateral sclerosis
    Jeremy M Shefner
    Department of Neurology, Upstate Medical University, Syracuse, New York 13210, USA
    Muscle Nerve 34:603-7. 2006
    ..This suggests that MUNE has potential efficacy as a useful functional outcome measure in both animal and human studies of ALS...
  4. ncbi request reprint 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...
  5. ncbi request reprint Motor unit number estimation
    Jeremy M Shefner
    Clinical Neurophysiology Laboratory, MDA ALS Research and Treatment Center, Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210, USA
    Phys Med Rehabil Clin N Am 14:243-60. 2003
    ..MUNE offers promise in the study of neuromuscular disease, enabling the collection of novel data in the living patient unobtainable by any other method...
  6. doi request reprint Designing clinical trials in amyotrophic lateral sclerosis
    Jeremy M Shefner
    Department of Neurology, State University of New York Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210, USA
    Phys Med Rehabil Clin N Am 19:495-508, ix. 2008
    ..While no design achieves the perfect balance of sensitivity and efficiency, clinical trialists continue to work toward the goals of smaller and shorter trials so that more compounds can be studied concurrently...
  7. doi request reprint Commentary on 'a novel, efficient, randomized selection trial comparing combinations of drug therapy for ALS'
    Jeremy M Shefner
    Department of Neurology, SUNY Upstate Medical University, Syracuse, New York 13210, USA
    Amyotroph Lateral Scler 9:254-6. 2008
  8. ncbi request reprint Statistical motor unit number estimation and ALS trials: the effect of motor unit instability
    Jeremy M Shefner
    Department of Neurology, SUNY Upstate Medical University, Syracuse, NY 13210, USA
    Suppl Clin Neurophysiol 60:135-41. 2009
    ..However, MUNE as a theoretical outcome measure remains extremely attractive and new techniques must be sought so that it can reach its full potential as a valid and reproducible outcome measure in ALS clinical trials...
  9. ncbi request reprint Recent MUNE studies in animal models of motor neuron disease
    Jeremy M Shefner
    Department of Neurology, SUNY Upstate Medical University, Syracuse, NY 13210, USA
    Suppl Clin Neurophysiol 60:203-8. 2009
    ..Even in models that have subtle or imperceptible phenotypes, MUNE can show reductions in motor unit compliment. Thus, it is a valuable addition to the armamentarium of investigative tools when studying models of motor unit loss...
  10. pmc 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...
  11. ncbi request reprint Comparison of incremental with multipoint MUNE methods in transgenic ALS mice
    Jeremy M Shefner
    Department of Neurology, Upstate Medical University, 750 East Adams Street, Syracuse, New York 13210, USA
    Muscle Nerve 25:39-42. 2002
    ..71 for single motor unit action potential (SMUAP) amplitude and 0.95 for MUNE. In this model, therefore, both MUNE methods yield similar estimates and are equally effective at documenting progression of a lower motor neuron disorder...
  12. ncbi request reprint 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...
  13. ncbi request reprint Degeneration of respiratory motor neurons in the SOD1 G93A transgenic rat model of ALS
    Jeronia Llado
    Department of Neurology, Johns Hopkins University 600 N Wolfe Street, Meyer 6 119, Baltimore, MD 21287, USA
    Neurobiol Dis 21:110-8. 2006
    ..Taken together, these data suggest that respiratory motor neuron loss results in significant electrophysiologic changes and diaphragmatic atrophy. These changes may play a significant role resulting in death of these animals...
  14. pmc Electrical impedance myography as a biomarker to assess ALS progression
    Seward B Rutkove
    Department of Neurology, Beth Israel Deaconess Medical Center, Boston, Massachusetts 02215, USA
    Amyotroph Lateral Scler 13:439-45. 2012
    ..In conclusion, EIM can serve as a useful ALS biomarker that offers the prospect of greatly accelerating phase 2 clinical trials...
  15. pmc Electrical impedance myography to assess outcome in amyotrophic lateral sclerosis clinical trials
    Seward B Rutkove
    Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA
    Clin Neurophysiol 118:2413-8. 2007
    ..This study investigates whether EIM could provide a new outcome measure for use in ALS clinical trials work...
  16. ncbi request reprint Motor unit number estimation: summary
    Jeremy M Shefner
    SUNY Upstate Medical University, New York, USA
    Amyotroph Lateral Scler Other Motor Neuron Disord 3:S97-102. 2002
  17. doi request reprint Motor unit number estimation in the rat tail using a modified multipoint stimulation technique
    Lora J Kasselman
    Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, 330 Brookline Avenue, CCE 807, Boston, Massachusetts 02215, USA
    Muscle Nerve 40:115-21. 2009
    ....
  18. ncbi request reprint Motor unit number estimation in the evaluation of focal conduction block
    Devanand Jillapalli
    Department of Neurology, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, New York 13210, USA
    Muscle Nerve 27:676-81. 2003
    ....
  19. ncbi request reprint Single motor unit variability with threshold stimulation in patients with amyotrophic lateral sclerosis and normal subjects
    Devanand Jillapalli
    Department of Neurology, Upstate Medical University, 750 E Adams Street, Syracuse, New York 13210, USA
    Muscle Nerve 30:578-84. 2004
    ....
  20. ncbi request reprint Evaluation of patients with recurrent symptoms after ulnar nerve transposition
    Cristina I Matei
    Department of Neurology, Upstate Medical University, 750 East Adams Street, Syracuse, New York 13210, USA
    Muscle Nerve 30:493-6. 2004
    ..The prevalence of this medial location of the ulnar nerve in asymptomatic postsurgical patients is unknown...
  21. doi request reprint Characterizing spinal muscular atrophy with electrical impedance myography
    Seward B Rutkove
    Department of Neurology, Beth Israel Deaconess Medical Center, Shapiro 810, 330 Brookline Avenue, Boston, Massachusetts 02215, USA
    Muscle Nerve 42:915-21. 2010
    ..These results confirm that EIM can accurately categorize patients with SMA. Because EIM requires no patient effort and is rapid to apply, it may serve a useful role in future SMA clinical trials...
  22. ncbi request reprint Motor unit number estimation in neurologic disease
    Jeremy M Shefner
    Department of Neurology, Clinical Neurophysiology Laboratory and MDA ALS Research and Treatment Center, Upstate Medical University, State University of New York, Syracuse, New York 13210, USA
    Adv Neurol 88:33-52. 2002
    ..MUNE is a technology offering important promise in the study of neuromuscular disease, enabling the collection of novel data in the living patient unobtainable by any other method...
  23. ncbi request reprint 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
    ....
  24. ncbi request reprint 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...
  25. ncbi request reprint 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...
  26. ncbi request reprint 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...
  27. doi request reprint The ALS Nutrition/NIPPV Study: design, feasibility, and initial results
    Edward J Kasarskis
    Department of Neurology, University of Kentucky, Lexington, Kentucky, USA
    Amyotroph Lateral Scler 12:17-25. 2011
    ..The results of this study will allow us to model equations to predict the energy requirements in ALS and to plan a study of early intervention with NIPPV...
  28. ncbi request reprint Electrodiagnosis in common mononeuropathies and plexopathies
    Devanand Jillapalli
    Department of Neurology, Upstate Medical University, Syracuse, New York 13210, USA
    Semin Neurol 25:196-203. 2005
    ..The relevant anatomy and pathology of these disorders is reviewed in the context of electrodiagnosis and prognosis...
  29. ncbi request reprint Multi-drug therapy in amyotrophic lateral sclerosis: combinations of multiple, untested drugs should not be used at this time
    Jeremy M Shefner
    Department of Neurology, SUNY Upstate Medical University, Syracuse, New York, USA
    Muscle Nerve 30:676-8. 2004
  30. ncbi request reprint 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...
  31. ncbi request reprint Genetic ablation of NMDA receptor subunit NR3B in mouse reveals motoneuronal and nonmotoneuronal phenotypes
    Stephan Niemann
    RIKEN MIT Neuroscience Research Center, The Picower Institute for Learning and Memory, Department of Brain and Cognitive Sciences, Massachusetts Institute of Technology, 77 Massachusetts Avenue, Cambridge, MA 02139, USA
    Eur J Neurosci 26:1407-20. 2007
    ..This work is the first reporting of the functional significance of NR3B in vivo and may give insight into the contribution of genetic variability of NR3B in the phenotypic heterogeneity among human population...
  32. ncbi request reprint Trial of celecoxib in amyotrophic lateral sclerosis
    Merit E Cudkowicz
    Neurology Clinical Trials Unit, Massachusetts General Hospital, Harvard Medical School, Boston, 02172, USA
    Ann Neurol 60:22-31. 2006
    ..To determine whether chronic treatment with celecoxib, a cyclooxygenase-2 inhibitor that has been shown to be beneficial in preclinical testing, is safe and effective in amyotrophic lateral sclerosis (ALS)...
  33. ncbi request reprint Mice deficient in the Rab5 guanine nucleotide exchange factor ALS2/alsin exhibit age-dependent neurological deficits and altered endosome trafficking
    Shinji Hadano
    Department of Molecular Neuroscience, The Institute of Medical Sciences, Tokai University School of Medicine, Isehara, Kanagawa 259 1193, Japan
    Hum Mol Genet 15:233-50. 2006
    ....
  34. ncbi request reprint Lead exposure and amyotrophic lateral sclerosis
    Freya Kamel
    National Institute of Environmental Health Sciences, Research Triangle Park, NC 27709, USA
    Epidemiology 13:311-9. 2002
    ..Previous interview-based studies have suggested that exposure to neurotoxicants including metals might be related to ALS...
  35. pmc Amyotrophic lateral sclerosis, lead, and genetic susceptibility: polymorphisms in the delta-aminolevulinic acid dehydratase and vitamin D receptor genes
    Freya Kamel
    National Institute of Environmental Health Sciences, Research Triangle Park, North Carolina 27709, USA
    Environ Health Perspect 111:1335-9. 2003
    ..These findings suggest that genetic susceptibility conferred by polymorphisms in ALAD may affect ALS risk, possibly through a mechanism related to internal lead exposure...
  36. doi request reprint Arimoclomol at dosages up to 300 mg/day is well tolerated and safe in amyotrophic lateral sclerosis
    Merit E Cudkowicz
    Neurology Clinical Trials Unit, Massachusetts General Hospital, 13th Street, Charlestown, MA 02129, USA
    Muscle Nerve 38:837-44. 2008
    ..Arimoclomol CSF levels increased with dose. Arimoclomol was shown to be safe, and it crosses the blood-brain barrier. Serum pharmacokinetic profiles support dosing of three times per day. An efficacy study in ALS is planned...
  37. pmc Amyotrophic lateral sclerosis: an emerging era of collaborative gene discovery
    Katrina Gwinn
    National Institute for Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, United States of America
    PLoS ONE 2:e1254. 2007
    ..This resource should facilitate genetic discoveries which we anticipate will ultimately provide a better understanding of the biological mechanisms of neurodegeneration in ALS...