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Richard Moxley

TitleProfessor
InstitutionSchool of Medicine and Dentistry
DepartmentNeurology
AddressUniversity of Rochester Medical Center
School of Medicine and Dentistry
601 Elmwood Ave, Box 673
Rochester NY 14642
 
 Awards And Honors
1992     Elected by his peers for inclusion in Best Doctors in America  | America's Best Doctors
2005     Humanitarian of the Year Award  | The Rochester MDA for Research & Care of Patients
2006     1st Annual Excellence in Research Day - Honored for outstanding research and contributions  | University of Rochester
2007     Lifetime Achievement Award for Research and Treatment in Myotonic Dystrophy  | Myotonic Dystrophy Foundation
2008     Distinguished Faculty Award  | University of Rochester
2011     Distinguished Service Award  | Board of Trustees of the Rochester Academy of Medicine
2011     "Top Doctors"  | US News and World Report's
2013     Best Doctors  | America's Best Doctors
 
 Overview
Richard T. Moxley, III, M.D.

Contact Information

University of Rochester Medical Center
School of Medicine and Dentistry
601 Elmwood Ave., Box 673
Rochester, NY 14642

Appointment: (585) 275-1200

Admits to Strong Memorial Hospital

Patient Care Bio

Dr. Moxley is Professor of Neurology and Pediatrics at the University of Rochester School of Medicine and Dentistry. He has served as an Associate Chair of the Department of Neurology since 1997 and served as Acting Chair for several months in 2008. He also holds the Helen Aresty Fine and Irving Fine Chair in Neurology at the University of Rochester. He received his BA from Harvard College and his M.D. from the University of Pennsylvania. He completed internship at Pennsylvania Hospital, received two years of exercise cardiology training in the Heart Disease & Stroke Program of NIH and served during this time as an instructor in exercise physiology at George Washington University School of medicine. Subsequently, Dr. Moxley completed his residency in neurology at the Harvard Longwood Program, and completed a fellowship in Medicine (Endocrinology & Metabolism) at Johns Hopkins Medical Center before joining the faculty of the Department of Neurology at the University of Rochester Medical Center in 1974.

 
 Selected Publications
List All   |   Timeline
  1. Gadalla SM, Pfeiffer RM, Kristinsson SY, Björkholm M, Hilbert JE, Moxley RT, Landgren O, Greene MH. Quantifying cancer absolute risk and cancer mortality in the presence of competing events after a myotonic dystrophy diagnosis. PLoS One. 2013; 8(11):e79851.
    View in: PubMed
  2. Hilbert JE, Ashizawa T, Day JW, Luebbe EA, Martens WB, McDermott MP, Tawil R, Thornton CA, Moxley RT. Diagnostic odyssey of patients with myotonic dystrophy. J Neurol. 2013 Oct; 260(10):2497-504.
    View in: PubMed
  3. Kalman L, Tarleton J, Hitch M, Hegde M, Hjelm N, Berry-Kravis E, Zhou L, Hilbert JE, Luebbe EA, Moxley RT, Toji L. Development of a Genomic DNA Reference Material Panel for Myotonic Dystrophy Type 1 (DM1) Genetic Testing. J Mol Diagn. 2013 Jul; 15(4):518-25.
    View in: PubMed
  4. Hilbert JE, Johnson NE, Moxley RT. New insights about the incidence, multisystem manifestations, and care of patients with congenital myotonic dystrophy. J Pediatr. 2013 Jul; 163(1):12-4.
    View in: PubMed
  5. Landis SC, Amara SG, Asadullah K, Austin CP, Blumenstein R, Bradley EW, Crystal RG, Darnell RB, Ferrante RJ, Fillit H, Finkelstein R, Fisher M, Gendelman HE, Golub RM, Goudreau JL, Gross RA, Gubitz AK, Hesterlee SE, Howells DW, Huguenard J, Kelner K, Koroshetz W, Krainc D, Lazic SE, Levine MS, Macleod MR, McCall JM, Moxley RT, Narasimhan K, Noble LJ, Perrin S, Porter JD, Steward O, Unger E, Utz U, Silberberg SD. A call for transparent reporting to optimize the predictive value of preclinical research. Nature. 2012 Oct 11; 490(7419):187-91.
    View in: PubMed
  6. Heatwole C, Bode R, Johnson N, Quinn C, Martens W, McDermott MP, Rothrock N, Thornton C, Vickrey B, Victorson D, Moxley R. Patient-reported impact of symptoms in myotonic dystrophy type 1 (PRISM-1). Neurology. 2012 Jul 24; 79(4):348-57.
    View in: PubMed
  7. Das M, Moxley RT, Hilbert JE, Martens WB, Letren L, Greene MH, Gadalla SM. Correlates of tumor development in patients with myotonic dystrophy. J Neurol. 2012 Oct; 259(10):2161-6.
    View in: PubMed
  8. Gadalla SM, Lund M, Pfeiffer RM, Gørtz S, Mueller CM, Moxley RT, Kristinsson SY, Björkholm M, Shebl FM, Hilbert JE, Landgren O, Wohlfahrt J, Melbye M, Greene MH. Cancer risk among patients with myotonic muscular dystrophy. JAMA. 2011 Dec 14; 306(22):2480-6.
    View in: PubMed
  9. Tang ZZ, Yarotskyy V, Wei L, Sobczak K, Nakamori M, Eichinger K, Moxley RT, Dirksen RT, Thornton CA. Muscle weakness in myotonic dystrophy associated with misregulated splicing and altered gating of Ca(V)1.1 calcium channel. Hum Mol Genet. 2012 Mar 15; 21(6):1312-24.
    View in: PubMed
  10. Hilbert JE, Kissel JT, Luebbe EA, Martens WB, McDermott MP, Sanders DB, Tawil R, Thornton CA, Moxley RT. If you build a rare disease registry, will they enroll and will they use it? Methods and data from the National Registry of Myotonic Dystrophy (DM) and Facioscapulohumeral Muscular Dystrophy (FSHD). Contemp Clin Trials. 2012 Mar; 33(2):302-11.
    View in: PubMed
  11. Rubinstein YR, Groft SC, Chandros SH, Kaneshiro J, Karp B, Lockhart NC, Marshall PA, Moxley RT, Pollen GB, Miller VR, Schwartz J. Informed consent process for patient participation in rare disease registries linked to biorepositories. Contemp Clin Trials. 2012 Jan; 33(1):5-11.
    View in: PubMed
  12. Heatwole C, Johnson N, Goldberg B, Martens W, Moxley R. Laboratory abnormalities in patients with myotonic dystrophy type 2. Arch Neurol. 2011 Sep; 68(9):1180-4.
    View in: PubMed
  13. Moxley RT, Pandya S. Weekend high-dosage prednisone: a new option for treatment of Duchenne muscular dystrophy. Neurology. 2011 Aug 2; 77(5):416-7.
    View in: PubMed
  14. Udd B, Meola G, Krahe R, Wansink DG, Bassez G, Kress W, Schoser B, Moxley R. Myotonic dystrophy type 2 (DM2) and related disorders report of the 180th ENMC workshop including guidelines on diagnostics and management 3-5 December 2010, Naarden, The Netherlands. Neuromuscul Disord. 2011 Jun; 21(6):443-50.
    View in: PubMed
  15. Pearson TA, Fogg TT, Bennett N, Kieburtz K, Kitzman H, Moxley R, Puzas E. Building capacity across the spectrum of research translation: centers of excellence within the Rochester Clinical and Translational Science Institute. Clin Transl Sci. 2010 Dec; 3(6):272-4.
    View in: PubMed
  16. Gagnon C, Chouinard MC, Laberge L, Veillette S, Bégin P, Breton R, Jean S, Brisson D, Gaudet D, Mathieu J. Health supervision and anticipatory guidance in adult myotonic dystrophy type 1. Neuromuscul Disord. 2010 Dec; 20(12):847-51.
    View in: PubMed
  17. Heatwole CR, Eichinger KJ, Friedman DI, Hilbert JE, Jackson CE, Logigian EL, Martens WB, McDermott MP, Pandya SK, Quinn C, Smirnow AM, Thornton CA, Moxley RT. Open-label trial of recombinant human insulin-like growth factor 1/recombinant human insulin-like growth factor binding protein 3 in myotonic dystrophy type 1. Arch Neurol. 2011 Jan; 68(1):37-44.
    View in: PubMed
  18. Moxley RT, Pandya S, Ciafaloni E, Fox DJ, Campbell K. Change in natural history of Duchenne muscular dystrophy with long-term corticosteroid treatment: implications for management. J Child Neurol. 2010 Sep; 25(9):1116-29.
    View in: PubMed
  19. Babcock MA, Kostova FV, Moxley RT, Chamberlain JS, Maria BL. Muscular dystrophy: new opportunities for diagnosis and treatment. J Child Neurol. 2010 Sep; 25(9):1080-97.
    View in: PubMed
  20. Logigian EL, Martens WB, Moxley RT, McDermott MP, Dilek N, Wiegner AW, Pearson AT, Barbieri CA, Annis CL, Thornton CA, Moxley RT. Mexiletine is an effective antimyotonia treatment in myotonic dystrophy type 1. Neurology. 2010 May 4; 74(18):1441-8.
    View in: PubMed
  21. Logigian EL, Twydell P, Dilek N, Martens WB, Quinn C, Wiegner AW, Heatwole CR, Thornton CA, Moxley RT. Evoked myotonia can be "dialed-up" by increasing stimulus train length in myotonic dystrophy type 1. Muscle Nerve. 2010 Feb; 41(2):191-6.
    View in: PubMed
  22. Mueller CM, Hilbert JE, Martens W, Thornton CA, Moxley RT, Greene MH. Hypothesis: neoplasms in myotonic dystrophy. Cancer Causes Control. 2009 Dec; 20(10):2009-20.
    View in: PubMed
  23. Nakamori M, Sobczak K, Moxley RT, Thornton CA. Scaled-down genetic analysis of myotonic dystrophy type 1 and type 2. Neuromuscul Disord. 2009 Nov; 19(11):759-62.
    View in: PubMed
  24. Ciafaloni E, Fox DJ, Pandya S, Westfield CP, Puzhankara S, Romitti PA, Mathews KD, Miller TM, Matthews DJ, Miller LA, Cunniff C, Druschel CM, Moxley RT. Delayed diagnosis in duchenne muscular dystrophy: data from the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet). J Pediatr. 2009 Sep; 155(3):380-5.
    View in: PubMed
  25. Ciafaloni E, Moxley RT. Treatment options for Duchenne muscular dystrophy. Curr Treat Options Neurol. 2008 Mar; 10(2):86-93.
    View in: PubMed
  26. Birnkrant DJ, Panitch HB, Benditt JO, Boitano LJ, Carter ER, Cwik VA, Finder JD, Iannaccone ST, Jacobson LE, Kohn GL, Motoyama EK, Moxley RT, Schroth MK, Sharma GD, Sussman MD. American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation. Chest. 2007 Dec; 132(6):1977-86.
    View in: PubMed
  27. Moxley RT, Logigian EL, Martens WB, Annis CL, Pandya S, Moxley RT, Barbieri CA, Dilek N, Wiegner AW, Thornton CA. Computerized hand grip myometry reliably measures myotonia and muscle strength in myotonic dystrophy (DM1). Muscle Nerve. 2007 Sep; 36(3):320-8.
    View in: PubMed
  28. Gagnon C, Noreau L, Moxley RT, Laberge L, Jean S, Richer L, Perron M, Veillette S, Mathieu J. Towards an integrative approach to the management of myotonic dystrophy type 1. J Neurol Neurosurg Psychiatry. 2007 Aug; 78(8):800-6.
    View in: PubMed
  29. Heatwole CR, Moxley RT. The nondystrophic myotonias. Neurotherapeutics. 2007 Apr; 4(2):238-51.
    View in: PubMed
  30. Logigian EL, Ciafaloni E, Quinn LC, Dilek N, Pandya S, Moxley RT, Thornton CA. Severity, type, and distribution of myotonic discharges are different in type 1 and type 2 myotonic dystrophy. Muscle Nerve. 2007 Apr; 35(4):479-85.
    View in: PubMed
  31. Heatwole CR, Miller J, Martens B, Moxley RT. Laboratory abnormalities in ambulatory patients with myotonic dystrophy type 1. Arch Neurol. 2006 Aug; 63(8):1149-53.
    View in: PubMed
  32. Lin X, Miller JW, Mankodi A, Kanadia RN, Yuan Y, Moxley RT, Swanson MS, Thornton CA. Failure of MBNL1-dependent post-natal splicing transitions in myotonic dystrophy. Hum Mol Genet. 2006 Jul 1; 15(13):2087-97.
    View in: PubMed
  33. Udd B, Meola G, Krahe R, Thornton C, Ranum LP, Bassez G, Kress W, Schoser B, Moxley R. 140th ENMC International Workshop: Myotonic Dystrophy DM2/PROMM and other myotonic dystrophies with guidelines on management. Neuromuscul Disord. 2006 Jun; 16(6):403-13.
    View in: PubMed
  34. Hirtz D, Iannaccone S, Heemskerk J, Gwinn-Hardy K, Moxley R, Rowland LP. Challenges and opportunities in clinical trials for spinal muscular atrophy. Neurology. 2005 Nov 8; 65(9):1352-7.
    View in: PubMed
  35. Logigian EL, Blood CL, Dilek N, Martens WB, Moxley RT, Wiegner AW, Thornton CA, Moxley RT. Quantitative analysis of the "warm-up" phenomenon in myotonic dystrophy type 1. Muscle Nerve. 2005 Jul; 32(1):35-42.
    View in: PubMed
  36. Moxley RT, Ashwal S, Pandya S, Connolly A, Florence J, Mathews K, Baumbach L, McDonald C, Sussman M, Wade C. Practice parameter: corticosteroid treatment of Duchenne dystrophy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2005 Jan 11; 64(1):13-20.
    View in: PubMed
  37. Jiang H, Mankodi A, Swanson MS, Moxley RT, Thornton CA. Myotonic dystrophy type 1 is associated with nuclear foci of mutant RNA, sequestration of muscleblind proteins and deregulated alternative splicing in neurons. Hum Mol Genet. 2004 Dec 15; 13(24):3079-88.
    View in: PubMed
  38. Meola G, Moxley RT. Myotonic dystrophy type 2 and related myotonic disorders. J Neurol. 2004 Oct; 251(10):1173-82.
    View in: PubMed
  39. Logigian EL, Moxley RT, Blood CL, Barbieri CA, Martens WB, Wiegner AW, Thornton CA, Moxley RT. Leukocyte CTG repeat length correlates with severity of myotonia in myotonic dystrophy type 1. Neurology. 2004 Apr 13; 62(7):1081-9.
    View in: PubMed
  40. Meola G, Sansone V, Perani D, Scarone S, Cappa S, Dragoni C, Cattaneo E, Cotelli M, Gobbo C, Fazio F, Siciliano G, Mancuso M, Vitelli E, Zhang S, Krahe R, Moxley RT. Executive dysfunction and avoidant personality trait in myotonic dystrophy type 1 (DM-1) and in proximal myotonic myopathy (PROMM/DM-2). Neuromuscul Disord. 2003 Dec; 13(10):813-21.
    View in: PubMed
  41. Bachinski LL, Udd B, Meola G, Sansone V, Bassez G, Eymard B, Thornton CA, Moxley RT, Harper PS, Rogers MT, Jurkat-Rott K, Lehmann-Horn F, Wieser T, Gamez J, Navarro C, Bottani A, Kohler A, Shriver MD, Sallinen R, Wessman M, Zhang S, Wright FA, Krahe R. Confirmation of the type 2 myotonic dystrophy (CCTG)n expansion mutation in patients with proximal myotonic myopathy/proximal myotonic dystrophy of different European origins: a single shared haplotype indicates an ancestral founder effect. Am J Hum Genet. 2003 Oct; 73(4):835-48.
    View in: PubMed
  42. Duong DJ, Moxley RT, Kellman RM, Pincus SH, Gaspari AA. Thalidomide therapy for cicatricial pemphigoid. J Am Acad Dermatol. 2002 Aug; 47(2 Suppl):S193-5.
    View in: PubMed
  43. Mankodi A, Takahashi MP, Jiang H, Beck CL, Bowers WJ, Moxley RT, Cannon SC, Thornton CA. Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy. Mol Cell. 2002 Jul; 10(1):35-44.
    View in: PubMed
  44. Moxley RT, Meola G, Udd B, Ricker K. Report of the 84th ENMC workshop: PROMM (proximal myotonic myopathy) and other myotonic dystrophy-like syndromes: 2nd workshop. 13-15th October, 2000, Loosdrecht, The Netherlands. Neuromuscul Disord. 2002 Mar; 12(3):306-17.
    View in: PubMed
  45. Meola G, Sansone V, Marinou K, Cotelli M, Moxley RT, Thornton CA, De Ambroggi L. Proximal myotonic myopathy: a syndrome with a favourable prognosis? J Neurol Sci. 2002 Jan 15; 193(2):89-96.
    View in: PubMed
  46. Mankodi A, Urbinati CR, Yuan QP, Moxley RT, Sansone V, Krym M, Henderson D, Schalling M, Swanson MS, Thornton CA. Muscleblind localizes to nuclear foci of aberrant RNA in myotonic dystrophy types 1 and 2. Hum Mol Genet. 2001 Sep 15; 10(19):2165-70.
    View in: PubMed
  47. Musser WS, Barbano RL, Thornton CA, Moxley RT, Herrmann DN, Logigian EL. Distal myasthenia gravis with a decrement, an increment, and denervation. J Clin Neuromuscul Dis. 2001 Sep; 3(1):16-9.
    View in: PubMed
  48. Fenichel GM, Griggs RC, Kissel J, Kramer TI, Mendell JR, Moxley RT, Pestronk A, Sheng K, Florence J, King WM, Pandya S, Robison VD, Wang H. A randomized efficacy and safety trial of oxandrolone in the treatment of Duchenne dystrophy. Neurology. 2001 Apr 24; 56(8):1075-9.
    View in: PubMed
  49. Forbes GB, Griggs RC, Moxley RT, Thornton CT, Tawil R. K-40 and dual-energy X-ray absorptiometry estimates of lean weight compared. Normals and patients with neuromuscular disease. Ann N Y Acad Sci. 2000 May; 904:111-4.
    View in: PubMed
  50. Sansone V, Griggs RC, Moxley RT. Hypothyroidism unmasking proximal myotonic myopathy. Neuromuscul Disord. 2000 Mar; 10(3):165-72.
    View in: PubMed
  51. Meola G, Sansone V, Perani D, Colleluori A, Cappa S, Cotelli M, Fazio F, Thornton CA, Moxley RT. Reduced cerebral blood flow and impaired visual-spatial function in proximal myotonic myopathy. Neurology. 1999 Sep 22; 53(5):1042-50.
    View in: PubMed
  52. Duong DJ, Spigel GT, Moxley RT, Gaspari AA. American experience with low-dose thalidomide therapy for severe cutaneous lupus erythematosus. Arch Dermatol. 1999 Sep; 135(9):1079-87.
    View in: PubMed
  53. Moxley RT. 54th ENMC International Workshop: PROMM (proximal myotonic myopathies) and other proximal myotonic syndromes. 10-12th October 1997, Naarden, The Netherlands. Neuromuscul Disord. 1998 Oct; 8(7):508-18.
    View in: PubMed
  54. Moxley RT, Udd B, Ricker K. Proximal myotonic myopathy (PROMM) and other proximal myotonic syndromes. Neuromuscul Disord. 1998 Oct; 8(7):519-20.
    View in: PubMed
  55. AFM/MDA 1st International Myotonic Dystrophy Consortium Conference 30 June-1 July 1997, Paris, France. Neuromuscul Disord. 1998 Aug; 8(6):432-7.
    View in: PubMed
  56. Menken M, Goldblatt D, Moxley RT, Hachinski V. Managed care and the survival of neurology referral centers. A commitment to centers of excellence. Arch Neurol. 1997 Nov; 54(11):1349-50.
    View in: PubMed
  57. Thornton CA, Wymer JP, Simmons Z, McClain C, Moxley RT. Expansion of the myotonic dystrophy CTG repeat reduces expression of the flanking DMAHP gene. Nat Genet. 1997 Aug; 16(4):407-9.
    View in: PubMed
  58. Lane R, Carey N, Orrell R, Moxley RT. Claude Monet's vision. Lancet. 1997 Mar 8; 349(9053):734.
    View in: PubMed
  59. DiDonato CJ, Ingraham SE, Mendell JR, Prior TW, Lenard S, Moxley RT, Florence J, Burghes AH. Deletion and conversion in spinal muscular atrophy patients: is there a relationship to severity? Ann Neurol. 1997 Feb; 41(2):230-7.
    View in: PubMed
  60. Moxley RT. Carrell-Krusen Symposium Invited Lecture-1997. Myotonic disorders in childhood: diagnosis and treatment. J Child Neurol. 1997 Feb; 12(2):116-29.
    View in: PubMed
  61. MOxley RT. Proximal myotonic myopathy: mini-review of a recently delineated clinical disorder. Neuromuscul Disord. 1996 Mar; 6(2):87-93.
    View in: PubMed
  62. Moxley RT. The myotonias: their diagnosis and treatment. Compr Ther. 1996 Jan; 22(1):8-21.
    View in: PubMed
  63. Thornton C, Griggs RC, Moxley RT. PROMM syndrome (Ricker's disease) Ann Neurol. 1995 Aug; 38(2):273.
    View in: PubMed
  64. Moxley RT, Ricker K. Proximal myotonic myopathy. Muscle Nerve. 1995 May; 18(5):557-8.
    View in: PubMed
  65. Ricker K, Koch MC, Lehmann-Horn F, Pongratz D, Speich N, Reiners K, Schneider C, Moxley RT. Proximal myotonic myopathy. Clinical features of a multisystem disorder similar to myotonic dystrophy. Arch Neurol. 1995 Jan; 52(1):25-31.
    View in: PubMed
  66. Rifai Z, Welle S, Moxley RT, Lorenson M, Griggs RC. Effect of prednisone on protein metabolism in Duchenne dystrophy. Am J Physiol. 1995 Jan; 268(1 Pt 1):E67-74.
    View in: PubMed
  67. Moxley RT. Evaluation of neuromuscular function in inflammatory myopathy. Rheum Dis Clin North Am. 1994 Nov; 20(4):827-43.
    View in: PubMed
  68. Ricker K, Moxley RT, Heine R, Lehmann-Horn F. Myotonia fluctuans. A third type of muscle sodium channel disease. Arch Neurol. 1994 Nov; 51(11):1095-102.
    View in: PubMed
  69. Moxley RT. Potential for growth factor treatment of muscle disease. Curr Opin Neurol. 1994 Oct; 7(5):427-34.
    View in: PubMed
  70. Ricker K, Koch MC, Lehmann-Horn F, Pongratz D, Otto M, Heine R, Moxley RT. Proximal myotonic myopathy: a new dominant disorder with myotonia, muscle weakness, and cataracts. Neurology. 1994 Aug; 44(8):1448-52.
    View in: PubMed
  71. Prior TW, Papp AC, Snyder PJ, Sedra MS, Western LM, Bartolo C, Moxley RT, Mendell JR. Heteroduplex analysis of the dystrophin gene: application to point mutation and carrier detection. Am J Med Genet. 1994 Mar 1; 50(1):68-73.
    View in: PubMed
  72. Burghes AH, Ingraham SE, Kóte-Jarai Z, Rosenfeld S, Herta N, Nadkarni N, DiDonato CJ, Carpten J, Hurko O, Florence J, et al. Linkage mapping of the spinal muscular atrophy gene. Hum Genet. 1994 Mar; 93(3):305-12.
    View in: PubMed
  73. Thornton CA, Griggs RC, Moxley RT. Myotonic dystrophy with no trinucleotide repeat expansion. Ann Neurol. 1994 Mar; 35(3):269-72.
    View in: PubMed
  74. Thornton CA, Johnson K, Moxley RT. Myotonic dystrophy patients have larger CTG expansions in skeletal muscle than in leukocytes. Ann Neurol. 1994 Jan; 35(1):104-7.
    View in: PubMed
  75. Rifai Z, Kingston WJ, McCraith B, Moxley RT. Forearm 3-methylhistidine efflux in myotonic dystrophy. Ann Neurol. 1993 Nov; 34(5):682-6.
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  76. Tawil R, Moxley RT, Griggs RC. Acetazolamide-induced nephrolithiasis: implications for treatment of neuromuscular disorders. Neurology. 1993 Jun; 43(6):1105-6.
    View in: PubMed
  77. Kissel JT, Lynn DJ, Rammohan KW, Klein JP, Griggs RC, Moxley RT, Cwik VA, Brooke MH, Mendell JR. Mononuclear cell analysis of muscle biopsies in prednisone- and azathioprine-treated Duchenne muscular dystrophy. Neurology. 1993 Mar; 43(3 Pt 1):532-6.
    View in: PubMed
  78. Griggs RC, Moxley RT, Mendell JR, Fenichel GM, Brooke MH, Pestronk A, Miller JP, Cwik VA, Pandya S, Robison J, et al. Duchenne dystrophy: randomized, controlled trial of prednisone (18 months) and azathioprine (12 months) Neurology. 1993 Mar; 43(3 Pt 1):520-7.
    View in: PubMed
  79. Livingston JN, Unger JW, Moxley RT, Moss A. Phosphotyrosine-containing proteins in the CNS of obese Zucker rats are decreased in the absence of changes in the insulin receptor. Neuroendocrinology. 1993 Mar; 57(3):481-8.
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  80. Torres CF, Moxley RT. Early predictors of poor outcome in congenital fiber-type disproportion myopathy. Arch Neurol. 1992 Aug; 49(8):855-6.
    View in: PubMed
  81. Fenichel GM, Florence JM, Pestronk A, Mendell JR, Moxley RT, Griggs RC, Brooke MH, Miller JP, Robison J, King W, et al. Long-term benefit from prednisone therapy in Duchenne muscular dystrophy. Neurology. 1991 Dec; 41(12):1874-7.
    View in: PubMed
  82. Fenichel GM, Mendell JR, Moxley RT, Griggs RC, Brooke MH, Miller JP, Pestronk A, Robison J, King W, Signore L, et al. A comparison of daily and alternate-day prednisone therapy in the treatment of Duchenne muscular dystrophy. Arch Neurol. 1991 Jun; 48(6):575-9.
    View in: PubMed
  83. Griggs RC, Moxley RT, Mendell JR, Fenichel GM, Brooke MH, Pestronk A, Miller JP. Prednisone in Duchenne dystrophy. A randomized, controlled trial defining the time course and dose response. Clinical Investigation of Duchenne Dystrophy Group. Arch Neurol. 1991 Apr; 48(4):383-8.
    View in: PubMed
  84. Griggs RC, Moxley RT, Mendell JR, Fenichel GM, Brooke MH, Miller PJ, Mandel S, Florence J, Schierbecker J, Kaiser KK, et al. Randomized, double-blind trial of mazindol in Duchenne dystrophy. Muscle Nerve. 1990 Dec; 13(12):1169-73.
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  85. Moxley RT, Arner P, Moss A, Skottner A, Fox M, James D, Livingston JN. Acute effects of insulin-like growth factor I and insulin on glucose metabolism in vivo. Am J Physiol. 1990 Oct; 259(4 Pt 1):E561-7.
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  86. Slot JW, Moxley R, Geuze HJ, James DE. No evidence for expression of the insulin-regulatable glucose transporter in endothelial cells. Nature. 1990 Jul 26; 346(6282):369-71.
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  87. Ricker K, Moxley RT. Autosomal dominant cramping disease. Arch Neurol. 1990 Jul; 47(7):810-2.
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  88. Torres CF, Moxley RT. Hypothyroid neuropathy and myopathy: clinical and electrodiagnostic longitudinal findings. J Neurol. 1990 Jul; 237(4):271-4.
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  89. Moss AM, Unger JW, Moxley RT, Livingston JN. Location of phosphotyrosine-containing proteins by immunocytochemistry in the rat forebrain corresponds to the distribution of the insulin receptor. Proc Natl Acad Sci U S A. 1990 Jun; 87(12):4453-7.
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  90. Kieburtz K, Ricotta JJ, Moxley RT. Seizures following carotid endarterectomy. Arch Neurol. 1990 May; 47(5):568-70.
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  91. Ricker K, Lehmann-Horn F, Moxley RT. Myotonia fluctuans. Arch Neurol. 1990 Mar; 47(3):268-72.
    View in: PubMed
  92. Moxley RT. Functional testing. Muscle Nerve. 1990; 13 Suppl:S26-9.
    View in: PubMed
  93. Minaker KL, Flier JS, Landsberg L, Young JB, Moxley RT, Kingston WJ, Meneilly GS, Rowe JW. Phenytoin-induced improvement in muscle cramping and insulin action in three patients with the syndrome of insulin resistance, acanthosis nigricans, and acral hypertrophy. Arch Neurol. 1989 Sep; 46(9):981-5.
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  94. Moxley RT, Ricker K, Kingston WJ, Böhlen R. Potassium uptake in muscle during paramyotonic weakness. Neurology. 1989 Jul; 39(7):952-5.
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  95. Mendell JR, Moxley RT, Griggs RC, Brooke MH, Fenichel GM, Miller JP, King W, Signore L, Pandya S, Florence J, et al. Randomized, double-blind six-month trial of prednisone in Duchenne's muscular dystrophy. N Engl J Med. 1989 Jun 15; 320(24):1592-7.
    View in: PubMed
  96. Brooke MH, Fenichel GM, Griggs RC, Mendell JR, Moxley R, Florence J, King WM, Pandya S, Robison J, Schierbecker J, et al. Duchenne muscular dystrophy: patterns of clinical progression and effects of supportive therapy. Neurology. 1989 Apr; 39(4):475-81.
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  97. Ricker K, Moxley RT, Rohkamm R. Rippling muscle disease. Arch Neurol. 1989 Apr; 46(4):405-8.
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  98. Griggs RC, Wood DS. Criteria for establishing the validity of genetic recombination in myotonic dystrophy. Neurology. 1989 Mar; 39(3):420-1.
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  99. Griggs RC, Pandya S, Florence JM, Brooke MH, Kingston W, Miller JP, Chutkow J, Herr BE, Moxley RT. Randomized controlled trial of testosterone in myotonic dystrophy. Neurology. 1989 Feb; 39(2 Pt 1):219-22.
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  100. Unger J, McNeill TH, Moxley RT, White M, Moss A, Livingston JN. Distribution of insulin receptor-like immunoreactivity in the rat forebrain. Neuroscience. 1989; 31(1):143-57.
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  101. Kingston WJ, Moxley RT. Treatment of muscular dystrophies. Gen Pharmacol. 1989; 20(3):263-8.
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  102. Fenichel GM, Brooke MH, Griggs RC, Mendell JR, Miller JP, Moxley RT, Park JH, Provine MA, Florence J, Kaiser KK, et al. Clinical investigation in Duchenne muscular dystrophy: penicillamine and vitamin E. Muscle Nerve. 1988 Nov; 11(11):1164-8.
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  103. Kingston WJ, Moxley RT. Inflammatory myopathies. Neurol Clin. 1988 Aug; 6(3):545-61.
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  104. Ricker K, Rohkamm R, Moxley RT. Hypertrophy of the calf with S-1 radiculopathy. Arch Neurol. 1988 Jun; 45(6):660-4.
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  105. Hyser CL, Province M, Griggs RC, Mendell JR, Fenichel GM, Brooke MH, Miller JP, Polakowska R, Doherty RA, Quirk S, et al. Genetic heterogeneity in Duchenne dystrophy. Ann Neurol. 1987 Oct; 22(4):553-5.
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  106. Hyser CL, Doherty RA, Griggs RC, Mendell JR, Polakowska R, Quirk S, Brooke MH, Fenichel GM. Carrier assessment for mothers and sisters of isolated Duchenne dystrophy cases: the importance of serum enzyme determinations. Neurology. 1987 Sep; 37(9):1476-80.
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  107. Brooke MH, Fenichel GM, Griggs RC, Mendell JR, Moxley RT, Miller JP, Kaiser KK, Florence JM, Pandya S, Signore L, et al. Clinical investigation of Duchenne muscular dystrophy. Interesting results in a trial of prednisone. Arch Neurol. 1987 Aug; 44(8):812-7.
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  108. Mendell JR, Province MA, Moxley RT, Griggs RC, Brooke MH, Fenichel GM, Miller JP, Kaiser KK, King W, Robison J, et al. Clinical investigation of Duchenne muscular dystrophy. A methodology for therapeutic trials based on natural history controls. Arch Neurol. 1987 Aug; 44(8):808-11.
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  109. Moxley RT, Kingston WJ, Griggs RC, Livingston JN. Lack of rapid enhancement of insulin action after oral glucose challenge in myotonic dystrophy. Diabetes. 1987 Jun; 36(6):693-701.
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  110. Moxley RT, Brooke MH, Fenichel GM, Mendell JR, Griggs RC, Miller JP, Province MA, Patterson V. Clinical investigation in Duchenne dystrophy. VI. Double-blind controlled trial of nifedipine. Muscle Nerve. 1987 Jan; 10(1):22-33.
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  111. Griggs RC, Halliday D, Kingston W, Moxley RT. Effect of testosterone on muscle protein synthesis in myotonic dystrophy. Ann Neurol. 1986 Nov; 20(5):590-6.
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  112. Kingston WJ, Moxley RT, Griggs RC. Effect of testosterone on whole body amino acid utilization in myotonic dystrophy. Metabolism. 1986 Oct; 35(10):928-32.
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  113. Moxley RT, Kingston WJ, Minaker KL, Corbett AJ, Rowe JW. Insulin resistance and regulation of serum amino acid levels in myotonic dystrophy. Clin Sci (Lond). 1986 Oct; 71(4):429-36.
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  114. Madsen KS, Miller JP, Province MA. The use of an extended baseline period in the evaluation of treatment in a longitudinal Duchenne muscular dystrophy trial. Stat Med. 1986 May-Jun; 5(3):231-41.
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  115. Kingston WJ, Livingston JN, Moxley RT. Enhancement of insulin action after oral glucose ingestion. J Clin Invest. 1986 Apr; 77(4):1153-62.
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  116. Kingston WJ, Moxley RT. Treatment of muscular dystrophies and inflammatory myopathies. Clin Neuropharmacol. 1986; 9(4):361-72.
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  117. Griggs RC, Kingston W, Herr BE, Forbes G, Moxley RT. Lack of relationship of hypogonadism to muscle wasting in myotonic dystrophy. Arch Neurol. 1985 Sep; 42(9):881-5.
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  118. Kingston WJ, Livingston JN, Moxley RT. Intravenous glucose infusion fails to alter monocyte insulin-binding affinity in normal subjects. Horm Metab Res. 1985 Sep; 17(9):464-6.
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  119. Griggs RC, Kingston W, Herr BE, Forbes G, Moxley RT. Myotonic dystrophy: effect of testosterone on total body potassium and on creatinine excretion. Neurology. 1985 Jul; 35(7):1035-40.
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  120. Moxley RT. Absence of major side effects of nifedipine following treatment of Duchenne dystrophy. Pediatrics. 1985 Jun; 75(6):1168-9.
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  121. Moxley RT, Kingston W, Griggs RC. Abnormal regulation of venous alanine after glucose ingestion in myotonic dystrophy. Clin Sci (Lond). 1985 Feb; 68(2):151-7.
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  122. Griggs RC, Mendell JR, Brooke MH, Fenichel GM, Miller JP, Province M, Moxley RT, Huntzinger D, Vaughn A, Cohen M, et al. Clinical investigation in Duchenne dystrophy: V. Use of creatine kinase and pyruvate kinase in carrier detection. Muscle Nerve. 1985 Jan; 8(1):60-7.
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  123. Mendell JR, Griggs RC, Moxley RT, Fenichel GM, Brooke MH, Miller JP, Province MA, Dodson WE. Clinical investigation in Duchenne muscular dystrophy: IV. Double-blind controlled trial of leucine. Muscle Nerve. 1984 Sep; 7(7):535-41.
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  124. Livingston JN, Kingston WJ, Finocchiaro L, Moxley RT. Rapid increase in the insulin sensitivity of rat adipocytes after intravenous glucose administration. Endocrinology. 1984 Jul; 115(1):55-9.
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  125. Corbett A, Kingston W, Griggs RC, Moxley RT. Effect of acetazolamide on insulin sensitivity in myotonic disorders. Arch Neurol. 1984 Jul; 41(7):740-3.
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  126. Moxley RT, Corbett AJ, Minaker KL, Rowe JW. Whole body insulin resistance in myotonic dystrophy. Ann Neurol. 1984 Feb; 15(2):157-62.
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  127. Riggs JE, Schochet SS, Fakadej AV, Papadimitriou A, DiMauro S, Crosby TW, Gutmann L, Moxley RT. Mitochondrial encephalomyopathy with decreased succinate-cytochrome c reductase activity. Neurology. 1984 Jan; 34(1):48-53.
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  128. Harris JP, Nanda NC, Moxley R, Manning JA. Myocardial perforation due to temporary transvenous pacing catheters in pediatric patients. Cathet Cardiovasc Diagn. 1984; 10(4):329-33.
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  129. Riggs JE, Griggs RC, Moxley RT. Dissociation of glucose and potassium arterial-venous differences across the forearm by acetazolamide. A possible relationship to acetazolamide's beneficial effect in hypokalemic periodic paralysis. Arch Neurol. 1984 Jan; 41(1):35-8.
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  130. Torres C, Moxley RT, Griggs RC. Quantitative testing of handgrip strength, myotonia, and fatigue in myotonic dystrophy. J Neurol Sci. 1983 Jul; 60(1):157-68.
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  131. Bach PR. Determination of nifedipine in serum or plasma by reversed-phase liquid chromatography. Clin Chem. 1983 Jul; 29(7):1344-8.
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  132. Moxley RT, Griggs RC, Forbes GB, Goldblatt D, Donohoe K. Influence of muscle wasting on oral glucose tolerance testing. Clin Sci (Lond). 1983 Jun; 64(6):601-9.
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  133. Brooke MH, Fenichel GM, Griggs RC, Mendell JR, Moxley R, Miller JP, Province MA. Clinical investigation in Duchenne dystrophy: 2. Determination of the "power" of therapeutic trials based on the natural history. Muscle Nerve. 1983 Feb; 6(2):91-103.
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  134. Griggs RC, Forbes G, Moxley RT, Herr BE. The assessment of muscle mass in progressive neuromuscular disease. Neurology. 1983 Feb; 33(2):158-65.
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  135. Livingston JN, Moxley RT. Glucose ingestion mediates a rapid increase in the insulin responsiveness of rat adipocytes. Endocrinology. 1982 Nov; 111(5):1749-51.
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  136. Corbett AJ, Griggs RC, Moxley RT. Skeletal muscle catabolism in amyotrophic lateral sclerosis and chronic spinal muscular atrophy. Neurology. 1982 May; 32(5):550-2.
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  137. Torres CF, Griggs RC, Moxley RT, Bender AN. Hypokalemic periodic paralysis exacerbated by acetazolamide. Neurology. 1981 Nov; 31(11):1423-8.
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  138. Riggs JE, Moxley RT, Griggs RC, Horner FA. Hyperkalemic periodic paralysis: an apparent sporadic case. Neurology. 1981 Sep; 31(9):1157-9.
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  139. Riggs JE, Griggs RC, Moxley RT, Lewis ED. Acute effects of acetazolamide in hyperkalemic periodic paralysis. Neurology. 1981 Jun; 31(6):725-9.
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  140. Moxley RT, Livingston JN, Lockwood DH, Griggs RC, Hill RL. Abnormal regulation of monocyte insulin-binding affinity after glucose ingestion in patients with myotonic dystrophy. Proc Natl Acad Sci U S A. 1981 Apr; 78(4):2567-71.
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  141. Pottick LA, Moxley RT, Livingston JN. Tissue differences in insulin receptors: acute changes in insulin binding characteristics induced by wheat germ agglutinin. Diabetes. 1981 Mar; 30(3):196-202.
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  142. Griggs RC, Donohoe KM, Utell MJ, Goldblatt D, Moxley RT. Evaluation of pulmonary function in neuromuscular disease. Arch Neurol. 1981 Jan; 38(1):9-12.
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  143. Brooke MH, Griggs RC, Mendell JR, Fenichel GM, Shumate JB. The natural history of Duchenne muscular dystrophy: a caveat for therapeutic trials. Trans Am Neurol Assoc. 1981; 106:195-9.
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  144. Griggs RC, Pandya S, Moxley RT, Forbes G, VanDyke DH, Pearce FJ. Treatment of myopathic carnitine deficiency: quantitation of response to prednisone and carnitine. Trans Am Neurol Assoc. 1981; 106:199-202.
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  145. Griggs RC, Moxley RT, Forbes GB. 3-methylhistidine excretion in myotonic dystrophy. Neurology. 1980 Dec; 30(12):1262-7.
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  146. Moxley RT, Griggs RC, Goldblatt D. Muscle insulin resistance in myotonic dystrophy: effect of supraphysiologic insulinization. Neurology. 1980 Oct; 30(10):1077-83.
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  147. Lewis ED, Griggs RC, Moxley RT. Regulation of plasma potassium in hyperkalemic periodic paralysis. Neurology. 1979 Aug; 29(8):1131-7.
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  148. Ross AJ, Herr BE, Norwood ML, Donohoe KM, Moxley RT. Neuromuscular diagnostic procedures. Nurs Clin North Am. 1979 Mar; 14(1):107-21.
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  149. Moxley RT, Griggs RC, Markesbery WR, Vangelder V. Metabolic implications of distal atrophy. Carbohydrate metabolism in centronuclear myopathy. J Neurol Sci. 1978 Dec; 39(2-3):247-59.
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  150. Griggs RC, Moxley RT, Lafrance RA, McQuillen J. Hereditary paroxysmal ataxia: response to acetazolamide. Neurology. 1978 Dec; 28(12):1259-64.
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  151. Moxley RT, Griggs RC, Goldblatt D, VanGelder V, Herr BE, Thiel R. Decreased insulin sensitivity of forearm muscle in myotonic dystrophy. J Clin Invest. 1978 Oct; 62(4):857-67.
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  152. Griggs RC, Moxley RT, Riggs JE, Engel WK. Effects of acetazolamide on myotonia. Ann Neurol. 1978 Jun; 3(6):531-7.
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  153. Moxley RT, Lockwood DH, Amatruda JM, Tobin JD, Pozefsky T. Loss of insulin response to ingested amino acids after jejunoileal bypass surgery for morbid obesity. Diabetes. 1978 Feb; 27(2):78-84.
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  154. Riggs JE, Griggs RC, Moxley RT. Acetazolamide-induced weakness in paramyotonia congenita. Ann Intern Med. 1977 Feb; 86(2):169-73.
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  155. Griggs RC, Moxley RT, Riggs JE, Engel WK. Effect of acetazolamide on myotonia. Trans Am Neurol Assoc. 1977; 102:133-5.
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  156. Moxley RT. Research trends pertinent to the management of neuromuscular diseases. Adv Neurol. 1977; 17:13-24.
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  157. Lockwood DH, Amatruda JM, Moxley RT, Pozefsky T, Boitnott JK. Effect of oral amino acid supplementation on liver disease after jejunoileal bypass for morbid obesity. Am J Clin Nutr. 1977 Jan; 30(1):58-63.
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  158. Moxley RT. Metabolic studies in muscular dystrophy: a role for insulin. Adv Neurol. 1977; 17:161-73.
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  159. Pozefsky T, Tancredi RG, Moxley RT, Dupre J, Tobin JD. Metabolism of forearm tissues in man. Studies with glucagon. Diabetes. 1976 Feb; 25(2):128-35.
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  160. Pozefsky T, Tancredi RG, Moxley RT, Dupre J, Tobin JD. Effects of brief starvation on muscle amino acid metabolism in nonobese man. J Clin Invest. 1976 Feb; 57(2):444-9.
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  161. White JJ, Moxley RT, Pozefsky T, Lockwood DH. Transient kwaskiorkor: A cause of fatty liver following small bowel bypass. Surgery. 1974 Jun; 75(6):829-40.
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  162. Moxley RT, Pozefsky T, Lockwood DH. Protein nutrition and liver disease after jejunoileal bypass for morbid obesity. N Engl J Med. 1974 Apr 25; 290(17):921-6.
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  163. Durbeck DC, Heinzelmann F, Schacter J, Haskell WL, Payne GH, Moxley RT, Nemiroff M, Limoncelli DD, Arnoldi LB, Fox SM. The National Aeronautics and Space Administration-U.S. Public Health Service Health Evaluation and Enhancement Program. Summary of results. Am J Cardiol. 1972 Nov; 30(7):784-90.
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