Alan Pestronk: Bibliography

INVITED PROFESSORSHIPS, LECTURES AND SEMINARS

1. Invited Seminar, University of New Mexico. Title: "Myasthenia Gravis". Albuquerque, New Mexico, August 1976.

2. Invited Presentation, Peripheral Nerve Society, Biennial Conference. Title: "Motor Nerve Sprouting". Airlie, Virginia, August 1977.

3. Invited Discussant, U.S. Department of Labor. Title: "Epidemic of Neuropathy in Polyurethane Foam Plants". Washington, D.C., April 1978.

4. Invited Seminar, University of Michigan. Title: "Motor Neuron Sprouting and Acetylcholine Receptors". Ann Arbor, Michigan, April 1978.

5. Invited Lecture, University of Oregon Neuroscience Council. Title: "Epidemic of Toxic Neuropathy." Portland, Oregon, December 1979.

6. Invited Lecture, Muscular Dystrophy Association Quadrennial Meeting - "Diseases of the Motor Unit." Title: Motor Nerve Outgrowth. Key Biscayne, Florida, June 1980.

7. Invited Presentation, New York Academy of Science Quadrennial Meeting on "Myasthenia Gravis." Title: Experimental Treatments of Myasthenia Gravis. New York, New York, December 1980.

8. Invited Presentation, Gordon Conference on Aging. Title: "Aging and the Neuromuscular Junction." Ventura, California, February 1982.

9. Invited Lecture, Vth International Congress on Neuromuscular Disease. Title: "Sprouting and Regeneration of Motor Nerves." Marseille, France, September 1982.

10. Invited Presentation, Workshop on "Immune Responses to Cell Membrane Receptors and their Ligands as Causes of Neurologic Diseases." Title: Neurotransmitters as Trophic Factors. Rancho Mirage, California, February 1983.

11. Medical Leader - Neurology in China. Invited Lectures in Peking -Myasthenia Gravis, Tianjin - Botulism, Shanghai - Polymyositis, Dementia, April 1983.

12. Chairman - Sessions on Myasthenia Gravis, American Academy of Neurology, Dallas, April 1985.

13. Invited Presentation, New York Academy of Sciences Meeting on Myasthenia Gravis. Title: "Polymyositis." New York, New York, March 1986.

14. Visiting Professor, Department of Neurology, The University of New Mexico. Title: "Plasticity of the Neuromuscular Junction", June 1986.

15. Visiting Professor, Department of Neurology, Washington University-St. Louis. Title: "Lower Motor Neuron Syndromes" February. 1989.

16. Invited Presentation, American Academy of Neurology Clinical Neurochemistry Course. Title: "Botulinum Toxin," April 1989.

17. Invited Presentation, American Academy of Neurology Peripheral Neuropathy Course. Title: "Motor Neuropathies," April 1989.

18. Visiting Professor, "Anti GMI Ganglioside Antibodies," University of Maryland, May 1989.

19. Invited Presentation: "Anti-glycolipid Antibodies in Lower Motor Neuron Syndromes," Georgetown University School of Medicine, June 1989.

20. Annual Speaker: Muscular Dystrophy Association - Mississippi Valley Chapter, "Muscular Dystrophy", November 1989.

21. Invited Speaker: Conference on ALS Tucson AZ. Title: "Immunology of ALS," December 1989.

22. Invited Speaker: Dallas Immunological Society, "Antiglycolipid Antibodies," May 1990.

23. Visiting Professor: Iowa-Midwest Neurological Society, "Immune Neuropathies" "Motor Neuropathies," June 1990.

24. Visiting Professor: St. Louis University, "Immune Neuropathies," June 1990.

25. Invited Speaker: VII International Congress of Neuromuscular Disease, Munich, West Germany, "Immune Neuropathies," September 1990.

26. Invited Speaker: St. John's Mercy Medical Center, St. Louis, MO, "Immune Neuropathies and Antiglycolipid Antibodies," January 1991.

27. Invited Speaker: Gangliosides: The Pharmacology of Neuronal Plasticity, Rome, Italy, "Anti-ganglioside Antibodies," February 1991.

28. Invited Speaker: American Academy of Neurology, "Childhood CIDP," April 1991.

29. Course Director: American Academy of Neurology, "Seminar on ALS," April 1991.

30. Visiting Professor: Columbia Neurological Group, Columbia, MO "Diagnosis and Treatment of Immune Neuropathies," July 1991.

31. Visiting Professor: Massachusetts General Hospital - Harvard Medical School, Boston Mass, "Autoimmune Motor Neuropathies," October 1991.

32. Visiting Professor: University of Kentucky, Lexington, KY, "Motor Neuropathies," December 1991.

33. Visiting Professor: Vanderbilt School of Medicine, Nashville, TN., "Motor Neuropathies," February 1992.

34. Visiting Professor: Yale University, New Haven, CT., "Autoimmune Neuropathies," February 1992.

35. Invited Speaker: ALS Association,- Greater St. Louis Chapter - Support Group, "ALS - New Advances," St. Louis, MO, February 1992.

36. Visiting Professor: Indiana University, Indianapolis, IN, "Multifocal Motor Neuropathy," "Autoimmune Neuropathies," March 1992.

37. Invited Speaker: American Academy of Neurology Course - Immunosuppression, San Diego, CA "Polyneuropathies," May 1992.

38. Visiting Professor: University of Alberta, Edmonton, Alberta, Canada, "Autoimmune Neuropathies," June 1992.

39. Visiting Professor: University of Michigan, Ann Arbor, MI, "Motor Neuropathies" "Polyneuropathies and Autoantibodies," June 1992.

40. Invited Speaker: Multifocal Motor Neuropathy: Diagnostic Criteria and Therapeutical Strategies. Milan, Italy, "Antiglycolipid Antibodies in Multifocal Motor Neuropathy and Motor Neuron Disease," July 1992.

41. Invited Speaker: Association of Medical Laboratory Immunologists - Plenary Session, Alexandria, VA, "Neuropathy Associated With Monoclonal Gammopathies," August 1992.

42. Invited Speaker: Research Updates, Controversial Issues in ALS. Venice, Italy, "Autoantibodies in Motor Neuron Disease and Motor Neuropathy," September 1992.

43. Invited Speaker: Molecular Basis of Clinical Neurology, Madison WI, "Anti-GM1 Ganglioside Antibodies," October 1992.

44. Invited Speaker: Yearly Meeting - The Amyotrophic Lateral Sclerosis Association - Greater St. Louis Chapter, St. Louis MO, "ALS," December 1992.

45. Visiting Professor: Wayne State University, Detroit, MI, "Motor Neuropathies and Autoantibodies," January 1993.

46. Visiting Professor: St. John's Mercy Medical Center, St. Louis, MO, "Autoantibodies and Immune Polyneuropathies," March 1993.

47. Invited Speaker: Neuroimmunology for the Clinician, New Haven CT, "Immune Mediated Neuropathies," April 1993.

48. Invited Speaker: American Academy of Neurology Course - Immunosuppression, New York City, NY, "Autoimmune Polyneuropathies," May 1993.

49. Invited Speaker: Mechanisms and Immunotherapies in Immune-mediated Neurological Diseases, Amelia Island, FL, "Multifocal Motor Neuropathy with Conduction block: Mechanisms and Therapy," June 1993.

50. Visiting Professor: University of New Mexico, Albuquerque, NM, "Autoimmune Motor Neuropathies," "GALOP Syndromes and Immune Sensory Polyneuropathies," October 1993.

51. Visiting Professor: Johns Hopkins School of Medicine, Baltimore, MD, "GALOP Syndromes and Autoimmune Polyneuropathies," October 1993.

52. Visiting Professor: New Jersey College of Medicine and Dentistry, Newark NJ, "Autoimmune Polyneuropathies," March 1994.

53. Invited Speaker and Symposium Chairman: VIII International Congress of Neuromuscular Disease, Kyoto, Japan, "Multifocal Motor Neuropathy and Conduction Block," July, 1994.

54. Invited Speaker: "Immune neuropathies" Orlando, FL September 1994.

55. Invited Lecture: "Treatable Motor Neuropathies", St. Louis Neurological Society, St. Louis, MO, September 1994.

56. Invited Lecturer: Myasthenia Gravis Course, "Surgery of the thymus gland", American College of Surgeons, Chicago, IL, October 1994.

57. Invited Lecturer: "ALS with paraproteins and autoantibodies" Course: Current issues in the pathogenesis and therapy of ALS, Marseille, France, October 1994.

58. Visiting Professor: "Immune sensory neuropathies" National Institutes of Health, Washington, D.C., February 1995.

59. Invited Lecturer: "Muscle pathology", Course: Adult and pediatric neurology in primary care medicine. Methodist Hospital of Indiana, IN, May 1995.

60. Invited lecture: "Immune neuropathies", Course: Immunosuppressive treatment, American Academy of Neurology, Seattle, WA, May 1995.

61. Participant: Workshop on Clinical research in ALS, Rochester, MN, June 1995.

62. Invited Lecturer: "Peripheral neuropathies", Course: Contemporary clinical neurology symposium, Vanderbilt University School of Medicine, Hilton Head Island, SC, July 1995.

63. Invited Lecturer: "Sensory polyneuropathies", University of Vermont School of Medicine, August 1995.

64. Visiting Professor: "Immune Neuropathies", Instituto De Ciencias Neurologicas, Lima, Peru, August 1995.

65. Visiting Professor: "Immune Neuropathies", State University of New York at Stony Brook, Stony Brook, NY, September 1995.

66. Invited Lecturer: Course: 1995 Diabetes Update "Diabetic Neuropathies" Cape Girardeau, MO, September 1995.

67. Visiting Professor: "Post paralysis paralysis", " Polyneuropathies and autoantibodies", Vanderbilt University School of Medicine, Nashville, TN, October 1995.

68. Visiting Professor: "Myasthenia Gravis", St. John's Medical Center, St. Louis, MO, January 1996.

69. Invited Lecturer: "Multifocal motor neuropathies", Course: Neuroimmunology and therapy of immune-mediated neurological and neuromuscular disease, San Francisco, CA , March 1996.

70. Invited Lecturer: "Immune neuropathies", Course: Immunosuppressive treatment, American Academy of Neurology, San Francisco, CA, March 1996.

71. Invited Lecturer: "Anti-GM1 antibodies", Course:, Antinerve Antibodies American Academy of Neurology, San Francisco, CA, March 1996.

72. Visiting Professor: Lendowne-Rogowski Lecture "Autoimmune neuropathies", Yale University School of Medicine New Haven, CT, April 1996.

73. Visiting Professor: "Internist's approach to neuromuscular diseases", VA Hospital, St. Louis, MO, June 1996.

74. Invited Lecturer: National Institutes of Allergy and Infectious Diseases workshop; Development of Guillain-Barre Syndrome Following Campylobacter Infection, "Anti-GM1 antibodies in GBS", Bethesda, MD, August 1996.

75. Visiting Professor: "Antibody mediated Neuropathies"; "Weakness in the ICU", Emory University School of Medicine, Atlanta, GA, October, 1996.

76. Invited Speaker: "Immune neuropathies and autoantibodies", St. Vincent's Hospital, New York, NY, December 1996.

77. Invited Speaker: "Immune neuropathies and autoantibodies", Garden City, New York, Dinner Speaking Program, December, 1996.

78. Invited Speaker: "Advances in ALS" Greater St. Louis ALS Association, St. Louis, MO, February 1997

79. Visiting Professor: "Antibody-mediated neuropathies", University of Missouri Columbia, MO, March 1997.

80. Visiting Professor: "Immune neuropathies and autoantibodies", Massachusetts General Hospital, Boston, MA, March 1997.

81. Seminar Speaker: "Diagnosing immune neuropathies", Baltimore, MD, April 1997.

82. Invited Speaker: "Immune neuropathies and autoantibodies" Neuroimmunology Course, Boston, MA, April 1997.

83. Invited speaker: "Advances in neuromuscular research", 1997 MDA Awareness Seminar, St. Louis, MO, May 1997

84. Visiting Professor: "Immune demyelinating polyneuropathies", Allegheny Hospital, Thomas Jefferson School of Medicine, Philadelphia, PA, June 1997

85. Platform Presenter: "Measurement of anti-GM1 antibodies", Peripheral Nerve Society, Cambridge, England, July 1997

86. Invited Speaker: "Demyelinating neuropathies and autoantibodies", South Carolina Neurologic Society, Hilton Head Island, SC, August 1997.

87. Visiting Professor: "Demyelinating polyneuropathies and autoantibodies", Northwestern School of Medicine, Chicago, IL, October 1997

88. Visiting Professor: "Immune demyelinating polyneuropathies", University of Washington School of Medicine, Seattle Washington, December 1997

89. Speaker: "Immune Neuropathies", Grand Rounds, Washington University School of Medicine, January 1998.

90. Visiting Faculty: "Immune Neuropathies", "DNA repeat sequences and neurological disorders", Southern Clinical Neurological Society, Bay of Huatulco, Mexico, January 1998.

91. Invited participant: "Neuropathy Centers Meeting", Dorado Beach, Puerto Rico, January 1998.

92. Speaker: "DNA repeat sequences and neurological disorders", Grand Rounds, Washington University School of Medicine, February 1998.

93. Visiting Professor: "Demyelinating polyneuropathies and autoantibodies", Mt. Sinai School of Medicine, New York City, NY, February 1998.

94. Chairman: NIH Special Study Section, March 1998.

95. Invited Participant: "Expert panel consensus on the use of IVIG for Neurologic diseases. Washington, DC, April 1998.

96. Visiting Professor: "Demyelinating polyneuropathies and autoantibodies", SUNY Syracuse, NY, April 1998.

97. Invited speaker: "Myasthenia Gravis", Neuroimmunology Course, American Academy of Neurology, Minneapolis, Minnesota, April 1998. 98. Invited instructor: "Computers in Neurology", Beginner and Advanced Computer Course, American Academy of Neurology, Minneapolis, Minnesota, April 1998.

99. Visiting Professor: "Immune-mediated demyelinating polyneuropathies", Cleveland Clinic, Cleveland, Ohio, May 1998.

100. Swank Visiting Professor: "Autoantibodies and polyneuropathies", " Inflammatory myopathies" University of Oregon, Portland, Oregon, June 1998.

101. Visiting Professor: "Immune demyelinating polyneuropathies", University of Florida, Gainesville, Florida, July 1998.

102. Invited speaker: "Weakness in the intensive care unit", Department of Anesthesia, Washington University, St. Louis, July 1998.

103. Invited participant: "Gene therapy for muscular dystrophy", Muscular Dystrophy Association, Tucson, Arizona, August 1998.

104. Invited speaker: "Anti-sulfatide antibodies and peripheral neuropathy", Peripheral Nerve Society, Epidaurus, Greece, August 1998.

105. Workshop leader: "Antiglycolipid antibodies and polyneuropathies", "Paraneoplastic neuropathies", International Congress of Neuromuscular Disease, Adelaide, Australia, September 1998.

106. Invited speaker: "Lower Motor Neuron Syndromes", International Congress of Neuromuscular Disease, Adelaide, Australia, September 1998.

107. Visiting Professor: "Immune demyelinating polyneuropathies", University of Oklahoma, Oklahoma City, Oklahoma, October 1998.

108. Invited speaker: "Paraneoplastic neuropathies" Presidential symposium, American Academy of Neurology, Montreal, Quebec, Canada, October 1998.

109. Visiting Professor: "Immune demyelinating polyneuropathies", University of Texas, Southwestern, Dallas, Texas, November 1998.

110. Visiting Professor: "Immune demyelinating polyneuropathies", Yale University School of Medicine, New Haven, Connecticut, December 1998.

111. Visiting Professor: "Immune demyelinating polyneuropathies", University of California, San Diego, San Diego California, January 1999.

112. Invited speaker: "Chronic immune demyelinating neuropathies" Course: Neuroimmunology and therapy of immune-mediated neurological and neuromuscular disease, Toronto, Ontario, Canada, April 1999.

113. Course Director "Muscle Biopsy", Dinner seminar, American Academy of Neurology, Toronto, Ontario, Canada, April 1999.

114. Invited speaker: "Autoantibodies and demyelinating neuropathies" Quest Diagnostics, San Juan de Capistrano, CA, April 1999.

115. Visiting professor: "Chronic immune demyelinating ", "Paraneoplastic neuromuscular syndromes", University of Miami, Miami, FL. June 1999.

116. Visiting professor: "Chronic immune demyelinating neuropathies", University of North Carolina, Chapel Hill, NC, September 1999.

117. Invited speaker: "Chronic immune demyelinating neuropathies", Duke University; Durham, NC, September 1999.

118. Invited speaker: "Chronic immune demyelinating neuropathies", University of Melbourne, Melbourne, Victoria, Australia, October 1999.

119. Visiting professor: "Chronic immune demyelinating neuropathies", SUNY Buffalo, Buffalo, NY, November 1999.

120. Visiting professor: "Chronic immune demyelinating neuropathies", University of Texas, Houston, TX, December 1999.

121. Invited speaker: "Chronic immune demyelinating neuropathies" "Inflammatory myopathies", Neurological Update 2000, University of Miami, Miami, FL, January 2000.

122. Visiting Professor: "Chronic immune demyelinating neuropathies", Harvard-Longwood Boston, MA, March 2000.

123. Visiting Professor: "Chronic immune demyelinating neuropathies", University of California, Davis, Sacramento, CA, March 2000.

124. Invited speaker: "Chronic immune demyelinating neuropathies" Kaiser-Permanente Neurology Symposium, Santa Monica, CA, March 2000.

125. Program chairman and speaker: "Myasthenia Gravis 2000" Myasthenia Gravis: Clinical and Basic Concepts, Myasthenia Gravis Foundation of America, St. Louis, MO, April 2000.

126. Invited speaker: "Neuromuscular disorders: Principles of therapy" Course: Immunosuppressive treatment for neurologists, San Diego, CA, April 2000.

127. Course Director "Muscle Biopsy", Dinner seminar, American Academy of Neurology, San Diego, CA, May 2000.

128. Visiting Professor: "Chronic immune demyelinating neuropathies", University of Colorado, Denver, CO, June 2000.

129. Invited speaker: "Antibodies and Polyneuropathies" Kroc Neuroscience Symposium 2000; Johns Hopkins School of Medicine, Baltimore, MD, June 2000.

130. Visiting Professor: "Chronic immune demyelinating neuropathies", University of California, San Francisco, CA, September 2000.

131. Invited speaker: "Immune-mediated Demyelinating Polyneuropathies" "Multifocal Motor Neuropathy" First Portuguese Congress on Neuromuscular Diseases, Estoril, Portugal, October 2000.

132. Visiting Professor: "Chronic immune demyelinating neuropathies", Southern Illinois University, Springfield, IL, November 2000.

133. Invited speaker: "Chronic immune demyelinating neuropathies", North Carolina Neurological Society Annual Meeting, Greensboro, NC, November 2000.

134. Invited participant: "Childhood CIDP", 88th ENMC Conference, Naarden, Netherlands, December 2000.

135. Invited speaker: "Chronic immune demyelinating neuropathies", Phoenix AZ, February 2001.

136. Invited speaker: "New concepts in immune myopathies", St. Johns Hospital Medical Grand Rounds, St. Louis, MO, February 2001.

137. Invited speaker: "Neuromuscular disease and the internet: Learning and teaching", Israeli Neuromuscular Society, Tel-Aviv, Israel, March 2001.

138. Invited speaker: "Chronic immune demyelinating neuropathies" "New concepts in immune myopathies", Israeli Society of Clinical Neurophysiology, Tel-Aviv, Israel, March 2001.

139. Invited speaker: "Treatment of immune mediated neuromuscular disorders", Symposium: "Glaucoma: Learning from other neurodegenerations" Sarasota, FL March 2001.

140. Visiting Professor: "Chronic immune demyelinating neuropathies: Diagnosis and Treatment", Johns Hopkins School of Medicine, Baltimore, MD, April 2001.

141. Invited speaker: "Neuromuscular disorders: Principles of therapy" Course: Immunosuppressive treatment for neurologists, Philadelphia, PA, May 2001.

142. Course Director "Nerve and Muscle Biopsy", Dinner seminar, American Academy of Neurology, Philadelphia, PA, May 2001.

143. Invited speaker: "Neuromuscular disease and the internet: Learning and teaching" Course: Computers and Neurology, Philadelphia, PA, May 2001.

144. Visiting Professor: "Chronic immune demyelinating neuropathies: Diagnosis and Treatment", Northwestern School of Medicine, Chicago, IL, May 2001.

145. Invited speaker: "Painful Neuropathies", Pain management group, Washington University School of Medicine, St. Louis, MO, June 2001.

146. Visiting Professor: "Evaluation of Neuromuscular Disorders" "Muscular Dystrophies" "Chronic immune demyelinating neuropathies" "Immune myopathies" Contemporary Neurology Course, Vanderbilt School of Medicine, Hilton Head, SC, July 2001.

147. Invited speaker: "Chronic Immune Motor Neuropathies", World Muscle Society, Snowbird, UT, September 2001.

148. Invited speaker: "Chronic Immune Motor Neuropathies", Grand Rounds, Neurology, Washington University School of Medicine, September 2001.

149. Invited speaker: "Chronic immune demyelinating neuropathies", Orange County Neurological Society, Costa Mesa, CA, October, 2001.

150. Visiting Professor: "Chronic immune motor neuropathies", University of California, Irvine, Orange, CA, October, 2001.

151. Invited speaker: "Chronic immune demyelinating neuropathies", Allegheny Hospital Pittsburgh, PA, October, 2001.

152. Invited speaker: "Rituxan treatment of IgM Associated Neuropathies", Maui, HI, January 2002.

153. Carrell-Krusen Lecturer: "New Concepts in Inflammatory Myopathies" University of Texas Southwestern, Dallas, TX, February, 2002.

154. Visiting Professor: "Immune Demyelinating Neuropathies: Diagnosis and Treatment" University of Texas Southwestern, Dallas, TX, February, 2002.

155. Invited speaker: "New Concepts in Inflammatory Myopathies" University of Melbourne, Melbourne, Victoria, Australia, March, 2002.

156. Visiting Professor: "Immune Demyelinating Neuropathies: Diagnosis and Treatment" New Jersey College of Medicine and Dentistry, Newark, NJ, March, 2002.

157. Invited speaker: "IgM associated Peripheral Neuropathy" International Waldenström's Macroglobulinemia Foundation, Las Vegas, NV, April, 2002.

158. Course Director "Practical Immunotherapy of Neuromuscular Disorders", American Academy of Neurology, Denver, CO, April 2002.

159. Invited speaker: "Neuromuscular Disorders" Course: Crossfire, Denver, CO, April 2002.

160. Visiting Professor: "Immune Demyelinating Neuropathies: Diagnosis and Treatment" University of Maryland College of Medicine, Baltimore, MD, May, 2002.

161. Invited speaker: "Neuromuscular Disease and the Internet", International Congress of Neuromuscular Diseases, Vancouver, BC, Canada, July 2002.

162. Visiting Professor: "Immune Demyelinating Neuropathies: Diagnosis and Treatment" Baylor College of Medicine, Houston, TX, August, 2002.

163. Invited speaker: "Antigenic targets of IgM M-proteins in Waldenström's macroglobulinemia", 2nd International Workshop on Waldenström's macroglobulinemia, Athens, Greece, September 2002.

164. Invited speaker: "Demyelinating neuropathies with conduction block" University of Melbourne, Melbourne, Victoria, Australia, October, 2002.

165. Visiting Professor, Davee Guest Lecturer: "New Concepts in Inflammatory Myopathies", Northwestern School of Medicine, Chicago, IL, November 2002.

166. Invited speaker: "New Concepts in the diagnosis and treatment of Inflammatory Myopathies", Department of Neurology Grand Rounds, Washington University School of Medicine, Saint Louis, MO, January 2003.

167. Invited speaker: "New Concepts in the diagnosis and treatment of Inflammatory Myopathies", San Diego Neurological Society, San Diego, CA, February 2003.

168. Visiting Professor, "Immune Demyelinating Neuropathies: Diagnosis and Treatment" University of California, San Diego, CA, February 2003.

169. Visiting Professor, "Immune Demyelinating Neuropathies: Diagnosis and Treatment" University of Massachusetts, Worcester, MA, March 2003.

170. Invited speaker: "New Concepts in the diagnosis and treatment of Inflammatory Myopathies", Division of Rheumatology Grand Rounds, Washington University School of Medicine, Saint Louis, MO, April 2003.

171. Course Director "Practical Immunotherapy of Neuromuscular Disorders", American Academy of Neurology, Honolulu, HI, April 2003.

172. Visiting Professor, "Immune Demyelinating Neuropathies: Diagnosis and Treatment" Medical College of Wisconsin, Milwaukee, WI, May 2003.

173. Visiting Professor, "Immune Demyelinating Neuropathies: Diagnosis and Treatment" Hahnemann School of Medicine, Philadelphia, PA, June 2003.

174. Visiting Professor, "Immune Demyelinating Neuropathies: Diagnosis and Treatment" University of Wisconsin, Madison, WI, August 2003.

175. Visiting Professor, "Immune Demyelinating Neuropathies: Diagnosis and Treatment" University of Kansas, Kansas City, MO, September 2003.

176. Course Director "Practical Immunotherapy of Neuromuscular Disorders", American Academy of Neurology, San Francisco, CA, April 2004.

177. Visiting Professor, "Immune Demyelinating Neuropathies: Diagnosis and Treatment" University of Illinois, Chicago, IL, October 2004.

178. Invited speaker: "Chronic immune demyelinating neuropathies" "Inflammatory myopathies, New Concepts", Neurological Update 2005, University of Miami, Miami, FL, January 2005.

179. Visiting Professor, "Immune Demyelinating Neuropathies: Diagnosis and Treatment" Duke University, Durham, NC, March 2005.

180 Visiting Professor, "Immune and Inflammatory Myopathies" University of Oklahoma, Oklahoma City, OK, March 2005.

181. Course Director "Practical Immunotherapy of Neuromuscular Disorders", American Academy of Neurology, Miami, FL, April 2005.

182. Visiting Professor, "Immune Demyelinating Neuropathies: Diagnosis and Treatment" University of Calgary, Calgary, Alberta, Canada, May 2005.

183. Visiting Professor, "Immune Demyelinating Neuropathies: Diagnosis and Treatment" Medical College of Virginia, Richmond, VA, May 2005.

184. Visiting Professor, "Immune Demyelinating Neuropathies: Diagnosis and Treatment" North Shore University Hospital, Great Neck, NY, June 2005.

185. Invited speaker: "Auto-antibodies in chronic neuromuscular disorders: Insights into diagnosis, treatment and pathogenesis" Neurology Grand Rounds Washington University School of Medicine, St. Louis, MO, October 2005.

186. Invited speaker: "Chronic immune demyelinating neuropathies" "Immunotherapy in Neuromuscular Disorders", World Congress of Neurology, Sydney, Australia, November 2005.

187. Invited speaker: "Pathology-based classification of Immune Myopathies" Internal Medicine Grand Rounds Washington University School of Medicine, St. Louis, MO, February 2006.

188. Visiting Professor, "Immune Demyelinating Neuropathies: Diagnosis and Treatment" Albert Einstein School of Medicine, Bronx, NY, March 2006.

189. Invited speaker: "Immune and Inflammatory Myopathies", American Academy of Neurology, San Diego, CA, April 2006.

190. Visiting Professor, "Immune and Inflammatory Myopathies" University of Pennsylvania School of Medicine, Philadelphia, PA, May 2006.

191. Invited speaker: "Immunotherapy in Neuromuscular Disorders", International Congress of Child Neurology, Montreal, Quebec, Canada, June 2006.

192. Invited speaker: "Immune Demyelinating Neuropathies: Diagnosis and Treatment" "Meet the Expert: Immune and Inflammatory Myopathies" International Congress of Neuromuscular Disease, Istanbul, Turkey, July 2006.

193. Visiting Professor, "Immune Demyelinating Neuropathies: Diagnosis and Treatment" University of Colorado School of Medicine, Denver, CO, September 2006.

194. Invited speaker: "Immune Demyelinating Neuropathies: Diagnosis and Treatment" Orange County Neurological Society, Irvine, CA, October 2006.

195. Visiting Professor, "Immune and Inflammatory Myopathies" University of California, Irvine School of Medicine, Irvine, CA, October 2006.

196. Invited speaker: "Autoantibodies and Neuromuscular disorders", "Immune and Inflammatory myopathies" University of Miami School of Medicine, Miami, FL, January 2007.

197. Visiting Professor, "Immune and Inflammatory Myopathies" University of Tennessee School of Medicine, Memphis, TN, Feb 2007.

198. Visiting Professor, "Autoantibodies and Neuromuscular disorders" Northwestern University School of Medicine, Chicago, IL, Mar 2007.

199. Invited speaker: "Autoantibodies and Neuromuscular disorders", American Academy of Neurology, Boston, MA, April 2007.

200. Visiting Professor, "Immune and Inflammatory Myopathies" Southern Illinois University School of Medicine, Springfield, IL, May 2007.

201. Invited speaker: "Autoantibodies and Neuromuscular disorders", Neurology Info 2007, Ho Chi Minh City, Vietnam, July 2007.

202. Invited speaker: "Autoantibodies and Neuromuscular disorders", Puerto Rican Academy of Neurology, San Juan, PR, January 2008.

203. Visiting Professor, "Immune Demyelinating Neuropathies: Diagnosis and Treatment" University of Kentucky School of Medicine, Lexington, KY, Mar 2008.

204. Visiting Professor, "Autoantibodies and Neuromuscular disorders" University of Michigan School of Medicine, Ann Arbor, MI, Apr 2008.

205. Invited speaker: "Muscle Biopsy and Neuromuscular disorders", "Immune and Inflammatory Myopathies" Mount Sinai School of Medicine, New York, NY, May 2008.

206. Visiting Professor, "Immune and Inflammatory Myopathies", "Immune Demyelinating Neuropathies: Diagnosis and Treatment" University of Nebraska School of Medicine, Omaha, NE, June 2008.

207. Invited speaker: "Acid maltase deficiency", National Women's Medical College, Tokyo, Japan, June 2008.

208. Visiting Professor, Royal College Lecturer: "Immune and Inflammatory Myopathies", "Immune Demyelinating Neuropathies", "Autoantibodies and Neuromuscular disorders" University of British Columbia, Vancouver, BC, Canada, March 2009.

209. Invited Speaker: "Muscle Biopsy in Immune and Inflammatory Myopathies" Myositis Association, Saint Louis, MO, September 2010.

210. Invited Speaker: "Autoantibodies and Neuromuscular Disease" American Academy of Electrodiagnostic Medicine, Quebec City, Quebec, October 2010.

211. Invited speaker: "Autoantibodies and Neuromuscular Disease" "Pathological Diagnosis of Immune and Inflammatory Myopathies" Israel Clinical Neurology and Neurophysiology Meeting. Tel Aviv, Israel, February 2011.

212. Invited Speaker: "Dermatomyositis: Pathology and Pathogenesis" Hadassah University, Jerusalem, Israel, February 2011.

213. Visiting Professor: "Pathological Diagnosis of Immune and Inflammatory Myopathies" Johns Hopkins School of Medicine, Baltimore, MD, March 2011.

214. Visiting Professor: "Dermatomyositis and other Immune Myopathies" University of Melbourne School of Medicine, Melbourne, Vic, Australia, April 2011.

215. Invited Speaker: "Pathological Diagnosis of Immune and Inflammatory Myopathies" American Academy of Neuropathology, Seattle WA June 2011.

216. Invited Speaker: "Practical Treatments for Neuromuscular Disorders" American Academy of Electrodiagnostic Medicine, San Francisco, CA September 2011.

217. Invited Speaker: "Autoantibodies in Neuromuscular Disorders" "Immune Demyelinating Neuropathies" Israel Neuromuscular Conference, Tel Aviv, Israel, February 2012.

218. Visiting Professor: "Autoantibodies in Neuromuscular Disorders" New York University School of Medicine, New York, NY, March 2012.

219. Visiting Professor: "Autoantibodies in Neuromuscular Disorders" Saint Louis University School of Medicine, Saint Louis, MO, May 2012.

220. Visiting Professor: "Pathological Diagnosis of Immune and Inflammatory Myopathies" University of Kansas School of Medicine, Kansas City, KS, June 2012.

221. Invited speaker: "Pathological Diagnosis of Immune and Inflammatory Myopathies" The European NeuroMuscular Centre (ENMC), Amsterdam, Netherlands, December 2012.

222. Visiting Professor: "Pathological Diagnosis of Immune and Inflammatory Myopathies" Saint Louis University School of Medicine, Saint Louis, MO, May 2013.

223. Invited speaker: "Pathologic Diagnosis of Immune and Inflammatory Myopathies" World Muscle Society, Asilomar, CA October 2013.

224. Visiting Professor: "Immune and Inflammatory Myopathies", "Neuromuscular Examination" University of Washington School of Medicine, Seattle, WA, March 2014.

225. Visiting Professor, "Immune and Inflammatory Myopathies", "Neuromuscular Examination" University of Nebraska School of Medicine, Omaha, NE, May 2014.

226. Invited speaker: "Pathologic Diagnosis of Immune and Inflammatory Myopathies" UC Irvine Neuromuscular Colloquium, Huntington Beach, CA, June 2014.

227. Invited speaker: "Pathologic Diagnosis of Immune and Inflammatory Myopathies"; "Immune Demyelinating Neuropathies", Neurology Update 2015; University of Miami, Miami, FL, January 2015.

228. Visiting Professor: "Pathologic Diagnosis of Immune and Inflammatory Myopathies", University of Colorado, Denver, CO, March 2015.

229. Visiting Professor: "Immune and Inflammatory Myopathies", University of Alabama, Birmingham, Birmingham, AL, April 2015.

230. Visiting Professor: "Immune and Inflammatory Myopathies"; "Muscle biopsies", University of Manitoba, Winnipeg, Manitoba, Canada, May 2015.

231. Invited speaker: "Pathologic Diagnosis of Immune and Inflammatory Myopathies", Myositis Night, Montreal, Quebec, Canada, May 2015.

232. Visiting Professor: "Immune and Inflammatory Myopathies", " Approach to Neuromuscular Patient", "Muscle biopsies": Tulane University, New Orleans, LA, May 2015.

233. Visiting Professor: "Immune and Inflammatory Myopathies": University of New Mexico, Albuquerque, NM, June 2015.

234. Invited speaker: "Pathologic Diagnosis of Immune and Inflammatory Myopathies" 3rd Ottawa International Conference on Neuromuscular Biology, Disease & Therapy. Ottawa, Canada, September 2015.

235. Invited speaker: "Pathologic Diagnosis of Immune and Inflammatory Myopathies" "Muscle Pathology" XXVIII World Congress of the World Association of Societies of Pathology and Laboratory Medicine. Cancun, Mexico, November 2015.

236. Visiting Professor: "Pathologic Diagnosis of Immune and Inflammatory Myopathies": Yale University, New Haven CT. March 2016.

237. Invited Speaker: "Pathologic Diagnosis of Immune and Inflammatory Myopathies": Saint Louis University, Department of Rheumatology, Saint Louis, MO. April 2016.

238. Visiting Professor: "Pathologic Diagnosis of Immune and Inflammatory Myopathies": University of Iowa, Iowa City, IA. May 2016.

239. Invited speaker: "Pathologic Diagnosis of Immune and Inflammatory Myopathies": Cuban Neurological Association, Havana Cuba, June 2016.

240. Visiting Professor: "Pathologic Diagnosis of Immune and Inflammatory Myopathies": Dartmouth University, Hanover NH. August 2016.

241. Invited speaker: "Diagnostic testing for Inclusion Body Myositis": American Academy for Neuromuscular and Electrodiagnostic Medicine. New Orleans, LA. September 2016.

242. Invited speaker: "Immune Myopathies with Necrosis" European Neuromuscular Centre. Amsterdam, The Netherlands. October 2016

243. Visiting Professor: Nigel Harris Lecturer, "Pathologic Diagnosis of Immune and Inflammatory Myopathies": University of Louisville, Louisville, KY. October 2016.

244. Visiting Professor: "Pathologic Diagnosis of Immune and Inflammatory Myopathies": Mount Sinai School of Medicine, New York, NY. October 2016.

245. Visiting Professor: "Pathologic Diagnosis of Immune and Inflammatory Myopathies": Department of Medicine (Rheumatology) Yale School of Medicine, New Haven, NY. November 2016.

246. Visiting Professor: "Autoantibodies in Neuromuscular Disorders" Saint Louis University School of Medicine, Saint Louis, MO, January 2017.

247. Visiting Professor: "Pathologic Diagnosis of Immune and Inflammatory Myopathies"; "Autoantibodies in Neuromuscular Disorders": Hospital for Special Surgery, New York, NY. February 2017.

248. Invited speaker: "Muscle Biopsy in Immune Myopathies": American Academy for Neuromuscular and Electrodiagnostic Medicine. Phoenix, AZ. September 2017.

249. Invited Speaker: "Pathologic Diagnosis of Immune and Inflammatory Myopathies"; "Autoantibodies in Neuromuscular Disorders": 4th Western Canadian Neuromuscular Conference. Edmonton, AB Canada October 2017.

250. Visiting Professor: "Pathologic Diagnosis of Immune and Inflammatory Myopathies"; Royal Melbourne Hospital, University of Melbourne, Melbourne, Victoria, Australia February 2018.

251. Invited Speaker: "Sarcopenia"; "Chronic Immune Motor Neuropathies": International Congress of Neuromuscular Disorders. Vienna, Austria. July 2018.

252. Invited Speaker: "Neuromuscular Examination: Performance & Interpretation": Neurohospitalist Society, Saint Louis, MO. October 2018.

253. Invited Speaker: "Autoantibodies in Neuromuscular Disorders": Texas Neurological Society, Austin, TX. February 2019.

254. Invited Professor & Consultant: "Immune Myopathies: Pathologic Analysis Directs Diagnosis and Treatment", "Muscle Biopsy", "Nerve Biopsy", "Autoantibodies in Neuromuscular Disorders: Clues to diagnosis and treatment", "Sarcopenia", "Clinical Approach to Neuromuscular Disorders", "Demyelinating Polyneuropathies", "Myasthenia Gravis"; Singapore Ministry of Health, Tan Tock Seng Hospital, National Union Hospital, Singapore General Hospital; Singapore, February 2019.

255. Invited Speaker: "Immune Myopathies: Pathologic Analysis Directs Diagnosis and Treatment" Grand Rounds; Cedars-Sinai Medical Center, Los Angeles, CA, April 2019.

256. Invited Speaker: "Immune Myopathies: Pathologic Analysis; New Syndromes"; International Congress of Neuromuscular Disorders. May 2021.

257. Invited Speaker: "Immune Myopathies: Pathologic Analysis, New Syndromes"; What's in a Nerve? HIEM: New Treatable Neuropathy; University of California, Irvine; June 2021.

258. Invited Speaker: "What’s in a Nerve? Pathology Analysis Improved; A New Treatable Neuropathy." Advances in Neurology, University of Louisville, Kentucky; October 2021

259. Invited Speaker: "What's in a Nerve? Pathology Analysis: Improved with Frozen sections; A New Treatable Neuropathy." International Congress of Neuromuscular Diseases July 2022

260. Invited Speaker: "What's in a Nerve? Analysis with Frozen sections Reveals a New Treatable Neuropathy." Grand Rounds; Albert Einstein Medical Center. November 2022.

261. Invited Speaker & 20th Athasit Oration: "What's in a Nerve? Analysis with Frozen Sections Reveals a New Treatable Neuropathy; "Immune Myopathies: Pathologic Analysis Directs Diagnosis and Treatment"; "Immune Myopathies: Muscle Pathology-Defined Clinical Syndromes"; "Autoantibodies in Neuromuscular Disorders: Clues to Diagnosis and Treatment." Neurological Society of Thailand. Bangkok, Thailand. March 2023.

ABSTRACTS

1. Pestronk, A., Drachman, D.B. (1975) Trophic regulation of ACh receptors: Partial effect of ACh transmission. Neurology 25:373.

2. Pestronk, A., Drachman, D.B., and Griffin, J.W. (1976) Trophic regulation of ACh receptors: Partial effect of usage. Neurology 26:348.


3. Toyka, K.V., Griffin, D.E., Pestronk, A., Fischbeck, K.H., Kao,I., and Drachman, D.B. (1976) Myasthenia gravis: passive transfer from man to mice with immunoglobulins. Fed Proc 23:437.

4. Pestronk, A., and Drachman, D.B. (1977) Disuse of muscle evokes nerve sprouting. Neurology 27:393-394.

5. Pestronk, A., and Drachman, D.B. (1978) Motor nerve terminal sprouting and acetylcholine (ACh) receptors. Neurology 28:355.

6. Pestronk, A., and Drachman, D.B. (1978) The effect of aging on nerve sprouting. Ann Neurol 4:164.

7. Pestronk, A., and Drachman, D.B. (1978) Acetylcholine receptors induce morphological changes in motor nerve terminals. Presented at IVth International Congress on Neuromuscular Disease, Montreal, Quebec, Canada.

8. Pestronk, A., Keogh, J.P., and Griffin, J.W. (1979) Dimethylamino-propionitirile (DMAPN) intoxication: a new industrial neuropathy. Neurology 29:549.

9. Pestronk, A., Drachman, D.B., Stanley, E.F., and Adams, R.N. (1980) Treatment of experimental autoimmune myasthenia gravis (EAMG) using single, high-dose cyclophosphamide. Neurology 30:388.

10. Hemphill, M., Pestronk, A., Walsh, T., Parhad, I.M., Clark, A., Rosenshein, N. (1980) Sensory neuropathy in cis platinum chemotherapy. Neurology 30:429.

11. Parhad, I.M., Pestronk, A., Price, D.L., and Drachman, D.B. (1980) Membrane myopathy: morphologic similarities to Duchenne's muscular dystrophy. Neurology 30:422.

12. Pestronk, A., Drachman, D.B., Stanley, E.F. (1980) Therapeutic strategies in experimental autoimmune myasthenia gravis. Presentation at New York Academy of Sciences. New York, New York.

13. Stanley, E.F., Pestronk, A., Drachman, D.B., Griffin, J.W., and Price, D.L. (1980) The role of ACh transmission in the neural regulation of muscle resting membrane potential. Soc for Neuroscience, Cincinnati, Ohio. Soc Neurosci Abst 6:384.

14. Pestronk, A., Drachman, D.B., Josifek, L.F. (1981) Measurement of junctional ACh receptors in myasthenia gravis: diagnostic value and clinical correlates. Neurology 31(2):83.

15. Pestronk, A., (1981) "Hidden" a-bungarotoxin binding sites in rat skeletal muscle in vivo. Soc Neurosci Abst 7:701.

16. Pestronk, A., and Drachman, D.B. (1982) Reduction of acetylcholine receptors (AChRs) in polymyositis. Neurology 32:A120.

17. Pestronk, A., and Drachman, D.B. (1982) Therapeutic strategy eliminates anti-AChR antibody response in experimental myasthenia gravis. Vth International Congress on Neuromuscular Diseases.

18. Drachman, D.B., Pestronk, A., Tutschka, P. (1982) Cyclosporin A in the treatment of experimental autoimmune myasthenia gravis. Vth International Congress on Neuromuscular Diseases.

19. Pestronk, A., and Drachman, D.B. (1984) Motor nerve sprouting. Effects of a-bungarotoxin and anti-AChR antibody. Soc for Neurosci 10:1020 (Part II).

20. Pestronk, A., and Drachman, D.B. (1984) Motor nerve sprouting: effects of a-bungarotoxin and anti-AChR antibody. Ann Neurol 16:147. 21. Pestronk, A., Tech, R., and Drachman, D.B. (1985) Effects of dimethyl sulfoxide on the humoral immune response to acetylcholine receptors. Neurology 35:101.

22. Cornblath, D., Pestronk, A., Yee, W.C., and Kuncl, R.W. (1985) Congenital myasthenia with increased numbers of acetylcholine receptors at neuromuscular junction. Muscle & Nerve 8:611.

23. Yee, W.C., and Pestronk, A. (1985) Motor nerve terminal outgrowth induces rapid modification of junctional acetylcholine receptor clusters in adult mammalian skeletal muscle. Soc for Neurosci 11:1146.

24. Griffin, J.W., Hoffman, R.N., Gold, B.G., Pestronk, A., Price, D.L., and Cork, L.C. (1986) Somatofugal axonal atrophy: a sensitive early marker of axonal dysfunction. ANA Meeting. Ann Neurol. 20:131A.

25. Yee, W.C., and Pestronk, A. (1986) Mechanisms of postsynaptic plasticity: modification of junctional acetylcholine receptor clusters induced by motor nerve terminal outgrowth. VI International Congress on Neuromuscular Disease.

26. Griffin, J.W., Gold, B.G., Pestronk, A., Stanley, E.F., Price, D.L., and Fahnestock, K. (1986) Experimental axonal atrophy produced by botulinum toxin. Neurology 36:80.

27. Kuncl, R.W., Pestronk, A., Cornblath, D.R., and Yee, W.C. (1986) Morphometric analysis of a congenital myasthenic syndrome characterized by increased acetylcholine receptors. Neurology 36:162.

28. Feldman, E.L., Hoffman, P.N., Pestronk, A., and Griffin, J.W. (1986) Cytoskeletal organization during axonal outgrowth. Soc for Neurosci 12:277.

29. Pestronk, A., and Drachman, D.B. (1986) Mechanism of action of lithium on acetylcholine receptor metabolism in skeletal muscle. Soc for Neurosci 12:308. 30. Gold, B.G., Griffin, J.W., Pestronk, A., Hoffman, P.N., Stanley, E.F., and Price, D.L. (1986) Somatofugal axonal atrophy produced by botulinum toxin. Soc for Neurosci 12:1108.

31. Yee, W.C., and Pestronk, A. (1986) Mechanisms of postsynaptic plasticity: modification of junctional acetylcholine receptor clusters induced by motor nerve terminal outgrowth. Soc for Neurosci 12:1106.

32. Alderson, K., Pestronk, A., Griffin, J.W., and Drachman, D.B. (1987) Motor nerve terminal sprouts express local signals for Schwann cell mitogenesis and myelination. Neurology 37:355.

33. Pestronk, A. (1987) The first neurology book: "De cerebri morbis..." (1549) by Jason Pratensis. Neurology 37:240.

34. Pestronk, A., and Drachman, D.B. (1987) Motor nerve outgrowth: reduced capacity for sprouting in the terminals of longer axons. Neurology 37:254.

35. Drachman, D.B., DeSilva, S., Ramsey, D., and Pestronk, A. (1987) "Sero-negative" myasthenia gravis: a humorally-mediated variant of myasthenia. Neurology 37:214.

36. Corey, A.L., Pestronk, A., Richman, D.P., and Drachman, D.B. (1987) Quantitation of acetylcholine receptors per neuromuscular junction in rats with passively induced experimental myasthenia. Neurology 37:303.

37. Pestronk, A., Cornblath, D.R., Ilyas, A.A., Baba, H., Quarles, R.H., Griffin, J.W. (1987) A treatable chronic, multifocal, motor polyneuropathy associated with antibodies to a defined neural antigen. Ann Neurol 22:119A

38. Yee, W.C., Pestronk, A. (1987) Cytoskeletal elements in motor nerve outgrowth in vivo: changes during axonal regeneration and terminal sprouting. Soc for Neurosci 13(2):1479.

39. Yee, W.C., Pestronk, A., Alderson, K. (1988) Regional heterogeneity in the distal motor axon: three zones with distinctive components. Neurology 38(1):189.

40. Pestronk, A., Adams, R.N., Clawson, L., Cornblath, D.R., Drachman, D.B., Griffin, D., Kuncl, R.W. (1988) Multifocal motor neuropathy: clinical features of patients with anti-GM1 ganglioside antibodies. Neurology 38(1):251.

41. Alderson, K., Pestronk, A., Griffin, J.W., Drachman, D.B. (1988) Motor nerve terminal morphology: a new diagnostic technique in peripheral neuropathy. Neurology 38(1):351.

42. Pestronk, A. (1988) The first neurology book written in English: De morbis capitis... (1650), by Robert Pemell. Neurology 39(1):385.

43. Pestronk, A., Adams, R.N., Clawson, L., Cornblath, D., Kuncl, R.W., Griffin, D., Drachman, D.B. (1988) Serum antibodies to GM1 ganglioside in amyotrophic lateral sclerosis. Ann Neurology 24:122.

44. Yee, W.C., Pestronk, A., Hahn, A.F. (1988) Laminin promotes motor nerve sprouting in vivo. Soc for Neurosci 14:1170.

45. Baba, H., Ilyas, A., Pestronk, A., Cornblath, D., Griffin, J., Quarles, R. (1988) Antibodies GM1 ganglioside in multifocal motor neuropathy. Am Soc Neurochem 19, 193.

46. Pestronk, A., Chaudhry, V., Feldman, E., Griffin, J.W., Cornblath, D.R., Denys, E.H., Glasberg, M., Kuncl, R.W., Olney, R.K., Yee, W.C. (1989) Lower motor neuron syndromes defined by patterns of weakness, nerve conduction abnormalities, and high titers of antiglycolipid antibodies. Ann Neurol 26:121-122.

47. Rothstein, J.D., Kuncl, R.W., Clawson, L.L., Cornblath, D.R., Drachman, D.B., Pestronk, A. (1989) Cerebrospinal fluid excitatory amino acids in amyotrophic lateral sclerosis. Ann Neurol 26:144.

48. Alderson, K., Griffin, J.W., Pestronk, A. (1989) Axonal swellings in neuromuscular disease: A common reflection of neurofilament transport alteration? Ann Neurol 16:146.

49. Drachman, D.B., Alving, C., McIntosh, K.R., Pestronk, A., Adams, R.N. (1989) Gangliosides elicit T-cell-independent antibody responses. Ann Neurol 26:150.

50. Alderson, K., Pestronk, A., Drachman, D.B. (1989) Abnormal myelination of distal motor nerves in amyotrophic lateral sclerosis and peripheral neuropathies. Ann Neurol 26:166-167.

51. Feldman, E.L., Pestronk, A., Bromberg, M.B., Albers, J.W. (1989) Multifocal demyelinating polyneuropathy: An electrodiagnostic and therapeutic profile. Neurology 39:319.

52. Pestronk, A., Adams, R.N., Kuncl, R.W., Drachman, D.B., Clawson, L.L., Cornblath, D. (1989) Differential effects of Prednisone and Cyclophosphamide on autoantibodies in human neuromuscular disorders. Neurology 39:219.

53. Cornblath, D.R., Kuncl, R.W., Mellitus, E.D., Quaskey, S.A., Clawson, L., Drachman, D.B. and Pestronk, A. (1990) Nerve conduction studies in amyotrophic lateral sclerosis (ALS). Neurology 40:4585.

54. Chaudhry, V., Pestronk, A., Drachman, D. (1990) Different patterns of glycolipid antibody reactivity: lower motor neuron syndromes vs immunization. Neurology 40:1185.

55. Sesodia, S., Nemeth, P.M., Choksi, R.M., Pestronk, A. (1990) High energy metabolism in severely atrophic muscle fibers in dermatomyositis. Society for Neuroscience.

56. Pestronk, A., Li, F., Griffin, J., Feldman, E., Cornblath, D., Trotter, J., Windsor, B. (1990) Antibodies to myelin-associated glycoprotein and sulfate in predominantly sensory polyneuropathies. American Neurological Association 28:239.

57. Moxley, R.T., Lorenson, M., Griggs, R.C., Mendell, J.R., Fenichel, G.M., Brooke, M. H., Miller, J. P. and CIDD Group. (1990) Decreased breakdown of muscle protein after prednisone therapy in Duchenne dystrophy (DD): Journal of Neurol Science 98:295.

58. Griggs, R.C. Moxley, R.T., Pandya, S., Mendell, J.R., King, W.M., Signore, L., Fenichel, G.M., Robison, J.R., Brooke, M.H., Matheson, N., Pestronk, A., Florence, J., Schierbecker, J., Miller, J.P., Wilson, B., Baty, J. (1991) Randomized, controlled trial of prednisone and azathioprine in Duchenne dystrophy. Neurology (Abstract) 41 (Suppl 1) 183S.

59. Fenichel, G.M., Florence, J., Pestronk, A., Miller, J.P., Baty, J., Wilson, B., Schierbecker, J. (1991) Prednisone slows strength decline in Duchenne Muscular Dystrophy: Two-year observation. Neurology 41 (Suppl 1) 182S.

60. Kuncl, R.W., Drachman, D.B., Wittstein, I., Adams, R., Wiggins, W., Avila, O., Pestronk, A., McIntosh, K., Maurath, d., DeSilva, S. (1991) Novel therapy of experimental myasthenia gravis by reducing endocytosis of acetylcholine receptors. Neurology 41 (Suppl 1) 140S.

61. Glasberg, M.R., Pestronk, A. (1991) Cyclophosphamide treatment in ALS patients with elevated anti-GM, ganglioside antibody titers. Neurology 41 (Suppl 1) 956S.

62. Sawchak, J.A., Sher, J.H., Kula, R.W., Shafiq, S.A., Norman, M., Pestronk, A. (1991) Expression of prenatal myosin heavy chain in myofibers of a subgroup of patients with centronuclear myopathy. Neurology 41(Suppl 1) 1069P.

63. Simmons, Z., Feldman, E.L., Pestronk, A. (1992) Heterogeneity in polyneuropathy associated with elevated anti-MAG titers with and without IgM monoclonal gammopathy. Neurology 42(3):333.

64. Connolly, A.M., Pestronk, A. (1992) High-titer serum anti-tubulin antibodies are a marker for chronic inflammatory demyelinating polyneuropathy (CIDP). Neurology 42(3):409.

65. Olney, R.K., Pestronk, A. (1992) Prednisone treatment of multifocal predominantly motor neuropathy. Neurology 42(3):178.

66. Pestronk, A., Zhu, S., Feldman, D.L. (1992) A distinctive pattern of serum IgM reactivity to GM1 ganglioside and cyclophilin in multifocal motor neuropathy (MMN). Neurology 42(3):177.

67. ALS CNTF Treatment Study(ACTS) Phase I-II Study Group: Cedarbaum, J.M., Charatan, M., Stambler Tarrytown, NY; Brooks, B.R., Sanjak, M., Madison WI; Mitsumoto, H., Szirony, K. Cleveland, OH; Neville, H., Ringel, S., Brinkmann, J., Denver, CO: Pestronk, A., Florence, J., St. Louis, MO; J. Wittes, Brittain, E., Washington, DC. (1993) The amyotrophic lateral sclerosis functional rating scale (ALSFRS): prospective validation and changes over time. Neurology 43: A417.

68. ALS CNTF Treatment Study(ACTS) Phase I-II Study Group: Cedarbaum, J.M., Charatan, M., Stambler Tarrytown, NY; Brooks, B.R., Sanjak, M., Madison WI; Mitsumoto, H., Szirony, K. Cleveland, OH; Neville, H., Ringel, S., Brinkmann, J., Denver, CO: Pestronk, A., Florence, J., St. Louis, MO; J. Wittes, Brittain, E., Washington, DC. (1993) Recombinant human ciliary neurotrophic factor (rHCNTF) in amyotrophic lateral sclerosis (ALS) patients: Phase I-II safety, tolerability and pharmacokinetic studies. Neurology 43:A416.

69. Lopate, G., Pestronk, A., Zhu, S., Choksi, R., Li, F., Beiser, K. (1993) A pattern of serum IgM anti-GM1 antibody binding with specificity for motor neuropathy syndromes. Neurology 43:A343.

70. Al-Lozi, M., Pestronk, A., Flaris, N., Yee, W.C., Cooper, J. (1993) SAMM syndrome: a subacute myopathy with myosin-deficient fibers. Neurology 43:A279.

71. Pestronk, A., Choksi, R., Bieser, K., Goldstein, J.M., Adler, C.H., Caselli, R.J., George, E.B. (1993) GALOP Syndrome: A treatable autoimmune gait disorder with late-age onset. Ann Neurol 34:269.

72. Kornberg, A., Pestronk, A., Bieser, K., Ho, T.W., McKhann, G.M., Griffin, J.W., Cornblath, D.R., Wu, H.S. (1993) The clinical correlates of high titer IgG anti-GM1 antibodies. Ann Neurol 34:268.

73. Parks B.J., Pestronk A., Goldstein J.M. (1994) Electrodiagnostic features of neuropathy associated with antibodies to sulfatide. Muscle and Nerve 17:1119

74. Blume, G., Pestronk, A., Goodnough, L.T. (1995) Anti-MAG-associated polyneuropathies: Improvement following immunotherapy with monthly plasma exchange and intravenous cyclophosphamide. Neurology 45, 32P

75. Brooks, B.R., and the ALS CNTF treatment study (ACTS) Phase II-III study group. Phase II-III double-blind, placebo-controlled clinical trial of subcutaneous administration of recombinant human ciliary neurotrophic factor (rHCNTF) in amyotrophic lateral sclerosis. Neurology 45, 1017P.

76. Blume, G., Pestronk, A., Johns, D.R. (1996) Polymyositis with excessive numbers of cytochrome oxidase-negative muscle fibers has characteristic clinical features and a poor response to Prednisone. Neurology 46, A116.

77. Parks, B.J., Lopate, G., Goldstein, J., Yee, W-C, Pestronk, A. (1996) Antisulfatide antibodies and polyneuropathy syndromes: Electrophysiologic features in patients with and without serum IgM M proteins. Neurology 46(2), A234.

78. Al-Lozi, M.T., Pestronk, A. (1996) Myopathies with serum IgM binding to the BJ antigen and IgM deposition in muscle endomysium. Neurology 46(2), A344

79. Lopate, G., Parks, B.J., Goldstein, J. Pestronk, A., (1996) Antisulfatide antibodies and polyneuropathy syndromes: Clinical features in patients with and without serum IgM M-proteins. Neurology 46(2), A380.

80. Pestronk, A., Choksi, R., Blume, G., Lopate, G. (1997) Multifocal motor neuropathy: serum IgM anti-GM1 ganglioside antibodies detected using modified ELISA methods in 85% of patients. Peripheral Nerve Society

LETTERS TO THE EDITOR:

1. Pestronk, A. (1990) A note about Genica Corporation. Also reply from the Editor-in-Chief Neurology 1323.

2. Pestronk, A. (1992) Monoclonal gammopathy and neuropathy. Ann Neurol 31:689.

3. Pestronk, A, Kornberg AJ. The clinical correlates of IgG antiganglioside antibodies (a reply). Muscle & Nerve 1995, December.

4. Pestronk, A. Testing for serum IgM binding to GM1 ganglioside in clinical practice Neurology 2000 54: 2353-2354.

BOOK REVIEWS:

1. Pestronk, A. (1997) Histology of the nervous system of man and vertebrates. Neurology:48;299.

CHAPTERS & INVITED REVIEWS

1. Drachman, D.B., Kao, I., Pestronk, A., and Toyka, K.V. (1976) Myasthenia gravis as a receptor disorder. Ann NY Acad Sci 274:226-234.

2. Drachman, D.B., Kao, I., Pestronk, A., Toyka, K.V., Griffin, J.W., and Winkelstein, J.A. (1976) Myasthenia gravis: a human disorder of ACh receptors. In Pathogenesis of Human Muscular Dystrophies. Ed. L.P. Rowland. Excerpta Medica, Elsevier/North Holland, Inc. NY, pp. 111-120.

3. Griffin, J.W., Price, D.L., Hoffman, P.N., Clark, A.W., Drachman, D.B., and Pestronk, A. (1979) Toxic axonal neuropathies. In Current Topics in Nerve and Muscle Research. Eds. A. Aguayo and G. Karpati. Excerpta Medica, Amsterdam, pp. 282-291.

4. Drachman, D.B., Pestronk, A., and Stanley, E.F. (1979) Pathogenesis of myasthenia gravis as a disorder of acetylcholine receptors. Neurotoxins 3:237-244.

5. Pestronk, A., Keogh, J., and Griffin, J.W. (1980) Dimethylaminopropionitrile. In Environmental and Clinical Neurotoxicology. Eds. P.S. Spencer and H.H. Shaumburg. Williams and Wilkens, Baltimore, Maryland, pp.424-429.

6. Pestronk, A., and Drachman, D.B. (1981) Therapeutic strategies in experimental autoimmune myasthenia gravis. Ann NY Acad Sci 337:278-291.

7. Drachman, D.B., Adams, R.N., Josifek, L.F., Pestronk, A., and Stanley, E.F. (1981) Antibody mediated mechanisms of ACh receptor loss in myasthenia gravis: clinical relevance. Ann NY Acad Sci 337:175-188.

8. Drachman, D.B., Pestronk, A., and Stanley, E.F. (1982) Neurotrophic interactions between nerves and muscles: role of acetylcholine. In Diseases of the Motor Unit. Ed. D.L. Schotland. John Wiley and Sons, Inc., NY. Chap. 8, pp.107-118.

9. Drachman, D.B., Pestronk, A., Stanley, E.F., and Adams, R.N. (1982) Mechanisms of acetylcholine receptor loss in myasthenia gravis. In Diseases of the Motor Unit. Ed. D.L. Schotland. John Wiley and Sons, Inc., NY. Chap. 16, pp. 215-232.

10. Pestronk, A., and Drachman, D.B (1982) Sprouting and regeneration of motor nerves. In Diseases of the Motor Unit. Ed. D.L. Schotland. John Wiley and Sons, Inc., NY. Chap. 13, pp. 173-185.

11. Pestronk, A., and Drachman, D.B. (1984) Regulation of motor nerve outgrowth. In Neuromuscular Diseases. Eds. G. Serratrice, D. Cros, C. Desnuelle, J.L. Gastaut, J.F. Pellissier, J. Pouget, A. Schiano. Raven Press, NY, pp. 407-413.

12. Drachman, D.B., Stanley, E.F., Pestronk, A., (1984) Neural regulation of muscle properties. In Neuromuscular Diseases. Eds. G. Serratrice, D. Cros, C. Desnuelle, J.L. Gastaut, J.F. Pellissier, J. Pouget, A. Schiano. Raven Press, NY, pp. 415-424. 13. Miller, N., Pestronk, A. (1985) Therapy of myasthenia gravis. In Current Ocular Therapy 2. Eds. F.T. Fraunfelder, F.H. Roy, W.B. Saunders Co., pp. 193-194.

14. Pestronk, A. (1985) Polymyositis. In Current Therapy in Neurologic Disease. Ed. R.T. Johnson. B.C. Decker, Inc., Philadelphia, pp. 380-383. 15. Pestronk, A., and Drachman, D.B. (1987) Antibody-mediated membrane abnormalities in polymyositis: reduction of acetylcholine receptors by immunoglobulin. Ann NY Acad Sci 505:357-367.

16. Drachman, D.B., De Silva, S., Ramsay, D., and Pestronk, A. (1987) Humoral pathogenesis of myasthenia gravis. Ann NY Acad Sci 505:90-105.

17. Pestronk, A., Li, F. (1991) Motor neuron syndromes: association with antiglycolipid antibodies. In Motor Neuron Disease ed. L.P. Rowland et al, Raven Press, New York, pp. 427-432.

18. Pestronk, A. (1991) Motor neuropathies, motor neuron disorders, and antiglycolipid antibodies. Muscle & Nerve 14:1429-10.

19. Kuncl, R.W., Wittstein, I., Adams, R.N., Wiggins, W.W., Avila, O., Pestronk, A., McIntosh, K., Lucas, D., DeSilva, S., Lehar, M., Drachman, D.B. (1993) A novel therapy for myasthenia gravis by reducing the endocytosis of acetylcholine receptors. Ann New York Academy of Sciences 681:298-302.

20. Lopate, G., Pestronk, A. (1993) Myasthenia gravis. Hospital Practice 28:109-131.

21. Kornberg, A.J., Pestronk, A. (1993) Immune-mediated neuropathies. Current Opinion in Neurology 6:681-687.

22. Lopate, G., Pestronk, A. (1994) The myasthenic neuromuscular junction. In Handbook of Myasthenia Gravis, ed. R.P. Lisak, Marcel Dekker, Inc., New York, NY., pp. 225- 237.

23. Lopate G., Pestronk A. Chronic immune demyelinating neuropathies. Seminars in Neurol 1994;14(2):131-136.

24. Kornberg A.J., Pestronk A. Chronic motor neuropathies: diagnosis, therapy and pathogenesis. Ann Neurol 1995;37(S1):S43-S50.

25. Pestronk, A. Dimethylaminopropionitrile. Experimental & Clinical Neurotoxicology, Second Edition, Oxford University Press. 1997

26. Lee, KP, Yee, W-C, Pestronk, A. Myasthenia Gravis. Current Diagnosis 9, W.B. Saunders Co. 1996.

27. Pestronk, A. Chronic immune polyneuropathies and serum autoantibodies. Practical Neuroimmunology, eds LA Rolak, Y Harati, Butterworth Heinemann 1997;237-251.

28. Pestronk, A. Motor neuropathies and lower motor neuron syndromes. Continuum 1997;3:99-110.

29. Pestronk, A. Familial amyotrophic lateral sclerosis. Continuum 1997;3:71-74.

30. Pestronk, A. Multisystem disorders with prominent motor signs. Continuum 1997;3:75-77

31. Pestronk, A. Hereditary spastic paraplegia. Continuum 1997;3:78-82

32. Pestronk, A. Polyneuropathies and serum autoantibodies to MAG, GALOP antigen, sulfatide and GM1 ganglioside. SRL Hokan Quarterly 1998;22:117-129.

33. Multifocal Motor Neuropathy: Diagnosis and Treatment. Neurology 1998;51S;S22-S24.

34. Pestronk A. Muscle pain and cramps. In: Bradley WG, Daroff RB, Fenichel GM, Marsden CD, editors. Neurology in Clinical Practice 3rd edition, Boston, MA: Butterworth-Heinemann. 2000, pp 397-402.

35. Pestronk A. Autoantibodies and Immune Polyneuropathies. In: Katirji B, Kaminski HJ, Preston DC, Ruff RL, Shapiro BE, editors. Neuromuscular Disorders in Clinical Practice. Boston, MA: Butterworth-Heinemann. 2002. pp 64-73.

36. Mozaffar T, Pestronk A. Cancer-related muscle disease. In: Karpati G, editor. Structural and Molecular Basis of Skeletal Muscle diseases. Basel, Switzerland: ISN Neuropath Press. 2002. pp 266-269.

37. Pestronk A. Paraneoplastic Neuropathy Syndromes: Principles and Treatment. In: Noseworthy JH, editor. Neurological Therapeutics, Principles and Practice. London: Martin Dunitz. 2003. pp 2158-2165.

38. Kornberg AJ, Pestronk A. Antibody-associated polyneuropathy syndromes: principles and treatment. Semin Neurol. 2003;23:181-190.

39. Merrill LJ, Gurnett CA, Connolly AM, Pestronk A, Dobbs MB. Skeletal Muscle Abnormalities and Genetic Factors Related to Vertical Talus. Clin Orthop Relat Res 2010 Jul 20

40. Weihl CC, Pestronk A. Sporadic inclusion body myositis: possible pathogenesis inferred from biomarkers. Current Opinion in Neurology 2010;23:482-488

41. Varadhachary AS, Weihl CC, Pestronk A. Mitochondrial pathology in immune and inflammatory myopathies. Curr Opin Rheumatol 2010;22:651-657.

42. Lopate G, Pestronk A. Inflammatory Demyelinating Neuropathies. Curr Treat Options Neurol 2011;13:131-142.

43. Pestronk A. Acquired immune and inflammatory myopathies: pathologic classification. Curr Opin Rheumatol 2011;23:595-604.

44. Allenbach Y, Mammen AL, Benveniste O, Stenzel W; Immune-Mediated Necrotizing Myopathies Working Group. 224th ENMC International Workshop: Clinico-sero-pathological classification of immune-mediated necrotizing myopathies. Zandvoort, The Netherlands, 14-16 October 2016. Neuromuscul Disord 2018;28:87-99.

COLLABORATIONS

1. McDonald CM1, Wong B2, Flanigan KM3, Wilson R4, de Kimpe S5, Lourbakos A5, Lin Z5, Campion G5; DEMAND V study group, Collaborators: Iannaccone ST, Karachunski PI, Mathews KD, Henricson EK, Joyce NC, Pestronk A, Renfoe JB, Russman BS, Smith EC, So YT, Wang CH, Day JW, Sproule DM, Wagner KR. Placebo-controlled Phase 2 Trial of Drisapersen for Duchenne Muscular Dystrophy. Ann Clin Transl Neurol 2018;5:913-926.

2. Thangarajh M1, Elfring GL2, Trifillis P2, McIntosh J2, Peltz SW2; Ataluren Phase 2b Study Group.
Collaborators: Ryan MM, Kornberg AJ, RodriguezCasero V, Wray A, Jones KJ, North K, Goemans N, Buyse GM, Campbell C, Mah J, Sarnat H, Selby K, Voit T, Doppler V, De Castro D, Chabrol B, Levy N, Halbert C, Pereon Y, Magot A, Perrier J, Mahe JY, Schara U, Lutz S, Busse M, Marina AD, Kirschner J, Stanescu A, Pohl A, RensingZimmerman C, Bertini E, D'Amico A, Kofler A, Carlesi A, Bonetti AM, Santecchia L, Emma F, Bergami G, Mercuri EM, Vasco G, Bianco F, Mazzone ES, De Sanctis R, Alfieri P, Pane M, Messina S, Comi GP, Magri F, Lucchini V, Corti SP, Moggio MG, Sciacco M, Bresolin N, Prelle AC, Magri R, Virgilio R, Lamperti C, Nevo Y, DorWollman T, Vilchez J, Muelas N, Sevilla T, Smeyers P, de la Osa A, Colomer J, Ortez CI, Nascimento A, Febrer A, Medina J, Tulinus M, Thorarinsdottir B, Darin N, Sejersen T, Hovmoller M, Bushby K, Straub V, Guglieri M, Sarkozy A, Willis T, Eagle M, Mayhew A, Muntoni F, Cirak S, Manzur AY, Robb SA, Kinali M, Quinlivan RCM, Smith MR, Pandey R, Wong B, Collins J, Finkel R, Bonnemann C, Yang M, Foley AR, Yum S, Sampson J, Bromberg M, Swoboda K, Day J, Karachunski P, Mathews K, Bonthius D, Laubenthal KS, Darras B, Kang P, Parson J, Barohn R, Dasouki M, Anderson H, Burns J, Dimachkie M, Pasnoor M, Wang Y, Ciafaloni E, Heatwole C, Connolly A, Pestronk A, Al-Lozi M, Lopate G, Golumbek P, Sommerville B, Wang L, Wojcicka-Mitchell A, Godbey A, Harms M, Varadachary A, Iyadurai S, Rojas L, Iannacone S, Khonghatithum C, Sproule D, De Vivo D, Constantinescu A, McDonald C, Han J, Renfroe B, Russman B, Sussman M, BurnsWechsler S, Juel V, Hobson-Webb L, Smith E. The relationship between deficit in digit span and genotype in nonsense mutation Duchenne muscular dystrophy. Neurology 2018 Aug 22.

3. Conrado DJ, Larkindale J, Berg A, Hill M, Burton J, Abrams KR, Abresch RT, Bronson A, Chapman D, Crowther M, Duong T, Gordish-Dressman H, Harnisch L, Henricson E, Kim S, McDonald CM, Schmidt S, Vong C, Wang X, Wong BL, Yong F, Romero K; Duchenne Muscular Dystrophy Regulatory Science Consortium (D-RSC). Towards regulatory endorsement of drug development tools to promote the application of model-informed drug development in Duchenne muscular dystrophy. J Pharmacokinet Pharmacodyn 2019 May 24.

PATENTS
(1) Sulfatide antibody 6,020,140, issued 1995
(2) GALOP antibody 6,121,004, issued 2000
(3) GM1 ganglioside antibody 6,077,681, issued 2000
(4) TS-HDS antibody 7,175,989, issued 2007
(5) NS6S antibody 9,535,072, issued 2017

PEER-REVIEWED PUBLICATIONS

1. Toyka, K.V., Drachman, D.B., Pestronk, A., and Kao, I. (1975) Myasthenia gravis: passive transfer from man to mouse. Science 190:397-99.

2. Pestronk, A., Drachman, D.B., and Griffin, J.W. (1976) Effects of muscle disuse on acetylcholine receptors. Nature 260:352-353.

3. Toyka, K.V., Drachman, D.B., Pestronk, A., Fischbeck, K.H., and Kao, I. (1976) A transferable "myasthenogenic" factor in the serum of patients with myasthenia gravis. J Neurol 212:271-280

4. Pestronk, A., Drachman, D.B., and Griffin, J.W. (1976) Effect of botulinum toxin on trophic regulation of acetylcholine receptors. Nature 264:787-788.

5. Toyka, K.V., Drachman, D.B., Griffin, D.E., Pestronk, A., Winkelstein, J.A., Fischbeck, K.H., and Kao, I. (1977) Myasthenia gravis: study of humoral immune mechanisms by passive transfer to mice. N Engl J Med 296:125-131.

6. Pestronk, A., and Drachman, D.B. (1978) A new stain for quantitative measurement of sprouting at neuromuscular junctions. Muscle and Nerve 1:70-74.

7. Pestronk, A., Drachman, D.B. (1978) Motor nerve sprouting and acetylcholine receptors. Science 199:1223-1225.

8. Drachman, D.B., Adams, R.N., Stanley, E.F., and Pestronk, A. (1980) Mechanisms of acetylcholine receptor loss in myasthenia gravis. J Neurol Neurosurg Psychiatry 43:601-610.

9. Murphy, A., Drachman, D.B., Satya-Murti, S., Pestronk, A., and Eggelston, J. (1980) Critical reexamination of the thymus immunization model of myasthenia gravis. Muscle and Nerve 3:293-297.

10. Keogh, J.P., Pestronk, A., Wertheimer, D., and Moreland, R.(1980) An epidemic of urinary retention caused by dimethylaminopropionitrile. JAMA 243:746-749.

11. Pestronk, A., Drachman, D.B., and Griffin, J.W. (1980) Effects of aging on nerve sprouting and regeneration. Exp Neurol 70:65-82.

12. Pestronk, A., Drachman, D.B., Stanley, E.F., Price, D.L., and Griffin, J.W. (1980) Cholinergic transmission regulates extrajunctional acetylcholine receptors. Exp Neurol 70:690-696.

13. Pestronk, A., and Drachman, D.B. (1980) Lithium reduces the number of acetylcholine receptors in skeletal muscle. Science 210:342-343.

14. Pestronk, A., and Drachman, D.B. (1980) Dimethyl sulfoxide reduces titers of antireceptor antibody in experimental myasthenia gravis. Nature 288:733-734.

15. Drachman, D.B., Stanley, E.F., Pestronk, A., Price, D.L., and Griffin, J.W. (1982) Neurotrophic regulation of two properties of skeletal muscle by impulse-dependent and spontaneous ACh transmission. J Neurosci 2:232-243.

16. Pestronk, A., Parhad, I.M., Drachman, D.B., and Price, D.L. (1982) Membrane myopathy: morphologic similarities to Duchenne's muscular dystrophy. Muscle and Nerve 5:209-214.

17. Pestronk, A., Drachman, D.B., Stanley, E.F., and Adams, R.N. (1982) Treatment of ongoing experimental myasthenia gravis with short term high dose cyclophosphamide. Muscle and Nerve 5:79-84.

18. Pestronk, A., Drachman, D.B, Teoh, R., Adams, R.N. (1983) Combined short-term immunotherapy cures experimental autoimmune myasthenia gravis. Ann Neurol 14:235-241. 19. Pestronk, A., Pestronk, S. (1983) Goggle migraine. N Engl J Med 301:226.

20. Andrew, C.G., Drachman, D.B., Pestronk, A., Narayan, O. (1984) Susceptibility of skeletal muscle to Coxsackie A2 virus infection: effects of botulinum toxin and denervation. Science 223:714-716.

21. Dellon, A.L., Mackinnon, S.E., Pestronk, A. (1984) Implantation of sensory nerve into muscle: preliminary clinical and experimental observations on neuroma formation. Ann Plas Surg 12:30-34.

22. Pestronk, A., and Drachman, D.B. (1985) Polymyositis: reduction of acetylcholine receptors in myasthenia gravis: clinical correlates. Muscle and Nerve 8:233-239.

23. Pestronk, A., Drachman, D.B., and Self, S. (1985) Measurement of junctional acetylcholine receptors in myasthenia gravis: clinical correlates. Muscle and Nerve 8:245-251.

24. Pestronk, A., and Drachman, D.B. (1985) Motor nerve terminal outgrowth: effects of a-bungarotoxin and anti-AChR antibody. J Neurosci 5:751-758.

25. Pestronk, A. (1985) Intracellular acetylcholine receptors in skeletal muscle of the adult rat. J Neurosci 5:1111-1117.

26. Drachman, D.B., Adams, R.N., McIntosh, K., Pestronk, A. (1985) Treatment of experimental myasthenia gravis with Cyclosporin A. Clin Immunol and Immunopath 34:174-188.

27. Pestronk, A., Teoh, R., Sims, C., Drachman, D.B. (1985) Effects of dimethylsulfoxide on humoral immune responses to acetylcholine receptors in rats. Clin Immunol Immunopath 37:172-178.

28. Malinow, K. Yannakakis, G.D., Glusman, S.M., Edlow, D.W., Pestronk, A., Powell, D.L., Ramsey-Goldman, R., Eidelman, G.H., Medsger, T.A., Jr., Alexander, E.L. (1986) Subacute sensory neuropathy secondary to dorsal root ganglionitis in primary Sjorgren's syndrome. Ann Neurol 20:535-537.

29. Kuncl, R.W., Rechthand, E., Pestronk, A. (1986) Penicilliamine-induced myasthenia gravis associated with reduced acetylcholine receptors. Ann Neurol 20:740-743.

30. Pestronk, A., and Drachman, D.B. (1987) Mechanism of action on lithium on acetylcholine receptor metabolism in skeletal muscle. Brain Res 412:302-310.

31. Yee, W.C., and Pestronk, A. (1987) Mechanisms of postsynaptic plasticity: remodeling of the junctional acetylcholine receptor cluster induced by motor nerve terminal outgrowth. J Neurosci 7:2019-2024.

32. Pestronk, A. (1988) The first neurology book: De Cerebri Morbis... (1549) by Jason Pratensis. Arch Neurol 45:341-344.

33. Pestronk, A., and Drachman, D.B. (1988) Motor nerve outgrowth: reduced capacity for sprouting in the terminals of longer axons. Brain Res 463:216-222.

34. Yee, W.C., Drachman, D.B., Walser, M., and Pestronk, A. (1988) Effect of ketoleucine treatment on atrophy of skeletal muscle. Exp Neurol 99:1-9.

35. Pestronk, A., Cornblath, D.R., Ilyas, A.A., Baba, H., Quarles, R.H., Griffin, J.W., and Alderson, K. (1988) A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Ann Neurol 24:73-78.

36. Pestronk, A., Adams, R.N., Clawson, L., Cornblath, D.R., Kuncl, R.W., Griffin, D., and Drachman, D.B. (1988) Serum antibodies to GM1 ganglioside in amyotrophic lateral sclerosis. Neurology 38:1457-1461.

37. Pestronk, A. (1988) The first neurology book written in English: "De Morbis Capitis" (1650). Arch Neurol 46:215-220.

38. Ramsay, D.A., Drachman, D.B., and Pestronk, A. (1988) Rapid synthesis of acetylcholine receptors at neuromuscular junctions. Brain Res 46:215-220.

39. Yee, W.C., Pestronk, A., Alderson, K., Yuan, C.M. (1988) Regional heterogeneity in the distal motor axon: three zones with distinctive intrinsic components. J Neurocytol 17:649-656.

40. Pestronk, A., Adams, R.N. Kuncl, R.W., Drachman, D.B., Clawson, L.L., and Cornblath, D. (1988) Differential effects of prednisone and cyclophosphamide on autoantibodies in human neuromuscular disorders. Neurology 39:628-633.

41. Alderson, K., Pestronk, A., Yee, W.C., and Drachman, D.B. (1989) Silver cholinesterase immunocytochemistry: a new neuromuscular junction stain. Muscle and Nerve 12:9-14.

42. Kuncl, R.W., Pestronk, A., Alexander, E., Valee, D., Lane, J. (1989) The MRL +/+ mouse: a new model of tubular aggregates which are gender and age-related. Acta Neuropath 78:615-620.

43. Crain, E., McIntosh, K.R., Gordon, G., Pestronk, A., Drachman, D.B. (1989) The effect of thalidomide on experimental autoimmune myasthenia gravis. J Autoimmun 2:197-202.

44. McArthur, J.C., Marek, K., Pestronk, A., McArthur, J., Peroutka, S.J. (1989) Nifedipine in the prophylaxis of classic migraine: A crossover, double-masked, placebo-controlled study of headache frequency and side effects. Neurology 39:284-286.

45. Pestronk, A., Adams, R.N., Cornblath, D., Kuncl, R.W., Drachman, D.B., and Clawson, L. (1989) Patterns of serum IgM antibodies to GM1 and GD1a gangliosides in ALS. Ann Neurol 25:98-102.

46. Alderson, K., Yee, W.C., Pestronk, A. (1989) Reorganization of intrinsic components in the distal motor axon during outgrowth. J Neurocytol 18:541-552.

47. Baba, H., Daune, G.C., Pestronk, A., Cornblath, D.R., Chaudhry, V., Griffin, J.W., Quarles, R.H. (1989) Anti-GM1 ganglioside antibodies with differing fine specificities in patients with multifocal motor neuropathy. J Neuroimmunol 25:143-150.

48. Lipsky, N.G., Drachman, D.B., Pestronk, A., Shih, P. (1989) Neural regulation of mRNA for the a-subunit of acetylcholine receptors: Role of neuromuscular transmission. Exp Neurol 105:177-183.

49. Marcus, D.M., Latov, N., Hsi, B.P., Gillard, B.K., and Participating Laboratories. (1989) Report of a conference: Measurement and significance of antibodies against GM1 ganglioside. J Neuroimmunol 25:251-259.

50. Avila, O.L., Drachman, D.B., Pestronk, A. (1989) Neurotransmission regulates stability of acetylcholine receptors at the neuromuscular junction. J Neurosci 9:2902-2906.

51. Pestronk, A., Watson, D.F., Yuan, C.M. (1990) Neurofilament phosphorylation in peripheral nerve: changes with axonal length and growth state. J Neurochem 54:977-982.

52. Krarup, C., Stewart, J.D., Sumner, A.J., Pestronk, A., Lipton, S.A. (1990) A syndrome of asymmetrical limb weakness with motor conduction block. Neurology 40:118-127.

53. Pestronk, A., Chaudhry, V., Feldman, E.L., Griffin, J.W., Cornblath, D.R., Denys, E.N., Glasberg, M., Kuncl, R.W., Olney, R.K., Yee, W.C. (1990) Lower motor neuron syndromes defined by patterns of weakness, nerve conduction abnormalities and high titers of antiglycolipid antibodies. Ann Neurol 27:316-326.

54. Rothstein, J.D., Guochuan, T., Kuncl, R.W., Clawson, L., Cornblath, D.R., Drachman, D.B., Pestronk, A., Stauch, B.L., and Coyle, J.T. (1990) Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis. Ann Neurol 28:18-25.

55. Li, F., Pestronk, A. (1991) Autoantibodies to GM1 ganglioside: Different reactivity to GM1-liposomes in amyotrophic lateral sclerosis and lower motor neuron disorders. J Neurol Sci 104:209-214.

56. Pestronk, A., Li, F., Griffin, J., Feldman, E.L., Cornblath, D., Trotter, J., Zhu, S., Yee, W.C., Phillips, D., Winslow, B. (1991) Polyneuropathy syndromes associated with serum antibodies to sulfatide and myelin-associated glycoprotein. Neurol 41:357-362.

57. Griggs, R.C., Moxley, R.T., Mendell, J.R., Fenichel, G.M., Brooke, M.H., Pestronk, A., Miller, J.P., et al. (1991) Prednisone in Duchenne dystrophy: A randomized, controlled trial defining the time course and dose response. Arch Neurol 48:383-388.

58. Selnes, O.A., Pestronk, A., Hart, J., and Gordon, B. (1991) Limb ataxia without aphasia from a left-sided lesion in a right-hander. J Neurol Neurosurg Psychiat 54(8):734-737.

59. Fenichel, G.M., Mendell, H.R., Moxley, R.T., Griggs, R.C., Brooke, M.H., Miller, J.P., Pestronk, A., Robison, J., King, W., Signore, L., Pandya, M.S., Florence, J., Schierbecker, J., Wilson, B. (1991) A comparison of daily and alternate-day prednisone therapy in the treatment of Duchenne muscular dystrophy. Arch Neurology 48:575-579.

60. Feldman, E.L., Bromberg, M.B., Albers, J.W., Pestronk, A. (1991) Immunosuppressive treatment in multifocal motor neuropathy. Ann Neurol 30:397-401.

61. Cornblath, D.R., Kuncl, R.W. Mellits, E.d., Quaskey, S.A., Clawson, L., Pestronk, A. Drachman, D.B. (1992) Nerve conduction studies in Amyotrophic Lateral Sclerosis. Muscle & Nerve 15:1111-1115.

62. Chaudhry, V., Pestronk, A. (1992) Different patterns of glycolipid antibody reactivity: Lower motor neuron syndromes vs immunization. J Neuroimmunol 36:127-134.

63. Griggs, R.C., Moxley III, R.T., Mendell, J.R., Fenichel, G.M., Brooke, M.H., Pestronk, A., Miller, J.P., Cwik, V.A., Pandya, S., Robison, J., King, W., Signore, L., Schierbecker, J., Florence, J., Matheson-Burden, N., Wilson, B. (1993) Duchenne dystrophy: Randomized, controlled trial of prednisone (18 months) and azathioprine (12 months). Neurology 43:520-527.

64. Connolly, A.M., Pestronk, A., Trotter, J.L., Feldman, E.L., Cornblath, D.R., Olney, R.K. (1993) High-titer selective serum anti-b-tubulin antibodies in chronic inflammatory demyelinating polyneuropathy. Neurology 43:557-562.

65. Kornberg, A.J., Pestronk, A. (1994) The clinical and diagnostic role of anti-GM1 antibody testing. Muscle and Nerve 17:100-104.

66. Kornberg, A.J., Pestronk, A., Bieser, K., Ho, T.W., McKhann, G.M., Wu, H.S., Jiang, Z. The clinical correlates of high titer IgG anti-GM1 antibodies. Ann Neurol 1994;35:234-237.

67. Al-Lozi, M., Pestronk, A., Yee, W.C., Flaris, N., Cooper, J. (1994) Rapidly Evolving Myopathy with Myosin-Deficient Muscle Fibers. Ann Neurol 94:35;273- 279.

68. Wax, MB, Barrett DA, Pestronk A. Increased Incidence of Paraproteinemia and Autoantibodies in Patients with Normal Pressure Glaucoma. Am J Ophthalmology 1994;5(117):561-568.

69. Elliott, J, Pestronk, A. Progression of Multifocal Motor Neuropathy During Apparently Successful Treatment with Human Immune Globulin. Neurology 1994;44:967-968.

70. Pestronk, A., Li, F., Bieser, K., Choksi, R., Whitton, A., Kornberg, A.J. (1994) Anti-MAG antibodies: Major effects of antigen purity and antibody cross-reactivity on ELISA results and clinical correlation. Neurology 1994;44:1131-1137.

71. Drachman, D.B., Chaudhry V., Cornblath, D., Kuncl R.W., Pestronk, A., et al. Trial of immunosuppression in ALS, using total lymphoid irradiation. Ann Neurol 1994;35:142-150.

72. Zielasek J., Ritter G., Magi S, Hartung H.P., Toyka K.V., and participating laboratories. A comparative trial of anti-glycoconjugate antibody assays: IgM antibodies to GM1. J. Neurol. 1994;241:475-480.

73. Al-Lozi M.T., Pestronk A., Yee W.C., Flaris N. Myopathy and paraproteinemia with serum IgM binding to a high molecular weight muscle fiber surface protein. Ann Neurol 1995;37:41-46.

74. Pestronk, A., Choksi, R., Bieser, K., Goldstein, J.M., Adler, C.H., Caselli, R.J., George, E.B. Treatable Gait Disorder and Polyneuropathy Associated with High Titer Serum IgM Binding to Antigens that Co-Purify with Myelin Associated Glycoprotein Muscle & Nerve 1994 17:1293-1300.

75. Pestronk A., Lopate G., Kornberg A.J., Elliott J.L., Blume G., Yee W-C, Goodnough L.T. Distal lower motor neuron syndrome with high titer serum IgM anti-GM1 antibodies: Improvement following immunotherapy with monthly plasma exchange and intravenous cyclophosphamide. Neurology 1994 44:2027-2031.

76. Romano C., Barrett D.A., Li Z., Pestronk A., Wax M.B. Anti-Rhodopsin antibodies in patients with normal pressure glaucoma. Investigative Opthal. 1995;10:1968-1975.

77. Cedarbaum, J.M., Charatan, M., Stambler, N., Lagunowich, L., Zhan, C., Radka, S., Morrisey, D., Lakings,D., Brooks, B.R., Sanjak, M., Pestronk, A., Florence, J., Mitsumoto, H., Szirony, K., Bittle, L., Ringel, S., Neville, H., Brinkmann, J. The Pharmacokinetics of Subcutaneously Administered Recombinant Human Ciliary Neurotrophic Factor (rHCNTF) in Patients with Amyotrophic Lateral Sclerosis: Relationship to Parameters of the Acute Phase Response. Clin Neuropharm 1996;18:500-532.

78. Cedarbaum JM, Brooks BR, Pestronk A, Mitsumoto H, Neville H, Wittes J. A phase I study of recombinant human ciliary neurotrophic factor (rHCNTF) in patients with amyotrophic lateral sclerosis. Clin Neuropharm 1995;18:515-532.

79. Blume G., Pestronk A., Goodnough L.T. Anti-MAG antibody associated polyneuropathies: improvement following immunotherapy with monthly plasma exchange and intravenous cyclophosphamide. Neurology 1995;45:1577-1580.

80. ALS CNTF Treatment Study (ACTS) Phase I-II Study Group. The amyotrophic lateral sclerosis functional rating scale. Arch. Neurol. 1996;53:141-147.

81. ALS CNTF Treatment Study (ACTS) Study Group: A double-blind placebo-controlled clinical trial of subcutaneous recombinant human ciliary neurotrophic factor (rHCNTF) in Amyotrophic Lateral Sclerosis. Neurology, 1996;46:1244-1249.

82. Connolly, AM, Pestronk, A, Planer GJ, Yue J, Mehta S, Choksi R. Congenital muscular dystrophy syndromes distinguished by alkaline and acid phosphatase, merosin, and dystrophin staining. Neurology 1996;46:810-814.

83. Nevo, Y, Pestronk, A, Kornberg AJ, Connolly AM, Yee WC, Iqbal I, Shield LK. Childhood chronic inflammatory demyelinating neuropathies: Clinical course and long-term follow-up. Neurology 1996;47:98-102.

84. Santorelli, FM, Sciacco, M. et al. Multiple mitochondrial DNA deletions in sporadic inclusion body myositis: A study of 56 patients. Ann. Neurol 1996;39:789-795.

85. Nevo, Y, Pestronk A. Neonatal perifascicular myopathy. Pediatric Neurology 1996;15:150-152.

86. Nevo, Y, Pestronk A, Lopate G, Carroll SL. Neuropathy of metachromatic leukodystrophy: improvement with immunomodulation. Pediatric Neurology 1996;15:237-239.

87. Kornberg, AJ, Pestronk A, Blume GM, Lopate G, Yue J, Hahn A. Selective staining of the cerebellar molecular layer by serum IgG in Miller-Fisher and related syndromes. Neurology 1996;47:1317-1320.

88. Connolly, AM, Pestronk, A, Mehta S, Yee WC, Green BJ, Fellin C, Olney RK, Miller RG, Devor WN. Serum IgM monoclonal autoantibody binding to the 301-314 amino acid epitope of b-tubulin: Clinical correlation with slowly progressive demyelinating polyneuropathy. Neurology 1997;48:243-248.

89. Blume, G, Pestronk, A, , Frank, B, Johns, DR. Polymyositis with cytochrome oxidase negative muscle fibers: Early quadriceps weakness and poor response to immunosuppressive therapy. Brain 1997;120:39-45.

90. Duggan, DJ, Gorospe, R, Fanin, MS, Hoffman, EP, Angelini, C, Pegoraro, E, Noguchi, S, Ozawa, E, Pendlebury, W, Waclawik, AJ, Duenas, DA, Hausmanowa- Petrusewicz, I, Fidzianska, A, Bean, SC, Haller, JS, Bodensteiner, J, Greco, CM, Pestronk, A, Berardinelli, A, Gelinas, DF, Abram, H, Kuncl, R. Mutations in the sarcoglycan genes in patients with myopathy. N Engl J Med 1997;336:618-624.

91. Pestronk, A, Choksi R, Blume, G, Lopate, G. Multifocal motor neuropathy: Serum IgM binding to a GM1-ganglioside containing lipid mixture but not to GM1 alone. Neurology 1997;48:1104-1106.

92. Fenichel, G, Pestronk, A, Florence, J, Robison, V, Hemelt, VB. A beneficial effect of oxandrolone in the treatment of Duchenne muscular dystrophy: A pilot study. Neurology 1997;48:1225-1226.

93. Lopate, G, Parks BJ, Goldstein, JM, Yee,, WC, Friesenhahn GM, Pestronk, A. Polyneuropathies associated with high-titer anti-sulfatide antibodies: Characteristics of patients with and without serum M-proteins. J Neurol Neurosurg Psychiatry 1997;62:581-585.

94. Connolly, AM, Pestronk, A, Mehta, S, Pranzatelli, MR, Noetzel, MJ. Serum autoantibodies in childhood opsoclonus-myoclonus syndrome: An analysis of antigenic targets in neural tissues. J Pediatr 1997;130:878-884.

95. Lopate, G, Pestronk, A, Kornberg, AJ. IgM anti-sulfatide autoantibodies: Patterns of binding to cerebellum, dorsal root ganglion and peripheral nerve. J Neurol Sci 1997;151:189-197.

96. Nevo, Y, Pestronk, A. Acute immune neuropathies: Serum antibodies to Campylobacter jejuni, Helicobacter pylori, and GM1 ganglioside. J Infect Dis 1997;176(Suppl2):S154-156

97. Pestronk, A., Choksi, R. Multifocal motor neuropathy: Serum IgM anti-GM1 ganglioside antibodies in most patients detected using covalent linkage of GM1 to ELISA plates. Neurology 1997;49:1289-1292

98. Al-Lozi, MT, Pestronk, A, Choksi, R. A skeletal muscle-specific form of decorin is a target antigen for a serum IgM M-protein in a patient with a proximal myopathy. Neurology 1997;49:1650-1654

99. Connolly, AM, Pestronk, A. Anti-tubulin autoantibodies in acquired demyelinating polyneuropathies. J Infect Dis 1997;176(Suppl2):S157-159

100. Levin, MI, Mozaffar, T, Al-Lozi, MT, Pestronk, A. Paraneoplastic necrotizing myopathy: Clinical and pathological features. Neurology 1998;50:764-767.

101. Stambler, NL, Charatan, M, Cedarbaum, JM and the ALS CNTF Study Group. Prognostic indicators of survival in ALS. Neurology 1998;50:66-72.

102. Al-Lozi, MT, Hemelt VB, Pestronk, A. Steroid-responsive myopathy with deficient chondroitin sulfate C in skeletal muscle connective tissue. Neurology 1998;50:526-529.

103. Lopate, G, Pestronk, A, Yee, WC. N lines in a myopathy with myosin loss. Muscle Nerve 1998;21:1216-1219.

104. Mozaffar, T, Pestronk, A. Clinical correlates of granulomas in muscle. J. Neurol 1998; 245:519-524.

105. Connolly, AM, Pestronk, A, Mehta, S, Al-Lozi, M. Autosomal recessive muscular dystrophy with primary a-sarcoglycan (adhalin) deficiency responsive to prednisone. Muscle Nerve 1998;21:1549-1553.

106. Levine, T, Pestronk, A. Inflammatory myopathy with cytochrome oxidase negative muscle fibers: Methotrexate treatment. Muscle Nerve 1998;12:1724-1728.

107. Pestronk, A, Choksi, R, Yee, WC, Kornberg, AJ, Lopate, G, Trotter J. Serum antibodies to heparan sulfate glycosaminoglycans in Guillain-Barré syndrome and other demyelinating polyneuropathies. J. Neuroimmunol 1998;91:204-209.

108. Connolly AM, Chez MG, Pestronk, A, Arnold ST, Mehta, S, Deuel RK. Serum autoantibodies to brain in Landau-Kleffner variant, autism, and other neurologic disorders. J Pediat 1999;134:607-613.

109. Levine, TD, Pestronk, A. IgM antibody-related polyneuropathies: Treatment with B-cell depletion chemotherapy using rituximab. Neurology 1999;52:1701-1704.

110. Nevo, Y, Al-Lozi, M, Parsadanian, AS, Elliott, JL, Connolly, AM, Pestronk. A. Mutation analysis in Emery-Dreifuss muscular dystrophy. Pediatric Neurology 1999;21:456-459.

111. Patton BL, Connolly, AM., Martin PT, Cunningham JM, Mehta S, Pestronk A, Miner JH, Sanes JR. Distribution of ten laminin chains in dystrophic and regenerating muscles. Neuromuscular Disorders 1999;9:423-433.

112. Calhoun RF, Ritter JH, Guthrie TJ, Pestronk A, Meyers BF, Patterson GA, Pohl MS, Cooper JD. Results of transcervical thymectomy for myasthenia gravis in 100 consecutive patients. Annals of Surgery 1999;230:555-561.

113. The BDNF Study Group (Phase III). A controlled trial of recombinant methionyl human BDNF in ALS. Neurology 1999;52:1427-1433.

114. Mozaffar T, Pestronk A. Myopathy with anti-Jo-1 antibodies: pathology in perimysium and neighbouring muscle fibres. J Neurol Neurosurg Psychiatry 2000;68:472-478.

115. Chiesa R, Pestronk A, Schmidt RE, Tourtellotte WG, Ghetti B, Piccardo P, Harris DA. Primary myopathy and accumulation of prp(sc)-like molecules in peripheral tissues of transgenic mice expressing a prion protein insertional mutation. Neurobiol Dis 2001;8:279-288.

116. Fenichel GM, MD, Griggs RC, Kissel J, Kramer, TI, Mendell JR, Moxley RT, Pestronk A, Sheng K, Florence J, King WM, Pandya MS, Robison VD, Wang H. A randomized efficacy and safety trial of oxandrolone in the treatment of Duchenne dystrophy. Neurology 2001;56:1075-1079.

117. Lopate G, Kornberg AJ, Yue J, Choksi R, Pestronk A. Anti-myelin associated glycoprotein antibodies: variability in patterns of IgM binding to peripheral nerve. J Neurol Sci 2001;188:67-72.

118. Connolly AM, Keeling RM, Mehta S, Pestronk A, Sanes JR. Three mouse models of muscular dystrophy: the natural history of strength and fatigue in dystrophin-, dystrophin/utrophin-, and laminin alpha2-deficient mice. Neuromuscul Disord 2001;11:703-712.

119. Kovach MJ, Waggoner B, Leal SM, Gelber D, Khardori R, Levenstien MA, Shanks CA, Gregg G, Al-Lozi MT, Miller T, Rakowicz W, Lopate G, Florence J, Glosser G, Simmons Z, Morris JC, Whyte MP, Pestronk A, Kimonis VE. Clinical Delineation and Localization to Chromosome 9p13.3-p12 of a Unique Dominant Disorder in Four Families: Hereditary Inclusion Body Myopathy, Paget Disease of Bone, and Frontotemporal Dementia. Molecular Genetics and Metabolism 2001;74:458-475.

120. Lopate G, Choksi R, Pestronk A. Severe sensory ataxia and demyelinating polyneuropathy with IgM anti-GM2 and GalNAc-GD1A antibodies. Muscle Nerve 2002;25;828-836.

121. Connolly AM, Keeling RM, Streif EM, Pestronk A, Mehta S. Complement 3 deficiency and oral prednisolone improve strength and prolong survival of laminin alpha2-deficient mice. J Neuroimmunol 2002;127:80-87.

122. Miller T, Al-Lozi MT, Lopate G, Pestronk A. Myopathy with antibodies to the signal recognition particle: clinical and pathological features. J Neurol Neurosurg Psychiat 2002;73:420-428.

123. Pestronk A, Choksi R, Logigian E, Al-Lozi MT. Sensory Neuropathy with Monoclonal IgM Binding to a Trisulfated Heparin Disaccharide. Muscle Nerve 2003;27:188-95.

124. Pestronk A, Florence J, Miller T, Choksi R, Al-Lozi MT, Levine TD. Treatment of IgM antibody-associated polyneuropathies using Rituximab. J Neurol Neurosurg Psychiat 2003;74:485-489.

125. Stone MJ, McElroy YG, Pestronk A, Reynolds JL, Newman JT, Tong AW. Human monoclonal macroglobulins with antibody activity. Semin Oncol 2003;30:318-324.

126. Gertz MA, Anagnostopoulos A, Anderson K, Branagan AR, Coleman M, Frankel SR, Giralt S, Levine T, Munshi N, Pestronk A, Rajkumar V, Treon SP. Treatment recommendations in Waldenström's macroglobulinemia: Consensus Panel Recommendations from the Second International Workshop on Waldenström's Macroglobulinemia. Semin Oncol 2003;30:121-126.

127. Watts GDJ, Thorne M,Kovach MJ, Pestronk A, Kimonis VE. Clinical and genetic heterogeneity in chromosome 9p associated hereditary inclusion body myopathy: exclusion of GNE and three other candidate genes. Neuromuscular Disorders 2003;13:559-567.

128. Pestronk A, Florence J, Levine T,Al-Lozi MT, Lopate G, Miller T, Ramneantu I, Waheed W,Stambuk M. Sensory exam with a quantitative tuning fork Rapid, sensitive and predictive of SNAP amplitude. Neurology 2004;62:461-464.

129. Watts GDJ, Wymer J, Kovach MJ, Mehta SG, Mumm S, Darvish D, Pestronk A, Whyte MP, Kimonis VE. Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein. Nature Genetics 2004;36:377-381.

130. Meininger V, Bensimon G, Bradley WG, Brooks BR, Douillet P, Eisen AA, Lacomblez L, Leigh PN, Robberecht W and other investigators. Efficacy and safety of xaliproden in amyotrophic lateral sclerosis: results of two phase III trials. Amyotroph Lateral Scler Other Motor Neuron Disord 2004;5:107-117.

131. Simpson D, Estanislao L, Evans S, McArthur J, Marcus K, Truffa M, Lucey B, Naismith R, Lonergan JT, Clifford D and the HIV Neuromuscular Syndrome Study Group. HIV associated neuromuscular weakness syndrome. AIDS 2004;18:1403-1412.

132. Shefner JM, Cudkowicz ME, Schoenfeld D, Conrad T, Taft J, Chilton M, Urbinelli L, Qureshi M, Zhang H, Pestronk A, Caress J, Donofrio P, Sorenson E, Bradley W, Lomen- Hoerth C, Pioro E, Rezania K, Ross M, Pascuzzi R, Heiman-Patterson T, Tandan R, Mitsumoto H, Rothstein J, Smith-Palmer T, Macdonald D, Burke D. A clinical trial of creatine in ALS. Neurology 2004;63:1656-1661.

133. Lopate G, Pestronk A, Al-Lozi M. Treatment of Chronic Inflammatory Demyelinating Polyneuropathy With High-Dose Intermittent Intravenous Methylprednisolone. Arch Neurol 2005;62:249-254.

134. Lopate G, Pestronk A, Evans S, Li L, Clifford D. Anti-sulfatide antibodies in HIV-infected individuals with sensory neuropathy. Neurology 2005;64:1632-1634.

135. Escolar DM, Buyse G, Henricson E, Leshner R, Florence J, Mayhew J, Tesi-Rocha C, Gorni K, Pasquali L, Patel KM, McCarter R, Huang J, Mayhew T, Bertorini T, Carlo J, Connolly AM, Clemens PR, Goemans N, Iannaccone ST, Igarashi M, Nevo Y, Pestronk A, Subramony SH, Vedanarayanan VV, Wessel H; CINRG Group. CINRG randomized controlled trial of creatine and glutamine in Duchenne muscular dystrophy. Ann Neurol 2005;58:151-155.

136. Ferrante KL, Shefner J, Zhang H, Betensky R, O'Brien M, Yu H, Fantasia M, Taft J, Beal MF, Traynor B, Newhall K, Donofrio P, Caress J, Ashburn C, Freiberg B, O'Neill C, Paladenech C, Walker T, Pestronk A, Abrams B, Florence J, Renna R, Schierbecker J, Malkus B, Cudkowicz M. Tolerance of high-dose (3,000 mg/day) coenzyme Q10 in ALS. Neurology 2005;65:1834-1836.

137. Weihl CC, Dalal S, Pestronk A, Hanson PI. Inclusion body myopathy associated mutations in p97/VCP impair endoplasmic reticulum associated degradation. Hum Mol Genet 2006;15:189-199

138. Levine T, Pestronk A, Florence J, Al-Lozi MT, Lopate G, Miller T, Ramneantu I, Waheed W, Stambuk M, Stone MJ, Choksi R. Peripheral neuropathies in Waldenström's macroglobulinaemia. J Neurol Neurosurg Psychiatry. 2006;77:224-228.

139. Lopate G, Pestronk A, Al-Lozi M, Lynch T, Florence J, Miller T, Levine T, Rampy T, Beson B, Ramneantu I. Peripheral neuropathy in an outpatient cohort of patients with Sjögren's syndrome. Muscle Nerve. 2006;33:672-676.

140. Strom, R G; Derdeyn, C P; Moran, C J; Cross, D T; Esper, G J; Mazumdar, A; Al-Lozi, M; Lopate, G; Pestronk, A. Frequency of spinal arteriovenous malformations in patients with unexplained myelopathy. Neurology 2006;66:928-931.

141. Pestronk A, Kos K, Lopate G, Al-Lozi MT. Brachio-cervical inflammatory myopathies: Clinical, immune, and myopathologic features. Arthritis Rheum 2006;54:1687-1696.

142. Sampson JB, Chin SS, Clayton FC, Pestronk A, Swoboda KJ, Flanigan KM. An unusual pathologic feature associated with dermatomyositis. Neuromuscul Disord 2006;16:391-393.

143. Weihl CC, Connolly AM, Pestronk A. Valproate may improve strength and function in patients with type III/IV spinal muscle atrophy. Neurology 2006;67:500-501.

144. Forman MS, Mackenzie IR, Cairns NJ, Swanson E, Boyer PJ, Drachman DA, Jhaveri BS, Karlawish JH, Pestronk A, Smith TW, Tu PH, Watts GD, Markesbery WR, Smith CD, Kimonis VE. Novel Ubiquitin Neuropathology in Frontotemporal Dementia With Valosin-Containing Protein Gene Mutations. J Neuropathol Exp Neurol. 2006;65:571-581.

145. Moore SA, Shilling CJ, Westra S, Wall C, Wicklund MP, Stolle C, Brown CA, Michele DE, Piccolo F, Winder TL, Stence A, Barresi R, King N, King W, Florence J, Campbell KP, Fenichel GM, Stedman HH, Kissel JT, Griggs RC, Pandya S, Mathews KD, Pestronk A, Serrano C, Darvish D, Mendell JR. Limb-Girdle Muscular Dystrophy in the United States. J Neuropathol Exp Neurol 2006;65:995-1003.

146. Galvin JE, Palamand D, Strider J, Milone M, Pestronk A. The muscle protein dysferlin accumulates in the Alzheimer brain. Acta Neuropathol (Berl) 2006;112:665-671.

147. Cudkowicz ME, Shefner JM, Schoenfeld DA, Zhang H, Andreasson KI, Rothstein JD, Drachman DB, Northeast ALS Consortium. Trial of celecoxib in amyotrophic lateral sclerosis. Ann Neurol 2006;60:22-31.

148. Baloh RH, Schmidt RE, Pestronk A, Milbrandt J. Altered axonal mitochondrial transport in the pathogenesis of Charcot-Marie-Tooth disease from mitofusin 2 mutations. J Neurosci 2007;27:422-430

149. Baloh RH, Rakowicz W, Gardner R, Pestronk A. Frequent atrophic groups with mixed-type myofibers is distinctive to motor neuron syndromes. Muscle Nerve 2007;36:107-110

150. Weihl CC, Miller SE, Hanson PI, Pestronk A. Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice. Hum Mol Genet 2007;16:919-928

151. Sampson JB, Smith SM, Smith AG, Singleton JR, Chin S, Pestronk A, Flanigan KM. Paraneoplastic myopathy: response to intravenous immunoglobulin. Neuromuscul Disord 2007;17:404-408

152. Baloh RH, Salavaggione E, Milbrandt J, Pestronk A. Familial parkinsonism and ophthalmoplegia from a mutation in the mitochondrial DNA helicase twinkle. Arch Neurol 2007;64:998-1000.

153. Dunckley T, Huentelman MJ, Craig DW, Pearson JV, Szelinger S, Joshipura K, Halperin RF, Stamper C, Jensen KR, Letizia D, Hesterlee SE, Pestronk A, Levine T, Bertorini T, Graves MC, Mozaffar T, Jackson CE, Bosch P, McVey A, Dick A, Barohn R, Lomen-Hoerth C, Rosenfeld J, O'connor DT, Zhang K, Crook R, Ryberg H, Hutton M, Katz J, Simpson EP, Mitsumoto H, Bowser R, Miller RG, Appel SH, Stephan DA. Whole-Genome Analysis of Sporadic Amyotrophic Lateral Sclerosis. N Engl J Med 2007;357:775-788.

154. Gwinn K, Corriveau RA, Mitsumoto H, Bednarz K, Brown RH Jr, Cudkowicz M, Gordon PH, Hardy J, Kasarskis EJ, Kaufmann P, Miller R, Sorenson E, Tandan R, Traynor BJ, Nash J, Sherman A, Mailman MD, Ostell J, Bruijn L, Cwik V, Rich SS, Singleton A, Refolo L, Andrews J, Zhang R, Conwit R, Keller MA; ALS Research Group. Amyotrophic lateral sclerosis: an emerging era of collaborative gene discovery. PLoS ONE. 2007;2:e1254.

155. Gordon PH, Moore DH, Miller RG, Florence JM, Verheijde JL, Doorish C, Hilton JF, Spitalny GM, MacArthur RB, Mitsumoto H, Neville HE, Boylan K, Mozaffar T, Belsh JM, Ravits J, Bedlack RS, Graves MC, McCluskey LF, Barohn RJ, Tandan R; Western ALS Study Group. Efficacy of minocycline in patients with amyotrophic lateral sclerosis: a phase III randomised trial. Lancet Neurol 2007;6:1045-1053.

156. Gitcho MA, Baloh RH, Chakraverty S, Mayo K, Norton JB, Levitch D, Hatanpaa KJ, White CL 3rd, Bigio EH, Caselli R, Baker M, Al-Lozi MT, Morris JC, Pestronk A, Rademakers R, Goate AM, Cairns NJ. TDP-43 A315T mutation in familial motor neuron disease. Ann Neurol 2008;63:535-538

157. Wagner KR, Fleckenstein JL, Amato AA, Barohn RJ, Bushby K, Escolar DM, Flanigan KM, Pestronk A, Tawil R, Wolfe GI, Eagle M, Florence JM, King WM, Pandya S, Straub V, Juneau P, Meyers K, Csimma C, Araujo T, Allen R, Parsons SA, Wozney JM, Lavallie ER, Mendell JR. A phase I/II trial of MYO-029 in adult subjects with muscular dystrophy. Ann Neurol. 2008;63:561-571

158. Kroos M, Pomponio RJ, van Vliet L, Palmer RE, Phipps M, Van der Helm R, Halley D, Reuser A, GAA Database Consortium. Update of the Pompe disease mutation database with 107 sequence variants and a format for severity rating. Hum Mutat 2008;29:E13-26.

159. Zaidman CM, Holland MR, Anderson CC, Pestronk A. Calibrated quantitative ultrasound imaging of skeletal muscle using backscatter analysis. Muscle Nerve 2008;38:893-898.

160. Gordon PH, Cheung YK, Levin B, Andrews H, Doorish C, Macarthur RB, Montes J, Bednarz K, Florence J, Rowin J, Boylan K, Mozaffar T, Tandan R, Mitsumoto H, Kelvin EA, Chapin J, Bedlack R, Rivner M, McCluskey LF, Pestronk A, Graves M, Sorenson EJ, Barohn RJ, Belsh JM, Lou JS, Levine T, Saperstein D, Miller RG, Scelsa SN. Combination Drug Selection Trial Study Group. A novel, efficient, randomized selection trial comparing combinations of drug therapy for ALS. Amyotroph Lateral Scler 2008;9:212-222.

161. Weihl CC, Temiz P, Miller SE, Watts G, Smith C, Forman M, Hanson PI, Kimonis V, Pestronk A. TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia. J Neurol Neurosurg Psychiatry 2008;79:1186-1189.

162. Wokke JH, Escolar DM, Pestronk A, Jaffe KM, Carter GT, van den Berg LH, Florence JM, Mayhew J, Skrinar A, Corzo D, Laforet P. Clinical features of late-onset Pompe disease: a prospective cohort study. Muscle Nerve 2008;38:1236-1245.

163. Temiz P, Weihl CC, Pestronk A. Inflammatory myopathies with mitochondrial pathology and protein aggregates. J Neurol Sci 2009;278:25-29.

164. Nozaki K, Pestronk A. High Aldolase with Normal Creatine Kinase in serum predicts a Myopathy with Perimysial Pathology. J Neurol Neurosurg Psychiatry 2009;80:904-908.

165. Weihl CC, Pestronk A, Kimonis VE. Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia. Neuromuscul Disord 2009;19:308-315.

166. Kaufmann P, Thompson JL, Levy G, Buchsbaum R, Shefner J, Krivickas LS, Katz J, Rollins Y, Barohn RJ, Jackson CE, Tiryaki E, Lomen-Hoerth C, Armon C, Tandan R, Rudnicki SA, Rezania K, Sufit R, Pestronk A, Novella SP, Heiman-Patterson T, Kasarskis EJ, Pioro EP, Montes J, Arbing R, Vecchio D, Barsdorf A, Mitsumoto H, Levin B; QALS Study Group. Phase II trial of CoQ10 for ALS finds insufficient evidence to justify phase III. Ann Neurol 2009;66:235-244.

167. Zaidman CM, Al-Lozi M, Pestronk A. Peripheral nerve size in normals and patients with polyneuropathy: An ultrasound study. Muscle Nerve 2009 Aug 20.

168. Margeta M, Connolly AM, Winder TL, Pestronk A, Moore SA. Cardiac pathology exceeds skeletal muscle pathology in two cases of limb-girdle muscular dystrophy type 2I. Muscle Nerve 2009;40:883-889.

169. WenKaspar R, Allen HD, Ray WC, Alvarez CE, Kissel JT, Pestronk A, Weiss RB, Flanigan KM, Mendell JR, Montanaro F. Analysis of Dystrophin Deletion Mutations Predicts Age of Cardiomyopathy Onset in Becker Muscular Dystrophy. Circ Cardiovasc Genet 2009;2:544-551.

170. Flanigan KM, Dunn DM, von Niederhausern A, Soltanzadeh P, Gappmaier E, Howard MT, Sampson JB, Mendell JR, Wall C, King WM, Pestronk A, Florence JM, Connolly AM, Mathews KD, Stephan CM, Laubenthal KS, Wong BL, Morehart PJ, Meyer A, Finkel RS, Bonnemann CG, Medne L, Day JW, Dalton JC, Margolis MK, Hinton VJ, Weiss RB; the United Dystrophinopathy Project Consortium. Mutational spectrum of DMD mutations in dystrophinopathy patients: application of modern diagnostic techniques to a large cohort. Hum Mutat 2009;30:1657-1666.

171. Buchsbaum R, Kaufmann P, Barsdorf AI, Arbing R, Montes J, Thompson JL; QALS Study Group. Web-based data management for a phase II clinical trial in ALS. Amyotroph Lateral Scler. 2009;10:374-377.

172. Gurnett CA, Desruisseau DM, McCall K, Choi R, Meyer ZI, Talerico M, Miller SE, Ju JS, Pestronk A, Connolly AM, Druley TE, Weihl CC, Dobbs MB. Myosin binding protein C1: a novel gene for autosomal dominant distal arthrogryposis type 1. Hum Mol Genet 2010;19:1165-1173.

173. van der Ploeg AT, Clemens PR, Corzo D, Escolar DM, Florence J, Groeneveld GJ, Herson S, Kishnani PS, Laforet P, Lake SL, Lange DJ, Leshner RT, Mayhew JE, Morgan C, Nozaki K, Park DJ, Pestronk A, Rosenbloom B, Skrinar A, van Capelle CI, van der Beek NA, Wasserstein M, Zivkovic SA. A randomized study of alglucosidase alfa in late-onset Pompe's disease. N Engl J Med 2010;362:1396-1406.

174. Pestronk A, Schmidt RE, Choksi R. Vascular pathology in dermatomyositis and anatomic relations to myopathology. Muscle Nerve 2010;42:53-61.

175. Aggarwal SP, Zinman L, Simpson E, McKinley J, Jackson KE, Pinto H, Kaufman P, Conwit RA, Schoenfeld D, Shefner J, Cudkowicz M; Northeast and Canadian Amyotrophic Lateral Sclerosis consortia. Safety and efficacy of lithium in combination with riluzole for treatment of amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial. Lancet Neurol 2010;9:481-488.

176. Pestronk A, Chuquilin M, Choksi R. Motor neuropathies and serum IgM binding to NS6S heparin disaccharide or GM1 ganglioside. J Neurol Neurosurg Psychiatry 2010;81:726-730.

177. Soltanzadeh P, Friez MJ, Dunn D, von Niederhausern A, Gurvich OL, Swoboda KJ, Sampson JB, Pestronk A, Connolly AM, Florence JM, Finkel RS, Bönnemann CG, Medne L, Mendell JR, Mathews KD, Wong BL, Sussman MD, Zonana J, Kovak K, Gospe SM Jr, Gappmaier E, Taylor LE, Howard MT, Weiss RB, Flanigan KM. Clinical and genetic characterization of manifesting carriers of DMD mutations. Neuromuscular Disorders 2010;20:499-504.

178. Harms MB, Allred P, Gardner R Jr, Fernandes Filho JA, Florence J, Pestronk A, Al-Lozi M, Baloh RH. Dominant spinal muscular atrophy with lower extremity predominance: Linkage to 14q32. Neurology 2010;75:539-546.

179. Zaidman CM, Connolly AM, Malkus EC, Florence JM, Pestronk A. Quantitative ultrasound using backscatter analysis in Duchenne and Becker muscular dystrophy. Neuromuscular Disorders 2010;20:805-809..

180. Weihl CC, Miller SE, Zaidman CM, Pestronk A, Baloh RH, Al-Lozi M. Novel GNE mutations in two phenotypically distinct HIBM2 patients. Neuromuscul Disorders 2011;21:102-105.

181. Flanigan KM, Dunn DM, von Niederhausern A, Soltanzadeh P, Howard MT, Sampson JB, Swoboda KJ, Bromberg MB, Mendell JR, Taylor L, Anderson CB, Pestronk A, Florence J, Connolly AM, Mathews KD, Wong B, Finkel RS, Bonnemann CG, Day JW, McDonald C, Weiss RB; the United Dystrophinopathy Project Consortium. Nonsense mutation-associated Becker muscular dystrophy: interplay between exon definition and splicing regulatory elements within the DMD gene. Hum Mutat 2011;32:299-308.

182. Pegoraro E, Hoffman EP, Piva L, Gavassini BF, Cagnin S, Ermani M, Bello L, Soraru G, Pacchioni B, Bonifati MD, Lanfranchi G, Angelini C, Kesari A, Lee I, Gordish-Dressman H, Devaney JM, McDonald CM; On behalf of the Cooperative International Neuromuscular Research Group. SPP1 genotype is a determinant of disease severity in Duchenne muscular dystrophy. Neurology 2011;76:219-226.

183. Zaidman CM, Malkus EA, Siener C, Florence J, Pestronk A, Al-Lozi M. Qualitative and Quantitative Skeletal Muscle Ultrasound in Late-Onset Acid Maltase Disease. Muscle Nerve 2011;44:418-423.

184. Spurney CF, Rocha CT, Henricson E, Florence J, Mayhew J, Gorni K, Pasquali L, Pestronk A, Martin GR, Hu F, Nie L, Connolly AM, Escolar DM; the Cooperative International Neuromuscular Research Group (CINRG) Investigators. CINRG pilot trial of coenzyme Q10 in steroid-treated Duchenne muscular dystrophy. Muscle Nerve 2011;44:174-178.

185. Escolar DM, Hache LP, Clemens RR, Cnaan A, McDonald CM, Viswanathan V, Kornberg AJ, Bertorini TE, Nevo Y, Lotze T, Pestronk A, Ryan MM, Monasterio E, Day JW, Zimmerman A, Arrieta A, Henricson E, Mayhew J, Florence J, Hu F, Connolly AM. Randomized, blinded trial of weekend vs daily prednisone in Duchenne muscular dystrophy. Neurology 2011;77:444-452.

186. Miller RG, Moore DH, Forshew DA, Katz JS, Barohn RJ, Valan M, Bromberg MB, Goslin KL, Graves MC, McCluskey LF, McVey AL, Mozaffar T, Florence JM, Pestronk A, Ross M, Simpson EP, and Appel SH, On behalf of the WALS Study Group. Phase II screening trial of lithium carbonate in amyotrophic lateral sclerosis: Examining a more efficient trial design. Neurology 2011;77:973-979.

187. Forsha D, Li JS, Smith PB, van der Ploeg AT, Kishnani P, Pasquali SK; Clemens P, Escolar DM, Leshner RT, Laforêt P, Pestronk A, Wasserstein M, van der Ploeg A, Rosenbloom B, Culper E, Mengel E, Hopkin R, Casey R, Charrow J, Sillence D, Lemieux B, Sims K, Scott CR, Durieu I, Furby A, Zagnoli F, Barohn R, Nations S, Pyeritz R, Edgar T, Barship B, Olsen M, Tita J, Schaefer GB, Aleck K. Cardiovascular abnormalities in late-onset Pompe disease and response to enzyme replacement therapy. Genet Med 2011;13:625-631.

188. Harms MB, Sommerville RB, Allred P, Bell S, Ma D, Cooper P, Lopate G, Pestronk A, Weihl CC, Baloh RH. Exome sequencing reveals DNAJB6 mutations in dominantly-inherited myopathy. Ann Neurol 2012;71:407-416.

189. Pestronk A, Schmidt RE, Choksi RM, Sommerville RB, Al-Lozi MT. Clinical and laboratory features of neuropathies with serum IgM binding to TS-HDS. Muscle Nerve 2012;45:866-872.

190. Harms MB, Ori-McKenney KM, Scoto M, Tuck EP, Bell S, Ma D, Masi S, Allred P, Al-Lozi M, Reilly MM, Miller LJ, Jani-Acsadi A, Pestronk A, Shy ME, Muntoni F, Vallee RB, Baloh RH. Mutations in the tail domain of DYNC1H1 cause dominant spinal muscular atrophy. Neurology 2012;78:1714-1720

191. Shefner J, Cedarbaum JM, Cudkowicz ME, Maragakis N, Lee J, Jones D, Watson ML, Mahoney K, Chen M, Saikali K, Mao J, Russell AJ, Hansen RL, Malik F, Wolff AA; Neals/Cytokinetics Study Team: Atassi N, Bedlack R, Boylan K, Bradshaw D, Goslin K, Heiman Patterson T, Jackson C, Kasarskis EJ, Katz J, Levine T, Maragakis NJ, Pestronk A, Simmons Z. Safety, tolerability and pharmacodynamics of a skeletal muscle activator in amyotrophic lateral sclerosis. Amyotroph Lateral Scler 2012;13:430-438.

192. van der Ploeg AT, Barohn R, Carlson L, Charrow J, Clemens PR, Hopkin RJ, Kishnani PS, Laforêt P, Morgan C, Nations S, Pestronk A, Plotkin H, Rosenbloom BE, Sims KB, Tsao E. Open-label extension study following the Late-Onset Treatment Study (LOTS) of alglucosidase alfa. Mol Genet Metab 2012;107:456-461

193. Zaidman CM, Holland MR, Noetzel MJ, Park TS, Pestronk A. Newborn brachial plexus palsy: Evaluation of severity using quantitative ultrasound of muscle Muscle Nerve 2013;47:246-254

194. Flanigan KM, Ceco E, Lamar KM, Kaminoh Y, Dunn DM, Mendell JR, King WM, Pestronk A, Florence JM, Mathews KD, Finkel RS, Swoboda KJ, Gappmaier E, Howard MT, Day JW, McDonald C, McNally EM, Weiss RB, for the United Dystrophinopathy Project. LTBP4 genotype predicts age of ambulatory loss in Duchenne Muscular Dystrophy. Ann Neurol 2012; Online November.

195. Lopate G, Streif E, Harms M, Weihl C, Pestronk A. Cramps and small fiber neuropathy. Muscle Nerve 2013;48:252–255

196. Henricson EK, Abresch RT, Cnaan A, Hu F, Duong T, Arrieta A, Han JJ, Escolar DM, Florence JM, Clemens PR, Hoffman EP, McDonald CM, CINRG Investigators: ... Pestronk A ,... The CINRG duchenne natural history study: Glucocorticoid treatment preserves clinically-meaningful functional milestones and reduces rate of disease progression as measured by manual muscle testing and other commonly-used clinical trial outcome measures. Muscle Nerve 2013;48:55-67.

197. McDonald CM, Henricson EK, Abresch RT, Han JJ, Escolar DM, Florence JM, Duong T, Arrieta A, Clemens PR, Hoffman EP, Cnaan A, CINRG Investigators: ... Pestronk A,... The cooperative international neuromuscular research group Duchenne natural history study-- a longitudinal investigation in the era of glucocorticoid therapy: design of protocol and the methods used. Muscle Nerve 2013;48:32-54.

198. Manousakis G, Koch J, Sommerville RB, El-Dokla A, Harms MB, Al-Lozi MT, Schmidt RE, Pestronk A. Multifocal radiculoneuropathy during ipilimumab treatment of melanoma. Muscle Nerve 2013;48:440-444.

199. Kim HJ, Kim NC, Wang YD, Scarborough EA, Moore J, Diaz Z, MacLea KS, Freibaum B, Li S, Molliex A, Kanagaraj AP, Carter R, Boylan KB, Wojtas AM, Rademakers R, Pinkus JL, Greenberg SA, Trojanowski JQ, Traynor BJ, Smith BN, Topp S, Gkazi AS, Miller J, Shaw CE, Kottlors M, Kirschner J, Pestronk A, Li YR, Ford AF, Gitler AD, Benatar M, King OD, Kimonis VE, Ross ED, Weihl CC, Shorter J, Taylor JP. Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS. Nature 2013;495:467-473.

200. Sommerville RB, Zaidman CM, Pestronk A. Coenzyme Q10 deficiency in children: Frequent type 2C muscle fibers with normal morphology. Muscle Nerve Muscle Nerve 2013;48:722-726..

201. Miller TM, Pestronk A, David W, Rothstein J, Simpson E, Appel SH, Andres PL, Mahoney K, Allred P, Alexander K, Ostrow LW, Schoenfeld D, Macklin EA, Norris DA, Manousakis G, Crisp M, Smith R, Bennett CF, Bishop KM, Cudkowicz ME. An antisense oligonucleotide against SOD1 delivered intrathecally for patients with SOD1 familial amyotrophic lateral sclerosis: a phase 1, randomised, first-in-man study. Lancet Neurol 2013;12:435-342.

202. Harms MB, Cady J, Zaidman C, Cooper P, Bali T, Allred P, Cruchaga C, Baughn M, Libby RT, Pestronk A, Goate A, Ravits J, Baloh RH. Lack of C9ORF72 coding mutations supports a gain of function for repeat expansions in amyotrophic lateral sclerosis. Neurobiol Aging 2013;34:2234.e13-19.

203. Berry JD, Shefner JM, Conwit R, Schoenfeld D, Keroack M, Felsenstein D, Krivickas L, David WS, Vriesendorp F, Pestronk A, Caress JB, Katz J, Simpson E, Rosenfeld J, Pascuzzi R, Glass J, Rezania K, Rothstein JD, Greenblatt DJ, Cudkowicz ME. Design and initial results of a multi-phase randomized trial of ceftriaxone in amyotrophic lateral sclerosis. PLoS One 2013;8:e61177.

204. Zaidman CM, Seelig MJ, Baker JC, Mackinnon SE, Pestronk A. Detection of peripheral nerve pathology: Comparison of ultrasound and MRI. Neurology 2013:80:1634-1640.

205. Zaidman CM, Harms MB, Pestronk A. Ultrasound of inherited vs. acquired demyelinating polyneuropathies. J Neurol 2013;260:3115-3121.

206. Cudkowicz ME, van den Berg LH, Shefner JM, Mitsumoto H, Mora JS, Ludolph A, Hardiman O, Bozik ME, Ingersoll EW, Archibald D, Meyers AL, Dong Y, Farwell WR, Kerr DA; EMPOWER investigators, Carbonell, J G [corrected to Gamez, J]. Dexpramipexole versus placebo for patients with amyotrophic lateral sclerosis (EMPOWER): a randomised, double-blind, phase 3 trial. Lancet Neurol 2013;12:1059-1067.

207. Cady J, Koval ED, Benitez BA, Zaidman C, Jockel-Balsarotti J, Allred P, Baloh RH, Ravits J, Simpson E, Appel SH, Pestronk A, Goate AM, Miller TM, Cruchaga C, Harms MB. TREM2 Variant p.R47H as a Risk Factor for Sporadic Amyotrophic Lateral Sclerosis. JAMA Neurol 2014;71:449-453.

208. Zaidman CM, Pestronk A. Nerve Size in CIDP Varies with Disease Activity and Therapy Response Over Time: A Retrospective Ultrasound Study. Muscle Nerve. 2014;50:733-738.

209. Johnson JO, Pioro EP, Boehringer A, Chia R, Feit H, Renton AE, Pliner HA, Abramzon Y, Marangi G, Winborn BJ, Gibbs JR, Nalls MA, Morgan S, Shoai M, Hardy J, Pittman A, Orrell RW, Malaspina A, Sidle KC, Fratta P, Harms MB, Baloh RH, Pestronk A, Weihl CC, Rogaeva E, Zinman L, Drory VE, Borghero G, Mora G, Calvo A, Rothstein JD, Drepper C, Sendtner M, Singleton AB, Taylor JP, Cookson MR, Restagno G, Sabatelli M, Bowser R, Chio A, Traynor BJ. Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis. Nat Neurosci 2014;17:664-666.

210. Zhao Y, Cudkowicz ME, Shefner JM, Krivickas L, David WS, Vriesendorp F, Pestronk A, Caress JB, Katz J, Simpson E, Rosenfeld J, Pascuzzi R, Glass J, Rezania K, Harmatz JS, Schoenfeld D, Greenblatt DJ. Systemic pharmacokinetics and cerebrospinal fluid uptake of intravenous ceftriaxone in patients with amyotrophic lateral sclerosis. J Clin Pharmacol. 2014;54:1180-1187.

211. Dori A, Lopate G, Keeling R, Pestronk A. Myovascular innervation: Axon loss in small fiber neuropathies. Muscle Nerve 2015;51:514-521.

212. Cady J, Allred P, Bali T, Pestronk A, Goate A, Miller TM, Mitra R, Ravits J, Harms MB, Baloh RH. ALS onset is influenced by the burden of rare variants in known ALS genes. Ann Neurol 2014 Nov 7

213. Cai C, Alshehri A, Choksi R, Pestronk A. Regional Ischemic Immune Myopathy: A Paraneoplastic Dermatomyopathy. J Neuropathol Exp Neurol 2014 Nov 7

214. Cudkowicz ME, Titus S, Kearney M, Yu H, Sherman A, Schoenfeld D, Hayden D, Shui A, Brooks B, Conwit R, Felsenstein D, Greenblatt DJ, Keroack M, Kissel JT, Miller R, Rosenfeld J, Rothstein JD, Simpson E, Tolkoff-Rubin N, Zinman L, Shefner JM; Ceftriaxone Study Investigators. Safety and efficacy of ceftriaxone for amyotrophic lateral sclerosis: a multi-stage, randomised, double-blind, placebo-controlled trial. Lancet Neurol 2014;13:1083-1091

215. Weihl CC, Iyadurai S, Baloh RH, Pittman SK, Schmidt RE, Lopate G, Pestronk A, Harms MB. Autophagic vacuolar pathology in desminopathies. Neuromuscular Disorders 2014; December 16.

216. Spurney C1, Shimizu R, Morgenroth LP, Kolski H, Gordish-Dressman H, Clemens PR; CINRG Investigators. Cooperative International Neuromuscular Research Group Duchenne Natural History Study demonstrates insufficient diagnosis and treatment of cardiomyopathy in Duchenne muscular dystrophy. Muscle Nerve 2014;50:250-256

217. Bushby K1, Finkel R, Wong B, Barohn R, Campbell C, Comi GP, Connolly AM, Day JW, Flanigan KM, Goemans N, Jones KJ, Mercuri E, Quinlivan R, Renfroe JB, Russman B, Ryan MM, Tulinius M, Voit T, Moore SA, Lee Sweeney H, Abresch RT, Coleman KL, Eagle M, Florence J, Gappmaier E, Glanzman AM, Henricson E, Barth J, Elfring GL, Reha A, Spiegel RJ, O'donnell MW, Peltz SW, Mcdonald CM; PTC124-GD-007-DMD STUDY GROUP. Ataluren treatment of patients with nonsense mutation dystrophinopathy. Muscle Nerve 2014;50:477-487.

218. Weihl CC, Baloh RH, Lee Y, Chou TF, Pittman SK, Lopate G, Allred P, Jockel-Balsarotti J, Pestronk A, Harms MB Targeted sequencing and identification of genetic variants in sporadic inclusion body myositis. Neuromuscular Disorders 2015;25:289-296

219. Findlay AR1, Wein N, Kaminoh Y, Taylor LE, Dunn DM, Mendell JR, King WM, Pestronk A, Florence JM, Mathews KD, Finkel RS, Swoboda KJ, Howard MT, Day JW, McDonald C, Nicolas A, Le Rumeur E, Weiss RB, Flanigan KM; for the United Dystrophinopathy Project. Clinical phenotypes as predictors of the outcome of skipping around DMD exon 45. Ann Neurol 2015;77:668-674.

220. Renton AE, Pliner HA, Provenzano C, Evoli A, Ricciardi R, Nalls MA, Marangi G, Abramzon Y, Arepalli S, Chong S, Hernandez DG, Johnson JO, Bartoccioni E, Scuderi F, Maestri M, Gibbs JR, Errichiello E, Chiò A, Restagno G, Sabatelli M, Macek M, Scholz SW, Corse A, Chaudhry V, Benatar M, Barohn RJ, McVey A, Pasnoor M, Dimachkie MM, Rowin J, Kissel J, Freimer M, Kaminski HJ, Sanders DB, Lipscomb B, Massey JM, Chopra M, Howard JF Jr, Koopman WJ, Nicolle MW, Pascuzzi RM, Pestronk A, Wulf C, Florence J, Blackmore D, Soloway A, Siddiqi Z, Muppidi S, Wolfe G, Richman D, Mezei MM, Jiwa T, Oger J, Drachman DB, Traynor BJ. A Genome-wide Association Study of Myasthenia Gravis. JAMA Neurol 2015;72:396-404.

221. Goyal NA, Cash TM, Alam U, Enam S, Tierney P, Araujo N, Mozaffar FH, Pestronk A, Mozaffar T. Seropositivity for NT5c1A antibody in sporadic inclusion body myositis predicts more severe motor, bulbar and respiratory involvement. J Neurol Neurosurg Psychiatry 2016;87:373-378.

222. Liewluck T, Tian X, Wong LJ, Pestronk A. Dystrophinopathy mimicking metabolic myopathies. Neuromuscul Disord 2015;25:653-677.

223. Schweitzer GG1, Collier SL, Chen Z, Eaton JM, Connolly AM, Bucelli RC, Pestronk A, Harris TE, Finck BN. Rhabdomyolysis-Associated Mutations in Human LPIN1 Lead to Loss of Phosphatidic Acid Phosphohydrolase Activity. JIMD Rep 2015;23:113-122.

224. Alshehri A, Choksi R, Bucelli R, Pestronk A. Myopathy with anti-HMGCR antibodies: Perimysium and myofiber pathology.
Neurol Neuroimmunol Neuroinflamm 2015;2:e124

225. Bucelli RC, Arhzaouy K, Pestronk A, Pittman SK, Rojas L, Sue CM, Evilä A, Hackman P, Udd B, Harms MB, Weihl CW. SQSTM1 splice site mutation in distal myopathy with rimmed vacuoles. Neurology 2015;85:665-674

226. Tahsili-Fahadan P, Yahyavi-Firouz-Abadi N, Keyrouz SG, Pestronk A. Bicaudate infarcts in the setting of congenital absence of A1 segment. Neurol Clin Pract 2015;5:540-541.

227. Dori A, Lopate G, Choksi R, Pestronk A. Myelinated and unmyelinated endoneurial axon quantitation and clinical correlation.
Muscle Nerve 2016;53:198-204.

228. Dietz AR, Bucelli RC, Pestronk A, Zaidman CM. Nerve ultrasound identifies abnormalities in the posterior interosseous nerve in patients with proximal radial neuropathies. Muscle Nerve 2016;53:379-383

229. Albulym OM, Kennerson ML, Harms MB, Drew AP, Siddell AH, Auer-Grumbach M, Pestronk A, Connolly A, Baloh RH, Zuchner S, Reddel SW, Nicholson GA. MORC2 mutations cause axonal Charcot-Marie-Tooth disease with pyramidal signs. Ann Neurol 2016;79:419-427

230. Sansone VA, Burge J, McDermott MP, Smith PC, Herr B, Tawil R, Pandya S, Kissel J, Ciafaloni E, Shieh P, Ralph JW, Amato A, Cannon SC, Trivedi J, Barohn R, Crum B, Mitsumoto H, Pestronk A, Meola G, Conwit R, Hanna MG, Griggs RC; Muscle Study Group. Randomized, placebo-controlled trials of dichlorphenamide in periodic paralysis. Neurology 2016;86:1408-1416.

231. Weiss MD, Macklin EA, Simmons Z, Knox AS, Greenblatt DJ, Atassi N, Graves M, Parziale N, Salameh JS, Quinn C, Brown RH Jr, Distad JB, Trivedi J, Shefner JM, Barohn RJ, Pestronk A, Swenson A, Cudkowicz ME. A randomized trial of mexiletine in ALS: Safety and effects on muscle cramps and progression. Neurology 2016;86:1474-1481.

232. Bali T, Self W, Liu J, Siddique T, Wang LH, Bird TD, Ratti E, Atassi N, Boylan KB, Glass JD, Maragakis NJ, Caress JB, McCluskey LF, Appel SH, Wymer JP, Gibson S, Zinman L, Mozaffar T, Callaghan B, McVey AL, Jockel-Balsarotti J, Allred P, Fisher ER, Lopate G, Pestronk A, Cudkowicz ME, Miller TM. Defining SOD1 ALS natural history to guide therapeutic clinical trial design. J Neurol Neurosurg Psychiatry 2017;88:99-105.

233. van der Ploeg A, Carlier PG, Carlier RY, Kissel JT, Schoser B, Wenninger S, Pestronk A, Barohn RJ, Dimachkie MM, Goker-Alpan O, Mozaffar T, Pena LD, Simmons Z, Straub V, Guglieri M, Young P, Boentert M, Baudin PY, Wens S, Shafi R, Bjartmar C, Thurberg BL. Prospective exploratory muscle biopsy, imaging, and functional assessment in patients with late-onset Pompe disease treated with alglucosidase alfa: The EMBASSY Study. Mol Genet Metab 2016;119:115-123

234. Griggs RC, Miller JP, Greenberg CR, Fehlings DL, Pestronk A, Mendell JR, Moxley RT 3rd, King W, Kissel JT, Cwik V, Vanasse M, Florence JM, Pandya S, Dubow JS, Meyer JM. Efficacy and safety of deflazacort vs prednisone and placebo for Duchenne muscular dystrophy. Neurology 2016;87:2123-2131

235. Harris E, Bladen CL, Mayhew A, James M, Bettinson K, Moore U, Smith FE, Rufibach L, Cnaan A, Bharucha-Goebel DX, Blamire AM, Bravver E, Carlier PG, Day JW, Díaz-Manera J, Eagle M, Grieben U, Harms M, Jones KJ, Lochmüller H, Mendell JR, Mori-Yoshimura M, Paradas C, Pegoraro E, Pestronk A, Salort-Campana E, Schreiber-Katz O, Semplicini C, Spuler S, Stojkovic T, Straub V, Takeda S, Rocha CT, Walter MC, Bushby K; Jain COS Consortium. The Clinical Outcome Study for dysferlinopathy: An international multicenter study. Neurol Genet 2016;2:e89.

236. Bello L, Flanigan KM, Weiss RB, Spitali P, Aartsma-Rus A, Muntoni F, Zaharieva I, Ferlini A7, Mercuri E, Tuffery-Giraud S, Claustres M, Straub V, Lochmüller H, Barp A, Vianello S, Pegoraro E, Punetha J, Gordish-Dressman H, Giri M, McDonald CM, Hoffman EP; Dunn DM, Swoboda KJ, Gappmaier E, Howard MT, Sampson JB, Bromberg MB, Butterfield R, Kerr L, Pestronk A, Florence JM, Connolly A, Lopate G, Golumbek P, Schierbecker J, Malkus B, Renna R, Siener C, Finkel RS, Bonnemann CG, Medne L, Glanzman AM, Flickinger J, Mendell JR, King WM, Lowes L, Alfano L, Mathews KD, Stephan C, Laubenthal K, Baldwin K, Wong B, Morehart P, Meyer A, Day JW, Naughton CE, Margolis M, Cnaan A, Abresch RT, Henricson EK, Morgenroth LP, Duong T, Chidambaranathan VV, Biggar WD, McAdam LC, Mah J, Tulinius M, Leshner R, Rocha CT, Thangarajh M, Kornberg A, Ryan M, Nevo Y, Dubrovsky A, Clemens PR, Abdel-Hamid H, Connolly AM, Pestronk A, Teasley J, Bertorini TE, North K, Webster R, Kolski H, Kuntz N, Driscoll S, Carlo J, Gorni K, Lotze T, Day JW, Karachunski P, Bodensteiner JB. Association Study of Exon Variants in the NF-κB and TGFβ Pathways Identifies CD40 as a Modifier of Duchenne Muscular Dystrophy. Am J Hum Genet 2016;99:1163-1171

237. Pestronk A, Keeling R, Choksi R. Sarcopenia, Age, Atrophy, and Myopathy: Mitochondrial Oxidative Enzyme Activities. Muscle Nerve 2017;56:122-128

238. Argov Z, Caraco Y, Lau H, Pestronk A, Shieh PB, Skrinar A, Koutsoukos T, Ahmed R, Martinisi J, Kakkis E. Aceneuramic Acid Extended Release Administration Maintains Upper Limb Muscle Strength in a 48-week Study of Subjects with GNE Myopathy: Results from a Phase 2, Randomized, Controlled Study. J Neuromuscul Dis 2016;3:49-66

239. Hogarth MW, Houweling PJ, Thomas KC, Gordish-Dressman H, Bello L; Cooperative International Neuromuscular Research Group (CINRG)., Pegoraro E, Hoffman EP, Head SI, North KN. Evidence for ACTN3 as a genetic modifier of Duchenne muscular dystrophy. Nat Commun 2017 Jan 31;8:14143.

240. Cabrera-Serrano M, Junckerstorff RC, Alisheri A, Pestronk A, Laing NG, Weihl CC, Lamont PJ. Cystinosis distal myopathy, novel clinical, pathological and genetic features. Neuromuscul Disord. 2017;27:873-878.

241. Wambach JA, Stettner GM, Haack TB, Writzl K, Škofljanec A, Maver A, Munell F, Ossowski S, Bosio M, Wegner DJ, Shinawi M, Baldridge D, Alhaddad B, Strom TM, Grange DK, Wilichowski E, Troxell R, Collins J, Warner BB, Schmidt RE, Pestronk A, Cole FS, Steinfeld R. Survival among children with "Lethal" congenital contracture syndrome 11 caused by novel mutations in the gliomedin gene (GLDN). Hum Mutat. 2017;38:1477-1484.

242. Argov Z, Bronstein F, Esposito A, Feinsod-Meiri Y, Florence JM, Fowler E, Greenberg MB, Malkus EC, Rebibo O, Siener CS, Caraco Y, Kolodny EH, Lau HA, Pestronk A, Shieh P, Skrinar AM, Mayhew JE. Characterization of Strength and Function in Ambulatory Adults With GNE Myopathy. J Clin Neuromuscul Dis 2017;19:19-26.

243. Kafaie J, Al Balushi A, Kim M, Pestronk A. Clinical and Laboratory Profiles of Idiopathic Small Fiber Neuropathy in Children: Case Series. J Clin Neuromuscul Dis 2017;19:31-37.

244. McDonald CM, Henricson EK, Abresch RT, Duong T, Joyce NC, Hu F, Clemens PR, Hoffman EP, Cnaan A, Gordish-Dressman H; CINRG Investigators. Long-term effects of glucocorticoids on function, quality of life, and survival in patients with Duchenne muscular dystrophy: a prospective cohort study. Lancet 2018;391:451-461.

245. Bucelli RC, Pestronk A. Immune Myopathies with Perimysial Pathology (IMPP): Clinical and Laboratory Features. Neurol Neuroimmunol Neuroinflamm 2018;5(2):e434.

246. Moore UR, Jacobs M, Fernandez-Torron R, Jang J, James MK, Mayhew A, Rufibach L, Mittal P, Eagle M, Cnaan A, Carlier PG, Blamire A, Hilsden H, Lochmüller H, Grieben U, Spuler S, Tesi Rocha C, Day JW, Jones KJ, Bharucha-Goebel DX, Salort-Campana E, Harms M, Pestronk A, Krause S, Schreiber-Katz O, Walter MC, Paradas C, Hogrel JY, Stojkovic T, Takeda S, Mori-Yoshimura M, Bravver E, Sparks S, Diaz-Manera J, Bello L, Semplicini C, Pegoraro E, Mendell JR, Bushby K, Straub V. Teenage exercise is associated with earlier symptom onset in dysferlinopathy: a retrospective cohort study. J Neurol Neurosurg Psychiatry 2018;89:1224-1226

247. Garcia-Santibanez R, Zaidman CM, Sommerville RB, Lopate G, Weihl CC, Pestronk A, Bucelli RC. CANOMAD and other chronic ataxic neuropathies with disialosyl antibodies (CANDA). J Neurol 2018;265:1402-1409.

248. Oates EC, Jones KJ, Donkervoort S, Charlton A, Brammah S, Smith JE 3rd, Ware JS, Yau KS, Swanson LC, Whiffin N, Peduto AJ, Bournazos A, Waddell LB, Farrar MA, Sampaio HA, Teoh HL, Lamont PJ, Mowat D, Fitzsimons RB, Corbett AJ, Ryan MM, O'Grady GL, Sandaradura SA, Ghaoui R, Joshi HB, Marshall JL, Nolan MA, Kaur S, Punetha J, Töpf A, Harris E, Bakshi M, Genetti CA, Marttila M, Werlauff U, Streichenberger N, Pestronk A, Mazanti I, Pinner JR, Vuillerot C, Grosmann C, Camacho A, Mohassel P, Leach ME, Foley AR, Bharucha-Goebel D, Collins J, Connolly AM, Gilbreath HR, Iannaccone ST, Castro D, Cummings BB, Webster R, Lazaro L, Vissing J, Coppens S, Deconinck N, Luk HM, Thomas NH, Foulds NC, Illingworth MA, Ellard S, McLean CA, Phadke R, Ravenscroft G, Witting N, Hackman P, Richard I, Cooper ST, Kamsteeg EJ, Hoffman EP, Bushby K, Straub V, Udd B, Ferreiro A, North KN, Clarke NF, Lek M, Beggs AH, Bönnemann CG, MacArthur DG, Granzier H, Davis MR, Laing NG. Congenital titinopathy: Comprehensive characterisation and pathogenic insights. Ann Neurol 2018;83:1105-1124.

249. Diaz-Manera J, Fernandez-Torron R, LLauger J, James MK, Mayhew A, Smith FE, Moore UR, Blamire AM, Carlier PG, Rufibach L, Mittal P, Eagle M, Jacobs M, Hodgson T, Wallace D, Ward L, Smith M, Stramare R, Rampado A, Sato N, Tamaru T, Harwick B, Rico Gala S, Turk S, Coppenrath EM, Foster G, Bendahan D, Le Fur Y, Fricke ST, Otero H, Foster SL, Peduto A, Sawyer AM, Hilsden H, Lochmuller H, Grieben U, Spuler S, Tesi Rocha C, Day JW, Jones KJ, Bharucha-Goebel DX, Salort-Campana E, Harms M, Pestronk A, Krause S, Schreiber-Katz O, Walter MC, Paradas C, Hogrel JY, Stojkovic T, Takeda S, Mori-Yoshimura M, Bravver E, Sparks S, Bello L, Semplicini C, Pegoraro E, Mendell JR, Bushby K, Straub V; Jain COS Consortium. Muscle MRI in patients with dysferlinopathy: pattern recognition and implications for clinical trials. J Neurol Neurosurg Psychiatry. 2018;89:1071-1081.

250. Findlay AR, Harms MB, Pestronk A, Weihl CC. Homozygous recessive MYH2 mutation mimicking dominant MYH2 associated myopathy. Neuromuscul Disord 2018;28:675-679.

251. Moore U, Jacobs M, James MK, Mayhew AG, Fernandez-Torron R, Feng J, Cnaan A, Eagle M, Bettinson K, Rufibach LE, Lofra RM, Blamire AM, Carlier PG, Mittal P, Lowes LP, Alfano L, Rose K, Duong T, Berry KM, Montiel-Morillo E, Pedrosa-Hernandez I, Holsten S, Sanjak M, Ashida A, Sakamoto C, Tateishi T, Yajima H, Canal A, Ollivier G, Decostre V, Mendez JB, Sanchez-Aguilera Praxedes N, Thiele S, Siener C, Shierbecker J, Florence JM, Vandevelde B, DeWolf B, Hutchence M, Gee R, Prügel J, Maron E, Hilsden H, Lochmüller H, Grieben U, Spuler S, Tesi Rocha C, Day JW, Jones KJ, Bharucha-Goebel DX, Salort-Campana E, Harms M, Pestronk A, Krause S, Schreiber-Katz O, Walter MC, Paradas C, Hogrel JY, Stojkovic T, Takeda S, Mori-Yoshimura M, Bravver E, Sparks S, Diaz-Manera J, Bello L, Semplicini C, Pegoraro E, Mendell JR, Bushby K, Straub V. Assessment of disease progression in dysferlinopathy: A 1-year cohort study. Neurology 2019 Jan 9.

252. Malik A, Lopate G, Hayat G, Jones J, Atluri R, Malo B, Pestronk A. Prevalence of Axonal Sensory Neuropathy With IgM Binding to Trisulfated Heparin Disaccharide in Patients With Fibromyalgia. J Clin Neuromuscul Dis 2019;20:103-110.

253. Pena LDM, Barohn RJ, Byrne BJ, Desnuelle C, Goker-Alpan O, Ladha S, Laforêt P, Mengel KE, Pestronk A, Pouget J, Schoser B, Straub V, Trivedi J, Van Damme P, Vissing J, Young P, Kacena K, Shafi R, Thurberg BL, Culm-Merdek K, van der Ploeg AT; NEO1 Investigator Group. Safety, tolerability, pharmacokinetics, pharmacodynamics, and exploratory efficacy of the novel enzyme replacement therapy avalglucosidase alfa (neoGAA) in treatment-naïve and alglucosidase alfa-treated patients with late-onset Pompe disease: A phase 1, open-label, multicenter, multinational, ascending dose study. Neuromuscul Disord 2019;29:167-186.

254. Pestronk A, Sinha N, Alhumayyd Z, Ly C, Schmidt R, Bucelli R. Immune Myopathy with Large-Histiocyte-Related Myofiber Necrosis. Neurology 2019;92:e1763-e1772.

255. Shefner JM, Cudkowicz ME, Hardiman O, Cockcroft BM, Lee JH, Malik FI, Meng L, Rudnicki SA, Wolff AA, Andrews JA; VITALITY-ALS Study Group. A phase III trial of tirasemtiv as a potential treatment for amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2019;20:584-594.

256. Babu S, Macklin EA, Jackson KE, Simpson E, Mahoney K, Yu H, Walker J, Simmons Z, David WS, Barkhaus PE, Simionescu L, Dimachkie MM, Pestronk A, Salameh JS, Weiss MD, Brooks BR, Schoenfeld D, Shefner J, Aggarwal S, Cudkowicz ME, Atassi N. Selection design phase II trial of high dosages of tamoxifen and creatine in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2020;21:15-23.

257. Levine TD, Kafaie J, Zeidman LA, Saperstein DS Massaquoi R, Bland RJ, Pestronk A. Cryptogenic Small Fiber Neuropathies: Serum Autoantibody Binding to TS-HDS and FGFR-3. Muscle Nerve 2020;61:512-515.

258. Pestronk A. Chronic Graft Versus Host Myopathies: Noninflammatory, Multi-Tissue Pathology With Glycosylation Disorders. J Neuropathol Exp Neurol 2020;79:102-112

259. Chung HL, Wangler MF, Marcogliese PC, Jo J, Ravenscroft TA, Zuo Z, Duraine L, Sadeghzadeh S, Li-Kroeger D, Schmidt RE, Pestronk A, Rosenfeld JA, Burrage L, Herndon MJ, Chen S; Members of Undiagnosed Diseases Network, Shillington A, Vawter-Lee M, Hopkin R, Rodriguez-Smith J, Henrickson M, Lee B, Moser AB, Jones RO, Watkins P, Yoo T, Mar S, Choi M, Bucelli RC, Yamamoto S, Lee HK, Prada CE, Chae JH, Vogel TP, Bellen HJ. Loss- or Gain-of-Function Mutations in ACOX1 Cause Axonal Loss via Different Mechanisms. Neuron 2020;106:589-606.e6

260. Miller T, Cudkowicz M, Shaw PJ, Andersen PM, Atassi N, Bucelli RC, Genge A, Glass J, Ladha S, Ludolph AL, Maragakis NJ, McDermott CJ, Pestronk A, Ravits J, Salachas F, Trudell R, Van Damme P, Zinman L, Bennett CF, Lane R, Sandrock A, Runz H, Graham D, Houshyar H, McCampbell A, Nestorov I, Chang I, McNeill M, Fanning L, Fradette S, Ferguson TA. Phase 1-2 Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS. N Engl J Med 2020;383:109-119.

261. Green JD, Barohn RJ, Bartoccion E, Benatar M, Blackmore D, Chaudhry V, Chopra M, Corse A, Dimachkie MM, Evoli A, Florence J, Freimer M, Howard JF, Jiwa T, Kaminski HJ, Kissel JT, Koopman WJ, Lipscomb B, Maestri M, Marino M, Massey JM, McVey A, Mezei MM, Muppidi S, Nicolle MW, Oger J, Pascuzzi RM, Pasnoor M, Pestronk A, Provenzano C, Ricciardi R, Richman DP, Rowin J, Sanders DB, Siddiqi Z, Soloway A, Wolfe GI, Wulf C, Drachman DB, Traynor BJ. Epidemiological evidence for a hereditary contribution to myasthenia gravis: A retrospective cohort study of patients from North America. BMJ Open 2020;10(9):e037909.

262. Trikamji B, Pestronk A. Treatable, Motor-Sensory, Axonal Neuropathies with C5b-9 Complement on Endoneurial Microvessels. Muscle Nerve 2021;63:506-515.

263. Moore U, Jacobs M, Fernandez-Torron R, LLauger Rossello J, Smith FE, James M, Mayhew A, Rufibach L, Carlier PG, Blamire AM, Day JW, Jones KJ, Bharucha-Goebel DX, Salort-Campana E, Pestronk A, Walter MC, Paradas C, Stojkovic T, Mori-Yoshimura M, Bravver E, Pegoraro E, Mendell JR, Bushby K, Straub V, Diaz-Manera J. Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy. Front Neurol 2020;11:613446.

264. Ikenaga C, Findlay AR, Goyal NA, Robinson S, Cauchi J, Hussain Y, Wang LH, Kershen JC, Beson BA, Wallendorf M, Bucelli RC, Mozaffar T, Pestronk A, Weihl CC. Clinical utility of anticytosolic 5'-nucleotidase 1A antibody in idiopathic inflammatory myopathies. Ann Clin Transl Neurol 2021;8:571-578.

265. Jacobs M, James MK, Lowes LP, Alfano LN, Eagle M, Lofra RM, Moore U, Feng J, Rufibach LE, Rose K, Duong T, Bello L, Pedrosa-Hernández I, Holsten S, Sakamoto C, Canal A, Práxedes NS, Thiele S, Siener C, Vandevelde B, DeWolf B, Maron E, Guglieri M, Hogrel JY, Blamire AM, Carlier PG, Spuler S, Day JW, Jones KJ, Bharucha-Goebel DX, Salort-Campana E, Pestronk A, Walter MC, Paradas C, Stojkovic T, Mori-Yoshimura M, Bravver E, Díaz-Manera J, Pegoraro E, Mendell JR, Mayhew AG, Straub V. Assessing Dysferlinopathy Patients Over Three Years With A New Motor Scale. Ann Neurol 2021;89:967-978.

266. Moore U, Gordish H, Diaz-Manera J, James MK, Mayhew AG, Guglieri M, Fernandez-Torron R, Rufibach LE, Feng J, Blamire AM, Carlier PG, Spuler S, Day JW, Jones KJ, Bharucha-Goebel DX, Salort-Campana E, Pestronk A, Walter MC, Paradas C, Stojkovic T, Mori-Yoshimura M, Bravver E, Pegoraro E, Lowes LP, Mendell JR, Bushby K, Straub V. Miyoshi myopathy and limb girdle muscular dystrophy R2 are the same disease. Neuromuscul Disord 2021;31:265-280.

267. Pestronk A, Choksi R. Pathology Features of Immune and Inflammatory Myopathies, Including a Polymyositis Pattern, Relate Strongly to Serum Autoantibodies. J Neuropathol Exp Neurol 2021;80:812-820.

268. Wencel M, Shaibani A, Goyal NA, Dimachkie MM, Trivedi J, Johnson NE, Gutmann L, WicklundMP, Bandyopadhay S, Genge AL, Freimer ML, Goyal N, Pestronk A, Florence J, Karam C Ralph JW, Rasheed Z, Currence M, Hopkins S, Mozaffar T. Investigating Pompe Prevalence in Neuromuscular Medicine Academic Practices (The IPaNeMA Study). Neurology Genetics 2021;7:e623.

269. Schoser B, Roberts M, Byrne BJ, Sitaraman S, Jiang H, Laforêt P, Toscano A, Castelli J, Díaz-Manera J, Goldman M, van der Ploeg AT, Bratkovic D, Kuchipudi S, Mozaffar T, Kishnani PS; PROPEL Study Group. Safety and efficacy of cipaglucosidase alfa plus miglustat versus alglucosidase alfa plus placebo in late-onset Pompe disease (PROPEL): an international, randomised, double-blind, parallel-group, phase 3 trial. Lancet Neurol 2021;20:1027-1037

270. Chia R, Saez-Atienzar S, Murphy N, Chiò A, Blauwendraat C; International Myasthenia Gravis Genomics Consortium, Roda RH, Tienari PJ, Kaminski HJ, Ricciardi R, Guida M, De Rosa A, Petrucci L, Evoli A, Provenzano C, Drachman DB, Traynor BJ. Identification of genetic risk loci and prioritization of genes and pathways for myasthenia gravis: a genome-wide association study. Proc Natl Acad Sci U S A. 2022;119:e2108672119.

271. Moore U, Fernandez-Torron R, Jacobs M, Gordish H, Diaz-Manera J, James MK, Mayhew AG, Harris E, Guglieri M, Rufibach LE, Feng J, Blamire AM, Carlier PG, Spuler S, Day JW, Jones KJ, Bharucha-Goebel DX, Salort-Campana E, Pestronk A, Walter MC, Paradas C, Stojkovic T, Mori-Yoshimura M, Bravver E, Pegoraro E, Lowes LP, Mendell JR, Bushby K; Jain COS Consortium, Bourke J, Straub V. Cardiac and pulmonary findings in dysferlinopathy: a 3-year, longitudinal study. Muscle Nerve 2022;65:531-540.

272. Mayhew AG, James MK, Moore U, Sutherland H, Jacobs M, Feng J, Lowes LP, Alfano LN, Muni Lofra R, Rufibach LE, Rose K, Duong T, Bello L, Pedrosa-Hernández I, Holsten S, Sakamoto C, Canal A, Sánchez-Aguilera Práxedes N, Thiele S, Siener C, Vandevelde B, DeWolf B, Maron E, Gordish-Dressman H, Hilsden H, Guglieri M, Hogrel JY, Blamire AM, Carlier PG, Spuler S, Day JW, Jones KJ, Bharucha-Goebel DX, Salort-Campana E, Pestronk A, Walter MC, Paradas C, Stojkovic T, Mori-Yoshimura M, Bravver E, Díaz-Manera J, Pegoraro E, Mendell JR, Straub V. Assessing the Relationship of Patient Reported Outcome Measures With Functional Status in Dysferlinopathy: A Rasch Analysis Approach. Front Neurol 2022;13:828525.

273. Statland JM, Campbell C, Desai U, Karam C, Díaz-Manera J, Guptill JT, Korngut L, Genge A, Tawil RN, Elman L, Joyce NC, Wagner KR, Manousakis G, Amato AA, Butterfield RJ, Shieh PB, Wicklund M, Gamez J, Bodkin C, Pestronk A, Weihl CC, Vilchez-Padilla JJ, Johnson NE, Mathews KD, Miller B, Leneus A, Fowler M, van de Rijn M, Attie KM. Randomized phase 2 study of ACE-083, a muscle-promoting agent, in facioscapulohumeral muscular dystrophy. Muscle Nerve 2022;66:50-62.

274. Thomas FP, Brannagan TH 3rd, Butterfield RJ, Desai U, Habib AA, Herrmann DN, Eichinger KJ, Johnson-Cl NE, Karam C, Pestronk A, Quinn C, Shy ME, Statland JM, Subramony SH, Walk D, Stevens-Favorite K, Miller B, Leneus A, Fowler M, van de Rijn M, Attie KM. Randomized Phase 2 Study of ACE-083 in Patients With Charcot-Marie-Tooth Disease. Neurology 2022;98:e2356-67.

275. Moore U, Caldas de Almeida Araújo E, Reyngoudt H, Gordish-Dressman H, Smith FE, Wilson I, James M, Mayhew A, Rufibach L, Day JW, Jones KJ, Bharucha-Goebel DX, Salort-Campana E, Pestronk A, Walter MC, Paradas C, Stojkovic T, Mori-Yoshimura M, Bravver E, Pegoraro E, Mendell JR, Bushby K, Blamire AM, Straub V, Carlier PG, Diaz-Manera J. Water T2 could predict functional decline in patients with dysferlinopathy. J Cachexia Sarcopenia Muscle 2022;13:2888-2897.

276. Garret M, Pestronk A. Sarcoidosis, granulomas and myopathy syndromes: A clinical-pathology review. J Neuroimmunol. 2022;373:577975.

277. Moore U, Fernández-Simón E, Schiava M, Cox D, Gordish-Dressman H, James MK, Mayhew A, Wilson I, Guglieri M, Rufibach L, Blamire A, Carlier PG, Mori-Yoshimura M, Day JW, Jones KJ, Bharucha-Goebel DX, Salort-Campana E, Pestronk A, Walter MC, Paradas C, Stojkovic T, Bravver E, Pegoraro E, Mendell JR, Bushby K, Diaz-Manera J, Straub V; Jain COS Consortium. Myostatin and follistatin as monitoring and prognostic biomarkers in dysferlinopathy. Neuromuscul Disord 2023;33:199-207.

278. Pestronk A, Schmidt RE, Bucelli R, Sim J. Schwann Cells and Myelin in Human Peripheral Nerve: Major Protein Components Vary with Age, Axon Size and Pathology. Neuropathol Appl Neurobiol 2023 Mar 3:e12898.

279. Expanding the muscle imaging spectrum in dysferlinopathy: description of an outlier population from the classical MRI pattern. Llansó L, Moore U, Bolano-Diaz C, James M, Blamire AM, Carlier PG, Rufibach L, Gordish-Dressman H, Boyle G, Hilsden H, Day JW, Jones KJ, Bharucha-Goebel DX, Salort-Campana E, Pestronk A, Walter MC, Paradas C, Stojkovic T, Mori-Yoshimura M, Bravver E, Pegoraro E, Mendell JR, Straub V, Díaz-Manera J. Neuromuscul Disord 2023;33:349-357.

280. Rebelo AP, Tomaselli PJ, Medina J, Wang Y, Dohrn M, Nyvltova E, Denzi M, Garrett M, Smith S, Pestronk A, Li C, Ruiz A, Jacobs E, Feely SME, França MC, Gomes MV, Santos D, Kumar S, Lombard DB, Saporta M, Hekimi S, Barrientos A, Weihl C, Shy M, Marques W, Zuchner S. Biallelic variants in COQ7 cause distal hereditary motor neuropathy with upper motor neuron signs. Brain 2023 May 12.

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