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Shaped Neuromuscular Disease in North America Richard J How the Vietnam War, Communism, Russia/USSR (and other despots) Shaped Neuromuscular Disease in North America Richard J. Barohn, M.D. Gertrude and Dewey Ziegler Professor of Neurology University Distinguished Professor Vice Chancellor for Research University of Kansas Medical Center Kansas City, KS KUMC Neurology Grand Rounds Kansas City, KS July 19th, 2019 Dr. Barohn served as a consultant for NuFactor and Momenta Pharmaceutical and receives research support from PTC Therapeutics, Ra Pharma, Orphazyme, Sanofi Genzyme, FDA OOPD, NIH, and PCORI. www.rrnmf.com > 2,000 Neuromuscular Health Care Professionals The Doctor Draft • The Doctor Draft began during Korean • Alternative to military: Public Health War-early 50s Service (PHS) as Commissioned • Continued during Vietnam War 1965- Officer-very selective 1973 • Could serve in PHS at various • Two year commitment to military locations armed forces -PHS Hospitals in major cities • Berry plan allowed doctors to defer -Indian Health Service Hospitals service until after training -National Communicable Disease Center (future CDC) -National Center for Urban and Industrial Health -NIH NIH Associate Training Program-ATP • Began in 1950s • Assigned to a Senior Staff • Clinical or Research or Staff Investigator/mentor Associates • Very competitive • After completing at least two • Associates chosen by the post doctoral years of Institute Scientific Director training/residency Number of Participants in NIH ATP • 1960: #68 associates • 1964-Gulf of Tonkin Resolution, authorizing The New president to take military action in Vietnam w/out York Times 25 May 1965 congressional approval • 1965: #178 associates • 1970: #206 associates • 1973: #229 associates Kissinger negotiated peace settlement in Paris -1974: #191 -1976: #108 The New York -1980:#76 Times 7 August 1964 NIH ATP Less than 5 % who applied got in heavily dominated by Eastern medical schools Between 1963-1975 In 1965, % of incoming associates % Alumni Participated : did their internship at: ➢ Harvard Medical School-12 ➢ 9.2 Hopkins to 20% ➢ 7.8 MGH ➢ U Michigan-.5 to 3.3% ➢ 6.5 Duke ➢ Northwestern- 0.5 to 3% ➢ 4.6 Bellevue ➢ UCSF- 0.4 to 2% ➢ 4 Brigham ➢ 3.9 Boston City ➢ 3.3 Bronx Municipal ➢ 1.3 U. Michigan ➢ 1.3 U. Minnesota NIH ATP • Two years at NIH but some extended • Some are still at NIH: Anthony Fauci was Clinical Associate 1968 to 1970 • Was a launching point for an academic research career as a physician • They referred to themselves as the “Yellow Berets”, serving in the “Battle of Bethesda” • Influenced most areas of medicine G. Milton Shy, MD 1919-1967 • Born in Trinidad, Colorado on Sept. 30, 1919 • Medical School- University of Oregon • Wounded US Army WW II as a doctor in Italy • Neurology training in Queens Square in London & Montreal Neurological Institute-McGill Univ. • 1951 Head of Neurology, University of Colorado • Described patients with neurodegenerative disorder ̶ Now called Shy-Drager Syndrome ̶ Orthostasis, ataxia PD, MND • 1953 to 1960 Intramural Clinical Director NINDB • Son is Michael Shy M.D., Charcot-Marie Tooth (CMT) expert at the University of Iowa G. Milton Shy, MD 1919-1967 • Muscle Disease interest at NINDB (NIH) • Described: – mitochondrial myopathy – central core myopathy – nemaline myopathy • EM of muscles • Intracellular recordings from nerves and muscles • Classified muscle disease by lesion site: muscle membrane, mitochondria, contractile proteins • 1961 Chair Neurology, U of Penn • 1967 Chair Neurology, Columbia / Prof NY Neurological Institute • Died of MI 3 weeks after he arrived, on September 25th, 1967. He was only 48 years old • Neuro house staff, Roger Rosenberg & Jerry Mendell tried to resuscitate him G. Milton Shy, MD A New Congenital Non- Publications Progressive Myopathy Nemaline Myopathy. A New Two Childhood Myopathies with Central Core Myopathy Congenital Myopathy Abnormal Mitochondria Brain. 1956 Dec;79(4):610-21. Brain. 1963 Dec;86:793-810. Brain. 1966 Mar;89(1):133-58. G. Milton Shy, MD 1919-1967 Students: • Lewis P. Rowland (Penn/Columbia) -Famously left NIH when he refused to sign document related to finding communists • W. King Engel (USC) • Daniel Drachman (Johns Hopkins) • David Drachman (UMass) • Andrew G. Engel (Mayo) • Donald Silberberg (Perelman SOM Univ. Penn) • Stan Appel (Houston Methodist) • Berch Griggs (Rochester) W. King Engel, MD • Born in St. Louis, MO; University City High School • Chief Neurology NINDS Intramural 1963-1978; at NIH till 1981 • Revolutionized muscle histology & classification of muscle disease • Prednisone in MG • Acetazolamide in periodic paralysis • RRF in mitochondrial myopathy • Married to Valerie Askanas, MD,PhD • 1981 – 2016 USC • Now USC Professor Emeritus • Retired with Dr. Askanas in Michigan W. King Engel, MD • Robert C. Griggs (Rochester NY) • Teepu Siddique (Northwestern) Trainees at NIH: • Michael Brooke (deceased) • John Trotter (deceased) (Wash U) • Bob Rolofs (deceased) • Jerry Mendell (OSU) • Jerry Resnick (Connecticut) • Jack Warmolts (OSU/deceased) • Walter Camp (Connecticut) • Steve Ringel (U of Colo.) • Michel Reznik (Belgium) • John Griffin (Johns Hopkins/deceased) • Leon Hogenhouis (Holland) • George Karpati (McGill/deceased) • Irwin Brody (deceased) • Gerald Fenichel (Vanderbilt) • Jose Carlo (Puerto Rico) • Tulio Bertorini (U of Tenn.) • Roger Kula (New York) • Dale McFarlin (NIH) • Ted Norris (LA/deceased) • Michel Fardeau (France) • Shalini Mahajan (Cedar Sinai, LA) • David Pleasure (U. Penn) • Said Beydoun (USC) • John Morgan-Hughes (England) • Jay Cook (LA) • Barry Festoff (KU) • Shin Oh (Univ. Alabama) • Marinos Dalakas (Jefferson and Greece) • Bruce Adornato (Palo Alto) • Ben Brooks ( North Carolina) • John Whitaker (Univ. Alabama/deceased) W. King Engel, MD The histographic analysis Publications of human muscle biopsies with regard to fiber types 2. Diseases of the 3. Myotonias, myasthenia 1. Adult male and upper and lower gravis, and hypokalemic 4. Children's female motor neuron periodic paralysis. biopsies. Neurology. 1969 Neurology 1969 Neurology. 1969 Neurology. 1969 Mar;19(3):221-33 April; 19(4): 378 May;19(5):469-77 Jun;19(6):591-605. W. King Engel Publications N Engl J Med 1972; Jan 6; (286):17-20 N Engl J Med 1968; Mar 14;(278):582-586 ENGEL TRICHROME STAINING RAGGED RED FIBERS Courtesy Drs Askanas and Engel CIDD: Clinical Investigation of Duchenne Muscular Dystrophy Griggs, Mendell, Fenichel, Moxley, Brooke Arch Neurol. 1987;44:812-817 Arch Neurol. 1991;48:575-579 Arch Neurol. 1987:44(8):812-7. Neurology 1993;43:520-7 Landmark Paper N Engl J Med 1989; 320:1592-7 Michael H. Brooke, MD Landmark Books A Clinician’s view of Muscle Biopsy: Neuromuscular Diseases A Modern Approach 2nd Edition Other Books by Shy & K. Engel Students Myology: Neuromuscular Diagnosis and Basic and Management of Clinical 1994 Peripheral Nerve Disorders 2001 Evaluation and Treatment of Neuromuscular Myopathies 1995 Disorders 2008 Pathology of Skeletal Muscle 2001 DeJong’s The Neurologic Examination 2019 Neuro Family Tree: Decedents of Shy through Engel, Mendell, & Griggs Milton Shy King Engel Mendell Griggs Ersin Tan M. Wicklund A. Amato J. Kissel Z. Sahenk R. Barohn M. Collins R. Tawil C. Thorton J. Statland N. Johnston S. Iannacone M. Rose R. Orrell A. Mankodi UTHSCSA: Harvard UTSW KUMC C. Jackson B&W/MGH Fellows-Too Okankwo-Onuigbo R. Raja J. Janas C. Greenfield many to count Z. Elchami R. Paduga L. Ridings S. Knobler H. Ali B. Zaminiferki N. Jetha N. Baker B. Marky D. Aggarwal R. Nieto S. Nations Y. Wang A. Abuzinadah D. Drampitz B. Atkinson L. Rashid K. Johnson T. Salmon J. Herold S. Khan D. Jabari R. Escobar C. Cha R. Mummaneni E. Bachir V. Venkatesh D. Saperstein G. Miller I. Muzyka A. Haq H. Alkersam Z. Haddad B. Bittel R. Van der Y. J. Southwell M. Tahmasbi Sohi Sluis Lindenhaum G. Lian L. Nayak J. Bauerle C. Ugokew H. Kabbani O. Jawdat S. Rao S. Kojan C. Milford T. Quesnell Z. Cheema Z. Nanijani S. Kamar K. Veerapeneni S. Manek S. Mathews H. Anderson A. Hamasaki S. Glusman S. Alseoudi R. Murphy J. Trivedi M. Pasnoor D. Kimple J. Katz D. Dumitru M. Varon T. Burka S.Chandrashekhar K. Syed Mentorship is the key to Career Success! Escape from U.S.S.R. & Communism Russian Revolutions: • 1905-Failed • 1914: Martial Law declared by Russian Empire • 1917:Tsar Nicholas abdicates and Bolsheviks/ Vladimir Lenin takes power • 1918-Tzar & family executed • 1918-1922: Civil war culminating in formation of U.S.S.R. Peter J. Dyck, MD • PJ Dyck born 1927 between the Black and Caspian Seas in U.S.S.R. • Father: Jacob Dyck, teacher and Mennonite minister • 1929-Jacob’s name appeared on a list as an enemy of the people because of his faith • Went to Moscow to get passports and then fled to Canada • Lived in Saskatchewan in a sod hut • Univ. of Saskatchewan BA/MD/internship/Int. Med./Neurology/Neuropathology • Mayo Clinic Fellow and Staff Peter J. Dyck, MD Thermal discs • Single handedly developed field of nerve disease and pathology • Sensory testing quantification • Fellows too many to count Toluidine blue stained Teased Nerve Fibers plastic sections Peter J. Dyck, MD Landmark CIDP Publications Mayo Clinical Proceedings 1975;50:621-637 Ann Neurol 1994;36:838-845 Ann Neurol N Engl J Med 1982;11:136-141 1986;314:461-465 George Karpati, MD and Escape from Hungary USSR occupied Hungary since end of WW2 • 1955 –U.S.S.R. created Warsaw Pact, binding Hungary and U.S.S.R. and satellite states in Central/Eastern Europe Hungarian Revolution/Uprising • October 1956 • Led to Soviet crackdown and military occupation and repression George Karpati, MD B:1934 in Debrecen, Hungary D: MI-February 6, 2009 in Montreal, Canada • Jewish…during WW2 at age 8 he was on his way in a cattle car to death camp. A “sleepy railway switch operator accidentally deflected George’s train to a forced labor camp” But his father was later killed in a concentration camp • After the war, remained in Hungary, went to college/medical school • In medical school at age 22 when U.S.S.R.
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