G. Milton Shy M.D.1919-1967

• B. Trinidad, Colorado

• Med School Oregon

• Wounded US Army WW II as a Dr. in Italy

• Neurology training in Queens Square & Montreal Neurological Institute

• 1951 Head of Neurology, U Colorado

• Described pts with neurodegenerative disorder

Now called Shy-Drager Syndrome

Orthostasis, ataxia PD, MND

• 1953 to 1960 Intramural Clinical Director NINDB

• Son is Michael Shy MD, CMT expert at U Iowa

G. Milton Shy MD 1919-1967

• Muscle Disease interest at MNDB (NIH)

• Described:

– mitochondrial myopathy ,

– central core myopathy

– nemaline myopathy

• EM of muscles

• Intracellular recordings from nerves & 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

• Dies of MI in office on top floor, 3 weeks after he arrived. 47 years old

• Neuro house staff tried to resuscitate him (Roger Rosenberg & Jerry Mendell)

G. Milton Shy M.DPublications

.

Two Childhood Myopathies with Abnormal Mitochondria

Brain. 1966 Mar;89(1):133-58.

Nemaline Myopathy. A New Congenital Myopathy

Brain. 1963 Dec;86:793-810.

A New Congenital Non-Progressive MyopathyCentral Core Myopathy

Brain. 1956 Dec;79(4):610-21.

G. Milton Shy M.D. 1919 - 1967

Students:• Andrew Engel (NIH)

• Daniel Drachman (NIH)

• David Drachman (NIH)

• Donald Silberberg (Perelman SOM Univ Penn)

• Lewis P. Rowland (Penn/Columbia)

• Stan Appel (Penn)

• Berch Griggs (NIH)

• Roger Rosenberg (Columbia)

• W. King Engel (NIH) (b. St. Louis)

– Chief Neurology NINDS 1963-1978 & at NIH till 1990

– Revolutionized muscle histology & classified muscle disease

– Steroids in MG

– Acetazolamide in periodic paralysis

– RRF in mitochondrial myopathy

– 1990 – Present at USC

W. King Engel M.D.Students:

• Robert C. Griggs (Rochester NY)

• Michael Brooke (Wash U)

• Jerry Mendell (OSU)

• Steve Ringel (U of Colo)

• John Griffin (Johns Hopkins)

• Geroge Karpati (McGill)

• Gerald Fenichel (Vanderbilt)

• Tulio Bertorini (U of Tenn)

• Dale McFarlin (NIH)

• Michel Fardeau (France)

• Fernando Tomé (France)

• David Pleasure (U. Penn)

• John Morgan-Hughes (England)

• Barry Festoff (KU)

• Marcus Dalakas (NIH)

W. King Engel M.D.Publications

.

The histographic analysis of human muscle biopsies with regard to fiber types

1. Adult male and female

Neurology. 1969 Mar;19(3):221-33

3. Myotonias, myasthenia gravis, and hypokalemic periodic paralysis.

Neurology. 1969 May;19(5):469-77

4. Children's biopsies.

Neurology. 1969 Jun;19(6):591-605.

Neurology 1969April; 19(4): 378

2. Diseases of the upper and lower motor neuron

.

W. King Engel Publications

N Engl J Med 1972; Jan 6; (286):17-20

Benefit from Alternate-Day Prednisone in Myasthenia Gravis

N Engl J Med 1968; Mar 14;(278):582-586

Acetazolamide Prophylaxis in Hypokalemic Periodic Paralysis

Neuro Family Tree: Decedents of Shy

Milton Shy

Lewis Rowland King Engel

Mendell

M. Wicklund A. Amato R. Barohn

UTHSCSA

Fellow

UTSW

Fellows

KUMC

Fellows

J. Kissel Z. Sahenk M. Collins

Griggs

Dr Barohn's Neurophysiology and Neuromuscular Fellows at UTHSCSA

and UTSW

Joanne Janas MD Jonathan Katz MDLarry Ridings MD C Fish Greenfield MDNabat Jetha MD Stacey B Knobler MDRoberto Nieto MD Noel Baker MDDaniel Krampitz MD Sharon P Nations MDThomas Salmon MD Bryan Atkinson MDRaul Escobar MD James Herold MDVenna Venkatesh MD Choon Cha MD Anwatul Haq MD David Saperstein MD Ralf Van der Sluis MD Husam Alkersam MDJay Bauerle MD Yelena Lindenhaum MDSurendra Rao MD Charles Ugokew MD Zabid Cheema MD Suleiman Kojan MDSalil Manek MD Zohair NanijianiMD Steven Glusman MD Sanil Mathews MDJaya Trivedi MD

Carlayne Jackson MD

Dr Barohn's Clinical Neurophysiology and Neuromuscular Fellows in Department of

Neurology at KUMC

Obi Okankwo- Onuigbo MD Gary Lian MD Ziad Elchami MD Haidar Kabbani MDHazem Ali MD Christopher Milford MDBlanco Marky DO Sanjeev Kamar MDYunxia Wang MD Heather Anderson MDLjaz Rashid MD Sarab Alseoudi MDSaud Khan MD Mamatha Pasnor MDReddiah Mummaneni MD Dan Dumitru MDGary Miller MD Kazi Syed MDZiad Haddad MD Faisal Raja MD James Southwell DO

.

R. Paduga MD B. Bittel MD B. Zaminiferki MD M. Tahmasbi Sohi MD D. Aggarwal MBBS L. Nayak MBBS A. Abuzinadah MBBS O. Jawdat MD K. Johnson MD T. Quesnell MD D. Jabari MD K. Veerapaneni MBBS E. Bachir MD A. Hamaski MD I. Muzyka MD D. Kimple MD

Michael H. BrookePublications

.

Clinical View of Neuromuscular Diseases

2ND Edition

Muscle Biopsy: A Modern Approach