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LETTER

A Further Fond Farewell to Shy and Drager

right arrow W. King Engel, MD

15 February 1997 | Volume 126 Issue 4 | Page 334


TO THE EDITOR:

The recent editorial, "Farewell to the ‘Shy-Drager Syndrome’" [1], prompts this brief illumination of the men involved in the discovery of the syndrome. I was in the group at the time of the syndromogenesis and knew the three participants-G. Milton Shy, Glenn A. Drager, and their patient-very well.

Milton Shy was a brilliant clinical investigator with a remarkable capacity to sense a new syndrome. He had recently described "central core disease" (I encouraged him to publish that name, which he had been using informally). Although the day's two muscle-pathology pundits believed the lesions were artifacts, Dr. Shy published his description of central core disease. That disease is now known to be associated with malignant hyperthermia, and the defective gene product is known to be the skeletalmuscle ryanodine receptor (calcium-release channel of the sarcoplasmic reticulum). Thus, central core disease could be "Shy's disease."

Glenn Drager was an excellent neuroanatomist and neuropathologist who studied the index patient's central nervous system in a detailed manner similar to that of the old Germanic school of neuropathology. I shared the "office" space (seven desks in a small windowless room) with that kind and gentle man.

Milton Shy was excited by neuromuscular diseases because one could apply state-of-the-art scientific techniques to the easily biopsied affected human muscle tissue. Today, his approach is more vibrant than ever in laboratories around the world. Brain and spinal cord diseases, such as the Shy-Drager orthostatic hypotension syndrome, were (and are) much more challenging to analyze.

Shy was a stimulating, probing, provocative, Socratic teacher who also had a deep personal concern for the members of his group. The example of his contagious enthusiasm for analyzing neuromuscular diseases launched many of our academic careers, and subsequently those of our trainees, the Shy lineage.

We all owe major respect to Dr. Shy (I could never bring myself to call him "Milton"), and with sadness I note the passing of the Shy-Drager eponym. And yet, in some Elysian Field, Milton Shy is nodding sagely, drawing softly on his pipe, for he too, eschewed eponyms whenever possible.


Author and Article Information
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University of Southern California School of Medicine, Los Angeles, CA 90017-1912.


REFERENCE
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1. Schatz IJ. Farewell to the "Shy-Drager" syndrome [Editorial]. Ann Intern Med. 1996; 124:74-5.

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