Medical Eponyms: Intrigue and Infighting Behind the Names

1. Medical Eponyms: Intrigue and Infighting Behind the Names Daniel A. Pollyea, MD University of Chicago March 7, 2006

2. What is an Eponym?

3. From the Greek eponymous • Epi = “upon” • Onyma = “name” • Allow for shorthand method to convey specific information • Particularly popular in the medical field, although many other every-day examples • Rudolf Diesel • Adolphe Sax • Jules Léotard

5. Medical Definition • “Name(s) of one or more individuals who devised or described an anatomic structure, a classification system, a disease, an injury, a principle, a physical sign or an operation technique” BUT… • “One of the following is usually true for eponyms: The eponymized person wasn’t the first describer; the eponymized person didn’t understand the discovery; the eponym’s current meaning is different from that which was described; the eponym has no historical basis.” –Mark M. Ravitch, MD

6. Eponyms in the Medical Literature • 1974 US National Institutes of Health conference held on the naming of diseases • JAMA published an article vowing to drop the possessive “s” (Archer J. Epitomes. JAMA 234:152, 1975) but journal editors over the years have been divided

7. Types of Eponyms • Named after male physicians (2,880 on whonamedit.com) • Named after female physicians (101) • First and last name • Austin Flint • Named after more than one person • Charcot-Marie-Tooth-Hoffman disease • Named after famous people with the disease • Lou Gerhig • Named after fictional characters • Ulysses

8. Case #1: Crohn’s Disease • Subacute chronic enteritis involving the terminal ileum and other parts of the GI tract (“lips to anus”), characterized by patchy, deep ulcers that may cause fistulas, with noncaseating granulomas • Symptoms include fever, diarrhea, cramping, abdominal pain and weight loss • Distinguished from UC because inflammation is transmural, and presence of “skip lesions”

9. Colonic ulceration in Crohn’s

10. Our Story Begins at Mt. Sinai Hospital in NYC, in 1930…

11. Granulomas of Crohn’s were thought to be of an infectious etiology:

12. Leon Ginzburg, MD

14. Case #2: Pickwickian Syndrome • Syndrome of hypoventilation and hypercapnia resulting in daytime somnolence, pulmonary hypertension and occasionally, polycythemia • Cause: Extreme obesity

15. 1837: Charles Dickens wrote The Posthumous Papers of the Pickwick Club

16. Literary Origin (and the modern-day version) “A most violent and startling knocking was heard at the door…The object that presented itself to the eyes of the astonished clerk was a boy- a wonderfully fat boy- habited as a serving lad, standing upright on the mat, with his eyes closed as if in sleep. He had never seen such a fat boy, in or out of a traveling caravan; and this, coupled with the calmness and repose of his appearance, so very different from what was reasonably to have been expected in the inflictor of such a knock, smote him with wonder. ”What’s the matter?” inquired the clerk. The extraordinary boy replied not a word; but he nodded once, and seemed, to the clerk’s imagination, to snore feebly…He breathed heavily, but in all other respects was motionless.” The clerk repeated the question thrice, and receiving no answer, prepared to shut the door, when the boy suddenly opened his eyes, winked several times, sneezed once, and raised his hand as if to repeat the knocking. “What the devil do you knock in that way for?” inquired the clerk, angrily. “Because master said, I wasn’t to leave off knocking till they opened the door, for fear I should go to sleep,” said the boy.

17. 119 Years Later… • “Extreme Obesity Associated with Alveolar Hypoventilation: A Pickwickian Syndrome” by CS Burwell, et al., 1956 • Described a 51-year-old business executive who stood 5’5” and weighed over 260 lbs.

18. Case #3: Brugada Syndrome • Right bundle branch pattern with ST elevation from V1-V3 • Associated with sudden cardiac death in young people without structural heart disease and a negative stress test • Often associated with syncope, VF/VT

19. Evolution of the Syndrome • 1953: Drs. Osher and Woff noted the characteristic EKG pattern, and considered it a normal variant • 1980’s: CDC in Atlanta noted a high incidence of young Thai immigrants dying of sudden cardiac death. In Thailand, this frequent condition was known as Lai Thai (death during sleep). Later this population would be found to have high frequencies of the mutation associated with Brugada.

20. 1986: Pedro Brugada treats a male Polish child with syncope whose sister died of sudden cardiac death. • With brother Josep, collected more cases of this characteristic EKG and its association with sudden cardiac death in young people

21. 1992: Brugada brothers, from Catalonia, Spain, describe the entity • 1998: Specific gene mutations in the Na-ion channels found to be responsible for the characteristic depolarization seen on EKG and the incidences of sudden death with no structural heart disease.

22. Francois Gigot de la Peyronie (1678-1747)

23. Case #4: Peyronie’s Disease • In a treatise on ejaculatory failure in 1743, Peyronie described a patient with “rosary beads” of scar tissue along the dorsum of his penis • Resulted in a curvature in the erect penis

24. Due to a connective tissue disorder of plaque formation in the corpora cavernosa • Affects .3-3% of men 40-60 years old • Can cause penile pain • 50% of cases spontaneously regress • Usually initiated by penile trauma during intercourse, resulting in plaque formation

25. Peyronie gets the eponym, but… • First described in 1587 by Giulio Casare Aranzi, a Bolognese anatomist • Thought to be punishment for incest, since the Byzantinian Emperor Heracles, who took his niece for his wife, was said to have developed a penile deformity that “caused him to urinate into his face”

26. 1942 review article speculated “when a youth is addicted regularly to sessions of prolonged ungratified sexual desires, his prostate, being in a constant state of engorgement, will develop prostatic hypertrophy” and result in Peyronie’s disease • Also cited “frigid” female partners, giving as evidence one man whose condition resolved after his wife became “more amorous.”

27. Treatment • “If treatment of the plaque with iodides is unsuccessful…or if the condition progresses…then the whole penis must promptly be amputated” • William J. Walsham, MD • London surgeon, 1903 • Vitamin E, various surgical techniques, straightening braces…

29. Case #5: Barrett’s Oesophagus • Change of the esophageal lining from squamous to columnar (which is found in the stomach) • Thought to be a complication of severe GERD • Risk factor for esophageal cancer (metaplasia)

31. Norman Barrett, an Australian surgeon, was the first to recognize these changes • However, he believed they occurred because these patients had a congenitally short esophagus, and the stomach had intruded into the chest to compensate • Dr. Jean-Louis Lortat-Jacob, a French surgeon, discovered the abnormalities around the same time as Barrett, and he correctly hypothesized that it was an acquired lesion • However, his paper was written in French. Barrett wrote his in English. The rest is history.

32. Eponyms Etiquette • It is poor form to name something after yourself • Follow the lead of Fred Askin: Make a long name • Described a “Small round blue cell tumor of the thoraco-pulmonary region.” • The next paper referred to “small round blue cell tumor of the thoraco-pulmonary region (Askin’s disease) • Now simply known as Askin’s disease

33. Tashima’s Syndrome • CK Tashima (JAMA 194:208;1965) identified Tashima’s Syndrome • “A physician’s search for a new sign, disease or syndrome to attach his name to.”