This is part of our Campus Spotlight on the University of North Carolina at Chapel Hill.
Familiar and well-recognized diseases may have interesting but little-known historical associations. The Health Sciences Library at the University of North Carolina at Chapel Hill possesses international medical theses previously owned by the New York Academy of Medicine (NYAM). The change of venue for these articles allowed accessibility in view of the NYAM’s lack of storage capability. This collection includes post-1801 theses written by medical students in various countries. I reviewed those written in Italian from the cities of Genoa, Padova, Pavia and Pisa. I was intrigued with the one entitled “Rilievi statistici clinici ed epidemiologici sulla malattia di Heine Medin in provincia di Padova” (Statistical, Clinical and Epidemiological Surveys on the Heine Medin disease in the Province of Padua) written by Girolamo Gottardi in 1931. The eponym, Heine-Medin disease, was unknown to me and thus I opted to examine the historical documentation of the illness and the origins of the eponym. This inquiry provided an opportunity to reflect on medical eponyms in general. The study of eponyms and nomenclature imparts insights into the evolution of specific clinical states.
Heine-Medin disease is an eponym for poliomyelitis, a well-known infectious multi-phasic viral disease. Its varied nomenclature, including the eponym that occurred prior to the general acceptance of the name “poliomyelitis,” resulted from fragmented research, a multitude of clinical manifestations, and geographically dispersed and relatively low volume epidemics. These factors, while not unique to poliomyelitis, were very prominent with this disease. While Heine and Medin were two men involved in the early elucidation of the disease, they were not the first to describe it, nor were they involved in finding the cure. Recent institutionalized bias against eponyms has discouraged their application but cogent rationalizations for their use have been articulated by prominent scholars such as McKissick, considered the father of medical genetics. A governing body for medical nomenclature does not exist, but editors of prominent journals have strongly voiced their opinions. Judith A. Whitworth, in September 2007, noted that as of that date there were 7,899 eponyms listed on the website whonamedit.com.
Heine-Medin disease, or poliomyelitis, is an infectious illness that is characterized by non-paralytic and paralytic stages, although it commonly aborts after the former without residual signs. Prior to 1909, when Popper and Landsteiner identified the virus, the clinically non-specific illness could be distinguished only by a paralysis if it occurred. Sporadic cases were identified but it wasn’t until the initiation of epidemics in the 1880s that there was a heightened medical, scientific and sociopolitical interest in the infection. Until that time, there was relative disinterest in poliomyelitis compared to other contagious diseases such as the plague, typhoid fever, malaria, and yellow fever, which were well-documented in fictional and non-fictional literature.
It is ironic that a disease that caused so much public health concern during the first half of the twentieth century should have scant historical documentation. Heine-Medin disease—or poliomyelitis—most likely has existed in an endemic or sporadic form since time in memoriam. Since “poliomyelitis pre-dates recorded history,”[1] its historical presence was inferred by the paralysis noted in mummies and by artistic and literary depictions. These were often based on retrospective interpretations of deformity and descriptions such as “lameness” and “lame.” Examples of these are few and in retrospect not always correct diagnostic of poliomyelitis. They include a shortened hypertrophied left leg in the skeleton of an Egyptian mummy from about 3700 BCE;[2] a shortened atrophied right leg with drop foot in a portrait of a young prince of the eighteenth dynasty of Egyptian rulers (between 1580 and 1350 BCE); talipes equinus (a foot deformity usually congenital) in the mummy of the Pharaoh Siptah of the nineteenth dynasty (died in 1209 BCE); and infantile paralysis that afflicted the Biblical character Mephibosheth (eleventh-tenth century B.C.E.), the son of Jonathan and grandson of Saul.
Centuries of art and literature reveal deformed limbs, such as clubfoot and ambulation with crutches, some of which have been speculated to have been caused by poliomyelitis. Examples include those of clubfoot by Hippocrates—a term he used to describe deformities that included flaccidity of the lower extremity as well as the ankle and foot—and those of Galen who remarked that there were two clubfoot entities, one congenital and the other occurring in infancy. [3] It is possible that a subgroup of these deformities resulted from poliomyelitis.
Other depictions include “Masaccio’s cripple” seen in the Branacci Chapel in Florence in the fresco “Saint Peter Healing the Sick with His Shadow” by Masaccio (1401-1428). However, “apart from suspected sporadic cases (clubbed feet in Egypt in 1580 BCE; Hippocrates and Galen descriptions; and maimed persons depicted by Bosch, Brueghel and others), there were no descriptions of poliomyelitis until its epidemic recognition in 1880.”[4] A more definite description of poliomyelitis prior to the epidemics was that of Sir Walter Scott, the nineteenth century writer. His clinical course as described by Robert Bartholow (1902) is consistent with poliomyelitis. Scott was afflicted with a fever at the age of eighteen months and a residual right leg disability in 1773. He was sent to live with his grandfather at Sandyknowe Farm (thirty miles southeast of Edinburgh) in the hope that fresh air and exercise would be therapeutic. Ironically, Lord George Gordon Byron (1788-1824), one of Britain’s greatest poets, had a limp of which he was very self- conscious. It was described as clubfoot but unlike Scott the etiology is less definitively a diagnostic of poliomyelitis.
Historical Clinical Symptomatology and Nomenclature
The first to describe a clinical disease entity consistent with Heine-Medin disease or poliomyelitis was Michael Underwood in 1789. He labeled the disease as “debility of the lower extremities.” Underwood was first to attach a scientifically appropriate label to a condition that hitherto was referred to as “lameness.” While stating that “the disorder intended here is not noticed by any medical writer within the compass of my reading, or is not so described as to ascertain the disease,”[5] he correctly observed that it attacked children previously affected by fever and that the paralysis (debility) often involved the lower extremities. The behavior of the disease over time that he observed was consistent with the classic signs and symptoms of the disease. His proposed treatment, although therapeutically futile, was appropriate to the state of medicine at the time. In his 1813 book Instituzione Chirurgicale, Giovanni Battista Monteggia, a physician from Lombardy, published “Paralisi ed Atrofia” (Paralysis and Atrophy)—a chapter in which he mentioned a syndrome very similar to the one Underwood had described. Apparently, his “compass” was limited because he stated that “non ancora trovata ne’libri” (I have not yet found in the books).[6]
Subsequently, several observers described the endemic or sporadic form and various names applied to it. Although additional characteristics of the disease would be considered, the core symptoms of fever in infancy and lower extremity (predominantly) paralysis described both by Underwood and Monteggia remained its bedrock. John R. Paul outlined the proposed names of the disease, their originators and dates: [7]
- Debility of the lower extremities, Underwood, 1789
- Lähmungszustände der unteren Extremitäten, Heine, 1840
- Morning paralysis, West, 1843
- Paralysie essentielle chez les enfants, Rilliet, 1851
- Paralysie atrophique graisseuse de l’enfance, Duchenne de Boulogne,1855
- Spinale Kinderlähmung, v Heine, 1860
- Tephromyelitis anterior acuta parenchymatose, Charcot, 1872
- Poliomyelitis anterior acuta, Kussmaul (quoted by Frey), 1874
- Heine-Medin disease, Wickman, 1907
- Infantile paralysis
- Poliomyelitis
- Polio
Monteggia was not mentioned since he never gave the disease a specific name. The above list highlights the multiple clinicopathological terminologies considered by the researchers or clinicians to be the most important feature prior to the proposed eponym of Heine-Medin disease. The last three in the list are those most widely accepted today. Diverse nationalities are represented including English, German, French and Swedish.
The Eponym: Heine-Medin Disease
The eponym Heine-Medin disease used by Girolamo Gottardi in his 1931 thesis epitomizes both characteristic and controversial issues of the use of eponyms in medical nomenclature. This eponym was not universally recognized and its use remained limited geographically and temporally. Today it is seen in the medical literature only as a historical reference.
Jacob von Heine was a German physician who specialized in orthopedics and rehabilitation and in that role was exposed to patients with flaccid paralysis. In 1840, without acknowledging previous reports such as those of Underwood, Monteggia and others, he published a monograph of twenty-nine patients. He described the typical symptoms, suggested treatment, and speculated that the anterior horns of the spinal cord were pathologically targeted. He emphasized the presence of a flaccid rather than a spastic paralysis and the absence of sensory symptoms. He was the first to report a large number of patients, described cogent clinical observations and proposed effective rehabilitative therapy. In an 1860 monograph he reported an additional 120 patients and called the disease “Spinale Kinderlähmung” (spinal infantile paralysis), an appropriate name that was to be in continuous use for many years. The term was accepted because of von Heine’s reputation and the lingering doubt that a disease with spinal symptoms could be the same as one with cerebral symptoms.
Between 1890 and 1905, clinical medicine made great strides both in Europe and in the United States. Data began to suggest that there was an association between spinal paralysis and certain forms of encephalitis. The same pathological process in some patients affected the spinal cord and in others the brain. Small epidemics were reported and evaluated. Sweden was at the forefront of advances in the disease, led by pediatrician Karl Oscar Medin who identified its various clinical features in “a more concise yet comprehensive way than ever been accomplished.”[8] His work received international attention, giving newfound exposure to this disease. Furthermore, “besides clarifying the picture of poliomyelitis, he succeeded in gaining for Sweden the unenviable reputation as the country where the worst epidemics occurred. Little did the medical profession realize that the rest of the world would soon catch up.”[9] Medin received many accolades and assured himself an honored place in the history of infantile paralysis. It is in 1907 that Ivar Wickman, one of his devoted students, suggested the eponym “Heine-Medin disease,” in honor of the two hitherto best-known and accomplished specialists of the disease.
Eponyms have an intriguing and interesting history in medicine. Wickman’s motivation for including Medin’s name in an eponym may have been, in addition to his extraordinary admiration for his mentor, ingratiatory. He had wished to succeed him as professor of children’s diseases at the Stockholm Faculty of Medicine. However, denied the appointment, he committed suicide at the age of forty-two. Paul H. Römer suggested a less self-serving motivation. Wickman tried to resolve the nomenclature quandary prompted by the new developments and observations. These included the affliction among adults and the dissemination of non-spinal pathology in the cerebrum, brainstem, meninges and white matter. He also had to account for the absence of paralysis in most cases. An eponym allowed for the circumvention of this dilemma caused by a disease that was “so kaleidoscopic in its symptomatology, so variable from the point of view of pathological evidence, and the etiology of which was so entirely unknown at that time.”[10] Furthermore, there were additional practical advantages. Specifically, Römer averred that adoption of the Heine-Medin appellation would acknowledge Wickman’s discovery that paralysis is an outstanding feature of the disease but not the most common. Römer opted to adopt the name because it would include all clinical manifestations, paralytic and non-paralytic, although he called the etiological agent “poliomyelitis virus.”
Victor A. McKusick, the father of medical genetics, in a brief commentary in 1998, summarized his thoughts about medical eponyms. For him, eponyms present several advantages including avoiding the inclusion of all manifestations of the disease in one name, steering clear of a term based on the presumed basic defect that may prove to be wrong, eluding “cumbersome, tongue-twister designations”[11] and terminology that may be offensive to patients or their families. A downside is the exclusion of other pioneers. In the case of Heine-Medin disease, these include Jean-Martin Charcot who identified the anatomical pathology (the ganglion cells), Ivar Wickman who contributed valuable epidemiology information, Karl Landsteiner who identified the poliovirus, and Adolph Strümpell who was the first to hypothesize that the disease was caused by an exogenous living organism. In fact, Strümpell’s contributions were significant. Working in Vienna in 1884, he gave the first account of a cerebral type of polio that was confirmed in 1885 by Pierre Marie, a pupil of Charcot. It became known as Strümpell-Marie form of poliomyelitis, or Strümpell’s disease II. These eponyms also proved to be ephemeral.
Unlike many medical eponyms that have become ingrained in the medical lexicon, Heine-Medin disease was never wholeheartedly accepted. The ambivalence was highlighted by the increasingly frequent adoption of the alternatives: poliomyelitis and polio. “For a brief period, it appeared that the name, poliomyelitis, would be superseded by Heine-Medin disease, which was introduced by the Swedish doctor, Ivar Wickman […]; Medin was Wickman’s teacher. This term was popular for a time in Europe and America. But only the name poliomyelitis has stood the test of time, although even this is often shortened to polio in common usage.”[12] One explanation is that its peak implementation during the first three decades of the 1900s paralleled Europe’s short prominence in poliomyelitis research. After the third decennium of the century, the research, the occurrence of large epidemics, the strong political support (encouraged by the stature of the afflicted President Roosevelt), and the funds shifted dramatically to the United States where neither Heine nor Medin was well known. Paradoxically, in the United States, eponyms were attached to the three viral types: Brünhilde (type I), Lansing (type II) and Leon (type III). All three were involved in research in the United States and all within a three-year period from 1937 to 1939. Even these eponyms were eventually dropped in preference to numerical identification (I, II, III).
Eponyms have been discredited for political reasons, as highlighted by the current debate about those diseases named in honor of persons who had a supportive Nazi affiliation. Examples include Julius Hallervorden (Hallervorden-Spatz syndrome—degeneration of the globus pallidus, red nucleus and substantia nigra), Julius Reiter (Reiter syndrome—reactive arthritis) and Hans Asperger (Asperger syndrome—milder form of the autism spectrum).[13] [14] However, none of the three eponyms associated with poliomyelitis were politically tainted. All had died before Hitler assumed power, Heine in 1879, Strümpell in 1925, and Medin in 1928.
As of September 2007 there were 7899 eponyms listed on the website whonamedit.com and those are “heavily influenced by local geography and culture.”[15] Eponyms may elapse from regular use over time. Heine-Medin honored two prominent physicians who were active in the early discovery and understanding of the disease even though they were not the ones who provided the crucial information that eventually resulted in the vaccine and near-eradication of the disease. In addition, neither was the first to describe the disease. In fact, in 1935, Wilburt C. Davison (Duke University Department of Pediatrics) stated that “Heine-Medin’s disease[…] should be discarded (in addition to the term acute wasting paralysis)[…] since[…] Underwood described it before Heine was born.”[16]
A review of seminal papers presented or published after Wickman’s recommendation that the disease be called Heine-Medin disease revealed an inconsistent use of the eponym and a confirmation of the decline in its usage over time. Writing on infantile paralysis in 1908, M. Allen Starr (Columbia University)[17] completely ignored the eponym although he used other eponyms, including the rarely used “poliomyelitis of Wernicke” and the “encephalitis of Strümpell.” A few years later Wickman himself not surprisingly entitled an article “On the Epidemiology of Heine-Medin’s Disease” (1913)[18] but referred to the disease in the text as poliomyelitis. Discussing “epidemic poliomyelitis” in a 1981 article, Thomas Gucker[19] mentioned Heine and Medin separately but did not use the eponym beyond the first sentence where it was placed in parentheses. Most recently, references to Heine-Medin disease as an historical alternative to poliomyelitis appeared in a brief note in the Journal of Neurology, Neurosurgery and Psychiatry [20] and an article in the World Journal of Virology.[21]
One of the potential drawbacks of eponyms is that they can be over-inclusive and lose scientific validity, as highlighted by F. Graham Crookshank’s expansion of variants constitutive of the Heine-Medin disease. Dr. Crookshank was a controversial—by virtue of his racial eugenic views—British epidemiologist and physician at the Hampstead General Hospital in London. He is not considered an important contributor to the field of poliomyelitis, although he wrote several articles and a monograph on epidemiology between 1919 and 1931 in which he expanded his conception of the Heine-Medin syndrome. While he resurrected maladies from the distant past that are of historic interest, he propagated misconceptions about and erroneous linkages to Heine-Medin disease. He lectured that it had been “with us for centuries”[22] in various guises. While this may be arguably accurate, his lists of “similar” diseases included among others Kriebelkrankheit (ergot poisoning); mal mazzuco (a form of influenza); raphania ( attributed to radish seeds in the threshed grain); a non-specified 1916 epidemic in America; the “Mysterious Disease” reported in Australia in 1917; a then recent epidemic in Great Britain that was “erroneously confused with Botulism;” several early English and continental epidemics during the fourteenth, fifteenth and sixteenth centuries symptomatically characterized by severe catarrhal “sweating” associated with neurologic symptoms (attributed at the time to certain specific foods or putrefaction of food); an 1842 illness with severe neurological symptomology in Italy; “sweating sickness” in 1529; “epidemical feavour” in 1661 described by Thomas Willis; “spinal typhoid” in Great Britain; and von Economo encephalitis. It is implausible that all these diseases were Heine-Medin disease or even a variant of it.
Buried in the annals of medical history, the story of Dr. Crookshank is a fascinating one. He was the only prominent scientist to have attempted to expand the concept of the Heine-Medin disease. Many of the diseases to which he attributed a similar etiology do in fact have non-infectious causes such as ergotism. Crookshank should be faulted for his overzealous expansion of the Heine-Medin disease but there may have been a kernel of truth in his hypotheses. Poliomyelitis in one form or another may have been the unidentified cause of some epidemics prior to the late 1800s.
Heine-Medin disease, known today primarily as poliomyelitis or polio, has a relatively short history as an eponymous entity. The origin of the eponym shares features with more familiar ones but it also encompasses unique ones. It was commonly used from its introduction in 1907 to the 1930s, particularly in Europe, then disappeared from the worldwide medical lexicon without making a significant mark. Recent references to it are few; in the 2000 book Neurological Eponyms[23] there is no mention of Heine-Medin disease. Eponyms are often overlooked in the study of disease states. Although often controversial and ephemeral, as with Heine-Medin disease, the historical context of eponyms is replete with scientific and curious facets of the evolution of disease. They are currently discouraged, but are not on the verge of vanishing from the medical lexicon.
Ugo Goetzl is a retired neurologist who is board-certified in both neurology and psychiatry. He is currently enrolled in the PhD graduate program in Italian Studies in the Department of Romance Studies at the University of North Carolina at Chapel Hill.
References
Bartholow, Roberts. “The Medical History of Sir Walter Scott.” The Southern California Practitioner XVII (1902):313-320.
Baicus, Anda. “History of Polio Vaccination.” World Journal of Virology 12 (2012):108-114.
Crookshank, F.G. Epidemiological Essays. MacMillan: New York, 1931.
——. “First Principles: and Epidemiology.” Proceedings of the Royal Society of Medicine 13 (1920): 159-184.
——. “The History of Epidemic Encephalomyelitis in Relation to Influenza, Parts I & II.” The Medical Press 108 (1919): 405-407, 423-426.
Davison, Wilbert C. “Poliomyelitis – A Résumé.” American Journal of Diseases of Children 52 (1936): 1158-1178.
Espinel, Carlos Hugo. “Massaccio’s Cripple: a Neurological Syndrome. Its Art, Medicine, and Values.” The Lancet. 346 (1995): 1684-1686.
Gottardi, Girolamo. “Rilievi statistici clinici ed epidemiologici sulla malattia di Heine Medin in provincia di Padova.” Università di Padova: Tesi delle Scuole di Perzionamento della Facoltà Medico-Chirurgica. Padova: CEDAM, 1931.
Gucker, Thomas, 3rd. “The Development of Our Knowledge of Anterior Poliomyelitis: An Account of The Advances Preceding and Including the Work of Medin.” Orthopedics 4 (1981): 773-781.
Hernigou, Philippe, et al. “History of Clubfoot Treatment, Part I: From Manipulation in Antiquity to Splint and Plaster in Renaissance Before Tenotomy.” International Orthopaedics 41 (2017): 1694-1704.
Koehler, Peter, et al. Neurological Eponyms. Oxford UP: Oxford, 2000.
McKusick, Victor A. “On the Naming of Clinical Disorders, with Particular Reference to Eponyms.” Medicine 77 (1998): 1-2.
Mitchell, John K. “Study of a Mummy Affected with Anterior Poliomyelitis.” Transactions of the Association of American Physicians 15 (1900): 134-136.
Monteggia, G.B. “Paralisi ed Atrofia.” Instituzione Chirurgicale. 2nd Ed. vol 1. Milano: Guiseppe Maspero, 1813.
Paul, John R. A History of Poliomyelitis. New Haven: Yale UP, 1971.
——. “Historical and Geographical Aspects of the Epidemiology of Poliomyelitis.” Yale Journal of Biology and Medicine 27 (1954): 101-113.
Pearce, J.M.S. “Poliomyelitis (Heine-Medin disease).” J Neurol Neurosurg Psychiatry 76 (2005): 128.
Römer, Paul H. Epidemic Infantile Paralysis (Heine-Medin Disease), trans. H. Ridley Prentice. New York: Wood, 1913.
Smallman-Raynor, M.R., Cliff, A.D. et al. Poliomyelitis: Emergence to Eradication. Oxford: Oxford UP, 2006.
Starr, M. Allen. “Epidemic Infantile Paralysis.” Journal of the American Medical Association LI (1908): 112-120.
Strous, Rael D., Edelman, Morris C. “Eponyms and the Nazi Era: Time to Remember and Time for Change.” The Israel Medical Association Journal 9 (2007): 207-214.
“The Story of a New Disease: The Chadwick Lectures – Abstract of Lecture, I, II, III.” The Medical Press 106 (1918): 291, 310, 329.
Underwood, Michael. “Debility of the Lower Extremities.” A Treatise on the Diseases of Children with General Directions for the Management of Infants from Birth. London: J. Matthews, 1789.
Wickman, Ivar, Maloney, J.W.J. “On the Epidemiology of Heine-Medin’s Disease.” Reviews of Infectious Diseases 2 (1980): 319-327.
Whitworth, Judith A. “Should eponyms be abandoned? No.” BMJ 335 (2007): 425.
Yeginsu, Ceylan. “Hans Asperger Aided Nazi Child Euthanasia, Study Says.” The New York Times, 19 April 2018. https://nyti.ms/2He2bi3.
[1] J.M.S. Pearce, Poliomyelitis (Heine-Medin disease), (J Neurol Neurosurg Psychiatry 76, 2005), 128.
[2] John K. Mitchell, Study of a Mummy Affected with Anterior Poliomyelitis, (Transactions of the American Physicians, 15, 1900).
[3] Philippe Hernigou, History of clubfoot treatment, part I: From manipulation in antiquity to splint and plaster in Renaissance before tenotomy, (International Orthopaedics, 41, 2017).
[4] Carlos Hugo Espinel, Massaccio’s Cripple; A Neurological Syndrome. Its Art, Medicine, and Values, (The Lancet, 346, 1995), 1686.
[5] Michael Underwood, A Treatise on the Diseases of Children with General Directions for the Management of Infants from Birth, (London, J. Matthews, 1789), D3.
[6] Giovanni Monteggia, Instituzione Chirurgicale, 2nd Ed. vol 1,(Milano, Maspero, 1813), 237.
[7] John Paul, A History of Poliomyelitis, (New Haven, Yale UP, 1971), 5.
[8] Ibid, 74.
[9] Ibid, 75.
[10] Paul Römer, Epidemic Infantile Paralysis (Heine-Medin Disease), (New York, Wood, 1913, 2.
[11] Victor McKusick, On the Naming of Clinical Disorders, with Particular Reference to Eponyms, Medicine 77, 1998, 1.
[12] M.R. Smallman-Raynor, et al, Poliomyelitis: Emergence to Eradication, (Oxford, Oxford UP, 2006), 6.
[13] Rael D. Strous, et al, Eponyms and the Nazi Era: Time to Remember and Time for Change, (The Israel Medical Association Journal 9, 2007).
[14] Ceylan Yeginsu, Hans Asperger Aided Nazi Child Euthansia, Study Says, (The New York Times, 2018).
[15] Judith Whitworth, Should eponyms be abandoned? No., (BMJ 335, 2007), 425.
[16] Wilbert Davison, Poliomyelitis – A Resume (American Journal of Diseases of Children, 52, 1936), 1158.
[17] Allen M. Starr, Epidemic Infantile Paralysis (Journal of the American Medical Association, 1908)
[18] Reprinted in the section “Classics in Infectious Diseases” in Reviews of Infectious Diseases 2,1980.
[19] Thomas Gucker, The Development of Our Knowledge of Anterior Poliomyelitis: An Account of the Advances Preceding and Including the Work of Medin (Orthopedics 4, 1981)
[20] JMS Pearce, Poliomyelitis (Heine-Medin Disease) (Journal of Neurology, Neurosurgery and Psychiatry 76, 2005).
[21] Anda Baicus, History of Polio Vaccination (World Journal of Virology 12, 2012).
[22] F. Graham Crookshank, Epidemiological Essays, (MacMillan, New York, 1931), 291.
[23] Peter J. Koehler, et al, Neurological Eponyms (Oxford UP, Oxford, 2000).
Photo: UNITED STATES OF AMERICA – CIRCA 1999: A stamp printed in USA dedicated to Polio Vaccine Developed | Shutterstock
Published on July 10, 2019
