Monday, March 19, 2018

DR. ALOIS ALZHEIMER

     In November 1901 Mrs. August D., age fifty-one, was admitted to the Asylum for the Insane and Epileptic in Frankfurt am Main with advanced dementia. Her husband, a railway clerk, had been aware of her deterioration for about eight months.  The senior
Auguste D, Alzheimer's original case
(Wikipedia)
physician of the Asylum, Dr. Alois Alzheimer, saw this as an unusual case and examined her in detail. In time, Mrs. Auguste D's problem would make the name Alzheimer a household word.
     Alois Alzheimer was born in Marktbreit, Germany, not far from Würzburg. Son of a notary, he received his secondary education at a gymnasium, then studied medicine in Berlin, Würzburg, and
Alois Alzheimer (Wikipedia)
Tübingen. He was an enthusiastic fraternity member and while fencing sustained a large gash over his left face. Since then he only allowed photographs from his right side. He took an interest in histology, having studied under the pioneer histologist Alfred von Kölliker,  and in  neuro-psychiatry, seeking an organic basis for mental illness. He obtained a position at the above-mentioned Asylum in Frankfurt, working under Dr. Emil Sioli.
 Emil Sioli was an early advocate of “open therapy” – substituting relaxing warm baths for restraints and using activities such as crafts and gardening as therapy in a time when almost no helpful medicines were available. Alzheimer was soon joined by
Franz Nissl (Wikipedia)
Franz Nissl, already known for his development of a neuron stain. He and Alzheimer became life-long friends and colleagues. They worked together on many histologic projects (usually at night, after clinical duties), published extensively, and both became recognized authorities in neuropathology.
     In 1892 Wilhelm Erb (of Erb’s paralysis) asked Alzheimer to travel to North Africa to care for a wealthy patient. The patient died en route to Europe but his widow, Cecilie, was taken with Alzheimer and eventually proposed to him. They were married in 1894 and lived happily until her relatively sudden death of unknown cause seven years later. 
     Alzheimer moved briefly to Heidelberg and in 1903 accepted a position under the famous psychiatrist Emil Kraepelin at the Royal Psychiatric Clinic in Munich. Since Kraepelin could not find funds for him Alzheimer, now wealthy, worked for no remuneration (imagine that today!). He built a large laboratory, mainly at his own expense. His genial personality and modern lab attracted excellent scientists such as Hans Creutzfeldt, Alfonse Jakob, and Frederik Lewey (Lewey bodies). Alzheimer published on histologic findings in alcoholism, epilepsy, and mental retardation, and was promoted to “chief physician”, finally earning a modest salary.
      About one third of patients in mental hospitals at the time suffered from general paresis. Most female patients were prostitutes and among males military officers, salesmen, and firefighters predominated. Priests were rarely afflicted. Syphilis was suspected as the cause but the evidence was circumstantial. To teach at the University of Munich Alzheimer had to submit a Habilitation thesis. He had studied 320 cases at autopsy over seven years and selected 26 for the thesis. The work,
Inflammatory thickening of pia mater in paresis
(from Alzheimer, Reference 5, Hathi Trust)
published in 1904, was considered a landmark paper. The very next year the treponema of syphilis was discovered by Fritz Schaudinn and Erich Hoffmann, and the year after that Wassermann announced a diagnostic test for syphilis.

     Back at the Asylum in Frankfurt, Auguste D. was deteriorating, and died in April, 1906. Alzheimer received her brain in Munich and on examination he found plagues, fibrillary tangles, and a paucity of neurons.
     Dr. Alzheimer presented the case of Auguste D. at a lecture in November, 1906, during a meeting of the Southwest German Psychiatrists. Much of the meeting was taken up with criticisms of Freud’s theory of the sexual basis for hysteria. Alzheimer’s paper, despite numerous illustrations, provoked no discussion. It was published the following year (1907) without illustrations in a German journal of psychiatry and “psycho-forensic” medicine. Alzheimer asked a coworker, Dr. Gaetano Perusini, to watch for similar cases at the Clinic, and after three more turned up the four were published in a serial work on the histology and histopathology of the cerebral cortex edited by
Plaque and fibrils seen by Alzheimer
(from Reference 6, Hathi Trust)

 Alzheimer and Franz Nissl. Three years later Emil Kraepelin, in the eighth edition of his popular psychiatry textbook, formally named the disorder “Alzheimer’s disease”. 
     Before long other reports drifted in, and eventually the gap between “pre-senile” dementia and the dementia of old age (“senile dementia”) narrowed. Today the disease is referred to as dementia of the Alzheimer type or Alzheimer’s dementia. The advancing age of the population plus a few cases in celebrities such as Rita Hayworth and Ronald Reagan have assured the disorder a prominent place in the public eye.
     In 1912, at the age of 48, Alzheimer was appointed full professor of psychiatry in Breslau, a prestigious post. Unfortunately his health began to fail shortly after and he died in 1915 of kidney failure.
     Was Alzheimer really the first to characterize the pathology
Oskar Fischer (Wikipedia)
of early dementia? In the journal Brain, 2009, Michel Goedert writes about Oskar Fischer, a Czech investigator at the now defunct German University in Prague, who made almost simultaneous observations. In 1907 – the same year as Alzheimer’s first publication on dementia, he reported on 16 cases of senile dementia (called “presbyophrenia” by him), 12 of whom had neuritic plaques and neurofibrils. Later papers of 1910 and 1912 enlarged the findings. The plaques were, in fact, often called “Fischer’s
Plaque and fibrils by Fischer
(from Reference 7, Hathi Trust)
plaques”, and "presbyophrenic dementia" and "Alzheimer’s disease" were terms used interchangeably for several years.  While Fischer felt that the plaques and fibrils were important in causing dementia, Alzheimer, mentioning Fischer, was less convinced of the causative role of plaques.
     Fischer, being Jewish, was eventually let go from the German University and perished in a concentration camp. After the war communism ruled in Czechoslovakia and the German University was never reopened. It is not surprising that Fischer’s name was forgotten until Dr. Goedert rescued him from archives in Prague.
    

SOURCES
1. Maurer, K and Maurer U, Alzheimer: The Life of a Physician and the Career of a Disease (Eng trans) 2003; Colombia Univ Press, NY.
2. Maurer K, et al, “Auguste D and Alzheimer’s Disease” 1997; Lancet 349:1546-9.
3. Goedert, M and Ghetti, B, “Alois Alzheimer: His Life and Times”. 2007; Brain Pathology 17: 57-62.
4. Goedert, M. “Oskar Fischer and the Study of Dementia” 2009; Brain 132: 1102-11.
5. Alzheimer, A. “Histologische Studien zur Differentialdiagnose der progressiven Paralyse” (Histologic studies in the differential
diagnosis of general paresis). 1904; Histologische und
Histopathologische Arbeiten. F Nissl, ed. 1: 1-297.
6. Alzheimer, A, “Über eigenartige Krankheitsfälle des späteren Alters”1911; Zeitschr für die gesamte Neurologie u Psychiatrie 4: 356-85.
7. Fischer, O. “Miliare Nekrosen mit drusigen Wucherungen der
Neurofibrillen, eine regelmässige Veränderung der Hirnrinde bei
seniler Demenz” (Miliary necroses with plaque-like growths in the
neurofibrils, a regular alteration of the cerebral cortex in senile
dementia) Monatsschrift Psychiatrie und Neurologie 1907; 22:36-    
72.
8. Perusini, G, “Über klinisch und histologisch eigenärtige psychische Erkrankungen des späteren Lebensalters”. 1909; in Histologische und Histopathologische Arbeiten. F Nissl, A Alzheiner, eds. 3: 297-351.


Monday, February 12, 2018

THE HIPPOCRATIC OATH

     Over centuries the Hippocratic Oath, sworn by doctors in ancient Greece, has acquired a patina of reverence as a statement of the moral integrity expected of medical healers. It outlines essential aspects of the doctor-patient relationship. Can physicians today can recite the Oath, and how many wonder about its origins?  (The full oath is reproduced below)
     Hippocrates was born on the island of Cos, in the Aegean Sea, about 469 BC, and died in 399 BC, the same year that Socrates was condemned to drink hemlock poison. Plato, Pericles,
Hippocrates, engraving by Peter Paul
Rubens (Wikipedia)
Sophocles, and Aristophanes were contemporaries and Greek culture flourished. Various writings attest that Hippocrates was a well-known physician and that he accepted payment for instruction. Nothing else is known for certain, except that Aristotle indicated he was “of short stature”. All likenesses seen in art and statuary appear to be artists’ imaginations.
     A collection of writings on medicine, the Hippocratic Corpus, survives today. It was written by an unknown number of unidentified physicians over an uncertain number of years, and appears in the Ionian dialect, the dialect spoken on Cos. Presumably Hippocrates was a contributor to the Corpus, but no one is sure. The writings eschew mention of divine intervention, sin, or superstitious curses as causes of illness. Sickness stems from natural causes, usually an imbalance of
Asklepion on Kos (Wikipedia)
The cult of Asclepius grew as Hippocrates ended his career
and invoked divine power for cures.
various “humors”. Eliciting a history of the illness and observation of the patient are the techniques of diagnosis. These features mark the Corpus as an original document of “modern” medicine.
     The Hippocratic Oath, one of the writings, was first mentioned by Scribonius Largus, a court physician to the Emperor Claudius, in 47 AD. It is divided into two parts. The first deals with the physician’s obligations to his profession and education and is self-explanatory. The second part sets ethical standards for the physician’s relation to patients, and begins with the requirement to not only benefit patients but not to harm them. This recalls the oft-quoted line from another part of the Corpus, “first do no harm”. 
Byzantine version of Hippocratic Oath
in form of a cross (Wikipedia)
     Next are the admonitions to never give a patient a deadly medicine and not to practice abortion. These are curious obligations. Giving a poisonous medicine could mean participation in a murder, which was definitely illegal, or participating in a suicide. Patient-requested suicide was not illegal, and was practiced to some extent, except by Pythagoreans, who opposed all forms of suicide. Determining the cause of death was not easy in those days, however, and the phrase may have been a way of encouraging doctors to avoid trouble in doubtful cases.
     Abortion was widely practiced in ancient Greece, and in one of the Hippocratic writings there is even a technique described: jump up and down, touching the buttocks with the heels at each leap. Infanticide was also practiced and not illegal as long as the infant was killed before it was first fed. It was recommended by Plato as part of his ideal state. Pythagoreans, however, did oppose abortions. The prohibitions against abortion and assisted suicide (if that was the intent) suggest to some that the Oath had Pythagorean origins.
     The oath not to use the knife, not even on sufferers from stone, is also curious. Medicine was not specialized in those days and Greek doctors practiced surgery as well as prescribing potions and diets. Once again a Pythagorean influence is suspected, as that sect considered it unethical to act in a way that would shed blood or possibly kill. Leaving that activity to someone else was permitted, however, as mentioned in the Oath.
     The final duties involve refraining from “wrong-doing” (corruption, theft, etc.), avoiding sexual contact with patients and their families, and maintaining confidentiality in all matters. They are easily understood and apply today. In ancient Greece, though, medicine was just one craft among many. There was no licensing and there were no guilds or laws regulating medical practice and no punishment for malpractice. The Canon, one of the Hippocratic writings, laments, “Although the art of healing is the most noble of all the arts, yet because of the ignorance of both its professors and its rash critics, it has at this time fallen into the least repute of them all.” The Oath, then, may have been a way to enhance the reputation of traditionally educated physicians and distinguish them from presumed quacks and charlatans. 
Galen and Hippocrates in imaginary conversation
12th Century mural, Anagni, Italy (Wikipedia)
Galen admired Hippocrates
     Other parts of the Corpus deal with the appearance and decorum of physicians, important in inspiring confidence and trust. He should be well-dressed and well educated, thoughtful but not stern, he should avoid excessive laughter (considered vulgar), be of high moral character, and be flexible in regard to fees. Manual dexterity in surgical procedures was important, to minimize pain. Skill in prognosis enhanced one’s reputation as much as a cure.
     Parts of the Oath, such as the prohibition on surgery, no longer mesh with today’s medicine. A modern oath was composed in 1964 by Louis Lasagna, Dean of Tufts Medical School, that is now used in many medical colleges. It, and the original, can be seen at: http://www.pbs.org/wgbh/nova/body/hippocratic-oath-today.html
Other versions exist.

The original Oath, taken from Wikipedia, is:
   I swear by Apollo the Healer, by Asclepius, by Hygieia, by Panacea, and by all the gods and goddesses, making them my witnesses, that I will carry out, according to my ability and judgment, this oath and this indenture.
   To hold my teacher in this art equal to my own parents; to make him partner in my livelihood; when he is in need of money to share mine with him; to consider his family as my own brothers, and to teach them this art, if they want to learn it, without fee or indenture; to impart precept, oral instruction, and all other instruction to my own sons, the sons of my teacher, and to indentured pupils who have taken the physician’s oath, but to nobody else.
   I will use treatment to help the sick according to my ability and judgment, but never with a view to injury and wrong-doing. Neither will I administer a poison to anybody when asked to do so, nor will I suggest such a course. Similarly I will not give to a woman a pessary to cause abortion. But I will keep pure and holy both my life and my art.
   I will not use the knife, not even, verily, on sufferers from stone, but I will give place to such as are craftsmen therein.
   Into whatsoever houses I enter, I will enter to help the sick, and I will abstain from all intentional wrong-doing and harm, especially from abusing the bodies of man or woman, bond or free.
   And whatsoever I shall see or hear in the course of my profession, as well as outside my profession in my intercourse with men, if it be what should not be published abroad, I will never divulge, holding such things to be holy secrets.
   Now if I carry out this oath, and break it not, may I gain for ever reputation among all men for my life and for my art; but if I break it and forswear myself, may the opposite befall me.[3] - Translation by James Loeb.

SOURCES:

Temkin, Owsei. Hippocrates in a World of Pagans and Christians. 1991.
Nutton, Vivian. Ancient Medicine. 2013
Hippocratic Writings. Penguin Classics
Nuland, Sherwin. Doctors: The Biography of Medicine. 1988. Chapter 
          One.
The Hippocratic Oath Today. (Nova).
Carrick, Paul. Medical Ethics in Antiquity: Philosophical Perspectives on
      Abortion and Euthanasia. 1985.



Thursday, January 11, 2018

REMBRANDT’S ANATOMY LESSON OF DR. TULP

     Rembrandt Harmenszoon van Rijn was still a young up-and-coming artist in 1632 when he received a commission from the Amsterdam Surgeon’s Guild to paint the Anatomy Lesson of Dr. Tulp. The painting is original in many ways and made his career.
Rembrandt Self Portrait one year before moving to
Amsterdam (NationalMuseum, Stockholm, Wikipedia)
     Rembrandt, at the time recognized but not famous, had moved to Amsterdam from Leiden only a year before the commission. Dr. Nicolaes Tulp, as Praelector of the Surgeons' Guild, was responsible for public dissection. Tulp was born in 1593 into a prosperous Amsterdam family as Claes Pieterszn. He studied medicine at Leiden University, finishing the three-year course at age 21. He practiced medicine and surgery in Amsterdam for the next 40 years and was by all accounts a hard-working, diligent physician with a large practice. He was community oriented, serving as a city councilor, burgomaster, and other offices during his life (while still in practice). His career coincided with Holland’s tulip mania and because he had placed a plaque painted with a tulip outside his
Page from Observationes Medicae showing ileo-
cecal valve (Hathi Trust)
office he was referred to as Dr. Tulp. He wrote a highly regarded book on medicine, Observationes Medicae that went through many editions. In it he gave a detailed description of the ileocecal valve, the most complete thus far published. He also described accurately beri beri, kidney stones, and angina, and gives many case histories – some studied at autopsy, anticipating Morgagni by many years. Tulp and others collaborated on the first Amsterdam pharmacopeia, helping to control indiscriminate use of medications. He lived to age 80.
     A public anatomy dissection had been performed annually in Amsterdam by the Praelector of the Surgical Guild since the latter half of the fifteenth century, using executed criminals. The event was held in January (to minimize decomposition of the body) in the Surgeons' Guild quarters in the weighing house (Waaghaus) of St.
St. Anthony's Gate, part of old city wall, where the Surgeons'
Guild was located (Wikipedia)
Anthony’s Gate. The building had originally been part of the old city wall. Guild members were required to attend and persons of note were invited. Talking and laughing were prohibited.
     Paintings of anatomy lessons were not new either. The Anatomy Lesson of Dr. Willem van der Meer and The Anatomy Lesson of Dr. Sebastion Egbertsz are two prior examples. In both, however, the static atmosphere predominates as the surgeons "look at the camera". The subjects generally posed
The Anatomy Lesson of Dr. Willem van der Meer, by Michiel and Pieter van
Mierevelt, for Surgeons' Guild in Delft, 1617. (Wikimedia Commons)
separately in the artist’s studio before the final version. 
Rembrandt, on the other hand, though he also used studio portraits, conceived a painting with action and narrative.
     The pale cadaver forms an emphatic diagonal. A dissection of  
The Anatomy Lesson of Dr. Tulp, by Rembrandt (Wikimedia Commons)
the forearm and hand, styled after a woodblock print in Vesalius’ atlas, is the center of attention. With his left hand in flexed position Tulp
From Vesalius' De Humani Corporis  Fabrica,
probably a source for arm dissection by Tulp
(Hathi Trust)
demonstrates the function of the flexor tendons that he holds in his right hand. The hand held a special place in anatomy circles, and Vesalius had described it as “the physician’s chief instrument”. It was common, too, at the onset of “anatomy lessons” to mention a dissection as showing the wonder of God’s creation, lending it a metaphysical bent.
     The viewers, all surgeons, are focused intently on the corpse’s hand, Tulp’s hand, or an anatomy book at the corpse’s feet, evoking a drama and intensity not seen in other works of the genre. Much ink has been spilt over the accuracy of the anatomic details of the depicted flexor tendons and muscles, but Rembrandt’s version is at least nearly correct. The dissection is out of order, however. Generally the abdomen was opened first, since in the days before preservatives that area decayed easily.
     Do we know who the cadaver was? Yes. His name was Adriaen Adriaensz, alias Aris Kint. He had been punished multiple times for theft, including floggings and possibly branding. The painting shows no sign of this, however, and neck marks from the hanging are not visible. Most intriguing is that X-ray studies have shown that the right arm was painted originally without a hand, the hand being added later(see Middelkoop et al). Amputating the hand of a thief before hanging was not unheard of at the time and may have been inflicted on Kint. A translation of the court record suggests that it was (Siegal). It is believed, however, that Rembrandt was not present at the actual dissection, so what he knew about the cadaver remains conjecture.
     X-ray studies have shown other items that were painted over. The head at the far left was a later addition, possibly by another artist. The added head destroys the original composition of the subjects set within two overlapping triangles. Other alterations show that the surgeon at the top originally wore a hat, and the paper with the list of names (at Tulp's right) originally showed an anatomic figure. A reconstructed version before alterations can be seen at the Schupbach reference, plate #1.
     A later painting by Rembrandt, The Anatomy Lesson of Dr. Jan Deijman (1656), shows the brain being uncovered following
Anatomy Lesson of Dr. Jan Deijman (1656) (Wikipedia)
dissection of the abdomen, as was the custom. The upper portion of the painting was damaged and is not shown. The position of the cadaver and its foreshortening are almost certainly derived from a painting by Mantegna (see illustration), a painter that Rembrandt admired, though he probably saw only a copy.
Lamentation over the Dead Christ by Andrea Mantegna (Wikimedia Commons)
     The Anatomy Lesson of Dr. Tulp not only established Rembrandt as a major painter, it has also kept historians of medicine and art busy for generations.

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SOURCES
       Middelkoop, N. et al. Rembrandt Under the Scalpel. 1998; Mauritshuis, The Hague.
       Siegal, N. The Anatomy Lesson. 2014; Doubleday.
       Simpson, D. “Nicolaes Tulp and the Golden Age of the Dutch Republic”. ANZ J Surg. 2007, 77: 1095-1101.
       Mellick, S. “Dr Nicolaes Tulp of Amsterdam, 1593-1674: Anatomist and Doctor of Medicine”. ANZ J Surg. 2007; 77: 1102-1109.
        Schupbach, W. “The Paradox of Rembrandt’s Anatomy of Dr. Tulp”. Medical History, Suppl 2, 1982.
       Goldwyn, R M. “Nicolaes Tulp (1593-1674)”. Medical History 1961; 5: 270-76.
       Cook, H. Matters of Exchange: Commerce, Medicine, and Science in the Dutch Golden Age. 2007; Yale U Press.
       Wallace, R. The World of Rembrandt: 1606-1669. 1968; Time-Life Books.
       Ormiston, R. Rembrandt: His Life and Work in 500 Images. 2012; Lorenz Books, London.

      Clark, K. Rembrandt and the Italian Renaissance. 1966; Norton Library.

Wednesday, December 13, 2017

CHRISTMAS, DICKENS, AND MEDICINE

     “God bless us, every one”, the final words spoken by Tiny Tim in Charles Dickens’ A Christmas Carol, still echo after over 150 years. It’s a tale of the power of Christmas to soften up a “squeezing, wrenching, grasping, scraping, clutching, covetous old sinner” like Ebeneezer Scrooge.
     Tiny Tim, Bob Cratchit’s little son, has aroused medical curiosity. He is depicted as small for his age and carried on his father’s shoulder. He “bore a little crutch and had his limbs supported by an iron frame”, and had a “withered little hand”. He often sits by himself and “thinks the strangest things you ever heard”, though not irrational thoughts. 
Tiny Tim on Bob Cratchit's shoulder (Wikipedia)
     What was wrong with Tiny Tim? The story does not say but there was fluctuation in his weakness and eventually the boy recovered. Donald W. Lewis, a pediatric neurologist, after ruling out tuberculosis of the spine and rickets by events in the story, made a case for renal tubular acidosis, favoring type I RTA. This disorder, by producing increased body acidification leads to growth retardation, osteomalacia, bone pain and pathologic fractures. A review of British pediatric texts of Dickens' time revealed that general treatments for almost any illness included fresh air and sunshine, a balanced diet, fish liver oils, and tonics for digestion. In Tim’s case treatment for rickets or TB might have been added, and rickets was managed the same way as scrofula. Such patients were believed to have an excess of acid and received alkaline carbonates such as bicarbonate of soda or other carbonates. This combination, especially the alkalinizing effect of bicarbonates, Dr. Lewis believed, could have led to Tiny Tim’s recovery.
     Medical problems pop up in much of Dickens’ fiction. (Someone even wrote a book about it.) Just to mention a few, we read about the fat, lethargic boy Joe in the Pickwick Papers, believed to have a “Pickwickian syndrome”. Other stories mention ataxic gait, gout, erysipelas, typhoid, dwarfism, opium use, and additional problems.
Charles Dickens, Photo of George Herbert Watkins
(Wikipedia)
     Children populated many of his novels and he took great interest in their welfare. He castigated child labor conditions at a time when children as young as seven worked in mines and other dangerous jobs. In 1850 in London about one half of all deaths were in children and yet there was no children’s hospital. Through the efforts of Dr. Charles West, assisted by Dr. Henry Bence-Jones (of the myeloma protein) and others, the Hospital for Sick Children went up in 1852 on Great Ormond Street, London, in a mansion that previously housed Queen Anne’s physician, Dr. Richard Mead, and his 100,000-volume library. Dickens raised funds for it by public speaking and a reading of A Christmas Carol.  
     The London of Dickens was pretty filthy. Thick smog all but obliterated sunlight much of the time. The gargantuan clouds of smoke pouring out from soft coal fires joined with Thames Valley mist to darken the streets, irritate the eyes, and create havoc for asthmatics. In poorer areas sanitation was almost absent and the Thames itself was a depository of tons of sewage. It took the “great stink” of 1858 to force members of Parliament, based on the Thames and literally holding handkerchiefs over their noses, to pass a bill authorizing a citywide sewage project. Housing was cramped, food often scarce, and water impure. People, including children, often walked miles to work.
     Dickens took an interest in public health. He was an anti-contagionist, attributing diseases of poverty to miasmas arising 
Joseph Southwood-Smith (Wellcome Library)
from unsanitary conditions. He befriended sanitarians such as Dr. Joseph Southwood-Smith, the famous public health advocate, fever expert, and co-believer in miasmas, and Edwin Chadwick, the
lawyer who authored the Report on the Sanitary Condition of the Labouring Population in Great Britain. The Report was a bombshell exposé that helped usher in better sanitation. Dickens supported their work with his own writings.
     Dickens was also close to Thomas Wakley. Wakley was a
Thomas Wakley (Wikipedia)
combative surgeon, reformer, coroner, Member of Parliament, and editor of Lancet at various times in his career. He used the Lancet as a platform for reforming medicine and public health.
     Dickens’ connections to medicine could go on, but that’s enough for now. Good health to all,

                     HAPPY HOLIDAYS
       and     A JOYOUS NEW YEAR.


SOURCES:
Hearn, Michael P., ed. The Annotated  
         Christmas Carol, by Charles Dickens. 2004.
Tomalin, Claire. Charles Dickens: A Life. 2011.
Flanders, Judith. The Victorian City: Everyday Life in Dickens’
         London. 2012.
Corton, Christine. London Fog: The Biography. 2015.
Cambridge, Nicholas. “From Mr. Pickwick to Tiny Tim: Charles
         Dickens and Medicine”. Lecture at Gresham College. available
Lewis, Donald W. “What was wrong with Tiny Tim?” Am J Dis
         Child 1992; 146(12): 1403-7.
Eysell, Joanne. A Medical Companion to Dickens's Fiction. 2005.


    



Thursday, November 16, 2017

SKIN DISEASE IN OLD VIENNA

     Imagine a king roaming around his realm in disguise to better know the problems of his subjects. It’s the stuff of a fairy tale, but it actually happened – in Vienna, where the Habsburg emperor Joseph II ruled from 1780-90. A liberal but impulsive monarch inspired by enlightenment ideas, he established in 1784 the Allgemeines Krankenhaus (General Hospital) as part of a larger program to deal with the sick and poor streaming into the city since the start of industrialization.  The hospital that Joseph created, using his own funds, was a makeover of an old almshouse built around several courtyards, redesigned to house about 2000 patients.
Allgemeines Krankenhaus (Wikipedia)
     In the decade 1836-46, under the influence of the enlightened vice-director of the faculty, Ludwig Baron von Türkheim, the medical school attached to the hospital was substantially reformed. Josef Skoda, the great diagnostician, was given his own chest service, and Carl Rokitansky created a pathology department (he eventually performed some 30,000 autopsies). Other specialty divisions were established, making the “second” Vienna Medical School one of the most advanced in the world, drawing on many thousands of patients seen each year at the hospital.
     Patients with skin diseases, before the 1840s, were generally placed in medical departments. Most skin conditions were thought to result from attempts of poisons or “corruptions” from within the body to escape through the surface. Thus skin lesions were often left alone so as not to impede this process. At the Allgemeines Krankenhaus the skin ward was next to Skoda’s chest ward, and Skoda asked one of his brightest graduating students, Ferdinand von Hebra, to take charge of the ward.
     Ferdinand Ritter von Hebra was born in Brünn, Moravia, in 1816. He attended high school at a monastery in Styria, then
(Wikipedia)
medical school in Vienna, graduating in 1841. Josef Skoda arranged for Hebra to be his assistant. Noticing that Hebra took an interest in the neglected dermatology patients Skoda urged him to study the patients, and supplied him with books.
      Hebra absorbed existing literature intensively - in particular the writings of two English physicians, Robert Willan and Thomas Bateman, and the Frenchmen, Jean Louis Alibert and Laurent-Théodore Biett, all of whom had attempted to bring some order out of the confusion of skin lesions. Alibert’s classification took the form of a tree, with the trunk representing the epidermis and dermis, and the branches various groups of diseases. All these classifications fell short, partly due to inadequate knowledge.
Arbre des Dermatoses by Alibert - illustrating
his classification (Wikipedia)
     Over time Hebra concluded that many skin ailments were of local origin and not expressions of poisons escaping from the inner body. This was most evident in the case of scabies, perhaps the most common disease he encountered (he is said to have seen 60,000 cases by 1860). The causative mite, Sarcoptes scabiei, though suspected for many years, had proved difficult to find. It was not until a student of Alibert, Simon Francois Renucci, in 1834 demonstrated the mite consistently (by looking between vesicles rather than inside them) at the St. Louis Hospital, Paris. His demonstration even survived a 300-franc bet that he was wrong.
     Hebra enlarged upon Renucci’s work by infecting himself several times and charting the course and cure of the disease. He did away with bleedings, laxatives, and most medicines taken internally, treatments reflecting the belief of internal disease as a cause. Instead he applied sulfur, in an ointment made from lard and potassium bicarbonate, and noted the necessity of treating all skin but the head. He acknowledged that “quacks and old women” had more sense than doctors in their use of topical therapies.
Vienna Medical Faculty
Hebra(second from left, rear), Skoda (second from left, front),
Rokitansky (center, front) ( from Google Books)
     Hebra’s work on scabies carried great weight in conveying the idea that the skin should be considered a separate organ subject to its own disorders, though he recognized that some skin problems were indeed manifestations of generalized disease. Soothing baths, oils, and sometimes more caustic applications were used, many of them tested systematically, and most internal remedies discarded.
     Hebra advanced the description and organization of dermatologic lesions. Terms such as erythema multiforme, lichen planus, impetigo herpetiformis, and rhinoscleroma are largely due to him. He began lectures on skin disease in his second year and was an exceptionally good lecturer, combining clarity, abundant
Erythema multiform (from Hebra: Atlas
der Hautkrankheiten,
Google Books)
material, and a ready wit. The lectures attracted students from home and abroad. He could also, in Sherlock Holmes fashion, tell a patient's occupation by examining their hands or feet. In 1845 Hebra’s ward was separated from Skoda’s to become an autonomous dermatology service, a moment considered by some as the “birthdate of dermatology” as a specialty. He published many articles and a few books, including a famous atlas of skin diseases. The latter was a collaboration with the artists Anton Elfinger, who was also a successful political cartoonist, and Carl Heitzmann. 

     Heitzmann had studied medicine under
From Atlas der Descriptiven Anatomie des Menschen
by Carl Heitzmann. (Hathi Trust)
both Hebra and Rokitansky and was even being considered as a successor to Rokitansky as chair of pathology. He never had formal art training but nevertheless published a surgical pathology text for medical students and later a two-volume human anatomy text with 600 illustrations that went through 9 editions. After failing to succeed Rokitansky he emigrated to New York where he practiced dermatology, published more papers, and was a founding member of the American Academy of Dermatology.
     An assistant to Hebra was Moritz Kaposi, whose name is
Moritz Kaposi (Wikipedia)
associated with a formerly rare but now common malignancy seen in HIV patients. Kaposi was gifted and hard-working, and finished volume 2 of Hebra’s Handbook of Dermatology after Hebra’s death. He also married the boss’ daughter.
     Ferdinand von Hebra died in 1880 of “dropsy”. A funeral procession a mile and a half long followed his coffin to its final resting place next to Carl Rokitansky. Von Hebra brought dermatology into the modern world, freeing it from many misconceptions and opening it to modern research methods.

SOURCES:
Crissey, J T. and Parish, L C. The Dermatology and Syphilology of the Nineteenth
         Century. 1981, Praeger Scientific. Especially chapter 4.
Lesky, E. The Vienna Medical School of the 19th Century. 1976, Johns Hopkins Univ
        Press.
Finnerud, C W: “Ferdinand von Hebra and the Vienna School of Dermatology”. AMA
        Arch Derm Syph.  1952, 66(2): 223-32.
Everett, M A: “Jean Louis Alibert: The Father of French Dermatology”. Int J Derm
       1984, 23: 351-6.
Neuburger, M. “Die Lehre von den Hautkrankheiten vor Hebra”. Wien Med Wochen 
          1928, 78: 641.
Friedman, R: “The Story of Scabies II”. Medical Life 1934, 41: 426-76. 
Friedman, R: “The Story of Scabies III”. Medical Life 1935, 42: 218-71.
Hackstock, I: “Carl Heitzmann (1836-1896): physician and illustrator”. Int J Derm
         1938, 37: 235-40.
Shelly, W B, and Crissey, J T: Classics in Clinical Dermatology, with Biographical
        Sketches. 1953, Charles Thomas.
Lesky, E. Meilensteine der Wiener Medizin. 1981, Verlag Wilhelm  
        Modrig.