Wednesday, January 01, 2003

'Pestilent Malignant Beams'

Pox: Genius, Madness, and the Mystery of Syphilis

By: Robert J. Joynt M.D., Ph.D.

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I saw my last case of general paresis, the personality and behavioral changes and slow mental deterioration that mark the third and final stage of syphilis, about 25 years ago. As a visiting professor of neurology, I was challenged to diagnose the illness of a neatly dressed, polite man who affected a French name and recounted to me his career as a movie director. He was at ease as he described, in lurid detail and with an occasional wink, his relationships with famous actors and actresses of the 1920s and 1930s. Frankly, I had already made a tentative diagnosis of paresis, based on what I had been told earlier about the patient’s history, which included working on a railroad. I had recalled an admonition of one of my old teachers that anyone who worked on the railroad should have his Wassermann reaction (the standard blood test for syphilis) checked—a sweeping, often inaccurate generalization, of course. Fortunately, patients like the man I interviewed that day, who shared the fate of many literary and musical geniuses and other notables of that and earlier eras, are rare today.

So why write a book about syphilis? The disease is now detectable and treatable in its early stages, before it affects the brain, and its incidence—with an occasional resurgence—has sharply dropped. There are several answers. In medical terms, syphilis remains a major health threat around the world; and there are 6,000 new cases a year in the United States. In scientific and historical terms, many questions about the disease have been answered, making this a story of the conquest of a once-feared, stigmatized, epidemic disorder that, like AIDS in our day, is sexually transmitted and, in its final stages, devastates the brain. Still, as Pox: Genius, Madness, and the Mystery of Syphilis makes clear, important questions remain about the disease.

Deborah Hayden is not a scientist but a talented amateur researcher with an apparent passion for her topic (she has lectured on “Syphilis and Creativity”). Her fund of information on the subject is remarkable. The history of syphilis involves medical science and the gamut of public health issues, but also speculation, secrecy, bigotry, quackery, genius, depravity, cruelty, and heartbreak. Hayden touches on them all, moving from hard facts of science and medicine to historical detective work about illustrious people who may or may not have had the disease. Pox is distinguished by careful, pertinent research about syphilis and the medical history of those possibly infected. For Hayden, a central question is how the disease affected the creativity of these noteworthy people.

The book comprises four sections: facts and speculations about the disease; nineteenth-century notables possibly infected; twentieth-century victims; and short vignettes of other possible sufferers. The organization of Pox would have been improved by putting the chapters of clinical description at the beginning, making more understandable the chapters on the origin and spread of the disease. The visuals are mostly portraits or photos of the afflicted. There is a long list of references.

A WORLDWIDE EPIDEMIC ARISES

Syphilis has been given many names—the French sickness, Italian pox, Neapolitan pox, Chinese ulcer, American pox, Spanish itch, and others—because no country wanted to claim it. Much debated is whether it originated in the New World and was introduced to Europe by sailors returning from Christopher Columbus’s voyage. Hayden presents the evidence on both sides.

Syphilis was identified as a distinct disease entity in the last years of the fifteenth century. As Hayden explains, contemporary descriptions of the initial genital lesion with subsequent skin rashes, fever, and various aches and pains are definitive. The first voyage of Columbus in 1492 arrived back in Spain the next year; some of his men were sick and sought medical attention. Subsequent voyages brought back more sufferers, and it was noted by Antonio de Torres in 1494 that “the men who mixed with the shameless and unchaste Indian women and behaved lewdly with them were struck down by this deplorable sickness.” From sailors and soldiers returning from the West Indies, the disease spread to Spanish prostitutes. The initial genital lesion is often not obvious in women, so the disease is detected in them at a later stage. About that time, Charles VIII of France was recruiting an international army, including Spaniards, for foreign invasions. With this army, the disease spread across Europe after the French invaded Naples in 1495. Answering the question of syphilis’s origin hinges on whether it existed in the Old World prior to that time— a vexed issue because the disease could have been confused with leprosy or yaws.

Syphilis causes characteristic changes in a victim’s bones, affording medical historians clues to the puzzle of its origins.

Syphilis causes characteristic changes in a victim’s bones, affording medical historians clues to the puzzle of its origin. A few years ago in England a cache of bones was found, some thought to have syphilitic lesions. These bones were carbon-dated back to the 1300s, prior to Columbus’s voyages. Based on this finding, it was announced with some brio that Columbus was not responsible for importing the disease to Europe. This claim has been disputed by Bruce Rothschild, a paleoanthropologist, who argues that the lesions were the result of yaws, not syphilis. He buttresses his case for a New World origin by identifying syphilitic lesions in pre-Columbian bones found in Hispaniola, the first major landfall of Columbus. Hayden argues, plausibly enough, that Columbus may have contracted syphilis during his second voyage, and that his subsequent bad health and eventual frailty may have been late complications of the disease.

Syphilis acquired its name in 1530 when Giorlamo Fracastoro, a physician and polymath, wrote a Latin poem, Syphilus sive morbus Gaelic. In it, Syphilus, a shepherd, curses the sun and destroys altars when the king’s sheep die during a drought sent by Apollo. Vengeful Apollo punishes the shepherd by afflicting him with a horrible infection: “darts forth such pestilent malignant beams/As sheds infection on air, earth and streams.”

Hayden describes how, starting in the early fifteenth century, the syphilis epidemic spread across Europe, then worldwide, along with European exploration. It was estimated, at one time, that two in ten people in Europe were infected. Because it was obvious that syphilis was sexually transmitted, people began to take vows of chastity and abstinence; there were broken relationships and suicides. Doctors gradually learned about the disease, and some began to specialize in treating it.

Eventually, in the nineteenth century, the lifelong course of syphilis was recognized and its progress classified into three stages. The initial, or primary, stage is marked by the appearance of the chancre, an open sore, usually in the genital area. With the spread of the spirochete (a bacterium), a secondary phase appears with fever, rash, and general systemic signs, such as ill-defined aches and pains. Many organs can be affected, producing meningitis, gastroenteritis, arthritis, and hepatitis. This stage subsides in days or months, although with occasional relapses. Often there is a long asymptomatic period, giving a false sense that the disease has run its course. Although the possibility of transmitting the infection decreases after a few years, some sufferers, often years or decades later, go on to a tertiary phase, in which the spirochete—long sequestered and inactive in certain tissues—attacks the nervous system, cardiovascular structures, or bone and skin. The nervous system complications may result in strokes; a gradual paralysis and clumsiness (tabes dorsalis); and personality and behavioral changes and brain deterioration (paresis). In paresis, the course of illness may take years before there is frank dementia. Alzheimer’s disease, in contrast, starts with memory loss and cognitive changes.

The symptoms of all these phases can mimic many other disorders. In 1879, Jonathan Hutchinson, a British physician, addressed the British Medical Association on “Syphilis as an Imitator.” The preeminent physician of his day, Sir William Osler, advised: “Know syphilis in all its manifestations and relations, and all other things clinical will be added unto you.” It is this deceptive aspect of the disease that led, and still may lead, to a missed diagnosis.

SCIENCE COUNTERATTACKS 

Information about the disease’s course came from many clinical observations, but also from conducting trials of treated versus untreated populations, with the “treatment” being whatever was considered most effective at the time. Hayden describes some of these, including the most notorious: “The Tuskegee Study of Untreated Syphilis in the Negro Male” conducted in Alabama from 1932 to 1973.

Organized and run by the U.S. Public Health Service, this study screened several thousand men for the presence of syphilis and selected two groups: 399 males with syphilis and 201 without syphilis. Admittedly, today’s elaborate guidelines for clinical studies did not exist then, but even so, the study’s methods were thoughtless, shoddy, and harmful. Subjects were not informed that they had the disease; those in the “untreated” group often received treatment; clinical follow-up was frequently incomplete;

and, worst of all, afflicted patients in the study were not given proven treatment when it became available (by the mid-1940s, penicillin was known to be an effective treatment). The Tuskegee study was terminated in 1973 only after a young public health doctor, Peter Buxtun, delved into it and blew the whistle to the media. A senate committee probed the project. The federal government eventually provided some compensation to survivors and their families, and, in 1997, President Clinton publicly acknowledged the fault of the government.

Treatments for syphilis began almost as soon as it was identified as a specific disease. Because syphilis is sexually transmitted, early measures were often directed toward the genital region, with elaborate application of various potions. Mercury, which had been used to treat leprosy and yaws, became the favored medicine for syphilis in the fifteenth century, in the form of a salve, pill, or vapor. The theory was that, because mercury causes intense salivation, the infection would pass out with the patient’s saliva. The large doses used at that time produced many side effects, some resembling the complications of syphilis itself, but mercury continued to be used well into the twentieth century.

A popular notion beginning in the seventeenth century was that the disease could be cured by having intercourse with a virgin, which led to many rapes and many infected women. 

A popular notion beginning in the seventeenth century was that the disease could be cured by having intercourse with a virgin, which led to many rapes and many infected young women. (This unfortunate belief still exists among some groups of people in connection with AIDS.) Arsenic was also tried as a treatment, again with many side effects. In 1909, Paul Ehrlich, searching for an effective therapy, developed a compound of arsenic called Salvarsan. It was not a cure, but it did slow the disease’s progression and prevent some complications. Alexander Fleming discovered penicillin in 1928, although its therapeutic properties were not put into use until 1940 and its specific effect on syphilis was not described until 1943. Penicillin remains the treatment of choice.

A treatment that Hayden did not discuss is fever therapy. In 1917, Julius von Wagner-Juarreg, a Viennese physician, introduced this approach, in which blood from a person with active malaria was injected into a patient with neurosyphilis. The malaria produced bouts of high fever, eventually brought under control by the use of quinine. The idea was that the high temperature would kill off the spirochetes. There were mixed reports of efficacy, and the treatment was discontinued in the mid-1950s because penicillin treatment proved so effective. For his contribution, Wagner-Juarreg received a Nobel Prize in Medicine in 1927.

The microorganism that causes syphilis was identified by Fritz Schaudinn, a German zoologist, in 1905. A spirochete is shaped like a corkscrew and swims by waving its flagella. The organism divides lengthwise about every 30 hours in its early active stage. Schaudinn named it Treponema pallidum, or Spirocheta pallida. Its evolution is still unknown, but it is related to other spirochetes that cause diseases such as yaws and the rarer bejel. Recently, the spirochete’s DNA, or genome pattern, was sequenced, which may yield still more accurate identification. If so, the bone lesions seen in human remains may be more accurately identified if spirochetal DNA can be found. This is unlikely, however, as the organism does not survive long when removed from living tissue.

A blood test for syphilis was developed in 1907 by a German biologist, August von Wassermann. It was effective in the first stages of the disease, but also produced some false negatives. In 1913, Hideyo Noguchi, a Japanese bacteriologist working at the Rockefeller Institute, found the spirochete in the brain of a paretic patient, thus identifying the late nervous system complications of the disease.

While syphilis may lead to gradual deterioration of the brain, there are earlier changes in personality, behavior, and energy, including feelings of grandeur and invincibility, with bursts of energy, but also depression and withdrawal.

FLAUBERT DEFINITELY, HITLER MAYBE 

After presenting these facts and speculations about the disease, Hayden turns to the medical history of notable men and women who may have had syphilis. The list is a hagiography of literary, musical, and other geniuses. While syphilis may lead to gradual deterioration of the brain, there are earlier changes in personality, behavior, and energy, including feelings of grandeur and invincibility, with bursts of energy, but also depression and withdrawal. Van Gogh, for example, talked shortly before his death about being charged with electricity. It is possible that some of these mental states could have led to heightened creativity.

Hayden is careful to begin the discussion with a chapter on the perils of assigning a diagnosis based on secondhand information and sketchy observations. This concern is especially appropriate for a chronic disease like syphilis, the Great Imitator, which mimics many other illnesses and for which positive tests were not developed until the beginning of the twentieth century (and even then were not always reliable). Also, the stigma of syphilis has always been great, so victims have naturally tended to be secretive about it, often not even confiding in their spouses, families, or friends. Doctors have frequently suppressed the information by keeping secret or cryptic records on their syphilitic patients.

Faced with these difficulties, the biographer nevertheless has some helpful resources. For many of these people, we have access to letters over many years, journals, observations of friends, hospital and medical records, and sometimes autopsy reports. Hayden sums up her conclusions as follows: Schubert, Flaubert, Baudelaire, Maupassant, Blixen, and Schumann had syphilis; Nietzsche probably had it; there is debate about Beethoven and Wilde; the issue has been raised discreetly about Mary Todd and Abraham Lincoln; among the various diagnoses given to Van Gogh, syphilis must be seriously considered; and it is strongly suspected in Hitler. In the chapters that consider the evidence for these judgments, Hayden’s scholarship comes to the fore. She found and examined enough material on each subject to construct a plausible medical profile.

Her first medical biography, of Beethoven (1770-1827), is the most detailed and compelling. Rumors about Beethoven center on two principal questions: Did he have syphilis, and was he poisoned? In 1991, two collectors purchased a lock of Beethoven’s hair; the answer was to come from analysis of its chemical content. An article duly appeared—“Beethoven’s Hair Tells All”—announcing, with some hubris, that mercury was not found, but a heavy concentration of lead was. Many concluded, therefore, that Beethoven did not have syphilis. Hayden objects to the article, pointing out that an antisyphilitic medicine like mercury would have been deposited in the hair, but would have had time to move further along the growing hair and eventually to have been cut off. Also, heavy metals from external sources may deposit on the hair and bear no relationship to what is taken into the body. The implications of the hair analysis have even become the subject of a book, Beethoven’s Hair, by Russell Martin, who advances the idea that the high content of lead accounted for many of Beethoven’s illnesses.

Is it not possible that Beethoven’s deafness and brain changes, both presumably due to syphilis, did make a great difference to his music?

Hayden brings a persuasive array of data to her case that Beethoven did have syphilis. She cites George Grove, of the well-known Grove’s Dictionary of Music and Musicians, who noted that the autopsy on Beethoven’s acoustic nerves found that they were atrophied and showed evidence of chronic inflammation. This is suggestive of syphilis. Grove also noted that Beethoven had at least two prescriptions for mercury ointment. Others, such as Andreas Bortelini, Beethoven’s doctor, and William Thayer, his biographer, share the opinion that Beethoven had the illness. Add to this that Beethoven was bedeviled most of his adult life by recurring illnesses, culminating in deafness and personality changes. Sir William Osler, an expert on syphilis, concluded, after examining the course of Beethoven’s many afflictions, that the diagnosis was syphilis. Hayden concludes by quoting Beethoven biographer George Marek: “It goes without saying that it makes not the slightest difference to the music if Beethoven’s complaints were due to spirochetes or to a hangnail.” Hayden does not take issue with this, but perhaps she should. It seems contrary to her view that the disease affects creativity both positively and negatively. Is it not possible that Beethoven’s deafness and brain changes, both presumably due to syphilis, did make a great difference to his music?

In similar fashion, Hayden examines several other cases. To her credit, she presents evidence and opinions inconsistent with her own. About James Joyce, Mary Todd Lincoln, and Abraham Lincoln, she is inconclusive. She constructs a telling argument that Hitler was syphilitic, but, although there were secondhand reports about a doctor who had treated Hitler, there was no confirmation of the disease. Karen Blixen (the writer Isak Denisen) was confirmed by a positive Wassermann test.

Prior to the twentieth century, there was no way to confirm the presence of syphilis, but there were fine clinical descriptions of the disease and these became more refined with time. By the mid-nineteenth century, the correlation of clinical descriptions with postmortem findings became common.

I have not seen, or not recognized, a case of neurosyphilis for years, and was intrigued by the author’s descriptions of these pitiful patients. Through Hayden, younger physicians and scientists will be introduced to a disease of once-epidemic proportions, which inevitably will bring to mind parallels with the present-day worldwide HIV/AIDS scourge. General readers will be interested in the social and historical aspects of the disease, and all of us will be encouraged to learn patience, tolerance, and justice toward patients who suffer from “this deplorable sickness.”

EXCERPT

From Pox: Genius, Madness, and the Mysteries of Syphilis, by Deborah Hayden. © 2003 by Deborah Hayden. Reprinted with permission from Basic Books.

Why did Hitler, who could have retained any doctor, choose Morell? Why did a public idol, who changed his white silk shirt twice a day, choose a notoriously unkempt and smelly man to watch over his health? The urgency of Hitler’s request, the magnitude of his fee, and the presenting symptoms suggest that he chose Morell, a syphilologist, for the simple reason that he feared the progression of syphilis. A villa was excessive payment for curing a skin condition and a stomachache. But was any fee too great for a man in Hitler’s position to contain and keep secret the horrors of late syphilis? Morell appealed to Hitler as well because he was willing to try alternative medical practices. He had an upscale, prestigious practice. And, unlike the majority of the doctors treating venereal disease at the time, he was not Jewish...

When Morell began his diary, Hitler was complaining of giddiness and a troublesome feeling over his left temple. He complained for several days of buzzing in the ears, a symptom he had experienced for years. Morell applied leeches to his temple to alleviate the buzzing. “Hitler sat in front of the mirror and watched fascinated as the leeches quenched their thirst on his blood.” That month as well he had a fever with shivering, chills, and vomiting. Morell called the episode encephalitis, an inflammation of the brain tissue. A second similar episode occurred in December. In March 1942 a note from Goebbels recorded that Hitler told him he was suffering from “the most violent dizzy spells.” Morell noted another significant medical event in July 1942 when Hitler was in Vinnitsa, his headquarters in the Ukraine: “Brain edema with impairment of vision in right eye and high blood pressure (over 170 mm.)” He called it a Russian headache, noting that it was not unlike meningitis. Hitler complained of impaired vision in his right eye. He was experiencing tremors and weakness in his left leg.

Over the next three years, Hitler was progressively incapacitated, with old symptoms recurring and new ones added. He complained of pressure on both sides of the head, insomnia, more dizziness, and throbbing headaches that lasted for days. He experienced jerkiness of the leg and a hand tremor. His speech lost volume. He had agonizing flatulence and contractions of the intestines. Pustules and furuncles appeared on the back of his neck. Morell administered electric warming pads and moist compresses to the stomach and liver, set up oxygen for him in his room, and sent him to a field hospital in Rastenburg for a skull X-ray that showed inflammation. His face became flat and lacking in expression. He was apathetic, lost in details; his memory was increasingly impaired. He had an episode of jaundice. His housekeeper, Anni Winter, described him as weak, with scarecrow arms that shook uncontrollably. He showed signs of premature aging. Saliva dribbled from the corner of his mouth...

The possibility that Hitler was suffering from late stage syphilis suggests that his various illnesses can be looked at again from the perspective of this diagnosis. Matching the dozens of signs and symptoms of Hitler’s physical state in those last years with a classic text of syphilology suggests a compelling likelihood that almost every part of his body was advertising a progressing late syphilis.  



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Scientific Advisory Board
Joseph T. Coyle, M.D., Harvard Medical School
Kay Redfield Jamison, Ph.D., The Johns Hopkins University School of Medicine
Pierre J. Magistretti, M.D., Ph.D., University of Lausanne Medical School and Hospital
Robert Malenka, M.D., Ph.D., Stanford University School of Medicine
Bruce S. McEwen, Ph.D., The Rockefeller University
Donald Price, M.D., The Johns Hopkins University School of Medicine

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