What do you suppose the following had in common: the American President Abraham Lincoln and his assassin, John Wilkes Booth, the German Emperor Frederick (Friedrich) III, the author of Household Management, Mrs Isabella Beeton, the impressionist painter Édouard Manet, the post-impressionist Henri de Toulouse-Lautrec, the gangster Al Capone, the composer Frederick Delius and the jazz pianist Scott Joplin, the Irish authors Oscar Wilde and James Joyce, the revolutionary Vladimir Lenin, the dictators Benito Mussolini and Idi Amin, the billionaire Howard Hughes, and the founder of the Daily Mail, Viscount Northcliffe? And, of course, Adolf Hitler. And countless multitudes, famous or unknown. In most cases the link is certain, in others it is compelling if circumstantial.
This story begins half a millennium ago shortly after Columbus had returned from the New World. An epidemic of a previously unknown disease broke out in Naples; it’s thought that it was brought back from the Americas by Columbus’s sailors; a sort of tit-for-tat, or Colombian exchange, for the smallpox and measles that they had introduced there, and which had devastated a population who had no natural immunity. This new disease, if indeed it was a new disease, spread rapidly throughout Europe. It gained political notoriety; the Dutch at war with Spain, called it ‘the Spanish disease’ for example, and others tarred their enemies in the same way.
Treatments then were as desperate as the disease. Mercury was a common specific, as a salve or by steaming in a cabinet. As a heavy metal, mercury is poisonous; it’s now uncertain how many sufferers died from the enthusiastic treatments rather than from the disease. It was said that a night with Venus led to a lifetime with Mercury.
It was the German pathologist Robert Koch who really established the germ theory of disease in the latter part of the 19th century, that tiny organisms rather than miasmas, ‘bad airs’ or evil vapours were responsible for infections. It took until the early years of the 20th century before the spirochaete, Treponema pallidum, was identified as the organism responsible for syphilis. At about the same time, a blood test for syphilis, the Wassermann reaction, was developed; and, after testing hundreds of arsenic compounds, Ehrlich 606 or Salvarsan was introduced. It’s a further sign of desperation that Julius Wagner-Jauregg introduced malaria therapy for syphilis; the idea was that giving patients malaria would raise their temperature sufficiently to kill the spirochaetes. He received the 1927 Nobel Prize for his work. Syphilis remains sensitive to penicillin, introduced in the 1940s. So ubiquitous was the problem that medical students were told to remember that ‘the Archbishop was once an undergraduate, and the Admiral was once a midshipman’.
The natural history of syphilis is classically described in three stages. A painless ulcer or chancre develops at the site of infection; this is primary syphilis. The chancre resolves, but is followed within a few weeks by the skin rashes of secondary syphilis. The afflicted is infectious at this time. Following a latent period, which may be many years, symptoms and signs of tertiary syphilis can become apparent. Any organ or tissue in the body can be affected, and the patterns of disease often mimic other conditions; not for nothing was syphilis known as ‘the great imitator’, and it was said that to know syphilis was to know medicine. Gummas, painless swellings, may be present, or the disease may affect the central nervous or cardiovascular systems. The afflicted are not infectious at this stage.
This natural history of the three stages of syphilis came from close observation of people over many years. As if this wasn’t enough, a formal, on-going study was begun in Tuskegee, Alabama in 1932; this involved poor African-American farm labourers. They were told (lied to) that they were being examined for ‘bad blood’. They were followed for four decades, and those that developed syphilis were given no treatment even when penicillin was available. The study only ended when it became public; it wasn’t until 1997 that Bill Clinton apologised to the few survivors. In the late 1940s, in a study in Guatemala, American officials actually infected soldiers, prostitutes and others with syphilis; no consent was obtained. The US apologised to Guatemala in 2010. Had Dr Mengele performed such unethical ‘experiments’ they would have been described as war crimes.
There was a short-lived notion in the later 19th century that ‘syphilis was good for you’; it was felt that syphilis made artists more creative. It was a rite of passage for a budding painter or author to become infected. The idea was total nonsense. In 1904 James Joyce wrote to Oliver St John Gogarty (Buck Mulligan) who was in Oxford, seeking advice. Gogarty wrote back about the necessity for chastity, and saying, ‘congratulations that our holy mother has judged you worthy of the stigmata…you have got a slight gleet from a recurrence of original sin’. ‘Gleet’ is the urethral discharge of gonorrhoea; the two diseases often coexisted, and it’s been suggested that Joyce got more than just a dose of ‘the clap’; he certainly had the opportunity.
If the brain is affected in tertiary syphilis, it results in general personality disturbances which may progress rapidly. A pathognomonic sign is the presence of Argyll Robertson pupils. Normally, the iris of the eye constricts in the reaction to bright light, or when accommodating to see close objects. Here, the light reaction is lost, the reaction to accommodation remains. Death is often described as being from a general paralysis or paresis, the general paresis of the insane (GPI). Death from a ‘paralysis’ was an euphemism for tertiary syphilis, and should be read as such in older biographies.
If the spinal cord is affected, a condition known as tabes dorsalis, there are characteristic changes in the gait which is described as ‘high-stepping’. ‘Lightening pains’, sudden, sharp, lancinating pains can be so severe as to fell the victim. Cardiovascular syphilis causes changes in the electrocardiograph, as well as aneurysms or dilatations of the major arteries.
Lincoln’s widow was described as dying from ‘a paralysis’. Almost certainly, she and Mrs Beeton were infected by their husbands. Manet had a lightening pain when walking in the street; he fell to the ground, but recognised immediately what it meant. In Hitler’s case, things are more circumstantial; he is thought to have contracted syphilis from a Jewish prostitute in Vienna in 1908. Mein Kampf has a section on syphilis, and much on the Jews. His personal physician was a dermatologist (and a quack). In Germany, specialists in skin conditions were also specialists in venereology because of the rashes in syphilis. Hitler had a splitting of the second heart sound, said to be typical of syphilis. James Joyce had multiple episodes of iritis (uveitis), a very painful and unpleasant inflammation of the eye; while this can be due to other diseases, at that time syphilis would have been the leading cause. At least one of the specialists who treated him thought Joyce had syphilis. Lord Horder, Viscount Northcliffe’s physician, thought that Northcliffe was too far gone for malarial therapy to be effective. Although Northcliffe is conventionally said to have died from sub-acute bacterial endocarditis, an infection of the heart valves, this is probably a cover story.
And the Emperor? Frederick was married to Queen Victoria’s eldest daughter, Vicky, the Princess Royal. They were the parents of Kaiser Bill. Frederick developed ‘laryngitis’ with hoarseness of the voice. He was examined by multiple German doctors; Victoria sent Morell Mackenzie, the foremost English expert. The Germans thought that the Crown Prince had a ‘tumour’ of the larynx or voice box, that is a cancer. The vocal cords had been cauterised to no real avail, and an operation was recommended. Mackenzie disagreed with all this, saying in as many words that the German physicians were eejits. There then followed a tortuous, protracted illness, with multiple interventions, culminating in a tracheostomy. Frederick eventually succeeded to the throne, but reigned for only 99 days before dying. The treatment of his illness was as much medical as it was political; neither he nor Rudolf Virchow, the pathologist, approved of the Iron Chancellor. Sir Morell published his version of events soon after the Emperor’s death; he was utterly excoriated for breaking the duty of confidentially to his patient, his own health declined and he died soon after. Apparently unrecognised by his critics, Mackenzie’s book reported inflammation of the laryngeal cartilages which was a indication of what he really thought to be the diagnosis, one that he had hidden in plain view; that the Emperor had died of tertiary syphilis of the larynx. While a death from a laryngeal cancer would have been unfortunate, it was only a cancer after all. But death from syphilis carried with it the incontrovertible stain of immorality and sin.
Robert Campbell is a retired surgeon.