Leaders prone to bouts of insanity

Beethoven's deafness, temper tantrums and irrational behaviour are well known. "On walks he bellowed so loudly that he stampeded cattle. The street children made fun of him, and once the police arrested him for staring in windows looking like a tramp," writes Deborah Hayden in Pox . His autopsy revealed that his "auditory nerves were shrivelled and lacking in the usual layer of myelin and that the left auditory nerve was much thinner than the right". But did he have syphilis?

Two physicians, Johann Wagner and Karl Rokitansky, performed the autopsy in Beethoven's house, where they sawed out portions of the temporal bone on both sides of his head. Later, the bones were stolen and the corpse disinterred. This and much other drama surrounds the stream of posthumous pronouncements on Beethoven's ailments and eccentricities that fascinate his listeners 200 years later. Otologist Sean Sellars is one who thinks that Beethoven might have had syphilis: "The postmortem finding of changes in the surround of the brainstem suggests a chronic localised meningeal reaction, thought to be syphilitic meningovasculitis. Syphilis was the cause that his contemporary medical attendants diagnosed and for which they treated him. His cirrhosis was at that time attributed to the same disease."

Having quoted this comment, Hayden analyses why, in the past three decades, opinion has turned vehemently against the syphilis diagnosis. She amasses quotes from letters, medical textbooks and patient records, as well as statements by any number of learned men, and concludes that for Beethoven (as for Adolf Hitler, Abraham Lincoln and several other famous names), "as much as we might like proof for a diagnosis of syphilis, there is none".

This meticulous referencing seems to give the lie to concerns expressed by others that the book is insufficiently scholarly. Without years of careful research and evaluation of a vast secondary, and in some cases primary, literature, Hayden could not have accomplished the publication of a book that I consider destined to become a classic of short biography in the newly emerging field of "medical symbiotics".

A crop of physician-authors, especially from the late 18th to the beginning of the 20th century, were identified as "syphilologists", a "respectable, challenging branch of medicine". The writings of these commentators represent major sources for Hayden's compelling narrative as she recounts the medical histories of the immensely talented authors, painters, musicians and activists who were afflicted.

She concentrates most on diagnoses in the 19th century, a time when clinical familiarity with syphilis was high. Evidence showed that all the afflicted passed through three stages. Immediately after infection there was the genital sore, which tends not to be painful and, in women, may go entirely unnoticed. The second stage was a terrible pox, fever and pain, which may emerge much later, with intermittent and extremely varied symptoms. The dreaded third stage, the "paresis" of untreated syphilis, is rarely seen in clinics nowadays. Paresis involves personality disturbances, reflex hyperactivity, eye abnormality, sensorium changes, intellectual impairment and slurred speech. Sometimes referred to as GPI, or general paralysis of the insane, paresis often begins with a dramatic delusional episode, such as identification with religious or royal figures, characterised by rage and violent acts. In the following months and years, dementia alternates with periods of such clarity that there seems to be a cure. Paralysis is progressive and the syphilitic often spend their last years in a mental institution. But most patients never manifest tertiary syphilis; those who do often display the symptoms years or decades after infection.

The cause of this venereal infection is a bacterium called Treponema pallidum . This is one of the anaerobic spirochaetes that genome sequencing has shown to lack four-fifths of the genes necessary for life outside the mammalian body.

Another associated syndrome is "tabes dorsalis". This manifests itself as a loss of locomotion ability and a progressive inability to coordinate bodily movements, and is explained by damage to the nerve cells of the spinal cord. The tabetic often experiences a stiffness of gait, which may progress to a stumbling, zigzag pattern of walking, sometimes with the dragging of a foot. Symptoms may include difficulty in urinating, impotence, blurred vision, numbness in the hands or feet and incorrect perception of ambient temperature change. Such papillary responses are often found in both tabes and paresis. Women, in general, are relatively less paretic and more prone to tabes.

Hayden describes such aspects of medical literature in a properly cautious, properly critical way. She provides detailed accounts of past and present medical practices from contemporaneous published accounts. It becomes clear that syphilis is still with us, despite the common idea that penicillin has got rid of it. I recommend Pox to anyone interested in the history of venereal disease.

Lynn Margulis is professor in the department of geosciences, University of Massachusetts, Amherst, US.