Russian to Conclusions
I thought I had Ivan’s case all figured out. I heard a rumor somewhere a few years ago that his sexual exploits led to a case of neurosyphilis that spread to his brain, a theory that could ostensibly explain some of his behavior. Plus, he had a traumatic childhood, which we’ve seen leaves people vulnerable to mental illness. But, being the good rigorous scientist that I am, when I began to search for evidence to support my theory that Ivan had neurosyphilis, I realized the issue was much more complicated than I anticipated.
First of all, there is a dire lack of information about the state of Ivan’s mental health from reputable sources. With each link I clicked on, I found new theories that claimed to be supported by a 1960s autopsy of Ivan’s body. They found evidence of syphilis. They found that he was poisoned. They found nothing at all, he must have been schizophrenic or a psychopath.
To make sense of these competing claims, all I had to do was read the autopsy myself. The problem? I could only find it in a journal called Canadian-American Slavic Studies that is inexplicably published in German and kept behind a paywall that my academic institution could not give me access to — no wonder there’s so much false information flying around about this man.
Rather than pay 20 dollars and dust off my high school foreign language skills, I scraped the bottom of the internet until I found a paper by Edward Keenan that described the autopsy findings. Keenan was a historian at Harvard who rocked the medieval Russian academic world when he exposed some documents that were used to paint Ivan as “Terrible” as actually fake. If there’s anyone I could trust, it would be this scholarly rebel. If Keenan’s interpretation of the autopsy is to be trusted, then Ivan did not have syphilis, but he did have something far more interesting.
Throw Me a Bone
I read from some other sources that the researchers who examined Ivan’s body (which was remarkably well preserved) found skeletal abnormalities. Keenan confirms this. He quotes the report as saying that his “right clavicle was abnormally shorter [than the left] and the left clavicle was larger and more massive [than the right]. The whole torso was noticeably asymmetrical. The spine, with its straight neck, had lost its flexibility as a result of the formation of numerous osteophytes. The whole spine was as if welded in a single position. The osteophytes on the vertebrae had fused…Around the joints of the long bones of the extremities were found ridge-shaped and lump-shaped growths…” I didn’t know what an osteophyte was, but I could tell it was not good.
After further research, I learned that osteophytes are what are more commonly referred to as bone spurs. These are outgrowths of bone near joints, and are associated with things like arthritis, where joint damage leads to destabilization. However, the differences between Ivan’s left and right clavicle suggested that this wasn’t simply a severe case of arthritis, but rather a developmental issue with the bones. I was convinced that the autopsy of Ivan’s body showed that he had several skeletal abnormalities. But what caused them, and what do they have to do with the state of his brain?
TB Tea
Keenan links the spinal abnormalities observed in Ivan’s remains to a series of letters exchanged between Ivan’s parents shortly after his birth. These letters are some of the very few primary sources we have about Ivan’s life, and they reveal he suffered from an illness that put the court on edge. Ivan’s mother says that her was suffering from convulsions, and then a large lump appeared on his neck. Soon after, it ruptured and began secreting pus and blood. Mysteriously, this sickness is referred to as “maka” in the letter. To the dismay of historians, this word is found nowhere else in the Russian language. But our brilliant protagonist Keenan links maka to a word from a Russian dialect meaning a hump of soil near a marsh, which is related to the Russian word for tuberculosis. Bullseye.
Tuberculosis (TB) is an infectious disease caused by Mycobacterium tuberculosis. Thanks to modern vaccines, TB is very rare today, but it was raging in the 16th century. Mycobacterium tuberculosis generally affects the lungs, but it can also spread to the cervical lymph modes, joints, bones, and abdomen. When TB infects the cervical lymph nodes, it produces growths much like the one Ivan had as a baby. Interestingly, this form of TB, called scrofula, is more common in children, and, up until the 1800s, was thought to be cured by the touch of a king. Oh, the irony!
Furthermore, while TB rarely affects the skeleton, when it does, the spine is the most common area of the body involved. Spinal tuberculosis is most common in children, and has severe consequences, including neurological symptoms due to spinal cord compression and spinal deformities, not unlike what Ivan’s remains showed. So it would be a pretty rare case, at least by today’s standards, but let’s roll with it. We think Ivan suffered from an early case of TB, causing severe spinal abnormalities. Now let’s finally get to what that has to do with his brain!
Mercury in Retrograde
As you can imagine, walking around with a body that was ravaged by TB was not a pleasant experience. Ivan was likely living with severe, chronic pain, and he medicated accordingly. Sources have described his affection for a stiff drink and ointments made with mercury. If you, like me, grew up living in fear that you were going to break your mercury-containing thermometer in chemistry class and kill everyone, you’re probably wondering why he would willingly use a toxin on his skin. In Ivan’s day and age, mercury was indeed used as a poison. A modern examination of the remains of one of his wives shows that she was definitely poisoned by Ivan’s enemies as he suspected. However, mercury was also a common medical ingredient, especially in treatments for syphilis. And the topical use of mercury is still ongoing, most often from beauty products produced in countries without strict safety regulations. Just this year, the Center for Disease Control reported a case of mercury poisoning in a woman using a foreign skin cream.
In case it wasn’t clear from the term “mercury poisoning”, mercury exposure is not a good thing, especially for the nervous system. Unlike many toxins, mercury can pass through the blood-brain barrier, a tightly controlled barricade that protects the brain from insult. Once there, it is converted to a stable form that keeps mercury in the brain longer. Mercury can bind to microtubules, which provide structural support for neurons, and impair neuronal signaling by altering the release of neurotransmitters and the activity of neurotransmitter receptors. In addition, it promotes the formation of reactive oxygen species, highly reactive forms of oxygen (as its name suggests) that can interact with and damage DNA. The overall effect? Cell death.
Because mercury has such extensive effects on the nervous system, it will come as no surprise that mercury poisoning can have psychiatric effects. The symptoms depend on the type of exposure, but most often include irritability, apathy, anxiety, cognitive impairment, decreased social inclination, and depression. A severe form called erethism or “Mad Hatter’s Disease” (so called because it was characterized in a group of hat factory workers exposed to mercury) manifests in severe mood instability, tantrums, and fighting. Rings a bell doesn’t it? Think about Ivan’s poor military decisions, his withdrawal from society, and the fact that he killed his son in a fit of rage. Debates exist about whether scientists found levels of mercury in Ivan’s remains consistent with poisoning, so take my theory with a grain of salt. But I think there are enough similarities between Ivan’s behavior and the psychiatric mercury poisoning to warrant further consideration.
Falling Tsar
Perhaps Ivan’s go-to pain killer wreaked havoc on his brain, causing a massive personality change that left him explosive and irritable. As we discussed, his remains revealed striking skeletal abnormalities, which, combined with historical documents, suggest that Ivan suffered from the chronic effects of spinal TB. His pain led him to depend on mercury (not to mention the alcohol, which we don’t even have time to discuss!), inducing neural death and impaired signaling. Imagine how history might have been different if the poor man had access to some Icy Hot. But I’ll leave that to Stef to discuss next week…
References
Azevedo, B. F., Furieri, L. B., Peçanha, F. M., Wiggers, G. A., Vassallo, P. F., Simões, M. R., . . . Vassallo, D. V. (2012). Toxic Effects of Mercury on the Cardiovascular and Central Nervous Systems. Journal of Biomedicine and Biotechnology,2012, 1-11. doi:10.1155/2012/949048
Benzagmout, M., S. B., Chakour, K., & Chaoui, M. E. (2011). Pott’s disease in children. Surgical Neurology International,2(1).
Grzybowski, S., & Allen, E. (1995). History and importance of scrofula. The Lancet,346(8988), 1472-1474. doi:10.1016/s0140-6736(95)92478-7
Keenan, R. (1993). Ivan IV and “The King’s Evil”: Ni maka li tu budet? Russian History,20(1), 5-13.
Notes from the Field: Methylmercury Toxicity from a Skin Lightening Cream Obtained from Mexico – California, 2019. (2019, December 19). Retrieved from https://www.cdc.gov/mmwr/volumes/68/wr/mm6850a4.htm?s_cid=mm6850a4_w
Panova, T. D., Dmitriev, A. Y., Borzakov, S. B., & Hramco, C. (2018). Analysis of arsenic and mercury content in human remains of the 16th and 17th centuries from Moscow Kremlin necropolises by neutron activation analysis at the IREN facility and the IBR-2 reactor FLNP JINR. Physics of Particles and Nuclei Letters,15(1), 127-134. doi:10.1134/s1547477118010132
Stier, P. A., & Gordon, R. A. (1998). Psychiatric aspects of mercury poisoning. Medical Update for Psychiatrists,3(5), 144-147. doi:10.1016/s1082-7579(98)00022-3
Uneasy Lies the Crown 
4 thoughts on “Autopsy Turvy”