In March of 2014, a 57 year old man arrived at an emergency room in Paris. He was sweating with a 101 degree fever and complained of generalized pain in his lower limbs. He also had a startlingly low heartbeat of 40 beats per minute. Most everything else was normal, save for elevated levels of ketones and glucose in his urine, both indicators of runaway type 1 diabetes. He had a clean, healed wound on his foot.
He was admitted to the ICU with a "presumptive diagnosis" of diabetes and unexplained respiratory alkalosis, which is where the pH balance of of a patient's blood is slightly acidic, the result of increased respiration—hyperventilating, in other words. The alkalosis, ketone, and glucose levels all returned to normal within a couple of days. It was, however, just the calm before the storm and would be several days before a final rabies diagnosis, which would only come on day 13.
The event is recounted in a paper accepted for publication in the Journal of Clinical Microbiology, courtesy of a research team led by Christian Brun-Buisson, director of the Infection Control Unit at Assistance Publique Hopitaux de Paris. The event marked the first rabies case in France since 2003 and an extremely rare reappearance of the disease in the developed world at-large, which partially accounts for the diagnosis lag.
What makes the whole thing really scary is that the patient never knew they'd been bitten. People don't die very much from rabies anymore for two reasons: intensive animal vaccination programs and post-exposure prophylaxis, e.g. if someone gets bitten by an animal that may have even the smallest chance of carrying rabies, it's possible to head off the illness' onset with a barrage of shots. Rabies is recognized as perhaps the most successful disease eradication effort in history.
For good reason. Rabies is fucking terrifying.
The French patient had recently spent six months in Mali, West Africa and, one month before returning to Europe, had cut himself on a tree branch. It was a minor injury and healed with self-care. The rabies strain in question was eventually matched with one that was known to be circulating among dogs in West Africa.
"After repeated interviews with the patient's relatives, no history of animal bite or exposure to known or suspected infected animals during his stay in Mali was elicited, although stray dogs and bats were reported from his residency area there," Brun-Buisson and his team report. It's a bit like in the Walking Dead when the survivors realize that everyone is infected, bite or no.
"Two days after admission, the patient began having bouts of hyperactivity, disorientation, and delirium with thoughts of impending death associated with persecution ideas, alternating with periods of drowsiness and returns to normal behavior when he seemed aware of his disorder and criticized it," the paper recalls. The patient began hypersalivating and would occasionally spit on hospital staff.
Brain scans, cerebrospinal fluid samples, and further blood tests offered no new clues. No herpes, syphilis, HIV, thyroid abnormalities: nothing. Test after test.
On day eight, the patient's condition deteriorated rapidly, with altered states of consciousness and cycles of tetraparesis, a condition in which all four limbs suddenly become extremely weak (flaccid). Soon he was unable to breath on his own and, by day nine, the patient was put onto mechanical ventilation. More tests: still nothing.
Four days later, skin samples from the patient were tested for rabies, followed by saliva samples and further cerebrospinal fluid samples. Positive, finally.
Soon afterwards, the patient fell into a deep coma. 19 days after admission, he died.
52 hospital employees were determined to be at risk of infection, having been exposed to the patient's bodily fluids, and received vaccines. Two had close enough contact to warrant the next step of receiving rabies immunoglobulins (antibodies).
The report summarizes:
As is often the case in rabies-free countries, the diagnosis of human rabies was considered late in the course of the disease, with several factors contributing to this delay. First, physicians have a very low index of suspicion for rabies in countries such as metropolitan France, where only sporadic imported cases are identified. Indeed, only 20 cases have been notified between 1970 and 2013, with the latest one dating back to 2003. Second, a history of animal bite or exposure to rabies was lacking, whereas most of the recent cases of human rabies imported to Western Europe have been associated with a dog bite.
Rabies is widely considered to be the most deadly (and oldest) infectious disease in existence. It is almost universally fatal, though some patients have survived thanks to preexisting antibodies. The one treatment protocol that's had any success at all, the Milwaukee protocol, has mostly been discarded by infectious disease specialists as ineffective. Having already began exhibiting symptoms, the patient was pretty much dead on arrival (and in the absence of a bite warning, long before even that).
The WHO calls rabies a "neglected" disease. While eradicated in the first-world, or very close to it, the disease still kills thousands in Asia and Africa—though likely many more—where it's primarily an affliction of the poor and vulnerable.
"It occurs mainly in remote rural communities where measures to prevent dog to human transmission have not been implemented," a fact-sheet explains. "Under-reporting of rabies also prevents mobilization of resources from the international community for the elimination of human dog-mediated rabies."
In Asia, a post-exposure rabies treatment can run $49, while average incomes hover around $1 or $2 per day. The WHO estimates that the disease potentially threatens three billion people in Asia and Africa on the regular, but notes that eradication is possible using cost-effective animal vaccination programs. "However," the organization notes, "recent increases in human rabies deaths in parts of Africa, Asia and Latin America suggest that rabies is re-emerging as a serious public health issue."