At the height of the Great War, the “sleeping plague” or Encephalitis lethargica (EL) appeared as mysteriously as it would vanish many years later. But not before it infected nearly 5 million people between 1915 to 1927.
People who were affected reported experiencing dramatic lethargy, or sleepiness, before plunging into a deep slumber, sometimes for decades.
Although this may be #lifegoals for those weary of everyday life, the reality was a nightmare. Sufferers of the disease literally became prisoners of their own body, seemingly frozen in their own bodies while the world around them forged on.
It came out of nowhere
The origins of the syndrome allegedly began when an unnamed soldier was evacuated from the frontlines of the Battle of Verdun in France. This was in World War I when the environment was extremely hospitable to viral outbreaks and soldiers would return home with all sorts of nasties, such as the Spanish Flu.
Safe from the heat of the battle, little did they know he was harbouring a new kind of threat. Overcome by extreme lethargy, the soldier soon plunged into a slumber so deep that no doctors could wake him.
Soon, the mysterious syndrome spread to 60 other soldiers, quickly moving on to civilians, and then throughout Europe. Once it reached New York, symptoms of the disease diversified as if it were producing different effects in different brain chemistries.
Von Economo’s brain-splitting diagnosis
Viennese neurologist Baron Constantin von Economo was the first to study and announce Encephalitis Letargica in his paper published in 1931.
Those in the acute phase of “von Economo’s disease” as it was also known, slept all day. Eerily, the body displayed signs of “sleeping” but the sleeper remained acutely aware of his or her surroundings.
Delerium, headache and muscle spasms, particularly in the oracular muscles were also common in the acute phases of the syndrome.
A commonly reported characteristic was the staring, unfocused look that came over EL patients, as if their eyes were “detached from their bodies”. On some occasions, sufferers would sniff and drool uncontrollably.
One-third of patients with EL usually succumbed during this acute phase due to respiratory failure. However, the multi-stage disease posed a risky gamble for the surviving patients and often cruelly gave them an illusion of recovery.
Those who thought to have escaped the clutches of the disease during the early phases would eventually develop severe Parkinson’s-like symptoms appearing between months or even 30 years later.
Patients with post-encephalitic parkinsonism (PEP) would convulse and twitch uncontrollably before reaching the end-stage— a permanent physical paralysis or the decades-long slumber.
New brainy frontiers
Von Economo’s brain autopsies confirmed that all patients with EL had an inflammation mapped within a small region of the brain—the sleep generating center above the hypothalamus.
Modern studies further refined Von Economo’s finding by zooming in on the sleep preventing center in the brain instead—that it was damage to this region that caused the “sleepiness” of EL sufferers and not the former.
Although his initial hypothesis of a sleep generating center was not quite accurate, Von Economo’s study on the disease provided significant insights into neurology and psychiatry, particularly the brain’s sleep centers and Parkinson’s disease.
The disease would have been easily forgotten in the annals of history if not for neurologist Oliver Sacks. In the 1960’s, he came across the paralysis-ridden patients with EL. He decided to try treating them with an experimental drug used to treat Parkinson’s, levodopa (L-dopa).
After all, they didn’t have anywhere else to be.
His success in rousing some of the patients was short-lived for they soon slipped back into their frozen states. Nevertheless, he kept the haunting memory of Economo’s disease alive for a generation.
The last “living statue”
Today, the mysterious plague remains largely forgotten even though it left many victims of the disease disabled decades after contracting it. Adolf Hitler was speculated to be a victim of EL.
The last known surviving victim was Philip Leather, who in 1933, became fully immobilized at age 13-years old and spent seventy years of his life as a “living statue”.
As a child, Leather was a reported child prodigy, a self-taught pianist before his behaviour rapidly deteriorated; stooped posture, inhibited speech and his eyes became partially paralyzed.
He reportedly remained in a mental state of his 13-year old self throughout his life and after his death in 2002, his brain was donated to medical science.
100 years later, the infectious disorder has only appeared once in human history— appearing in the epidemic form before seemingly vanishing. Until now, no one knows exactly how it happened or how to cure it as research was limited by the technology of their time.
As the last EL victim is no longer here to help scoot research along, it’s safe to say we’re unprepared if this antique disease decides to pay us another visit.