Encephalitis lethargica: The mysterious plague from 1916 that plunged people into decades-long slumber

World War I was a time when the environment became quite hospitable to bacterial and viral outbreaks. Around the world, soldiers were malnourished. They lived in filth and then returned home carrying with them unknown infections. It was the time when diseases like cholera and typhoid spread like wildfire. The Spanish flu created mass devastation by killing an estimated 50 million people in 1918 and 1919.

But that was not all. In 1916, an unknown soldier was evacuated from the vicious Battle of Verdun. The soldier brought with him a new kind of threat. The soldier was sent to doctors from Austria to Paris where he was thoroughly examined, but they were all confused. Nobody could explain the incredible inactivity that possessed the man. In short, all he did was sleep. This was just the beginning. More than 60 soldiers joined him. All attempts to wake them up were futile.

This new disease was a cruel puppeteer that had the potential to freeze people in time. On the other hand, the disease caused other people to sniff and drool until all they wanted was to die. Symptoms involved constant muscle spasms, mostly on the face, and an uninterested and unfocused look. The majority of the patients lost their lives. 

Von Economo
Image of Von Economo who first described the disease and his book. Image credit: 1,2

A neurologist in Vienna was noticing similar cases among civilians. Constantin von Economo was curious when medical clinics started to fill with people who were constantly nodding off.  Patients were explaining how they sometimes just fall asleep while chewing food at the dinner table! Von Economo noticed that all of them displayed tics (habitual spasmodic contraction of the muscles), and their eyes somehow seemed disconnected from their body. They were unfocused and uninterested.

Majority of those patients died due to paralysis of the respiratory system. The neurologist Von Economo cut away their brains trying to discover a clue to this weird disease. In the majority of them, he found a swollen hypothalamus, the part of the brain that controls sleep. Whatever struck the patients down was attacking how the brain regulates sleep.

Von Economo then published a paper announcing the arrival of a new disease. The disease came to be known as “Von Economo’s Encephalitis” in Vienna and “encephalitis lethargica” elsewhere.

After Vienna, Londo and New York began seeing cases. And the more it appeared, the more diverse its symptoms became. Some people developed affectations that were ostensibly amusing like jumping or hiccups, but they were so persistent that they appeared horrifying.

New York’s then-mayor William O’Dwyer saw his wife succumb to this disease. New symptoms started emerging as the disease went on to affect more people. Some people developed superficial affectations to actions such as jumping or hiccups. Others experienced extreme tiredness even though they were not doing anything. While others grew hyperactive rather than tired.

The disease seemed to produce different effects in different brain chemistries.

By 1929, the people diagnosed with encephalitis lethargica became an exclusive club. Very few new cases began to appear. Around one-third of the affected patients recovered for unknown reasons, one-third died, and the remaining third suffered from symptoms resembling Parkinson’s Disease. They remained frozen in their bodies for decades.

Post-encephalitic Parkinson's
Post-encephalitic Parkinson’s. Image credit: jnnp.bmj.com

In some patients, the inflammation in the brain was like a time bomb that was set to detonate later. After months or years when the patients thought they had recovered, symptoms started to appear that resembled Parkinson’s disease. The patients convulsed and twitched before they went into permanent, physical paralysis. They were aware of their surroundings but unable to move. Frozen in their bodies, they waited for something else to take their lives. And that meant sometimes waiting for decades.

In the 1960s, neurologist Oliver Sacks started treating the paralysis-affected patients with the drug L-dopa. He was able to rouse some of them, but eventually, all slipped back into the condition.

The neurologist and author Oliver Sacks came across the paralysis-affected patients at Beth Abraham Hospital in the Bronx in the 1960s. Some of the patients projected their sense of awareness by responding to music or catching a ball thrown at them. Sometimes outside stimulus was able to provoke a reaction, but nothing could alter the condition permanently.

“Once, a patient brought a dog to the hospital,” Sacks recalled in 1991. “The poodle jumped up on a woman who was always frozen, and suddenly she belted out that she loved animals. She started stroking the dog and laughing. When the animal went away, once again she was frozen.”

Treating them with the drug L-dopa, Sacks was able to rouse some patients. Many of the patients started to walk and developed full awareness, but eventually, they slipped back into condition.

If it was not for Oliver Sacks, encephalitis lethargica would have never come to be known outside of medical literature. Sacks’ 1973 book,  Awakenings, and the movie it inspired in 1990 brought into the limelight the forlorn terror of such a horrible disease that was so poorly understood. But considering the time when the disease appeared and its quick departure, no one was able to examine the disease in depth. Some foundations and wealthy sufferers funded research for research, but that too never seemed to outlive their benefactors.

The last known surviving victim, Philip Leather, was infected as an eleven-year-old and subsequently spent over seventy years as a virtual “living statue” until his death in 2003. In another case, a patient identified only as “Miss R.” complained of dreams that were predictive of the state in which she would later end up.

Philip Leather
Philip Leather. Image credit: bcc.co.uk

Philip Leather spent nearly his entire life in a state worse than death. He was a ward of the UK’s public health services for nearly 40 years. Toys and school books that he left behind when he fell into sleep at the age of 11 surrounded his bed.

In another incident and shortly before succumbing to the disease, a woman identified only as “Miss R.” had a series of dreams. In her dreams, she was imprisoned in an inaccessible castle, but the castle had the form and shape of herself. She dreamed that she had become a living, sentient statue of stone and that she had fallen into a sleep so deep that nothing could wake her. Her dreams were unsettlingly predictive of her own condition.

To understand this disease completely, researchers would require fresh encephalitis lethargica victims. Very few scientists wish to devote their career to such an antique and vicious disease. Moreover, funding is scarce. But an absence of research could leave medicine unprepared for another outbreak if someday it decides to haunt the world again.
[Source: wikipedia]

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