By the time Rosie pulled out her other eye — calmly, with a steady hand, as neat and clean as any surgeon with a scalpel — the collective shock of hospital staff had long since worn off. She’d already committed so many acts of self-harm that caregivers had grown numb to the sight of blood creeping along the bleached white sheets of her hospital bed.
Rosie was just a girl when it started: a mild fever, a twitching, spastic eye and exhaustion. She slept like she’d been anesthetized, a stillness without the fluttering and shifting of regular rest. When she woke, she terrorized her parents with tantrums, breaking windows and making threats.
But the harm, when it came, was only directed at herself. She once locked a bathroom door and pulled out two dozen of her teeth, each one hitting the sink with a tap.
She was hospitalized in New York, one of thousands of children effectively institutionalized because their families could not cope with their behavior. The first time she pulled out an eye, she denied doing it, explaining it must have fallen out while she was sleeping. The second time, she was manacled to the bed.
Of all her misfortunes, Rosie’s worst may have been falling gravely, inexplicably ill at a time when the world’s attention was consumed by World War I (and an influenza pandemic killing millions with brutal efficiency). She suffered from a relatively minor outbreak of a strange infection that attacked the brain and demanded resources medicine could not spare. It also seemed to diffuse quickly, throbbing with activity in the 1920s before it was thought to have disappeared.
But encephalitis lethargica, or “the sleepy sickness,” never really went away. Survivors struggled with residual fatigue and neurological issues. Others appeared to make a full recovery only to drift into a state of physical inertia, little more than a rigid sculpture of flesh that could do nothing but tear up when they heard doctors pronounce them hopeless. It was as if Clive Barker became a virologist and engineered a disease of exquisite suffering.
One hundred years later, we still don’t understand it. And it’s still not gone.
The Spread of the Sleepy Sickness
World War I could not have been more hospitable to viral and bacterial outbreaks. With malnourished soldiers from all over the world converging, living in squalor, then returning home, opportunistic infections had first-class accommodations. Typhus and cholera prospered; the Spanish flu ravaged, killing an estimated 50 million people in 1918 and 1919.
“It was of great concern to have an outbreak of disease in war,” says John Oxford, Ph.D., a virologist and professor at Queen Mary, University of London. “There were reports of the flu as early as 1915, but the name of the camp wasn’t mentioned. There was this secrecy.”
It wasn’t until an unknown soldier was evacuated from the vicious, sprawling Battle of Verdun in 1916 that a different kind of threat emerged. The man was shuttled from Austria to Paris, where doctors examined him and cocked their heads in confusion over his incredible lethargy. He slept constantly. So did the more than 60 soldiers who joined him. Nothing could rouse them.
The only thing separating them from a corpse was their breathing. Maybe it was mustard gas, doctors thought, which may have been better than saying they didn’t know.
In Vienna, a neurologist named Constantin von Economo was seeing similar cases in civilians. Clinics filled with patients nodding off, nervous spouses or parents explaining how they’d sometimes fall asleep walking or while chewing food. They displayed tics and repeated words. Their eyes seemed disconnected from their brain, unfocused and unregistering.
Almost half of them died, their respiratory systems paralyzed. Von Economo spirited away their brains hoping to find pieces of the puzzle. In most, he found a swollen hypothalamus, the part of the brain that controls sleep. Whatever struck the patients down, as this video shows, was attacking how their system regulated somnolence.
Von Economo wrote a paper announcing the arrival of a new disease that quickly became known in Vienna as Von Economo’s Encephalitis and elsewhere in the world as encephalitis lethargica — a swelling of the brain producing sleepiness.
“He described something similar to narcolepsy,” Oxford says. “It was a sudden sleep, with people rolling their eyes and going off even while standing up.”
London and New York began seeing cases; New York’s then-mayor, William O’Dwyer, saw his wife succumb. And the more it appeared, the more diverse the symptoms. Some people developed affectations that were superficially amusing — jumping, hiccups — but so persistent as to be horrifying.
Others grew hyper rather than tired. The disease delighted in producing different effects in different brain chemistries, a cruel puppeteer that could freeze one patient and cause another to sniff and drool until they wanted to die.
Children may have gotten the worst of it. Their evolving brains reacted strongly to infection, rendering their behavior heedless and sinister. Feces was smeared on walls; heads were stuffed into fires; siblings were sexually assaulted. Patients were either catatonic in bed or hysterical with violence, but generally retained their intellect. When the disease became more widely known — and feared — some doctors opted not to tell patients they were infected, hoping to spare them grief.
Queen Mary Hospital in London took six brain samples from expired patients and sealed them in wax. If they couldn’t find a cause or cure, maybe another generation could.
Frozen in their Bodies
By 1929, the people diagnosed with encephalitis lethargica were an exclusive club. New cases were dwindling. Roughly one-third had recovered for unknown reasons; one-third died and another third would soon wish they had.
In some patients, the brain inflammation set a timer for a delayed detonation. After a period of months or years where patients seemed to be fully recovered, symptoms resembling Parkinson’s appeared. Patients twitched and convulsed before settling into an effective physical paralysis. They were aware, but unmoving. Post-encephalitic and frozen, they remained trapped in their bodies until something else took their lives. That could mean decades of bed-ridden imprisonment.
The neurologist and author Oliver Sacks came across a small colony of these patients at Beth Abraham Hospital in the Bronx in the 1960s. Some projected their awareness by responding to music or catching a ball that was thrown to them; outside stimulus could provoke a reaction, but nothing could be initiated.
“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 became ambulatory and cognizant, before becoming tolerant and slipping back into condition.
If not for Sacks, it’s unlikely encephalitis lethargica would have crawled out of medical literature. His 1973 book, Awakenings, and the 1990 movie it inspired, described an abject terror that such an insidious disease was still so poorly understood. Given the climate of the time and its rapid departure, no one had been able to examine it up close in its acute stage for very long; foundations and research funded by a few wealthy sufferers never seemed to outlive their benefactors.
One by one, the physicians who had seen cases up close died of natural causes. (Von Economo was the first, in 1931.) By the time Sacks made his discovery, medical textbooks had stopped mentioning it.
Meanwhile, these victims aged in their beds, a handful still alive in the 1980s. Only Philip Leather, infected as a boy in the 1930s, saw the year 2000, having spent more than 70 years locked inside of his own body. When he died, his sister elected to donate his brain to science.
Leather’s brain was of recent vintage, but it wasn’t the only one. Researching both Spanish flu and encephalitis lethargica, Oxford came across the half-dozen brain samples stored at Queen Mary in London and pursued a molecular examination of the tissue. Some reports — like one from the Centers for Disease Control — had hypothesized it had been caused by the flu outbreak.
“We looked for the genetic footprint of flu but didn’t find it,” Oxford says. He turned to Philip’s brain, fresh and full of promise. There was no evidence of influenza there, either.
But, Oxford says, the circumstantial evidence is strong. “Maybe we didn’t use a sensitive enough technique to pick it up,” he says. The theory is that the strain — which affected nearly a half-billion — made its way to the brain of an unfortunate few, causing havoc in a relative handful.
In quarantined parts of the world, he says, no one got Spanish flu. And no one got encephalitis lethargica, either.
Lurking in the Shadows
Oxford visited Philip Leather shortly before his death in 2002. Having been a ward of the UK’s public health services for nearly 40 years, he lay in bed, surrounded by the toys and school books that were left behind when he fell into darkness at the age of 10.
Oxford showed him a picture given to him by Leather’s sister. It was of Philip as a child, ambulatory and cheerful. There was a flash of recognition on his face. It didn’t last long.
At around the same time, a pediatric neurologist named Russell Dale began seeing cases in the Great Ormond Hospital of London. Though sporadic, they still appear from time to time, all of them with a hallmark trio of psychiatric symptoms, movement disorders and sleep disturbances. MRIs were abnormal in a portion. Some patients responded to steroids to reduce inflammation; in others, L-dopa minimized the effects.
Dale looked at the cases and the ones of von Economo and discovered a common trait: Many patients had complained of a sore throat. It’s possible, he wrote, that a strain of streptococcus bacteria could be at the root of the infection, with the immune system’s overreaction to its presence attacking the basal ganglia that modulates movement and emotion.
To understand encephalitis lethargica better would, perversely, require fresh victims. That, or researchers would need to obtain more brain samples than the dozen or so that have been examined thus far.
“There must be some out there, but it takes effort to get them,” Oxford says. “You ask pathology museums and they say they haven’t got any, then you go and spend hours looking and it’s tucked away somewhere.”
Few are interested in devoting a scholar’s life to an antique, wholly unpleasant disease. Funding is scarce. But an absence of research could leave medicine unprepared for another outbreak. The Spanish flu is known as the “mother” virus, with its many descendants alive and well.
“You never truly know whether the thing could come back again,” Oxford says. “It’s a bit of a pity, and a bit of a danger.”
Of the surviving patient case histories, Rosie’s remains one of the most unique. After blinding herself, she went on to learn Braille, seemingly exempt from any depression or trauma. What happened to her next, like the disease itself, remains a mystery.