The Sleeping Disease That Made Living Statues
Imagine waking up one day in a hospital, everyone around you in shock, people smiling snapping pictures, and shaking each other's hand in congratulations. You’re confused, you don’t remember falling asleep in a hospital, and you don’t even remember being sick.
Finally, a nurse comes to your side to tell you, you were affected by a disease that put you to sleep… over forty years ago.
Today we’re looking at a largely forgotten sleeping disease that killed over one point six million people around the world in the early 1900s. Which is a hell of a lot of people.
First Case?
In 1917 a neurologist from Vienna, named Constantin Von Economo released a paper after witnessing an illness that was beginning to affect more and more people. While treating patients diagnosed with meningitis, multiple sclerosis, and delirium in a Psychiatric-Neurological Clinic he quickly realized all of their symptoms didn’t exactly match their diagnosis.
In particular, the patients presented with extreme sluggishness, and some were completely immobile but alive as if they were living statues. After the deaths of some patients, Dr. Economo noticed an inflammation of the patient’s brains, this is called encephalitis. He went on to call the disease Encephalitis Lethargica which translates to sleeping sickness.
“Frozen in Time”
Soon the disease would spread throughout Europe with no cure or full understanding of it’s cause and by 1919, the disease had spread all around the world. Millions of people began to experience symptoms such as light fevers, headaches, blurred vision, upper body weakness, and muscular pains.
These symptoms then evolved into slowed thinking, slowed movements, double vision, loss of interest in life, delirium, and paralysis.
For some, all of the symptoms progressed gradually but for others, on rare occasions, the symptoms advanced to sudden paralysis without warning.
People would be at home with everything seeming normal when their movements began to slow until they fully stopped and were unable to move. It didn’t matter what position they were in or what they were doing, encephalitis lethargica, or EL, could strike without warning. Others would describe them as frozen in time.
When they were once again able to move, some stated they were fully aware of what was going on, saying it was as if there was a force holding them still or their brains were preventing them from moving. This honestly describes my worst nightmare.
While some eventually woke up, others never did, as in the case of a young girl who presented with sudden paralysis in one side of her body while on her way back from a concert. It quickly spread to the other side of her body and within a half hour, she was asleep.
Twelve days later she died.
Gone
The disease affected over five million people of all ages all around the world over the course of the next ten years. Then in 1926, encephalitis lethargica disappeared with no known cause for its beginning or end.
But in that time over 1.6 million of those affected died, many from respiratory failure, with less than a million making full recoveries. The rest experienced symptoms throughout their lives with some displaying impulsive behaviors to the point where they were described as psychopathic.
Soon they began to display symptoms similar to Parkinson's disease such as tremors, stiffness, or slowed movement regardless of their age. Eventually many became catatonic or fell into comas, their loved ones fearing they would stay in this state for the rest of their lives.
A Possible Cure?
It wasn’t until the late 1960s, forty years after the outbreak ended, that neurologist Oliver Sacks decided to try a newly invented drug called L-Dopa. This drug increased the dopamine levels in a patient’s brain and had been shown effective in those suffering from Parkinson's disease.
Parkinson’s disease was known to deteriorate a part of the brainstem called the substantia nigra where dopamine affects the motivation of movement. Since the L-Dopa had proven useful in those cases and knowing movement was being limited in EL, Sacks believed the drug could help.
Miraculously the drug worked and patients began to wake for the first time in decades. Many reported not being aware of anything but others reported being awake throughout their entire catatonic state with varying degrees of memories.
But the celebrations were short-lived. Patients began experiencing side effects from L-dopa such as hallucinations and aggressive behavior. And soon the drug stopped working, no matter the dose the patients were deteriorating back to their previous catatonic states. Some were angry to have been awake for such a short period of time while others were happy to have experienced life one last time.
Oliver Sacks went on to write a book about the experience called Awakenings in 1973 which was then turned into an Academy Award-nominated film starring Robin Williams and Robert De Niro.
Forgotten
As for encephalitis lethargica, it’s still not known what causes the disease, the pandemic from 1915-1926 was largely overshadowed by the Spanish flu from 1918-1920 which killed upwards of 100 million people. Some scientists believe the Spanish flu may have actually played a role in creating a lowered resistance to EL, especially since some of the early symptoms of EL mirror that of the flu or a cold.
Because of this, virologist John Oxford believed the disease could return at any time and in 1993 he was proven right when a patient Becky Howells was diagnosed with EL. Since then Oxford compiled at least twenty other patients suffering from EL. With the work of others, he believes he discovered the cause of EL is a severe immune response to a streptococcus-like bacteria, called diplococcus.
While I couldn’t find anything officially stating that's the cause it makes sense but we’ll have to wait for future updates and here’s to hoping they make the names of these bacteria easier to say.