Physicians are dumbfounded by the repeated re-occurence of this disease; from the epidemics of the 1920’s to Oliver Sack’s research in the 1960’s to the cases discovered today. It appeared throughout history continuing to baffle physicians to this very day.
It asks the question of whether we will be unprepared should this disease arise in epidemic form?
Such forgettings are as dangerous as they are mysterious.
—DR OLIVER SACKS, 1973
This disease is known as “the forgotten epidemic,” the case studies mentioned in this book are all obtained from medical articles and journals during those time periods this includes personal accounts, family letters, and newspaper coverage.
Patient names were changed.
The disease behaved like a kaleidoscope and not just that it drastically changed the lives of the patients and doctors alike.
Case Study 1
Chapter 1
100,000 shells rained over the battlefields, mud, stones and splinters splashed and sprayed. The battle of Verdun of 1916 was unlike any other of its kind. By that I mean that it was the longest and most devastating battle for all of world history. The plan took place on Christmas night, the German command units would attack a French town fort. The crazy part was that no ground would be gained nor sought.
The war was meant to bleed the French army.
One wounded patient managed to reach the medical train from moving from the trenches to the hospital.
There were two physicians working in neuro-psychiatric hospitals; one in Paris and one in Vienna. The metal from the helmets made its mark on the skulls as well as the psyche. This inadvertently gave neurologists a chance to map out the brain, matching the parts to bodily functions, using the physical damage as a marker for a guide. Then, the psychological damage paved way for modern psychiatry.
A French physician who was also a pathologist and paediatrician, was working in the Paris clinic, his name was Jean René Cruchet. He was struck by the unusual set of symptoms that the wounded soldier from Verdun had. At first, he wondered if the culprit were mustard gas or a new chemical weapon installed the Germans.
There were 64 similar cases that Cruchet saw all in all and there was no standard diagnosis. The striking thing was that they had an unusal range of symptoms; some had fever others did not. Most of them however complained of headache and nausea.
The strangest conflicting clue was how much all of these soldiers would fall ASLEEP.
Chapter 2
A patient who was drowsy and confused wandered into a psychiatry clinic in Vienna in January of 1917, he was slumped up in his seat, his neck giving away with his head tilted off to his side, he could be roused though not fuly awake. He was a very sleepy civilian.
The Wagner-Jauregg clinic had seen numerous patients recently but it was obvious that this patient had nothing to do with contributing to the war. There was no physical trauma, no history of insanity, no single-handed infection known to cause these sort of symptoms. The doctors have not seen anything like this! His case would be followed by a dozen more.
In all of these cases, it was observed that there was change in the patient’s eyes. The eye muscles would malfunction, it would be dull and unresponsive, either that or the eyes would twitch by rolling back to the head. There were so many unusual symptoms at one point and it got even stranger from there.
There were hiccups resulting in death in one of the patients, other patients had bizarre tics; and then the sudden intake of schizophrenia patients and dementia patients who would salivate well into their catatonic state and in all of these cases an unusual sleepiness would accompany these unfortunate patients.
Catatonic State; it is a state of awakefulness but there is a lack of responsiveness towards people and the environment.
All of this were a complete mystery to the physicians at that time.
The one thing that would concern all of them was that there was no diagnosis.
In this very Vienna clinic, there was a young doctor named Constatin von Economo who first noticed that there was a common link to all with no common connection.
There was no neurological disease linked to indefinite sleep and disease to explain why the patient would awaken with such suddenness.
“We are dealing with sleeping sickness,” he said, bewildered.
In Vienna and throughout eastern Europe, groundbreaking work on brain study was taking place during the early decades of the twentieth century, Julius Wagner-Jauregg a neurologist for which the clinic was named after was experimenting with fevers to treat mental illness. He would go on to win the Nobel Prize for his experiments using malaria to treat neurosyphilis. In the neighbouring Germany, Emil Kraepelin, who was studying dementia praecox, the disease that would be later called schizophrenia, and Abis Alzhiemer was publishing papers that would bear his name.
Europe was also the birthplace of psycho-analysis thanks to Sigmund Freud and Carl Jung. According to Freud, exposure to so many taboo, immoral, violent actions was more than the human mind could take in many instances. The scores of patients with “war neurosis” seemed to support his theory as patients became catatonic, suffered nightmares, and saw recurring images of bayonets and mutilated bodies. They could not get the scent of the yellow clouds of mustard gas out of their nose, the feel of trench barbs out of their skin, or the staccato sound of continuous gunfire out of their ears.
The mortality of this strange new disease was high of about 40%. When the doctors autopsied the first victims, they found swelling and damage to the mid-section of the brain, which is the respective part controlling sleep.
With a clinic full of patients dying from sleep, von Economo searched medical texts for clues to what this epidemic could be. The culprit in any disease outbreak usually falls into one of three categories: an entirely new pathogen, a pathogen that has been around for some time but was only recently identified, or an old pathogen that has adapted and grown more dangerous. He could not find anything about this “sleeping epidemic” in the standard literature.
There are many things we do not know about the brain I guess even today!
Some papers mentioned a mass suicide in Scotland that pointed to a sleeping sickness disorder followed by mass hysteria. Similar instances of collective hysteria had surfaced in history, and all were related to the sleeping patterns. No medical texts describing the outbreak were widely published but it was recent enough to circulate in one’s mind of the previous generation.
There was a sleeping sickness that had occured in Italy and other parts of Europe during the 1890s flu epidemic and Von Economo had asked his mother who had remembered the nona epidemic all too well. Once she told him about the symptoms – they sounded hauntingly similar to what was going on in his clinic in Vienna.
The nona outbreak was the first time where doctors had questioned if there was a possiblity if the influenza and sleeping sickness had an association.
Von Economo quickly wrote a published paper that would be presented to the Vienna Psychiatry Society on spring of April 17th, 1917 announcing that he had identified this new disease. This was also published within days to that of Cruchet. In Vienna, it would be von Economo’s encephalitis and in France, it would be that of Cruchet’s.
To the whole world, it would be encephalitis lethargica.
As European doctors scrambled over to investigate its spread, and its first case discovery; it had mysteriously vanished with no new cases being reported at that time.
Chapter 3
The disastrous losses began to take a toll on the French soldiers, Britain would send its own troops into northern France to relieve the pressure by distracting Germany in battle.
The British and Germans met on a field near the river Somme despite this being a costly plan it proved effective.
There is a legend of Hitler that says of a British private coming in contact with a German courier, the German could not lift his rifle in the face of defeat, he was however spared by the Englishman. This would be a regretable choice given the causualities we will not mention thereafter.
It is worth mentioning however, that Hitler had a diverse medical case; one would suspect a whole spectrum of mental illnesses, syphilis, hysterical blindness, mustard gas poisoning, and the one and only encephalitis lethargica. One can agree that medical records have no valid logical reasoning towards cruel intentions now or does it?
The path between Verdun and the Somme was one but of many troop movements and battles that gave soldiers the chance to spread this sleeping sickness. The troops crowded together in camps, then trains and boats, giving the disease a chance to broaden its reach and offering the perfect entry into the United Kingdom. One ship after another departed from the coast and crossed the ragged whitecaps between France and England.
Our cases now shift to London, there’s patients with droopy eyelids, slurred speech and muscle weakness with filled healthcare bases. Sausage was blamed initially, other foodborne toxins were investigated as well. Things got complicated when exclusively breastfed babies experienced the same symptoms.
London Hospital had failed to locate this botulism thus ending the scare, instead tear gas and mustard gas were suspected or even a new chemical warfare.
Then, British doctors threw their hands up and acknowledged the very existence of an entirely new disease. In fact, those three epidemics of sleeping sickness during the seventeenth century may have very well been the inspiration for one of our most famous fairy tales: “Sleeping Beauty,” published in 1697. Likewise, Washington Irving wrote “Rip Van Winkle” while he was living in England in 1819, and it, too, is the story of someone awakened after years of sleep. The mid-1800s in Germany, physicians reported, seemed particularly rife with paralysis or coma caused by fevers. It was around that time that Edgar Allan Poe wrote two of his most haunting tales. In both stories, a character falls in and out of a catatonic, “death-like” trance from which he cannot be awakened, and Poe plays on one of society’s greatest fears at the time. One short story was “The Fall of the House of Usher”; the other was suitably titled “The Premature Burial.”
The English general practitioner A J Hall compared it to the story of Cinderella: “They could not find amongst the diseases of their acquaintance a foot which would go into this newly found glass slipper.” Hall’s first case, a boy, did not seem particularly sleepy; but he did have marked inability to move his muscles. He could barely lift an arm or leg off the bed. Other patients of Hall’s had fevers for weeks at a time or mumbled incoherently or had tremors. Most of the patients, however, showed the same lethargy. It was as if their sleep cycle was turned around—they stayed in a sleepy stupor all day and became delirious at night. Still other patients could sleep with their eyes open, with the chilling appearance of a corpse.
Worse, the cases in England were showing a mortality rate over 50 percent, even higher than the death rate in Vienna. Death, the Ministry of Health concluded, appeared due to paralysis of the respiratory system. In 1918, the first year the disease appeared on English soil, there were 538 cases; the number doubled in the coming months. The Ministry of Health also made it clear that “Medical Health officers are invited to take any action which they consider useful and practicable to secure post-mortem and pathological examinations by skilled workers who are investigating this disease.” Public health was of greater importance than individual wishes during an epidemic. The ministry made encephalitis lethargica a “reportable disease,” forcing physicians to inform the government of new cases.
As the disease spread, however, it took on new symptoms.
In a bizarre twist on the lethargy cases, some patients grew hyper-kinetic, unable to sleep. Their bodies twitched and muscles tensed. A broader range of uncontrollable tics occurred. If put to bed, a patient might begin rolling from side to side. A heightened sense of euphoria was reported in some cases and incessant talking in others. In one report, a boy was described as leaping from all fours into the air—like the mechanical toys sold on the London streets called “jumping beetles.” It would have been humorous had it not been for the look of terror on the boy’s face. He could not control himself or stop. As the number of cases increased, one British physician noted that with encephalitis lethargica, recovery, in the true meaning of the word, was becoming the exception rather than the rule.
For the first time physicians not only identified the disease outside of Vienna or Paris, but they also realized the symptoms were changing as it spread. By 1918, doctors in England noticed with alarm that the epidemic was exceeding the severity of the previous years. This would prove to be a foreboding discovery.
Encephalitis lethargica would soon spread to countries covering the globe, from Sweden to India, Egypt to China, Australia to Algeria, Uruguay to Persia. And, yet, there were still far more questions than answers about this global disease.
At one hospital, Queen Mary’s in London, pathologists autopsied some of the fatal encephalitis lethargica patients. They took tiny pieces of brain tissue, soaked them in formalin, and then coated them in wax. The samples, which looked like clouded pieces of ice with a kernel of brain matter in the center, were then placed in a wooden crate, which was stamped and marked as a postmortem sample from that year: PM 1918. Each crate was carried to the basement and stacked on shelves holding other boxes marked the same way, dating all the way back to the turn of the century. There it began, slowly, to collect dust for the next eighty years.
October 2003 era
Scientists trying pieces together whether influenza caused this or not were unsuccessful as Professor John Oxford from Queen Mary’s in London, was unable to isolate the “organism” from the brain segments of the samples collected back in 1920s. We do close this in a positive light (the series will be continuing!) Amazingly, scientific and historical research carried out at the Institute of Neurology and Great Ormond Street Hospital, London, respectively, showed that encephalitis lethargica was almost certainly a post-streptococcal phenomenon. It is thought to be solely a complex immune respose to the microbe.
What do you think this ‘microbe’ was? I mean its so baffling to think that modern scientific inventions are lacking in determing this ‘organism!’ It’s like we’ve uncovered almost every type and form of encephalitis causative agents except this one!
Source:
ASLEEP
THE FORGOTTEN EPIDEMIC THAT REMAINS ONE OF MEDICINE’S GREATEST MYSTERIES
Chapter 1- Chapter 3
https://www.rcpsych.ac.uk/mental-health/mental-illnesses-and-mental-health-problems/catatonia
https://pmc.ncbi.nlm.nih.gov/articles/PMC487753/