The early 20th century was dominated by tragedy. Titanic, World War I, the Spanish Flu. Millions were killed across the globe in events that we, rightly, remember to this day. However, the pain of the era did not end there. Between 1916 and 1930 an epidemic of Encephalitis lethargica, ‘Sleeping Sickness’ or ‘Sleepy Sickness’ surged through the population. It’s been largely forgotten, despite killing half a million and incapacitating hundreds of thousands. It’s even been accused of contributing to the outbreak of World War II and the rages of Adolf Hitler. If true, its death toll skyrockets.
Constantin von Economo, a neurologist in Vienna was first to report the disease in 1917. He named it after its primary symptoms, severe lethargy and drowsiness. Sleepy Sickness might not sound like the most terrifying plague to befall mankind but the reality for two-thirds of patients was deadly or life-altering.
Victims experienced a wide variety of symptoms, which made it hard to diagnose at first. During the initial acute phase, they would endure fever, movement disorders, delirium, headaches, paralysis, involuntary eye movements and extreme sleepiness. Their exhaustion was so severe that many could sleep for 18 hours a day and even fall into a coma-like state. Some, who entered the chronic phase of the illness, remained frozen, like living statues, for 40 years.
If you’ve heard of Sleeping Sickness you may know it from the 1973 book or 1990s film – Awakenings. These tell the story of Dr Oliver Sacks, a neurologist working with postencephalitic patients in America. He administered a new drug – Levodopa in an attempt to release the victims from their years of immobility. Remarkably, patients were successfully woken after their decade’s long sleep. Tragically, not all had a happy ending. Some experienced violent mood swings and one patient refused to accept that she was no longer in the 1920s. She stopped responding to the treatment.
The Different Forms of Encephalitis Lethargica
Von Economo classified the acute phase of the illness into three separate forms.
Firstly, The somnolent-ophthalmoplegic form. – The most common and, unfortunately, the most deadly version of the disease. Patients experiencing this suffer the extreme exhaustion for which the condition was named. Other symptoms include; Confusion, delirium, fever, meningeal irritation and nerve weakness, leading to abnormal eye movements.
More than 50% of people with this form die. However, survivors are also the most likely to suffer no long term side effects.
Secondly, The hyperkinetic form. – This variation of the disease is easily recognised by the wild and uncontrolled movements of the patients. The main symptom, chorea, is named from the Greek word for dance, chorós. This is because the movements make sufferers look like they’re dancing.
Other symptoms include jerks, contractions of the jaw, oscillations of the eyes, pain in the face and limbs, hallucinations and day-night sleep cycle reversal.
The final form of Sleepy Sickness is the amyostatic-akinetic form. It’s the least common type and notable due to the patients’ complete lack of movement. While their bodies are immobile their minds are fully functioning.
Other symptoms include rigidity, weakness and masked faces, where the muscles of the face stop functioning normally. This prevents the patient from making facial expressions and showing emotions, just as if they were wearing a mask.
The two-thirds who survive the acute phase may initially think themselves lucky. However, this is an exceptionally cruel disease that leaves victims living under a shadow. They’re left waiting for the axe to fall as years or even decades after the initial illness patients can, sometimes quite suddenly, enter the chronic phase. This brings a range of new symptoms that affect bodies and minds. Those who knew the patients in the original epidemic said they were changed from the person they once were. That person would never come back.
Postencephalitic Parkinsonism is the most common symptom of the chronic phase. It leads to extreme stiffness of the upper body. Patients are often rendered completely unable to move their arms. Interestingly, from a neurological point of view, this can often be overcome with an external stimulus, like a ball. A patient can be wheelchair-bound and unable to move or feed themselves. But when a ball is thrown to them they can suddenly catch it and return it without a problem.
Another common effect of the chronic phase is the involuntary movement of the eyes. Patients can enter an oculogyric crisis in which their eyes roll upwards. This can last a few seconds or many hours and occurs in up to 30% of patients. They remain fully conscious throughout the event but are completely unable to control their own eye movements.
The final and most distressing impacts of the disease are the psychiatric disorders. Some might be lucky enough to experience euphoria, and mildly damaging social disorders like silliness and excessive puns. – Yes really. The unlucky ones might demonstrate obsessive thoughts, impulsiveness, violence and cruelty to others. Many survivors from the 1920s found themselves committing a series of crimes such as theft and prostitution.
Another point of interest is that the impulsive behaviour is rarely for the patients own gain. For example, they might steal things they don’t want or need and simply forget about the items or give them away. When questioned past patients would simply say that they were compelled to take the item. Many patients also experience extreme apathy towards life. They lose the ability to find pleasure in anything. Some have reported the apathy to be a blessing as it’s prevented them from becoming depressed about their situation.
Other symptoms can be even more severe and reports from the 1920s are harrowing. Psychosis affects 30% of patients which can lead to violent outbursts, manic episodes and self-mutilation. In one previous case, an 8-year-old girl removed all of her teeth and both of her eyes.
What happened to the Patients of the 1920s epidemic?
When it became clear that patients had entered the chronic and degenerative phase of the illness they were told there was no cure. Many began freezing up, their bodies losing the ability to move over months or years. Lots became unable to talk or even change their facial expression. In one case, a woman could no longer swallow solid food. However, patients retained their intelligence and were fully aware of their surroundings, despite being unable to interact with them. Sufferers were sent to live out the rest of their lives in a chronic disease hospital. They would slowly become less mobile and rely on carers for every need.
In the 1960s a new drug, levodopa was developed for use with Parkinsons patients. One of the Sleepy Sickness sufferers was actually one of the first to hear about the new drug – dopamine – and its potential. He nicknamed it ressurectamine with the hope that it could bring him and his fellow sufferers back to life.
In the Summer of 1969, Dr Sacks administered the drug to a group of near-catatonic patients and the effects were instantaneous. Many rose from their wheelchairs and walked, sang and even danced. Initially, staff and patients were elated. Believing that their prayers had been answered and a cure had finally been found.
Unfortunately, despite the initial miraculous results, this was not the cure. The dopamine did return the patients’ ability to move but it did not mend the structural damage to the brain or remake the neural connections which had been lost. Some became manic, violent and angry. Others remained unable to control their movements and were left to endure extreme tremors or head rolling.
What Causes Encephalitis Lethargica?
During the 1920s more than 9000 scholarly articles were published on the subject but none could convincingly identify the cause. Scientists not only had no idea how to treat it they also had no clue what caused it. A sickness was spreading like wildfire, turning people into motionless zombies and they didn’t know if it was being transmitted by sneezing, petting stray dogs or eating over-ripe bananas.
There were many theories. Some thought that the fact the epidemic occurred at the same time as the Spanish Flu Pandemic was a bit too much of a coincidence. They proposed a link between the two. Others pointed to bacteria, viruses, auto-immune response and toxins as potential culprits. And to be completely honest we’re still not 100% sure.
Virologist, professor John Oxford felt that leaving a previous epidemic unsolved was a recipe for disaster. And I’ve got to agree. When the cases plummeted the world essentially said ’wow, that was horrible. Should we figure out how to stop it in case it comes back… Nah!’. And it’s not like it completely disappeared. Sporadic cases are still appearing and we have no cure. When a 23-year-old was diagnosed in 1993 Professor Oxford decided to act. He located brain tissue samples from the original patients of 1920 and began looking for traces of virus. He believed that the Spanish flu could be responsible but found no trace of any virus in the tissue.
As more patients started appearing, it caught the attention of Dr Russell Dale at Great Ormond Street Hospital. He began tracking down as many cases as he could until he had 20 patients in total. He worked together with Dr Andrew Church trying to find a link connecting all of the victims.
They had a breakthrough. They discovered that every one of the patients had suffered a sore throat before their Encephalitis Lethargica symptoms appeared. This kicked off the hunt for evidence of bacterial infection and they found it. A rare form of Streptococcus bacteria was present in all of their patients. That’s right, a variant of a very common bacteria normally responsible for a simple sore throat.
The theory is that some people experience an extreme immune response to the streptococcus bacteria. An immune response so severe that their bodies start attacking themselves. Did they manage to crack one of the biggest medical mysteries looming over humanity? Many believe so. However, their theory leaves many questions unanswered. Like, why did so many experience this immune reaction all at once? Why did the epidemic end so suddenly? And, why hasn’t occurred again?
How does Sleeping Sickness Kill You?
You might guess that it has something to do with sleeping. However, it’s not the exhaustion that kills you. The disease attacks the brain causing inflammation and bleeding. Sometimes the damage is so extensive it leads to respiratory failure. This is the cause of the 33% death rate.
Autopsies have revealed discrete lesions in the midbrain. Particularly, damage to the oculomotor nucleus explaining the eye movement disorders that many suffer with.
Damage can also be seen in the substantia nigra accounting for the movement and behavioural symptoms.
Did it Play a Role in World War II?
It’s known that Adolf Hitler had symptoms of Parkinson’s disease. Reports date back to 1934 when he was 45 years old. However, some sources indicate that he may have had transient symptoms starting in 1923 at age 34. This would be classed as Young Onset Parkinsonism and would align with the 1920s epidemic of Encephalitis Lethargica.
His most well-known symptom was a tremor in his left hand. He often kept it in his pocket or held a cane to steady it. He wanted to hide it so as not to be seen as senile or suffering from alcoholism. By 1942 he’d reduced his public appearances and would only allow himself to be photographed at angles that didn’t reveal it. By 1945 witnesses described him as shrivelling up like an old man. He stooped and his entire left arm was severely affected. He was only 56. Prime Minister of France, E. Daladier, also witnessed an oculogyric crisis in 1938. He reported that Hitler’s eyes turned suddenly upwards and other instances have been caught on film.
The transient nature of his symptoms, his involuntary upwards eye movements and his left side tremor lead the diagnosis towards postencephalitic Parkinsons. As does the timeline. 50% of diagnoses of Parkinsonism in his age group at the time were encephalitic. The jury is still out however on the cause of Hitler’s Parkinsonism. Was it idiopathic? – spontaneous and cause unknown. Or was it post Encephalitic? – a symptom of chronic Encephalitis Lethargica.
His hatred for others was established before 1918 so the illness could not be held responsible for his horrific acts. But could it go some way to explain his lies, uncontrolled rages, obsession and lack of remorse? Some think so. Frustratingly, only a portion of Hitler’s jaw and skull remains to be examined and so a full autopsy was never completed. It’s therefore incredibly likely that we will never know the cause of his Parkinsonism.
The accusations made against Encephalitis Lethargica and its role in World War II do not end there. It’s been suggested that U.S. President, Woodrow Wilson was another victim of the disease. Before the 1919 peace conference, he’d felt very strongly that any treaty made should not humiliate Germany. He believed that if the treaty was too harsh there would be potential for resentment to build and for the peace to be broken.
He began the peace conference arguing strongly against the French and the proposal of punitive reparations. However, he was struck down by sickness, becoming very ill. Though he recovered, those close to him reported a change in personality, lack of mental agility and said he would tire easily. There were also incidents of paranoia where he believed the French servants to be spies. Despite the worries of his staff, he continued the peace negotiations. He abandoned his original beliefs, yielded to the French demands and the Treaty of Versailles was signed. This has led some to ask the question… Did Encephalitis Lethargica impact the conditions of the treaty leading to the rise of Adolf Hitler and the start of World War II?
Why has it Been Forgotten?
It’s is a vicious and cruel disease that left wards of patients alive but frozen for the world to see. Why is it not in our history textbooks, taught in schools or referenced in relation to the Coronavirus pandemic? Why has it been forgotten?
Well, this is largely due to it being overshadowed by the far more deadly Spanish Flu. The 1918 influenza outbreak spread worldwide and lead to approximately 50 million deaths. One-third of the world’s population was infected with the virus. So while Sleepy Sickness was incredibly deadly the Spanish flu killed 100 times more people and affected everyone.
The second reason is its rapid disappearance. Almost as quickly as it began, the Sleepy Sickness disappeared. As there were no new patients there became more pressing medical issues to focus on.
Finally, it just left everyone baffled. Without an epidemic to get under control it lost the attention of the medical community and they turned their focus to problems they could actually solve.
Is there a Cure?
Not to freak you out, but no. That’s right, the incredibly common bacteria usually responsible for a scratchy throat has the potential to once again start spreading through the population, trapping people in their bodies. Things are not entirely hopeless though. If it is caused by bacteria, it’s likely to respond to antibiotics. Providing antibiotic resistance has not taken hold.
Another small ray of hope is that hyperkinetic patients have shown a response to steroids in some small studies. Potentially offering a new course of treatment for those left with involuntary movements and twitches.
So, have we seen the last of the Sleeping Sickness? Well, the good news is that the disease largely disappeared in 1940. The bad news is that 80 cases have been reported since then. However, due to the vast array of symptoms and lack of definitive diagnosis, it’s likely that some of these were not Encephalitis Lethargica. How these few cases have appeared in isolation instead of as part of an outbreak is not known. The sudden appearance and consequent disappearance remain one of the greatest medical mysteries to date. Therefore no, we can’t rule out another epidemic. However, we are much better equipped to deal with it than we were in the 1920s. So I’m keeping it fairly low on my list of things that keep me awake at night.