On this month's Morbidly Fascinating Page:

Most have heard about the 1918 Spanish Flu...but not about the Sleeping Sickness that sometimes followed

See actual patients in the youtube videos HERE and HERE


Above: Constantin Alexander von Economo, a psychiatrist and neurologist, reported in April 1917 about encephalitis lethargica in front of the Vienna Psychiatric Society. His primary description of the illness that raged in an epidemic in Europe and North America was named von Economo encephalitis. 



What was Encephalitis Lethargica (EL)?

Between 1916-1931, a mysterious illness swept across the globe, afflicting millions, killing one-third outright and leaving another third permanently disabled. Encephalitis lethargica (EL, also known as von Economo encephalitis) usually appeared in a person who had had recent symptoms of a sore throat or flu-like illness, and was characterized by the patient appearing to fall into a deep sleep that could last for weeks or months. Some patients were stricken so suddenly that they seemed to lose consciousness while in the midst of eating, their partially chewed food still in their mouths. Others developed oculogyric crises, with many also developing signs of Parkinson's disease. Half of those who survived (and up to 70% of children who survived) emerged with significant personality changes and/or other disabilities.

Was it caused by the Spanish Flu?

Since EL's prevalence in the 1920s, epidemiologic and clinical debate has persisted over whether it was caused by, potentiated by, or merely coincident with the Spanish influenza pandemic. Epidemiologic analyses generally suggest that the disorders were coincidental. However, recent studies now believe EL may have been a result of an immune reaction to strepotococcal infection, or sore throat. Many people contracted sore throats when they suffered from the Spanish Flu, so the two may have been related in that way.

How many people contracted EL?

The exact number of people infected is unknown, but it is estimated that over one million people in the United States contracted the disease during the epidemic, which directly caused more than 500,000 deaths. Most of those who survived EL never recovered their pre-morbid vigor. World-wide estimates are over five million.

What were the symptoms?

Encephalitis lethargica was characterized by high fever, sore throat, headache, lethargy, double vision, delayed physical and mental response, sleep inversion and catonia. In severe cases, patients entered a coma-like state. Many patients experienced abnormal eye movements (oculogyric crisis), upper body weakness, muscular pains, tremors, neck rigidity, and behavioral changes including psychosis. The disease attacks the brain, and left many victims in a statue-like condition, speechless and motionless, unless physically moved by another. Many times a clinician would move the arms, and the arms would freeze in place unless moved again.



Above: two examples of arms remaining frozen in place.



Above: Eye movements could become frozen in place.



Above: Expressions could become frozen in place.

Oliver Sacks


Above: Oliver Sacks was a consultant for the movie Awakenings starring Robin Williams

Oliver Sacks and the L-Dopa Experiment

Patients immobilized by EL were sent to institutions, which is where Oliver Sacks encountered them. In the late 1960s, the experimental drug L-DOPA was first given to Parkinson’s patients, with encouraging results. (Patients with Parkinson’s disease also show damage within the substantia nigra, the same brain area as EL patients.) Sacks eventually decided to treat a group of these patients at Beth Abraham with L-DOPA.

To Sacks’s great surprise, his patients awakened from their frozen states to act and move normally. Tragically, after a period of time L-DOPA’s effects began to wear off and the patients no longer responded to the drug, leaving them just as frozen inside their bodies as they had been before. In many cases, the drug not only wore off, but the patients began to show “strange, unstable states,” as Sacks put it. This story would become the basis of Sacks’s 1973 book, Awakeningswhich was later made into a movie.

What is L-DOPA?

L-Dopa—also known as levodopa, 3-hydroxy-L-tyrosine, and dozens of other names—has been known for at least 90 years as a naturally occurring amino acid that is the biological precursor to the neurotransmitter dopamine. L-Dopa is administered to increase dopamine levels in Parkinson’s disease patients; unlike dopamine, it can cross the blood–brain barrier.


About the movie titled Awakenings

The 1990 movie Awakenings is a dramatization of Dr. Oliver Sacks' 1973 memoir of the same name — and the true story behind the semi-fictional Dr. Sayer is just as fascinating. In 1990, viewers were treated to a dramatic story starring Robin Williams (who, even in a more serious role, added a touch of his particular sense of humor) and Robert De Niro. The pair play doctor and patient in a story that’s equal parts heartwarming and heartbreaking.

Awakenings follows neurologist Malcolm Sayer (played by Robin Williams), who, in 1969 while working at a hospital in the Bronx, began extensive research on catatonic patients who survived the 1917-1928 epidemic of encephalitis lethargica. Sayer learns of a new drug that helps patients suffering from Parkinson’s disease and believes it could be of use on catatonic patients.

He runs a trial on patient Leonard Lowe (played by Robert De Niro), who completely “awakens” and starts to show major improvements, but the experiments soon come across some obstacles that threaten the life quality of the patients who were just starting to deal with a new life in a new time. As detailed in Sacks' memoir, the drug and experiments shown in the movie are actually real, despite Awakenings being a fictional story.

Dr. Sayer is based on Dr. Oliver Sacks, a British neurologist, naturalist, historian, and writer, who authored various best-selling books recounting case studies of people with neurological disorders, including himself. Sacks suffered from prosopagnosia, also known as “face blindness,” a cognitive disorder of face perception that affects the ability to recognize familiar faces, including one’s own. This disorder was the basis for his book The Man Who Mistook His Wife for a Hat, published in 1985. Over a decade earlier, he wrote a book about the Awakenings true story, recounting the life stories of the victims of the 1920s encephalitis lethargica epidemic.

Leonard Lowe


Above: Leonard Lowe was played in the movie by Robert De Niro

Leonard Lowe, as well as many other patients, initially had a positive reaction to the drug and fully awoke, but just like in the movie version of Awakenings, Leonard began to become paranoid and developed severe tics, eventually regressing to his earlier catatonic state and passing away in 1981.

Could EL happen again?

Some doctors speculate that the encephalitis lethargica virus is only lying dormant. Oliver Sacks, the New York neurologist who wrote the book "Awakenings" upon which the film was based, warns that there is no reason to think that the mysterious virus is extinct.

Richard T. Johnson is the director of the Department of Neurology at Johns Hopkins School of Medicine and neurologist in chief at Johns Hopkins Hospital. "Were encephalitis lethargica to appear again," says Johnson, "scientists would be much better prepared than their colleagues were 75 years ago to stem an epidemic. We would be able to isolate a virus and devise a treatment," he says. "In the 1920s, the most they could do was inoculate rabbits and monkeys. There were no cell culture systems, no electron microscope and no molecular methods to look for the virus. We are much better equipped today."

See more HERE