The Best of Times, the Worst of Times

Neurology_0

During the late teens and early 20’s of the last century, the War to end all wars (a misnomer if there ever was one) had ended, and the Spanish Flu (that originated in Kansas) had run its devastating course. Perhaps no period in American history saw such abrupt changes to society as the period of the early 20th century.

A few more notable events include President Woodrow Wilson’s “Fourteen Points” speech, his plan to end war forever. The Fourteen Points were enthusiastically adopted by ambassadors worldwide and became the framework for the League of Nations. At its height, the League of Nations had 58 member states. It is notable that the United States never joined, as Americans had suffered civilian casualties in the war, and many citizens wanted to keep America out of European affairs.

On September 16, 1920, suspected Italian anarchists detonated an improvised explosive hidden in a horse-drawn cart on the busiest corner of Wall Street, and nearly 40 bystanders were killed, and over 100 were injured. This was the worst terrorist attack in American history until the Oklahoma City Bombing in 1995. This act of terrorism had repercussions that included a campaign to capture and deport suspected foreign radicals. Over the next few years, thousands of accused communists and anarchists across the country were arrested in raids. The man behind the raids, a young lawyer named J. Edgar Hoover, would become head of the Federal Bureau of Investigation.

1920 was also the start of the influence of the “Lost Generation,” American writers living in Europe fallowing World War I. Books published during this period include Main Street, a critical examination of small-town America by Sinclair Lewis; This Side of Paradise, the debut novel of F. Scott Fitzgerald; and Flappers and Philosophers, Fitzgerald’s first collection of fiction. Fitzgerald also introduced editors to the work of Ernest Hemingway, who would go on to have some success as well. These significant changes in thought were not limited to printed media.

In November of 1920, the first commercially-licensed radio station began broadcasting live results of the presidential election. The real-time transmission of news was unprecedented. The world was captivated by the idea of instant news, and radios, “the talking box”, became very popular; in 1922 alone, Americans bought over 100,000 radios. The next year, they purchased over half a million. By the mid-1920’s, the number of commercial radio stations had grown to over 700, covering virtually every town in America. The Walton’s, a popular dramatic series that played through the 1970’s and early 1980’s, often showed the family gathered around the “wireless”, as the radios were sometimes referred to, listening to popular shows or the news.

While Americans reveled at the end of the war, delighting in the pages of Fitzgerald, Hemingway, and Langston Hughes, a more ominous player was entering the American consciousness. Although not a writer, it would, nevertheless, make an enduring mark on history.

The disease started with a high fever and severe headache, often leading to double vision and slowing of physical response. Often it progressed to a general lethargy, and a need for constant sleep. In acute cases, this was followed by coma and death. The condition caused swelling in the hypothalamus, the part of the brain that controls sleep, and came to be known as Encephalitis Lethargica due to the lethargy that victims experienced.

Today, a century has passed, and it is still not understood where the condition came from or its cause, although many epidemiologists and virologists agree that it was most probably viral. The first known case involved an unknown soldier from the battle of Verdun in 1916. The man was originally hospitalized in Austria, and then later sent to Paris for examination. Doctors examining him were puzzled. He slept constantly, and even when awake, did not seem fully conscious. Soon another 60 soldiers joined him. Despite examination and attempt at treatment, over half died from respiratory failure. Postmortems found the swelling in the hypothalamus.

Almost as quickly as it struck, Encephalitis Lethargica faded from history, even though some victims lived on, often in perpetual sleep. It was not until the 1960s when neurologist and author Dr. Oliver Sacks discovered a group of patients living in a hospital in the Bronx that the condition would once again be the topic of discussion. Working with the patients in the hospital, Dr. Sacks found that most would respond to some form of stimuli. For example, several responded to hearing music, while others would catch a ball if it were tossed to them. They did not, however, throw the ball back nor initiate any actions themselves. Dr. Sacks recounted the story of a patient in another part of the hospital who brought a poodle in. When the dog jumped up on a woman who had always been rigid and unmoving, she suddenly started talking about how she loved animals, and laughed as she stroked the animal. Once the dog was removed, she returned to her rigid, frozen state.

Dr. Sacks originally believed the patients were suffering from some form of Parkinson’s disease, a chronic and progressive movement disorder affecting nearly one million people in the United States. The exact cause of Parkinson’s disease is unknown, and there is presently no cure. However, there are some treatment options, including medication and surgery that help manage the symptoms of tremors in the hands, arms, and face; slowness of movement; rigidity of limbs; and impaired balance and coordination. What is known about Parkinson’s disease is that it seems to involve the malfunction and death of neurons in the area of the brain known as the Substania Nigra. One of the functions of neurons in this area is the production and release of dopamine, a chemical messenger used to communicate with another part of the brain responsible for movement and coordination. As the disease progresses, dopamine production decreases, leaving the person unable to control his or her movements.

Dr. Sacks began treating the Encephalitis Lethargica patients with the then-experimental drug Levodopa, or L-dopa, a precursor to dopamine among other neurotransmitters known collectively as catecholamines. L-dopa increases the concentration of dopamine and was found to be an effective treatment for some of the symptoms of Parkinson’s disease and other dopamine-responsive conditions in the late 1960’s. Using L-dopa, Dr. Sacks was able to temporarily revive some of his patients. Most became ambulatory and talkative; most suffered Parkinson’s-like side effects, but this was preferred to the frozen state they had been trapped in for decades. Some asked to be taken off the medication because they preferred a trance existence to waking up decades later. But even those who wished to maintain the treatment became tolerant of the treatment and returned to their frozen condition.

Dr. Sack’s work with the Encephalitis Lethargica patients is chronicled in the film Awakenings with Robin Williams.  

The cause of Encephalitis Lethargica remains largely unknown, despite causing the death of over 5 million people worldwide. Encephalitis Lethargica has not been diagnosed since the end of the epidemic in 1927. This does not, however, mean it has disappeared from human history. Few things do.

For an interesting look into the amazing neurological writings and work of Dr. Oliver Sacks, see his books The Man who Mistook his Wife for a Hat, Awakenings, and Seeing Voices. 

 

 

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