Asleep: The Forgotten Epidemic That Remains One of Medicine's Greatest Mysteries (3 page)

In fact, medicine was a second career for von Economo, and he continually turned down promotions to pursue other interests. His first love was flying—in balloons, then in planes. He was the first in Vienna to attain an international aviation license. Von Economo also loved anything to do with science and learning in general. He had received a degree in engineering before achieving one in medicine. He was something of a left-brained Renaissance man—the kind of scientist who kept copies of the New Testament, the
Odyssey,
and Faust on his bedside table.

As for his professional life, von Economo didn’t really need one at all—although born in Romania, von Economo came from Greek aristocracy and married the daughter of an Austrian prince, becoming Baron Constantin von Economo. He lived a cosmopolitan life, traveling between estates in Italy and Greece and his home in Austria. His appearance was a nod to his Greek heritage—chestnut skin, dark hair parted and swept to one side, black eyes beneath heavy lids, a thick mustache that turned at the edges. He looked calm and dignified, a man who would be at ease in any company—whether European royalty or patients of a psychiatric clinic.

V
on Economo spent months flying with the Austrian air corps, but when he learned of his brother’s death in battle, he returned to Vienna to be with his grief-stricken parents. At their behest, he reluctantly transferred to a medical command in the city away from danger. When his wife asked him if he missed aviation, he thought for a moment and replied, “No, there is no longer so much new to do there.” The psychiatry clinic, and medicine as a whole, still provided plenty of uncharted territory. It was at the Wagner-Jauregg clinic that von Economo met his first sleeping sickness patient.

On that cold winter day in Vienna, in a hospital that looked out upon the graying snowcaps on rooftops and a sky laced with chimney smoke, the clinic was filling up with these patients literally falling asleep where they stood. Family members complained that a patient might fall asleep at dinner with food still in his mouth; or parents would shake and shout at a child who would not wake from her sleep.

Before long, the novelty of this disease evolved into something much more frightening—some of the patients fell asleep indefinitely, eventually dying. The mortality rate of this strange new disease was as high as 40 percent in the cases at the Vienna clinic. When the doctors autopsied the first victims, they found significant swelling and damage to the midsection of the brain—the part of the brain that controls 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.

Von Economo could find nothing like this sleeping epidemic in any of the standard literature. Some papers mentioned a mass suicide in Scotland that pointed to a sleeping sickness disorder followed by hysteria. The patients, as a group, jumped from their windows. Similar instances of collective hysteria surfaced in history, all related to sleep patterns. And von Economo did have a vague memory of an epidemic of sleep, called
“nona,”
when he was a child. No medical texts describing the outbreak were widely published, but it was recent enough still to circulate in the memory of the previous generation. The sleeping sickness had occurred in Italy and other parts of Europe during the 1890s just after a notable flu epidemic. Von Economo asked his mother, who remembered when the nona epidemic struck in western Europe, and she told him in detail about the symptoms—they sounded hauntingly similar to what he was seeing in his clinic in Vienna. The peasants called the disease “La Nonna,” which means “the grandmother” in Italian, but in the cities, the disease became known as nona, or “the living dead.” At the time, it was thought to be a neurological complication of the flu.

The nona outbreak became the first time doctors questioned whether or not influenza and this sleeping sickness were somehow connected, whether the flu had led to these unusually lethargic patients, or if this strange new disease was an epidemiological foreshadowing of an influenza pandemic. More than one hundred years later, the question would remain.

V
on Economo quickly pulled together a paper to present in spring to the Vienna Psychiatry Society, and on April 17, 1917, he announced that he had identified this new disease. His paper was published within days of the one published by the Parisian surgeon, Jean René Cruchet. In Vienna, the disease would become known as von Economo’s encephalitis; in France, the disease was named for Cruchet. To the world at large, a less controversial name developed that meant, literally, a swelling of the brain that makes one sleepy, or encephalitis
lethargica.

Of course, there was some opposition in Vienna to von Economo’s theory of the disease, particularly among psychoanalysts, who believed mental illness to be a result of deep-seated psychological problems, not an actual disease. But von Economo warned that any psychiatrist who based his studies entirely on the subconscious, ignoring organic causes like disease, would build his theories on sand and watch the “psychological constructions collapse like a house of cards.”

Von Economo began to discern a pattern of brain damage in his autopsies, and he suspected a virus was to blame. Before the age of the electron microscope, there was no way to identify a virus aside from infecting another animal and awaiting the outcome. He found that brain tissue of the encephalitis patients could pass the disease into monkeys, producing similar symptoms and suggesting that the culprit was both microscopic and virulent.

Von Economo also made an important discovery about the brain. He theorized that a certain part of the brain, the hypothalamus, was regulating the sleep in his encephalitis patients. The hypothalamus, small, almond-sized, and located in the middle of the brain, controls several automatic functions like body temperature, hunger, thirst, and sleep cycles. Depending on where the encephalitis lethargica damaged the hypothalamus, too much sleep or too little resulted. He believed the hypothalamus was the part of the brain responsible for two other known sleep disorders as well: narcolepsy and insomnia. Von Economo’s theory was visionary to say the least; seventy years would pass before advanced medicine could prove his theories true.

As the European doctors squabbled over what was causing encephalitis lethargica, how it spread, and who discovered the first case, the disease trumped the scientists. It vanished. No new cases appeared. The doctors felt relieved, fortunate even, that this seemed to be the end of a terrifying epidemic. This epidemic had been so frightening not because of the high death toll or the clip at which it spread, but because physicians had so few answers. At the same time, they must have felt some disappointment. The disease had come and gone before they ever had the chance to solve the mystery—it was a feeling that would be echoed again and again.

I
n Europe and elsewhere, fewpeople worried about the little-known epidemic of sleep. They were preoccupied with the rapidly spreading, extremely virulent influenza breaking out among troops in Europe and the United States. There is no definitive calculation, but the number of people about to die of influenza in 1918 would fall between 20 million and 100 million worldwide. The sleeping sickness faded quietly behind the voracious flu pandemic, a drop of water in a tempest. Surely, encephalitis lethargica was a bizarre and isolated event, like the previous ones in history. A sudden, mass hysteria. Short-lived and soon forgotten.

Then, an ill patient—delusional, feverish, and lethargic—arrived at London Hospital.

CHAPTER 3

The London Outbreak

W
orld War I, though sparked by an assassination, was really a war fought over alliances and power struggles. Two countries, Austria and Serbia, went to war. Thanks to complicated, and sometimes outdated, alliances, nine other countries went to war as well. Britain had an old but steadfast agreement to ally with France in the case of a war. So, during the first two years of World War I, Britain sent a respectable number of troops into Belgium and France. It wasn’t long before the Western Front became overwhelmed, and the British engaged fully.

As disastrous losses at Verdun took their toll on the French soldiers, Britain decided to send her own troops into northern France to relieve some of the pressure by distracting Germany in battle. The British and German troops met on a field near the river Somme in a battle that cost Britain more losses in a single engagement than in any other war. The British plan was costly, but ultimately effective. British troops finally began to overwhelm Germany.

In a fateful turn of events, legend has it that a British private came upon a wounded German courier during the battle. In the face of defeat, the courier did not even reach for his rifle. Looking him directly in the eye, the British soldier could not bear to pull the trigger on a wounded man in the field. Sympathy and respect for life overtook him, and he lowered his rifle. It would prove to be a regrettable decision; the German courier’s name was Adolf Hitler.

Hitler was hospitalized both after the battle near the Somme as well as during a later engagement. During a hospital stay toward the end of the war, his erratic and volatile temperament caught the notice of physicians and patients. Most of Hitler’s medical records were later destroyed, so no complete list of his symptoms or diagnosis exists, but several different diseases have been suspected: the whole spectrum of mental illnesses, syphilis, hysterical blindness, mustard gas poisoning, and encephalitis lethargica. Though it seems unlikely that any medical diagnosis can ever explain evil.

A
s Britain’s involvement in the war escalated, and more and more troops landed along the coast of France, injured or ill British soldiers on the front were sent home—by train to the coast then aboard the hospital ship and back across the English Channel. With so many troops traveling back and forth to England, every effort was made to prevent soldiers from carrying disease with them as well. Sick soldiers in the field hospitals had their uniforms removed and disinfected before they boarded the train for the coast. Once across the English Channel, the soldiers carrying infectious disease were sent to the clearing hospital to wait out the illness before they returned on sick leave to their homes in London or elsewhere in England.

Nonetheless, the exchange of troops became an exchange of microbes as well. As early as 1916, a British doctor named A. J. Hall read reports of a new illness spreading among the troops in France— one that sounded very much like what von Economo and Cruchet had reported.

The path between Verdun and the Somme was but one 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 in the United Kingdom. One ship after another departed from the coast and crossed the ragged whitecaps between France and England.

W
hen patients with drooping eyelids, slurred speech, and muscle weakness first began filling the waiting rooms of London clinics, sausage was blamed. With a wartime shortage of meat, questionable substitutes were often used, so botulism was the first diagnosis. Other foodborne toxins were investigated as well. To complicate things further, families who
had
shared the same food did not see multiple cases, but infants, who were exclusively breastfed, with no exposure to tainted foods,
were
experiencing the strange symptoms. When London Hospital failed to find the bacterium that caused botulism, it put an end to the botulism scare. After that, tear gas and mustard gas were suspected, or even a new type of chemical warfare. Finally, British physicians simply declared this to be an entirely new disease.

It would have been helpful to the British doctors to have some contact with Austria or even French physicians at this time and to know about the epidemic of sleep that had arisen and evaporated, but the war severed most medical communications. The war was disseminating the disease, but not the information about it. Von Economo’s paper, as well as the one published by Cruchet, received little attention across the Channel. Physicians were too overwhelmed with war injuries and fatalities to pay much attention to anything else. Yet encephalitis lethargica, which had taken root in Europe, was spreading as steadily, slowly, and unnoticed as a creeping vine.

Working with the Ministry of Health, the British physicians pulled old medical files, books, and published papers to search for information; they reviewed historical documents. Eventually, they traced cases of unexplained sleep as far back as 1657 in Copenhagen, 1658 and 1661 in England, and 1775 in London. In fact, those three epidemics of sleeping sickness during the seventeenth century may very well have 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.”