Get Well Soon

by Jennifer Wright

  I would never disparage von Economo, but I’m not sure anyone needed all of the Goethe and Homer and the numerous degrees to figure that out.

  When faced with the need for more specifics, von Economo consulted his mother. She told him about a sleeping sickness that had struck parts of Europe in the 1890s that was known as “Nona” or “the living dead.” In 1929 von Economo would claim, “Today, we can with some certainty maintain that Nona and Encephalitis Lethargica are identical diseases.”7

  Describing sufferers as “the living dead” is extremely alarming and unfortunately seems like an accurate characterization of the patients von Economo was seeing.

  EL didn’t necessarily end in death. Only four of von Economo’s initial eleven case studies died. Admittedly, that is not a great ratio.8 But after weeks or months many patients seemed to recover. No doubt von Economo and the doctors treating them, and certainly their families, rejoiced!

  They rejoiced too soon.

  Though the recovered patients might have seemed “normal” for a prolonged period, even years, few recovered permanently or became their old selves again. Children who had suffered from EL often emerged with altered—for the worse—personalities. Reports surfaced of children who had always been docile turning into truly terrifying adults years after they had “recovered” from the disease. Some children who had been gentle before EL smeared feces on the walls. Some tried to murder their siblings. Some tried to rape and mutilate other children. One tried to bite off another child’s penis. In 1928, in one famous case, a sufferer pulled out all her teeth and plucked out her eyes. One doctor claimed, “When the child’s personality changes so dramatically, they’ve ‘lost’ the original child forever.”9 Even more tragically, unlike psychopaths, the children were largely horrified by their impulses. They even asked to be put away for their own and others’ safety, and begged doctors to try to understand how little control they had over their impulses. The lucky ones ended up in asylums. The unlucky ones died or ended their lives in prison.10

  The aftereffects of EL seemed to manifest differently in adults. The most common chronic effect among adults was the development of Postencephalitic Parkinson’s disease. After seeming to recover, many patients then lapsed into an unresponsive state and remained that way for years, prompting von Economo to refer to them as “extinct volcanoes.”11 One patient described the state: “I ceased to have any moods. I ceased to care about anything. Nothing moved me—not even the death of my parents. I forgot what it felt like to be happy or unhappy. Was it good or bad? It was neither. It was nothing.”12 Immobile, unresponsive, and unable to fend for themselves, many would be confined to institutions, where they would need constant care for decades.13

  If von Economo’s theories about Nona are to be believed, there had likely been outbreaks of something similar to EL before. But no one recorded the condition in such scientific terms before the twentieth century. Molly Caldwell Crosby, the author of Asleep: The Forgotten Epidemic That Remains One of Medicine’s Greatest Mysteries, notes that numerous folktales point to incidences of a disease that closely resembles the one von Economo encountered. You can probably name a lot of them yourself. Let’s play a game where we can see how many folktales involving sleeping we can rattle off in under a minute:

  1. Sleeping Beauty

  2. Snow White

  3. Rip Van Winkle

  Okay, that is three. Good for us! Are there more? You bet.

  Physicians also reported people falling into catatonic stupors with surprising regularity in mid-nineteenth-century Germany. Those reports inspired two of Edgar Allan Poe’s most famous stories, “The Fall of the House of Usher” and “The Premature Burial.”14

  Von Economo’s new scientific methods—he had a microscope!—allowed him to pinpoint the physical cause of the disease more accurately than ever before. In his initial paper on the disease, he ruled out toxins caused by food poisoning or malnutrition by determining that his patients had no gastrointestinal disturbances. He researched his patients’ histories to make sure they hadn’t been exposed to poisons (for example, poison gas complications experienced by soldiers). He drew spinal fluid to ensure they were not suffering from influenza. They tested negative for syphilis. There was no polio epidemic in Vienna at the time, so it seemed unlikely they had polio. Von Economo did, however, find spherical bacteria formations when he took samples from his deceased patients of the membrane that encased the brain. Those samples would prove especially important as he would later go on to demonstrate that the disease could be transmitted to a monkey through insertion of that bacteria into its brain tissue.15

  And—this is especially interesting—due to the location of the patients’ brain damage, von Economo theorized that the affected brain region, the hypothalamus, regulates sleep. He would be proved correct on that supposition, although not for another seventy years.16

  Von Economo published his paper on April 27, 1917. Timing being everything, another scientist named Jean-René Cruchet had published a paper on the condition a few days prior. In it he described soldiers he had treated who seemed to be suffering from a disease similar to EL. He was not happy about von Economo getting credit for any research on the disease and suggested that, instead of calling it encephalitis lethargica, the world should call it “the disease of Cruchet.” Von Economo countered that maybe one of Cruchet’s forty patients actually had EL; the rest were probably simply suffering from wartime maladies.17 For a time, in Vienna the disease was known as von Economo’s encephalitis lethargica and in France as Cruchet’s disease. I am not paying much attention to Cruchet because he did not race balloons. If you want to call EL Cruchet’s disease, you can.

  Overseas people really weren’t calling it much of anything.

  If you have never heard of EL—and are marveling that you never knew of a disease that turns people into comatose “living dead” versions of their former selves or children of the damned—well, a lot of people didn’t know about it even as the outbreak was happening. Not because the death toll of EL wasn’t significant. It was. But it was overshadowed by World War I. (The war overshadowed everything during those years.) And if people weren’t paying attention to the war, they were focused on the epidemic of the Spanish flu, which was occurring at the same time. And, as we know, the death toll from that was shockingly high.

  Now, you might think to yourself, Those two diseases occurred at the same time—maybe they were related. Could EL be a symptom of influenza? You are smart! I, too, considered that possibility, but it didn’t exactly pan out. While there is still dispute as to whether the two are connected, von Economo at least strongly felt they weren’t. His patients tested negative for influenza. The first case of EL was studied in 1916, before the first reported case of the 1918 influenza epidemic. Moreover, von Economo noted that the high fevers, muscle pain, and upper respiratory symptoms (coughing or sneezing) that accompanied influenza were absent in the presentation of EL.18 If you are interested in following a line of thought on interrelated diseases, though, some scientists today think that EL is related to streptococcal bacteria, so that’s a fun thing to consider when you get strep throat.

  In 1918 the first cases of EL appeared in London, baffling clinicians there, who had minimal awareness of the outbreak in Vienna. Professor A. J. Hall wrote:

  They could not find amongst the diseases of their acquaintance a foot which would go into this newly found glass slipper. A search was therefore made amongst the diseases which, so to say, did not move in Court circles, and were not personally known to the seekers. Amongst those it was at first thought that the owner of the slipper had been found, and that her name was Botulism. After many painstaking efforts to force her foot into the slipper, it was found to be impossible, and the attempt was abandoned. Botulism was not Cinderella.19

  No, of course it wasn’t, because it was EL. Physicians, who initially declared that they were dealing with a wholly new disease,20 came to their senses and recognized: “For identification a
nd description, it was decided to follow von Economo in terming the illness encephalitis lethargica, a name which has the right of priority and indicates a characteristic clinical feature.”21

  Admittedly, some of the symptoms that sufferers were displaying in London were different from those that had first been observed in Vienna. For instance, the London incarnation of the disease sometimes manifested with bouts of hyperkinetic energy. One boy jumped up and down on all fours, obviously terrified and not in control of his actions at all. Others talked incessantly. But most presented with the same lethargy and sleepiness that had been observed by von Economo in Vienna. And in a supercreepy detail, a lot of the patients slept with their eyes open, which isn’t necessarily scarier than someone jumping uncontrollably but really adds to the whole “we’re in a horror movie” feel.

  In 1918 there were 538 cases of EL in England. The death rate crept up to 50 percent—mostly from paralysis of the respiratory system.22 And, even more terrifying, it had by now become very clear that among those patients who survived, most weren’t going to return to their normal selves. They would live out their lives in institutions.

  The disease kept spreading. It made its way to China, India, Australia, Sweden, Uruguay—all the places. In New York a neurologist met with a teenage girl’s family. She had been sleeping for two months and could respond only insofar as she could blink when told to or squeeze a hand on command. He regretfully told her family that nothing could be done. As he told them, the girl began to cry.

  There were some happier cases. In 1920 a woman with EL remained asleep for over three months. Her husband, aware of her great love of music, then hired a musician to play Schubert by her bedside. She woke up immediately and went on to make a full recovery. The London Times claimed, “This is the first case in the records of the New York Health Department of a cure in a case of Encephalitis Lethargica.”23 That may have been, and it’s a great story, but playing Schubert was not an especially successful cure with anyone else.

  Trials for a vaccine began. The Matheson Commission for the Study of Epidemic Encephalitis was founded in 1927, endowed by the industrialist William J. Matheson, who suffered from EL. Josephine B. Neal, who led the research, wrote four influential papers on the disease and became the world’s leading expert on EL—but the group had no success in finding a vaccine or a cure. Neal noted “how difficult it was to prove the efficacy of a treatment for EL because of EL’s course of natural remissions and progressions.”24 Though Neal continued to work on EL until her death in 1955, in 1942 she sadly wrote to all her patients that the commission had been shut down due to lack of funding.25

  Twenty-six years after the initial cases of the disease, A. J. Hall’s lament—“It may be that generations which follow us will see clearly where we can only grope darkly”—still seemed applicable.26 This poignant sentiment must resonate with medical researchers throughout every age.

  But … Remember that cool graph at the beginning of this chapter? Medicine changed really fast!

  Stories like the one about the influenza epidemic of 1918 are terrifying because we have become complacently accustomed to thinking that most diseases that killed people in the past are now treatable. That’s not the case with influenza! And that’s not even the case with EL. However, luckily for the sufferers of EL, medicine progressed so quickly in the twentieth century that its advances offered some relief during their lifetimes.

  If that does not immediately take your breath away, consider that a cure for syphilis only came about after people had been having noses grafted onto their syphilitic faces for five hundred years. In the twentieth century (and even now), a disease could be identified, dismissed as tragically untreatable and best left to future generations, and then fifty years later scientific advances would allow for radically, dramatically different treatments. At the beginning of the EL outbreak, it was a really big deal that von Economo had a microscope. In the following years the pace of developments in chemistry and medicine would be breathtaking.

  The story of EL is intertwined with the work of Oliver Sacks. Sacks was the coolest man ever to walk the earth. Sorry, Constantin von Economo, I have a fickle heart.

  Oliver Sacks was a neurologist and a wonderful author of medical narratives like The Man Who Mistook His Wife for a Hat. If you haven’t read it, do! You are reading this book, so you presumably like tales about strange medical conditions. All of his writing makes Sacks out to be a compassionate, smart, funny man, which is a view supported by his colleagues and patients and all who knew him.

  In his nonfiction account Awakenings, Sacks discusses how he “awakened” some of the longtime sufferers of EL. I will let him set the scene because I have nothing on him in the “writing nonfiction about medicine” game. Sacks explains in the foreword to Awakenings that early in 1969

  these “extinct volcanoes” erupted into life. The placid atmosphere of Mount Carmel was transformed—occurring before us was a cataclysm of almost geological proportions, the explosive “awakening,” the “quickening,” of eighty or more patients who had long been regarded, and regarded themselves, as effectively dead.27

  Sacks recalled it as “the most significant and extraordinary event in my life.” Yes, Oliver Sacks basically brought people back from the dead! Someone get out the confetti! Pop the champagne because we are bringing Lazarus back and you know that guy loves bubbly!

  Lazarus is wearing a cozy outfit and he is ready to par-TAY.

  Sacks’s patients’ miraculous awakenings seem especially unlikely when you consider the fact that by the 1960s, EL wasn’t even covered in medical school. This would have made von Economo really angry, as he once noted: “One thing is certain: whoever has observed without bias the many forms of encephalitis lethargica … must of necessity have quite considerably altered his outlook on neurological and psychological phenomena … Encephalitis lethargica can scarcely again be forgotten.”28 Wrong, Constantin. People often take whatever information about the world makes them feel scared or dumb and stuff it away as quickly as possible.

  It must therefore have been something of a surprise to Sacks to begin his appointment at Beth Abraham Home for the Incurables (renamed Mount Carmel in his book) and find eighty postencephalitic patients. He noted: “Almost half of these patients were immersed in states of pathological ‘sleep,’ virtually speechless and motionless, and requiring total nursing-care; the remainder were less disabled, less dependent, less isolated, and less depressed, could look after many of their own basic needs, and maintain a modicum of personal and social life.”29

  Sacks first attempted to give these patients a basic sense of self by trying to find their lost relatives and then persuading those people to visit. He also formed close relationships with them himself. He found that some of the patients were more responsive than initially considered. However, they were only reactive if stimulus seemed to come from the outside world.30

  As depicted in the movie Awakenings (1990), if you threw patients a ball, they could catch it. That wasn’t just the case with patients at Beth Abraham; a patient in the Highlands Hospital in London, if thrown a ball, would play with it so energetically that the staff nicknamed him Puskás (after the great Hungarian soccer player). Other patients could walk if you supported them. Unfortunately, they had no will to do so on their own. Sadly, basic responses to the outside world are not nearly enough to constitute a full life. There is a big difference between being able to catch a ball when someone tosses it at you and being able to say, “I would like to play with a ball now” and then get one.31

  Most of Sacks’s fellow doctors had dismissed these patients as chronic cases for which little could be done. The fact that Sacks took the time to understand their symptoms and interact with them at all is great. Let’s pause to say, “What a nice guy!” And then L-dopa came along. In 1969 Sacks began testing large doses of a new promising anti-Parkinson drug called Levodopa or, more commonly L-dopa, on the patients at Beth Abraham.

  The results were extr
aordinary. Patients who had been barely responsive for years remarkably returned to their old personalities, admittedly in much older bodies. A man who had raced cars until the 1930s awoke and, although he could no longer take to the road, began drawing highly realistic cars. Sacks reported: “His cars were accurate, authentic, and had an odd charm. When he was not drawing, he was talking, or writing—of ‘the old days’ in the twenties when he was driving and racing.”32 One woman woke and, though she could inform the doctors of the dates of momentous occasions like Pearl Harbor, believed that she was still age twenty-one and that the year was still 1926. Sacks wrote that she “spoke of Gershwin and other contemporaries as if they were still alive; of events in the mid-twenties as if they had just happened. She had obsolete mannerisms and turns of speech; she gave the impression of a ‘flapper’ come suddenly to life.” She could, understandably, not imagine what it was like to be older than 21 because she had never done any of the things that we associate with aging.33

  Sacks later recalled: “What I … regret, and what many of the patients did as well, was that this [L-dopa] was not available 10 or 20 years before, when they had not lost so many of their connections to the world.”34 He claimed that the twenty-one-year-old flapper was one of the patients whom he found the saddest, but I think that party-girl flappers are resilient and generally happy to be alive.

  No wonder this phenomenon was made into a movie! How terrible to awake feeling you had thirty or forty years of your life ripped away from you. But how amazing to see these people suddenly return to their full selves.