It was the last months of 1916. The First World War was about to enter its fourth year. That winter was to be known as “the winter of the German turnips.” The potato harvest, an essential element in the diet of Central Europe, had been ruined and in the market it was replaced by the turnip, with a much lower caloric intake. The situation for the population of Austria and Germany was desperate and hunger was seen at every corner . Historian John H. Morrow describes a Dantesque scene: “On one occasion in Berlin, a starving horse fell to the ground, and instantly a horde of women armed with kitchen knives surged from the apartments, shouting and shoving, they left the animal in the skeleton, and even collected the blood it shed with cups”. With temperatures reaching 30 degrees below zero, there were women who died in the queues waiting for the daily exiguation or in front of the death lists at the front.
This was the situation when a new disease appeared in Vienna , which rapidly spread throughout the world in the following three years. Apparently there had already been cases in the previous winter, but the chaos caused by the bloody war did not prevent awareness of it.
When the Austrian neurologist Constantin von Economo discovered that the brains of all deceased patients had damaged substantia nigra midbrain , it became clear that this was a strange new disease. Von Economo described in detail its symptoms, pathology, and histology, which became known as Von Economo disease. The neurologist published his findings in an article from 1917 with the title “Die Encephalitis lethargica”, and this is how the disease is known today: encephalitis lethargica . This is how he described it: “We are dealing with a type of sleeping sickness that has an unusual prolonged course.
The first symptoms are usually acute, with headache and general malaise. A state of drowsiness then sets in, often associated with delusions from which the patient can be easily aroused. The patient knows how to give relevant answers and understands the situation. This delusional sleepiness can lead to death quickly or within a few weeks. On the other hand, the disease can persist unchanged for weeks, or even months, with periods lasting from a day or even longer to profound fluctuations in a state of unconsciousness, ranging from simple drowsiness to deep coma. It was a disease that mainly affected young people and caused violent behavior in children, leaving those affected unable to move, keeping them in a state of drowsiness that lasted for years and decades .
Asleep, but aware, for decades
Those who survived seemed to make a full recovery and returned to their normal lives. However, most eventually developed neurological or psychiatric disorders , often after years of seemingly perfect health. Sometimes these disorders progressed rapidly, causing profound disability or death; in others it did so very slowly, and they remained ill for years or decades; and sometimes, after the initial attack, it subsided and disappeared. It was all very strange: it wasn’t a coma and it wasn’t a dream either, since the patient was aware of what was happening around him . It was popularly known as sleeping sickness, because they entered a state of minimal consciousness. Many of those who woke up -and the last ones did so in the 1960s- the disease left sequelae reminiscent of Parkinson’s.
In two years the disease spread throughout the world; by 1919 it had already reached the United States, Central America and India , sharing a table and tablecloth with the great pandemic that was ravaging the world, the sadly famous Spanish flu. In this case, the only thing that could be done was to accompany the patient and feed him .
And in 1928 the disease disappeared; it left just as it came, leaving behind a million dead and several million people affected. Neurologist Oliver Sacks recounted the story of several post-encephalitic patients at Beth Abraham Hospital in the Bronx, New York, in his book Awakenings . Sacks described them as awake but not fully aware : “They sat motionless and speechless all day in their chairs, totally lacking in energy, drive, initiative, motivation, appetite, affection, or desire; […] they were as insubstantial as ghosts and as passive as zombies”
To this day we do not know how it appeared or what caused it.
Referencia: Berger JR, Vilensky JA (2014) Encephalitis lethargica (von Economo’s encephalitis). Handb Clin Neurol. 123: 745-61. doi: 10.1016 / B978-0-444-53488-0.00036-5.
