By Diane Sparacino, Staff Writer
When Robert De Niro’s fragile character, Leonard, suddenly wakes from his 30-year slumber in the 1990 film, Awakenings, it was a heartbreakingly memorable moment. Following an influenza pandemic, Leonard and a group of similarly stricken patients had essentially been locked away from the world for decades, crippled by a mysterious disease that all but rendered them lifeless – unable to move or speak. After an observant doctor, (played by Robin Williams), takes a chance on a new drug, nothing short of a miracle takes hold; a mass awakening pulls the seemingly catatonic patients into consciousness – at least for a short time (Ebert, 1990).
Set in the Bronx in 1969, the story was based on Dr. Oliver Sacks’ real-life experiences working at a psychiatric hospital with a group of men and women suffering from encephalitis lethargica (EL). At the time, a brand new medication (L-dopa) was making the rounds and Sacks took note (Sacks, 1973; 1990). Originally developed to treat Parkinson’s disease, L-dopa (Levodopa) controls symptoms by raising the levels of dopamine in the brain. Though its effectiveness reportedly decreases after 4-5 years of use, more than 40 years after it was first introduced, it remains a favored treatment for Parkinson’s (NYT, 2011).
Today, new “awakenings” of a different sort continue to sprout up all over the world -- most recently as a result of popular sleep medications Ambien and Zolpidem (Interlandi, 2011). At the same time, the EL pandemic and its aftermath continue to puzzle researchers. Experts hope that history may provide clues to help them better understand brain function and a whole host of brain-based diseases.
A Global Health Crisis:
Just as WWI was drawing to a close, the Spanish Flu pandemic of 1918-19 struck. A disaster of epic proportions, recent estimates have put the flu’s death toll at 60-100 million worldwide. By contrast, the Black Plague of the 1300s killed an estimated 24 million worldwide (National Geographic, n.d.). The fast-moving epidemic came in three waves, with the mildest strain erupting in mid-year 1918, targeting the very young and the elderly. In the fall of 1918, a second wave brought a virulent strain resulting in a severe outbreak which attacked normally healthy individuals in the prime of life. In the spring of 1919, the final wave hit (HHS, n.d.).
An estimated 675,000 Americans died; at the time, the total population of the United States was only 105 million (HHS, n.d.). Some cities reported losing as many as 10,000 citizens per week to the disease (National Geographic, n.d.). In the end, the Spanish Flu (“La Grippe”), infected one-fifth of globe’s total population; twenty-eight percent of all Americans were infected, and 10 times the number who had died in WWI lost their lives to the disease. It has been cited by some as “the most devastating epidemic in recorded world history” (Billings, 1997; 2005).
Just after the last wave of the Spanish Flu died out in 1919, a new pandemic began. Known as “Sleepy Sickness,” because victims experienced lethargy and muscle weakness as the illness took hold, EL was a debilitating, Parkinson-like disease that affected brain function and all but shut-down motor skills. Rather than targeting the elderly or very young like many diseases, most of those stricken with EL were younger than 30 years of age (NeuRA, 2011). As it swept the globe, EL killed nearly one million and left millions more essentially frozen-in-time, living in virtual isolation for decades. Many ended up in institutions. Doctors did not know how to treat EL, let alone how to prevent it (BBC News, 2004). The largest outbreak occurred in the Northern Hemisphere from 1920-21; in 1924, the UK was hit with its own epidemic, but by the 1930s the outbreak finally dissipated (NeuRA, 2011). However, EL has not entirely disappeared, as scientists have logged nearly two dozen cases in England since the early 1990s (BBC News, 2004).
Today, nearly a century since it took hold, scientists continue to explore the causes of the EL epidemic and its similarities to other brain-based diseases. The Spanish influenza pandemic has been suspected, but scientists have been unable to connect the two. Conceding as much in The Journal of Neurovirology (2008), researchers reported, “Epidemiologic analyses generally suggest that the disorders were coincidental . . . there is little direct evidence supporting influenza in the etiology of EL” (McCall et. al., 2008). In the end, researchers admit that the lack of an identified cause is rather unsettling. The search for answers continues.
At Neuroscience Research Australia [NeuRA], brain expert Dr. Paul Foley is among those studying the EL pandemic. Using EL as a model to explore the possible link between infection and the brain, Foley and his colleagues hope to learn more about other brain-based diseases, including Parkinson’s, Alzheimer’s and multiple sclerosis (NeuRA, 2011).
Which brings us back to present day “awakenings,” the first of which was reported in 1999. A South African man, Louis Viljoen, was in a vegetative state for three years, after being struck by a truck. His doctor prescribed the sleep aid Zolpidem to help his patient with a bout of apparent insomnia. Only 20 minutes after Viljoen ingested the sleep aid, his once-vacant stare found focus and he miraculously began to talk (“Hello, Mummy” were his first words). He was also able to control his facial muscles and limbs, but only for a few hours at a time. Day after day, Zolpidem would revive the patient for short stints. Viljoen’s recovery was remarkable and steady. He could recount conversations that took place when he was thought to be vegetative, he recognized faces and voices and he was able to remember fragments of his former life. Viljoen was speaking in complete sentences and laughing at jokes. Eventually, the medication was no longer needed (Interlandi, 2011).
As doctors have tried similar therapies on their patients over the years, the results have been mixed. The medication tended to wear off quickly and didn’t work for some, but for others, the “effects were profound” (Interlandi, 2011). In the United States, an estimated 200,000 patients live in vegetative states. Research suggests that as many as 10% of the brain-injured population could be helped by similar medications, but only a small percentage of those would realize a recovery as profound as in Viljoen’s case. Last year, Philadelphia area scientists embarked on a large-scale clinical study of consciousness disorders and the drug Zolpidem (Interlandi, 2011).
As unexpected and joyful as an awakening may be for a patient’s family and loved ones, there are of course a host of moral, legal and ethical challenges to bear. For the medical profession, might this be the light at the end of the tunnel for patients once thought to be beyond the scope of consciousness? Then there are the quality of life issues: How much can a person regain of his/her former self after existing in a vegetative state for years? As the research begins to unlock the secrets of the consciousness, there are certain to be as many questions as there are answers.
BBC News. (2004). Mystery of the forgotten plague Retrieved from: http://news.bbc.co.uk/2/hi/health/3930727.stm
Billings, M. (1997, rev. ed. 2005). The Influenza Pandemic of 1918. Retrieved from: http://virus.stanford.edu/uda/
Ebert, R. (1990). www.rogerebert.suntimes.com. Awakenings (review). December 20, 1990. Retrieved from: http://rogerebert.suntimes.com/apps/pbcs.dll/article?AID=/19901220/REVIEWS/41223003/1023
Interlandi, J. (2011). The New York Times Magazine. A Drug That Wakes the Near Dead. Retrieved from: http://www.nytimes.com/2011/12/04/magazine/can-ambien-wake-minimally-conscious.html?_r=2&adxnnl=1&hpw=&adxnnlx=1325208392-wrVjzKYnlSB9Ld72Hb2GRw
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Neuroscience Research Australia [NeuRA], (2011). Mysterious ‘sleeping sickness’ pandemic offers insight into Parkinson’s. Retrieved from: http://www.neura.edu.au/news-events/news/mysterious-sleeping-sickness-pandemic-offers-insight-parkinson-s
Sacks, O. (1973, rev. ed. 1990). Awakenings. Retrieved from: http://www.oliversacks.com/books/awakenings/
The New York Times [NYT] Health Guide. (2011). Parkinson's Disease - Levadopa (l-Dopa); Parkinson’s Disease - Treatment. Review Date: 9/7/2011. Reviewed by Simon, H.MD, Editor-in-Chief, Associate Professor of Medicine, Harvard Medical School, and Zieve, D. MD, MHA, Medical Director, A.D.A.M., Inc. Retrieved from: http://health.nytimes.com/health/guides/disease/parkinsons-disease/levadopa-(l-dopa).html
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