Awakenings
Although the evidence suggests its impact may be abating, we are all well aware of the COVID-19 pandemic, a global disease resulting from infection by the severe acute respiratory syndrome coronavirus (SARS-CoV-2). First known to have emerged in an outbreak in Wuhan, China, in December 2019, it rapidly spread worldwide in early 2020. The World Health Organisation has determined it has caused more than 7 million confirmed deaths by early 2024, and it ranks it fifth in the list of the deadliest pandemics and epidemics in history. The worst of those we know about was the Black Death, an outbreak of the Bubonic Plague in the middle of the 14th Century, the same disease that spread through the world in the middle of the 5th Century (the Plague of Justinian) which ranks third. Second on the list is the Spanish Flu, which swept across the globe in 1918-1920, and the fourth was the HIV/Aids epidemic, which probably began in began in 1981, and continues today. All these events are horribly familiar, three of which occurred in the past one hundred years.
However, there have been some other epidemics in the past century that have received less press. Among these, one of the most remarkable, and least well understood, was Encephalitis Lethargica, an atypical form of encephalitis. It was a disease that attacked the brain, leaving people in a frozen, statue-like condition, often speechless and motionless. It spread around the world between 1915 and 1926, but attention was soon deflected from EL to the Spanish Flu. The exact number of people infected is unknown, but estimates suggest that more than one million people contracted the disease, which directly caused more than 500,000 deaths. Of the survivors, most never recovered to their prior vigour and alertness. An account of the lives of a number of victims forms the core of an extra-ordinary book, Awakenings, by Oliver Sacks. However, before I turn to his account, a few more facts about the overall impact.
Encephalitis lethargica is characterised by fever, lethargy, delayed physical and mental responses and catatonia. In some case victims enters a coma-like state, Parkinsonism, upper body weakness, tremors, rigidity, and even Klazomania (compulsive screaming). Patients became frozen, their bodies and reactions stuck at the age when they were infected, even though they physically age. Unsurprisingly, the timing of EL led to the belief it was connected to the Spanish Flu, but most modern research has argued against this. A recent study, in 2008, concluded: “the case against influenza [is] less decisive than currently perceived … there is little direct evidence supporting influenza having a role in the aetiology of EL”, and that “[a]lmost 100 years after the EL epidemic, its aetiology remains enigmatic.” While opinions on the relationship of encephalitis lethargica to influenza still remain divided, the preponderance of the literature appears sceptical.
It was also unusual in another sense. A German neurologist who examined hundreds of encephalitis lethargica patients in the 1920s, noted that their encephalitis lethargica typically evolved over time. The early symptoms would be dominated by sleepiness or wakefulness. Then a second stage of symptoms appeared, characteristically identified by weird and unpredictable eye movements. For many the third stage would be recovery. A 2010 Oxford University Press compendium quoted a researcher, writing in 1930, who stated, “we must confess that aetiology is still obscure, the causative agent still unknown, the pathological riddle still unsolved”, and asked, at the time of publication: “Does the present volume solve the ‘riddle’ of EL, which … has been referred to as the greatest medical mystery of the 20th century? Unfortunately, no: but inroads are certainly made here pertaining to diagnosis, pathology, and even treatment”. In 2012, Oliver Sacks noted there was evidence a virus was the probable cause of the disease, possibly an enterovirus variant.
This ‘new illness’ was first noted in the winter of 1916–1917 in Vienna and later in some other cities. It rapidly spread world-wide over the next three years. The number of people infected during the ten years of the pandemic is unknown (but, as already noted, estimates suggest more than 1 million people contracted the disease, and that more than 500,000 died). Encephalitis lethargica assumed its most virulent form between October 1918 and January 1919. The pandemic disappeared in 1927, as abruptly and mysteriously as it first appeared, although it is likely that the Spanish Flu virus accelerated the effects of whatever caused the encephalitis or lowered resistance to it in a catastrophic way.
Finally, and equally extraordinarily, many surviving patients of the 1915–1926 pandemic eventually seemed to make a complete recovery and return to their normal lives. Despite this, the majority of survivors subsequently developed neurological or psychiatric disorders, often appearing after years or decades of seemingly perfect health. Post-encephalitic syndromes varied widely: sometimes they proceeded rapidly, leading to profound disability or death; sometimes very slowly; sometimes they progressed to a certain point and then stayed at this point for years or decades; and sometimes, following their initial onslaught, they remitted and even disappeared completely. Often thought of as a disease of the past, it is still seen in occasional cases today.
Two flu epidemics, in 1957-8, and 1968-9, had pushed the Spanish Flu into the background by the end of the 1960’s, and encephalitic lethargica had disappeared from popular attention: it was relegated to treatment wards. However, it was an English doctor, then working in Mount Carmel Hospital in New York, who decided to see if he could work with some of the 200 patients in the city using a novel form of treatment. That doctor was Oliver Sacks, then a consultant neurologist, who decided to try administering a new drug, laevo-dihydroxyphenylalanine (L-DOPA), which had proven to be a ‘miracle drug’ for Parkinson’s patients. The results were bizarre, and Awakenings, his account of what happened, is a compelling read. As one commentator, Frank Kermode, an English Professor at Cambridge University, described it: “this doctor’s report … is written in a prose of such beauty that you might well look in vain for its equal among living practitioners of belles lettres”.
My 1973 first edition of Awakenings is dedicated to “To the patients whose lives are here depicted”. The title page quotes “… and now, a preternatural birth in returning to life from this sickness”. As we discover in reading his book, for some this was a bittersweet recovery.
The patients Sacks was to meet would sit motionless and speechless all day in their chairs, totally lacking energy, impetus, initiative, motive, appetite or desire. These ‘survivors’ had their thoughts and feelings unchangingly fixed at the point at which their long “sleep” had closed in on them during the 1920s, a time that would remain more real to them than any subsequent decade. While their minds remained clear and unclouded, they were inaccessible. Unable to work or see to their needs, they had been largely abandoned by their friends and families and had been put away in hospitals and nursing homes, largely forgotten.
Oliver Sacks was employed at Mount Carmel’s Beth Abraham care centre in New York’s Bronx and was given permission to experiment with the administration of L-Dopa to nearly 80 patients living there. The result was like an explosion, or perhaps like a rollercoaster ride. Some of L-Dopa’s side-effects had a frightening intensity: in one patient’s words: “I can no more control it than I could control a spring tide. I just ride it out and wait for the storm to clear… That L-Dopa, that stuff should be given its proper name – Hell-Dopa!”
While the L-Dopa released amazing reactions, Sacks was carefully taking notes. “I cannot think back on this time without profound emotion,” he wrote later. “It was the most significant and extraordinary moment in my life, no less than in the lives of our patients. All of us at Mount Carmel [Beth Abraham] were caught up with the emotion, the excitement, with something akin to enchantment, even awe.”
In the spring of 1969, he writes: “I moved to an apartment a hundred yards from the hospital and would sometimes spend 12 or 15 hours a day with our patients – observing them, talking with them, getting them to keep notebooks, and keeping voluminous notes myself, thousands of words each day. And if I had a pen in one hand, I had a camera in the other: I was seeing such things as had never, perhaps, been seen before – and which, in all probability, would never be seen again.” It was, said Sacks, his duty and his joy “to record and bear witness”.
To read the stories of the lives of Frances D, Magda B, Rolando P, Lucy K Margaret A, George W and the others is moving, sometimes shocking and occasionally extraordinarily tragic. As he explains, “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.” The strange, almost unbelievable histories Sacks tells of the patients at Mount Carmel are exemplified in his description of concluding moments of the life of Magda B, who had “a sudden premonition of death”. As he describes in Awakenings, Mrs B suffered progressive blindness, a touching tic and vivid dreaming. In other respects, her physical health was good. Then she contacted her daughters. “Come and see me today … there will be no tomorrow. No, I feel quite well … nothing is bothering me, but I know I shall die in my sleep tonight’.”
Sacks records that “her tone was quite sober and factual, wholly unexcited”. The medical team wondered if they had missed something, and decided to take cardiograms, some blood tests, and other checks. The results were ‘quite normal’. In the evening Mrs B went round the ward, with a laughter-silencing dignity, shaking hands and saying ‘Goodbye’ to everyone there. She went to bed,” Sacks continues, “and she died in the night.” To reread just this one case reminded me how just what an extraordinary account Sacks provides in Awakenings. It is full of similar moments of bizarre drama, in which you find you’re reading about strange and often disturbing events, often appearing more like moments from a fantasy novel. But it isn’t, it is a window into yet another area of mystery concerning the human brain.
There are twenty case studies in Awakenings. His account of Margaret A is typical, in that it describes amazing behaviour and reactions (to be clear, each case study is different in almost every respect, except for the strange ‘release’ that L-Dopa achieved), but her story proved more extreme than most. Margaret was a New Yorker, born in 1908, the youngest child of a poor Irish Immigrant family. In 1925, she fell ill, initially sleeping almost continually for ten weeks (apart from being woken to be fed) and fell into a state of depression. Then she appeared to have recovered, working as a secretary and having an enjoyable social life. However, by 1929, she began to show signs of wakefulness, put on 100 lbs in weight, had periods of depression. She managed to keep working until 1935, and then stayed at home, nursed by her mother, but with frequent hospital visits. In 1958, she was admitted to Mount Carmel. From her admission onwards, her behaviour was characterised by sleepiness, depression, and excessive water consumption. Her gait and posture were very rigid.
The initial administration of L-Dope in May 1968 seemed promising. She could walk more easily, dress herself, and talk more easily. Within a fortnight, she had become sociable, wanted to dance with the nurses, explained she felt a ‘star patient’, although she was having trouble sleeping. Shortly after this, she began to develop tics, was finding it difficult to sleep, and her almost manic behaviour was alternated with ‘frozen’ periods. By June, she started to come apart, saying of the L-Dopa, “It’s driving me mad, but I’ll die if you stop it.”
From that moment on, Sacks reported that she was almost never in a “well-modulated ‘middle’ state and has almost nothing in-between coma and hyper-vigilance”. Sacks goes on to report that: In the presence of excitement and perpetual contradiction, Miss A has split into a dozen Miss A’s – the drinker, the ticker, the stamper, the yeller, the swinger, the gazer, the sleeper, the wisher, the fearer, the lover, the hater, etc. – all struggling with each other to ‘possess’ her behaviour. Her real interests and activities have practically vanished, and have been replaced by absurd stereotypies, continually ground smaller in the mill of her being. She is completely reduced, for most of the time, to a ‘repertoire’ of a few dozen thoughts and impulsions, increasingly fixed in phrase and form, and repeated, compulsively, again and again. The original Miss A – so engaging and bright – has been dispossessed by a host of crude, degenerate sub-selves – a schizophrenic fission of her once-unified self”
This case study ends with another quite unsettling section. We learnt that she has a younger sister, who arrived once a month to take her out. They would go on excursions, eat out, and have a great time. In the book the sister explains they had a wonderful day, each time, and adds “She talked and laughed the way she used to in the old days, back in the twenties before she got ill … she goes mad in your madhouse because she is shut off from life.” What are we to make of this – and all the other cases Sacks describes.
Perhaps it is unfair to focus on this particular case as an example. Many of the other patients Sacks treated had equally bizarre reactions to L-Dopa, but in several cases the drug was to help them get back to a more viable way of life. Some were able to go back to work, marry, have children, and lead fairly normal lives. If Margaret A offers a sad, unresolved and ultimately unsatisfactory account, in almost every case the original Encephalitis Lethargica and the subsequent impact of L-Dopa left the individuals with scars of one kind or another, in some cases physical consequences, and for almost everyone psychological concerns and uncertainties.
However, Awakenings gives us something more. It was superbly written, a presentiment of the several books Oliver Sacks went on to author. His reflections on health, recovery and change take up the last 50 pages of Awakenings, in three sections, under the heading of Perspectives. The first part reflects on the process of Awakening and how it was experienced by his patients. The second is on the topic of Tribulation (and many had extraordinary journeys following that “flash-like drug-awakening of the Summer of 1969” ). Finally Sacks explores the varieties of Accommodation, and the very different responses from a return to an almost normal life through to some scarcely doing much more than surviving.
Given his mesmerising and occasionally dark account, any final comments have to come from Oliver Sacks. In 1982, he wrote: “I have become much more optimistic than I was when I […] wrote Awakenings,for there has been a significant number of patients who, following the vicissitudes of their first years on L-DOPA, came to do – and still do – extremely well. Such patients have undergone an enduring awakening, and enjoy possibilities of life which had been impossible, unthinkable, before the coming of L-DOPA”.
