Awakenings

1. Encephalitis Lethargica: The Sleeping Sickness Epidemic

A third of those affected died in the acute stages of the sleeping-sickness, in states of coma so deep as to preclude arousal, or in states of sleeplessness so intense as to preclude sedation.

The mysterious epidemic. Encephalitis lethargica, also known as the sleeping sickness, was a devastating neurological disorder that swept across the world between 1916 and 1927. This enigmatic disease affected nearly five million people, causing a wide range of symptoms that baffled medical professionals of the time.

Long-term consequences. Those who survived the acute phase often developed severe neurological complications, including:

• Parkinson-like symptoms
• Catatonia
• Tics and involuntary movements
• Behavioral and personality changes
• Extreme states of stillness or agitation

Many patients were left in a state of profound disability, requiring long-term institutional care. The aftermath of the epidemic created a unique population of patients who would later become the subjects of Dr. Oliver Sacks' groundbreaking work with L-DOPA.

2. L-DOPA: A Miracle Drug with Complex Effects

L-DOPA is a 'miracle-drug' – the term is used everywhere; and this, perhaps, is scarcely surprising, for the physician who pioneered its use – Dr George Cotzias – himself calls L-DOPA 'a true miracle-drug . . . of our age.'

The promise of L-DOPA. L-DOPA (levodopa) emerged as a revolutionary treatment for Parkinson's disease and post-encephalitic syndromes in the late 1960s. It offered hope to patients who had been trapped in frozen states for decades, promising to restore movement and function.

A double-edged sword. However, the effects of L-DOPA were far from simple:

• Initial responses were often dramatic and positive
• Long-term use led to complex side effects and complications
• Patients experienced unpredictable fluctuations in their symptoms
• The drug revealed deeper neurological and psychological issues

L-DOPA's introduction sparked intense debate in the medical community about its benefits and risks, challenging traditional notions of treatment and recovery for neurological disorders.

3. Awakening: The Dramatic Response to L-DOPA

Awakening consists of a change in awareness, of one's total relation to one's self and the world.

The miraculous revival. For many post-encephalitic patients, the initial response to L-DOPA was nothing short of miraculous. Patients who had been motionless and silent for decades suddenly:

• Regained the ability to move and speak
• Experienced a flood of emotions and memories
• Reconnected with their surroundings and loved ones

A complex transformation. However, this awakening was not simply a return to normalcy. It often involved:

• Intense physical and emotional experiences
• A confrontation with decades of lost time
• The challenge of adapting to a radically changed world

The awakenings revealed the profound plasticity of the human brain and raised philosophical questions about the nature of consciousness, identity, and time.

4. Tribulation: The Challenges of Continued L-DOPA Treatment

All patients, then, move into trouble on L-DOPA; not into 'side-effects,' but into radical trouble: they develop, once more, their 'propensenesse to diseases,' which can sprout and flower in innumerable forms.

The honeymoon ends. After the initial period of dramatic improvement, many patients began to experience significant challenges with continued L-DOPA use:

• Uncontrollable movements (dyskinesias)
• Rapid fluctuations between mobility and immobility
• Psychiatric symptoms such as hallucinations and mood swings
• The emergence or re-emergence of tics and compulsive behaviors

A precarious balance. Managing these complications became a complex balancing act for both patients and physicians. It often involved:

• Constant adjustments to medication dosages and timing
• Dealing with unpredictable and sometimes extreme symptom fluctuations
• Navigating the psychological impact of these ups and downs

The tribulations phase revealed the limitations of L-DOPA and highlighted the need for a more comprehensive approach to treatment.

5. Accommodation: Finding Balance in Long-Term L-DOPA Use

Health and disease are alive and dynamic, with powers and propensities and 'wills' of their own. Their modes of being are inherently antithetical: they confront one another in perpetual hostility – our 'Internal Militia,' in Sir Thomas Browne's words.

Adapting to a new normal. Over time, some patients were able to achieve a degree of stability in their L-DOPA treatment:

• Finding an optimal dosage and schedule
• Developing strategies to manage side effects
• Adjusting expectations and lifestyle to accommodate limitations

The role of resilience. Successful accommodation often depended on:

• The patient's individual physiology and disease progression
• Psychological factors such as attitude and coping mechanisms
• The support of caregivers and medical professionals

This phase emphasized the importance of personalized medicine and the need for a holistic approach to patient care that goes beyond just administering medication.

6. The Human Experience: Patients' Struggles and Triumphs

I have learned from them that the body can be tortured far more than I thought possible – that there are some Hells known only to neurological patients, in the almost inconceivable depths of certain neurological disorders.

The depths of suffering. Dr. Sacks' patients experienced extraordinary hardships:

• Decades of physical immobility and silence
• Profound isolation and loss of independence
• The psychological trauma of "awakening" to a changed world
• The rollercoaster of hope and disappointment with L-DOPA treatment

Resilience and humanity. Despite these challenges, many patients demonstrated remarkable strength:

• Maintaining their sense of self through years of illness
• Adapting to radical changes in their condition
• Finding meaning and moments of joy even in difficult circumstances

Their stories highlight the indomitable nature of the human spirit and the complex interplay between neurological function and personal identity.

7. Beyond Medicine: The Importance of Human Connection and Art

Kinship is healing; we are physicians to each other – 'A faithful friend is the physic of life' (Browne). The world is the hospital where healing takes place.

The power of relationships. Dr. Sacks observed that human connections played a crucial role in patients' well-being:

• Family visits often triggered dramatic improvements
• Supportive relationships within the hospital community were vital
• The doctor-patient relationship itself could be therapeutic

Art as medicine. Various forms of artistic expression proved remarkably effective in helping patients:

• Music could "awaken" patients from frozen states
• Physical activities like dancing sometimes overcame motor difficulties
• Creative pursuits provided an outlet for expression and a sense of purpose

These observations underscored the importance of a holistic approach to care that goes beyond pharmacological interventions.

8. The Limitations of Mechanical Medicine

We see, in Donne, a great variety of words relating to the nature of disease: disposition, inclination, propensity, complexion, constitution, etc., a richness of language which both distinguishes and unites two aspects of disease – its structure and strategy.

Beyond reductionism. Dr. Sacks argues against an overly mechanistic view of medicine:

• Neurological disorders cannot be reduced to simple chemical imbalances
• The complexity of human experience must be considered in treatment
• Patients are not merely collections of symptoms to be "fixed"

A call for holistic care. The L-DOPA experience demonstrated the need for:

• Individualized treatment plans that consider the whole person
• Recognition of the psychological and social aspects of illness
• An approach that values the patient's subjective experience

This perspective challenges the dominant paradigm in modern medicine and calls for a more nuanced, humanistic approach to patient care.

9. The Complexity of Neurological Disorders and Treatment

Perhaps all this sounds unduly poetic, but the fact of such awakening is easily confirmed, not only clinically, but physiologically too.

The intricate brain. Dr. Sacks' work revealed the immense complexity of neurological function:

• The same treatment could produce radically different effects in different patients
• Symptoms often involved a complex interplay of physical and psychological factors
• The brain's plasticity allowed for unexpected adaptations and recoveries

Challenging medical paradigms. This complexity necessitated:

• A more flexible and adaptive approach to treatment
• Recognition of the limitations of current medical knowledge
• Openness to unconventional therapies and explanations

The L-DOPA experience highlighted the need for humility in the face of neurological mysteries and a willingness to embrace new paradigms in neuroscience.

10. The Ethics of Experimental Treatment and Patient Care

I hesitated very greatly in regard to the original publication of our patients' 'story' and their lives. But they themselves encouraged me, and said to me from the first, 'Tell our story – or it will never be known.'

Navigating uncharted waters. The use of L-DOPA in post-encephalitic patients raised significant ethical questions:

• The risks and benefits of experimental treatments
• The role of patient consent in chronic, debilitating conditions
• The responsibility of physicians in managing patient expectations

Balancing hope and harm. Dr. Sacks grappled with:

• The potential for both dramatic improvement and severe side effects
• The psychological impact of "awakening" after decades of illness
• The ethics of continuing or discontinuing treatment in difficult cases

These dilemmas highlight the complex moral landscape of experimental treatments and the importance of transparent, compassionate communication with patients and their families.

11. The Long-Term Impact of L-DOPA on Post-Encephalitic Patients

I have continued to see a diminishing number of post-encephalitic patients at Mount Carmel, and at other places where I work in New York, and to keep in frequent touch with the post-encephalitics at the Highlands Hospital and elsewhere.

A dwindling population. Over the decades following the initial L-DOPA trials:

• The number of surviving post-encephalitic patients steadily decreased
• Long-term effects and complications of L-DOPA became apparent
• Some patients maintained significant benefits, while others struggled with side effects

Lasting lessons. The long-term follow-up of these patients provided valuable insights:

• The importance of individualized treatment and ongoing adjustment
• The potential for unexpected long-term adaptations and improvements
• The need for comprehensive care that addresses both physical and psychological needs

The experiences of these patients continue to inform our understanding of neurological disorders and the potential for recovery even after prolonged periods of disability.

Last updated: November 19, 2024