BRAINSPOTTING

Adventures in Neurology

A. J. Lees

• Editore: Notting Hill Editions
• Anno: 2022
• ISBN:
  9781912559374
• Argomento: Sciences biologiques
• Categoria: Science générale

As a trainee doctor, Andrew Lees was enthralled by his mentors: esteemed neurologists who combined the precision of mathematicians, ?the scrupulosity of entomologists and the solemnity of undertakers in their diagnoses and treatments. For them, there was no such thing as an unexplained symptom or psychosomatic problem – no difficult cases, only interesting ones – and it was only a matter of time before all disorders of the brain would be understood in terms of anatomical?electrical and chemical connections. Today, this kind of 'holistic neurology' is on the brink of extinction as a slavish adherence to protocols and algorithms – plus a worship of machines – runs the risk of destroying the key foundational clinical skills of listening, observation and imagination that have been at the heart of the discipline for over 150 years. In this series of brilliant, insightful and autobiographical essays, Andrew Lees takes us on a kind of Sherlock Holmes tour of neurology, giving the reader insight into – and defending – the deep analytical tools that the best neurologists still rely on to diagnose patients: to heal minds and to fix brains.

30

– Charcot’s Parrot –

In the course of my rotation through the different specialties at The London Hospital I had learnt about Charcot’s joint, his biliary triad, his crystals seen under the microscope in asthma and finally his eponymous neurological diseases. In the second half of the nineteenth century, Jean-Martin Charcot’s second sight had allowed him to see patterns of disease that no one before him had ever noticed and after I had finished my house jobs and become a doctor I left London with the intention of walking him back to life. I arrived in Paris in 1972 to continue my medical training and hoping that I could learn to perceive things my mind did not yet know.

I set off early beneath a sky rendered oppressive by the first suns of June. A south-westerly wind had swept away the clouds and above the city mist hung an azure wall. At the Pont de la Tournelle I stopped for a moment to watch the yawning river as it flowed on its journey to the blue roofs of Rouen. The cast iron gate of Le Jardin des Plantes was closed, but through its filigree I could see the allées where the naturalist Georges Cuvier had imagined a dinosaur from a single bone. I left the embankment at the Austerlitz terminus 31and sidled up the long boulevard that followed the Métro in the direction of the Hôpital de la Salpêtrière.

Charcot had first appreciated the opportunities for research at L’Hospice de la Vieillesse-Femmes de la Salpêtrière during the final year of his internship. In his thesis, in which he described how to distinguish chronic rheumatism from gout, he outlined the anatomo-clinical method that would be fundamental to all his future work. Practising nosography, as he referred to it, depended on the meticulous correlation of symptoms and signs with pathology. The first step was to document in detail the history and clinical signs over the extent of a person’s illness and then examine the brain and spinal cord after death. On leaving La Salpêtrière to continue his medical training, Charcot is reputed to have said: ‘I must return here one day and then stay.’

His next post was back at Hôpital de la Charité where he had worked as Pierre Rayer’s intern in 1851. Rayer was an outstanding diagnostician and anatomist with a particular interest in kidney disease who had recently been appointed Dean of the Faculty of Medicine in Paris. He had a strong interest in pathology and had also been elected as the first President of the Société de biologie. Impressed by his ability, Rayer proposed Charcot as a titular member of the society which provided his young assistant with the opportunity to present his scientific research in front of the 32greatest naturalists and physiologists of Paris. During this second spell at Hôpital de la Charité Charcot was promoted to associate professor, and at the age of thirty-seven, with the support of Rayer, he finally achieved the promotion he had been hoping for and was appointed chef de service at La Salpêtrière.

The hospice owed its name to its origin as a magazine for saltpetre. After several gunpowder explosions the arsenal was relocated, and its former site converted into a poorhouse for women. By the time of the First French Republic (1792–1804), it had expanded into a vast repository for every sort of female social misfit. The insane were shackled in rat-infested dungeons and fed through iron grilles. There was a block where orphans and the children of the destitute lived until they were old enough to go out to work in factories and some of its inmates had been admitted against their will on the whim of their husbands. It was a threatening world of its own, described in the newspapers as ‘The Versailles of Pain’.

By the time Jean-Martin Charcot returned to take charge in 1862, the criminals and most of the beggars had been moved to other facilities. It was still a dreaded, alien place, but little by little it had been transformed from the pandemonium of bedlam to a self-supporting colony. The residents of the hospice could be divided into three broad groups: elderly, frail women from the least favoured classes of society, whom desertion or ill fortune had placed under the protection 33of public charity; women of all ages with infirmity due to chronic incurable physical maladies; and lastly the mentally disturbed or inadequate. Shortly after he had returned to the hospice Charcot famously wrote about its research opportunities:

The clinical types available for study are represented by numerous examples, which enable us to study categorical disease during its entire course, so to speak, since the vacancies that occur in any specific disease are quickly filled in the course of time. We are, in other words, in possession of a sort of museum of living pathology of which resources are great.

With the assistance of his close friend and colleague Alfred Vulpian, he began at once to delineate the illnesses of all 2,635 female residents that were under their joint care, a project he would continue until his death thirty years later. Each morning he arrived promptly at the hospice in his carriage and drove through the Mazarin gate. After alighting and giving his two horses some sugar he walked across the courtyard to his office on the ground floor of the old Pariset division. It was here, on weekdays, that he would see patients brought to him from the wards, and each Sunday at 9.30 a.m. he taught his assistants using cases they had selected from the previous week’s admissions from the clinic. The spartan room had dark walls, a single window, a few chairs, a small table and a wardrobe. Rubens and Raphael reproductions 34hung on the wall. The first teaching case would be brought in and his nurse would proceed to remove the patient’s gown and nightdress. The intern then presented l’observation while Charcot sat drumming his fingers impatiently on the table. After a loaded pause, he might ask the patient a question about her family history or command her to make a movement. His penetrating gaze remained fixed on the scene in front of him. Then, following what seemed like an interminable silence, he voiced his opinion in no more than a few aperçus. To some of those present for the first time it felt as if he had come to his conclusions in the manner of a mystic. ‘In the final analysis’, he might say, ‘we see only what we are ready to see, what we have been taught to see. We eliminate and ignore everything that is not part of our own prejudices.’

Once the last patient had left his office, he would put on his coat and top hat and leave for home. On his way out he was sometimes accosted by a patient begging for alms. Ensconced in his carriage, he would immediately begin to read, becoming so engrossed that his coachman reported that he often did not step down for several minutes after arriving back at L’hôtel de Varengeville on the Boulevard St Germain. Once lunch was over he would retire to his salon to recommence his studies. Sometimes he would work into the early hours of the morning catching up with the latest research published in German, English, Spanish and Italian as well as in French, and when his attention 35began to flag he would break off to do some sketches.

Charcot cut open cadavers in a renovated kitchen behind his consulting room. If his lancet revealed an alteration in the colour, volume or texture of the brain he drew a picture of the abnormality in a notebook and then included it in his post-mortem findings inserted in the patient’s case records. He considered the autopsy to be an integral part of the clinical examination, the last service one could offer to the patient and her family and an invaluable lesson in humility. His modus operandi is epitomised by the case of Luc, a woman who despite her clumsiness he employed as his domestic servant. Charcot observed that her hands shook when she carried a cup and saucer and that if her hands were motionless her head shook. Her unsteadiness gradually increased and when she could no longer work he arranged for her to be admitted to the hospice under his care. When she died, he performed a postmortem examination and found numerous sclerotic plaques in the white matter of her brain and spinal cord, a finding that supported a condition he was in the course of differentiating from Parkinson’s disease, which would later come to be called multiple sclerosis. His decision to employ Luc probably arose as much from his single-minded curiosity as his charity.

In 1882, a new amphitheatre was built in honour of Charcot’s appointment as the first Professor of Diseases of the Nervous System and his weekly 36teaching sessions then became more structured but remained impromptu. Every Tuesday morning a large audience waited for him in silence and high expectation. At 9 a.m. he would enter the theatre with an entourage of clinical and laboratory chiefs, interns and visiting overseas fellows. He walked slowly across the dais to his chair, positioned to the left of the stage. The first patient would already be waiting in place. After a heavy pause he would begin to take their history:

Charcot: How old are you?

Patient: Thirty years.

Charcot: What do you do?

Patient: I am a tailor.

Charcot: Are you married?

Patient: My wife is dead.

Charcot: And when did your illness begin?

Patient: Thirteen months ago.

Charcot: And before that?

Patient: I had pain.

Charcot: What kind of pains?

Patient: Sudden stabbing pains in my legs that last at least twenty-four hours and during which the skin becomes sensitive. If I press hard on my legs it slightly relieves the pain. Charcot: And during this time you were still walking and working?

Patient: Yes.

There were times when Charcot was unable to come to an immediate decision. On other occasions he 37was forced to admit he could not explain logically how he had arrived at his diagnosis. He would remind his audience that patients were allowed to have diseases that were rare and might even suffer from maladies that had not yet been identified. In the early stages of nervous disease an incomplete clinical presentation was usual, but this did not mean that a diagnosis could not be suspected. When there was uncertainty, it was best to describe exactly what one had heard and seen without prejudice and leave it at that.

Charcot was aware of his gift of seeing things in a flash, and to assist his audience he amplified his observations with memorable verbal descriptions. He also made drawings in chalk on the blackboard, showed lantern slides and temperature charts, and prepared posters and plaster casts to embellish his demonstration. An example of his ability to recount clearly what he had heard or seen is provided in this observation of the speech of a patient with multiple sclerosis:

There is a symptom more frequently found than nystagmus … and this is a peculiar difficulty in enunciation … The affected person speaks in a slow drawling manner and sometimes almost unintelligibly. It seems as if the tongue has become ‘too thick’, and the delivery recalls that of an individual suffering from incipient intoxication. A closer examination shows that the words are as if measured or scanned, there is a pause after every syllable, and the syllables themselves are pronounced slowly.

38For some of his lessons, his clinical assistants would be asked to bring three or four patients into the amphitheatre at the same time. These people often looked completely different from one another but he knew, from his pathological work, that they represented different stages, or biological variations, of the same disease entity. If an example of a particular syndrome was not available for his presentation, he would imitate or pantomime an abnormal gait or a particular form of facial tic in order to embroider his description. His method owed little to inference or analysis but demanded extraordinary patience and a continuity of observation. In moments of reflection he would tell his disciples that he was nothing more than a photographer, but at the same time he was very aware of the limitations of static imagery and knew that pictures required captions, explanations and sequential categorisation for their clinical interpretation. Without his skill in focusing the camera’s gaze, and the exactness of his accompanying written descriptions, the fading daguerreotypes, preserved in the hospital’s iconography, would now be uninterpretable. Charcot’s observations were far more than textbook accounts or snapshots of poorly people lying in bed: instead each seemed like a tableau of a life imprinted on an autobiography.

Although he emphasised that attentive listening and astute observation were essential to his method he also told his pupils that occasionally a coup d’oeil was 39all that was necessary to make a confident diagnosis. In one of his Tuesday lectures devoted to Parkinson’s disease he said: ‘I have seen such patients everywhere on the streets of Rome, of Amsterdam, in Spain, it is always the same picture: they can be identified from afar, you do not need a medical history.’

His teaching sessions left indelible pictures in the minds of his assistants, which no amount of private study could ever hope to replicate. Sigmund Freud, who spent the winter of 1885–86 as a visitor in Charcot’s service, later wrote that he remembered coming out of one of these lessons fully sated with Charcot’s words ringing in his ears, as if he was leaving Notre Dame with a new idea of perfection.

The entrance of L’Hôpital de la Salpêtrière was guarded by a majestic greystone cuboid with narrow windows and four turreted chimneys. I walked through its arched porch and down the long path that led to the first line of hospital buildings. Beyond the Mazarin gate stood the cruciform chapel of Saint-Louis, with its iconic domed roof, four naves and bleached walls. As I continued on down a footpath bordered by lindens and plane trees that formed one side of a large rectangular garden, the doleful peal of the church bells broke the leaden silence. I then turned left into Rue de la Lingerie and directly in front of me was La Force, the former prison with its inner courtyards named after Manon Lescaut and the September Massacres, 40where prostitutes and heretics had been contained before their deportation to Canada and Louisiana. After a few more minutes I arrived at a brutalist faded edifice that smelt of feline ammonia. I climbed the stone steps, opened the glass door and walked down a narrow passage lined with offices. At the end of the corridor was a lower hallway that ran at a right angle and led to the clinics. I took the lift up to the ward.

In the nurses office, over a breakfast of croissants and coffee, I was introduced to my new colleagues. Once the pleasantries were over discussion returned to the previous night’s medical ‘take’ and specifically to an alcoholic, who had been admitted with amnesia due to acute Korsakoff’s syndrome. After about half an hour François Lhermitte, the chef de service, arrived. He had a bulging cranium and wore his buttoned-up white coat with the collar upturned. His glasses seemed to enhance the twinkling inquisitiveness of his eyes as he looked at me as if I was a specimen from an alien world. He began by talking about a recently published paper in Revue Neurologique describing a group of people with focal damage to the globus pallidus who had profound mental inertia and slowness of thinking but without evidence of amnesia or dementia. I knew that he had a particular interest in behavioural disorders linked to frontal lobe disease and that he was also very exercised over whether thought was possible without words. His father, Jean Lhermitte, had preceded him on the staff of the hospital, and was 41best remembered in England and the United States of America for his de...

Indice dei contenuti

• – Title Page –
• – Contents –
• – Dedication –
• – Preface –
• – Birdwatching on the Pavements –
• – Full of East End Promise –
• – Charcot’s Parrot –
• – This Is the Ritual –
• – The Lost Soul of Neurology –
• – Words –
• – The Dead Hospital –
• – Zadig and Voltaire –
• – Resurrection –
• – Machine Learning –
• – Acknowledgements –
• – About the Author –
• – Copyright –