M. Noirtier was sitting in a large wheelchair where they put him from morning till evening, in front of a mirror which reflected the whole apartment and allowed him to see who was coming in or going out, and what was happening around him, without attempting any movement: this was something that had become impossible for him. Motionless as a corpse, he greeted his children with bright, intelligent eyes….
The Count of Monte Cristo by Alexandre Dumas
Learning objectives
1. Review the portrayal of illness and disease within The Count of Monte Cristo
2. Examine the portrayal of Monsieur Noirtier De Villefort
3. Consider Dumas’ description and whether it matches ‘locked-in’ syndrome
Delving further into The Count of Monte Cristo by Alexandre Dumas, this post will examine another key character: Monsieur Noirtier De Villefort. Again, potential spoiler alert.
The novel is widely considered to contain the first literary description of ‘locked-in syndrome‘. The condition is still sometimes called ‘coma vigil’ or even ‘Monte Cristo’s syndrome’. In this classic portrayal, patients are conscious but unable to initiate any motor response beside eye and eyelid movement which can be used to communicate. In the book, the description continues:
Sight and hearing were the only two senses which, like two sparks, still lit up this human matter, already three-quarters remoulded for the tomb. Moreover, only one of these two senses could reveal to the outside world the inner life which animated this statue…
The Count of Monte Cristo by Alexandre Dumas
Image from https://www.gutenberg.org/files/1184/1184-h/1184-h.htm
Dumas’ inclusion of Noirtier is an intriguing instance of a literary description of a medical condition preceding the medical description. His prose conveys the condition with his typical style and is relatable to the average, non-medical reader. Indeed, quotes from the novel are still often included in medical articles regarding locked-in syndrome.
Question
What was known about the condition Dumas describes at the time the novel was written?
Dumas appears to be well ahead of his time with his description. Williams points out that Darolles reports a similar clinical entity around 1875 but this was about 30 years after the publication of The Count of Monte Cristo. Indeed, it wasn’t until 1966 that the term ‘locked-in syndrome’ was first defined by Plum and Posner. Caused by an insult to the ventral pons (usually vascular) it was originally described as manifesting with lower cranial nerve paralysis, and mutism with preservation of consciousness, vertical gaze, and upper eyelid movement. The definition was refined in 1987 as quadriplegia and anarthria with preserved consciousness.
Image from: https://www.researchgate.net/figure/Ventral-brainstem-lesion-in-the-locked-in-syndrome-as-shown-by-MRI-A-graphical_fig1_318611710 via Creative Commons Attribution 4.0 International.
In addition to our previous discussion regarding the meaning of apoplexy in The Count of Monte Cristo, the exposition of a stroke is explicit with regards to Noirtier. Indeed, Dumas must have possessed at least a rudimentary understanding of the aetiology leading to a locked-in state. Consider one section of the story where Gérard Villefort (Noirtier’s son) says the following when talking to the Count:
There are other things to fear, Monsieur,’ Villefort said, ‘apart from death, old age and madness. For example, apoplexy, that lightning bolt which strikes you down without destroying you, yet after which all is finished. You are still yourself, but you are no longer yourself: from a near-angel like Ariel you have become a dull mass which, like Caliban, is close to the beasts. As I said, in human language, this is quite simply called an apoplexy or stroke.
…and I shall show you my father, Monsieur Noirtier de Villefort, one of the most fiery Jacobins of the French Revolution…
Well, Monsieur, the rupture of a blood vessel in the brain put an end to all that, not in a day, not in an hour, but in a second. One day he was Monsieur Noirtier, former Jacobin, former senator, former carbonaro, who scorned the guillotine, the cannon and the dagger; Monsieur Noirtier, manipulator of revolutions; Monsieur Noirtier, for whom France was only a vast chessboard from which pawns, castles, knights and queens were to vanish when the king was mated. The next day, this redoubtable Monsieur Noirtier had become “poor Monsieur Noirtier”, a paralysed old man, at the mercy of the weakest being in his household, his granddaughter Valentine. In short, a silent, icy corpse who only lives without suffering to allow time for the flesh to progress easily to total decomposition.
The Count of Monte Cristo by Alexandre Dumas
The fact the Dumas was writing this in a popular novel approximately 120 years before the formal medical description feels rather remarkable.
Question
What variant of locked-in syndrome might Dumas have been describing?
There are different ways to categorise locked-in syndrome. One system is described by Smith and Delargy where there are three types and then further subdivided into transient and chronic:
1. Classic – Quadriplegia and anarthria with preserved consciousness and vertical eye movement
2. Incomplete – The same as classic but with remnants of voluntary movement other than vertical eye movement
3. Total – Total immobility and inability to communicate, with full consciousness.
Noirtier can only move his eyes and eye lids. Yet, with only this means of movement, Dumas is still able to convey the nature of his character:
The gesture of the hand, the sound of the voice and the attitude of the body may indeed have gone, but these powerful eyes made up for all: he commanded with them and thanked with them. He was a corpse with living eyes and, at times, nothing could be more terrifying than this marble face out of which anger burned or joy shone.
…The light in Noirtier’s eyes was savage. Something frightful must surely be taking place in the old man’s heart; and surely a cry of pain and anger was rising to his throat where, unable to escape, it suffocated him, because his face became purple and his lips turned blue…
The Count of Monte Cristo by Alexandre Dumas
Question
How do patients with locked-in syndrome communicate?
With eyes alone, Noirtier is able to communicate:
It was understood that when the old man meant ‘yes’, he would close his eyes, when he meant ‘no’ he would blink them repeatedly and, when he needed something, he would raise them upwards. If he wanted Valentine, he closed only the right eye; if he wanted Barrois, he closed the left. At Mme de Villefort’s suggestion, he blinked vigorously.
The Count of Monte Cristo by Alexandre Dumas
However, in reality, time to diagnosis can be considerable with one 2005 article stating distressingly, the diagnosis of locked-in syndrome on average takes over 2.5 months. In some cases it took 4-6 years before aware and sensitive patients, locked in an immobile body, were recognised as being conscious. Furthermore, it isn’t doctors who often notice patient awareness, but rather family members at the bedside.
Smith and Delargy point out that communication with eyes can soon become tiresome and that an agreed system of interpretation with effective closed questioning is required. An alphabet board (also known as a ‘spell board’ or ‘letter board’) allows communication beyond the binary yes/no. Once again, Dumas anticipates this and in the novel, Valentine uses a dictionary to assist Noirtier:
Valentine went to fetch a dictionary, which she put on a reading stand in front of Noirtier. She opened it and when she saw that he was looking attentively at the pages, she ran her finger up and down the columns. Over the six years during which Noirtier had been in his present unhappy state, the exercise had become so easy that she guessed the invalid’s thoughts as quickly as though he had been able to use the dictionary himself.
The Count of Monte Cristo by Alexandre Dumas
Image from https://www.gutenberg.org/files/1184/1184-h/1184-h.htm
Image from: https://pubmed.ncbi.nlm.nih.gov/15718541/
As we already know, Dumas was exposed to clinical medicine, sometimes attending medical rounds at the hospital with his friend and physician Thibaut. With the vivid and the seemingly clear description of locked-in syndrome, are we safe to assume that Dumas either encountered a patient with the condition or at least received a detailed description of it? His prose seems too accurate to be purely conjured up within his own imagination.
Question
Where do you learn about rare conditions?
In the late 1860s, Émile Zola’s Thérèse Raquin contains a character with a possible locked-in state following a stroke. Today, there are a number of fictional depictions of locked-in syndrome. Why the fascination? Haan suggests that being locked-in serves as a strong philosophical metaphor for human existence. What do you think? Please share them in the comments section below.
Always the same: perfectly clear in his mind, but still immobile and speechless.
The Count of Monte Cristo by Alexandre Dumas
Summary
Noirtier is considered the original description of locked-in syndrome. Not only did Dumas’ description proceed the medical one by some time, he also appears to have had a basic understanding of both the aetiology as well as the communication methods individuals in such situations could use. Dumas yet again leaves us with questions as to the extent of his clinical experience and the origins of the diagnosis regarding one of his most important characters.
Further resources
Locked-in syndrome in literature, cinema and television
https://www.researchgate.net/publication/224835900_Locked-in_syndrome_in_literature_cinema_and_television
Locked-in syndrome
https://pubmed.ncbi.nlm.nih.gov/15718541/
Locked-in syndrome: a review of 139 cases
https://pubmed.ncbi.nlm.nih.gov/3738962/
The locked-in syndrome: a review and presentation of two chronic cases
https://pubmed.ncbi.nlm.nih.gov/2152394/
The patient’s journey: Living with locked-in syndrome https://www.ncbi.nlm.nih.gov/pmc/articles/PMC558618/
Locked-in: the syndrome as depicted in literature https://pubmed.ncbi.nlm.nih.gov/24290474/
UNEXAMINED MEDICINE 