JMS Pearce
Hull, England
The soul is trapped in a body that no longer obeys its commands.
—A. Dumas, The Count of Monte Cristo, 1844
The pediatric neurologist Richard E. Nordgren and colleagues in 1971 described seven cases of what they called “The Locked In Syndrome.”1 Plum and Posner’s classic monograph comprehensively reviewed the condition and distinguished it from the persistent vegetative state.2 Patients with locked-in syndrome have no flicker of movement of their limbs, nor can they talk, yet they retain sentient consciousness. They are immobile and with difficulty can communicate by blinking and vertical eye movements.3 They are aptly described as “de-efferented.” The causative lesion is usually infarction, hemorrhage, or demyelination in the ventral pons or medulla; it spares the reticular activating system, responsible for maintaining alertness, thereby explaining why patients are conscious despite their paralysis. It also spares the tegmentum, preserving the supranuclear pathway for vertical eye movements. Medical texts before Nordgren failed to describe these distinctive clinical features, although since strokes have always been common, they undoubtedly occurred.
In literature
Plum and Posner prudently commented that claims for literary priority could have a treacherous career. The first descriptions of the locked-in syndrome may not have been by a doctor but by writers of novels, who presumably had encountered or heard of such cases, though their mechanism was unknown. In 1844, Alexandre Dumas, the prolific playwright and novelist, in The Count of Monte Cristo tells the tale of Noirtier de Villefort, a Bonapartist sympathizer, father of Gérard de Villefort, and grandfather of Valentine. After suffering a stroke, Noirtier can neither talk nor move, but can communicate by moving his eyelids and eyes. In Chapter 58, he is described:
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. … Sight and hearing were the only two senses, which, like two sparks, still lit up this human matter, already three-quarters remolded for the tomb.
Dumas understood the consequences of the locked-in syndrome4 when he related: “the soul is trapped in a body that no longer obeys its commands.”
Another fictional example is Émile Zola’s Thérèse Raquin, published in 1868.5 Thérèse and her passionate, adulterous lover, Laurent, murder Camille. Saddened by her son’s death, the pitiful mother, Madame Raquin, has a stroke:
Her tongue turned to stone. Her hands and feet stiffened. She was struck dumb and motionless.
After a partial respite, “she had only the language of her eyes, and her niece had to guess what she wanted.”
Later, “she could communicate quite easily with that imprisoned mind buried alive in a dead body. … She had learnt to use her eyes like a hand or a mouth, to ask and give thanks, and in a strange way made up for the organs she had lost.”
She discovered the lovers’ treachery in murdering her son and then “made frantic efforts to … put all her hatred into her eyes.” The wicked couple are eventually driven to take their own lives by poison. “Madame Raquin, stiff and silent, contemplated them at her feet, unable to feast her eyes enough, eyes that crushed them with a brooding hate.”
The embittered, loveless Mrs. Clennam in Charles Dickens’ Little Dorrit (1856) has a stroke, after which:
She never from that hour moved as much as a finger again, or had the power to speak one word. For upwards of three years she reclined in her wheeled chair, looking attentively at those about her, and appearing to understand what they said; but, the rigid silence she had held so long was evermore enforced upon her, and, except that she could move her eyes and faintly express a negative or affirmative with her head, she lived and died a statue.
A 1997 non-fictional autobiography The Diving Bell and the Butterfly was “dictated” by Jean-Dominique Bauby, an editor of the French magazine Elle, who aged forty-three had a stroke leaving him completely immobile apart from the ability to blink his left eye. He told his story by laboriously blinking, letter by letter, and letting his therapist transpose his thoughts into writing. Two days after the book was published, Bauby died of pneumonia. A prize-winning film was produced in 2007.
In clinical practice, recognition of the locked-in state is important if casual remarks at the bedside are not to be the unintended cause of grave hurt and mental anguish to a sentient, motionless patient.6 Patients thought to be in a coma should be instructed to raise their eyes and to blink; only in this way will the awareness of the alert but helpless patient be discovered. Further clues to localization may be provided by imaging and by characteristic patterns of disordered respiration seen in patients with lesions in the brainstem.2
It is important to distinguish the locked-in syndrome from transient periods of coma, a state of unrousable unresponsiveness, and from:
- Persistent (continuous) vegetative state,7 renamed unresponsive wakefulness syndrome, in which the patient survives for months or years, is awake but shows no outward sign of conscious intelligence. Some survive, usually showing improvement in the first year, but most die of the complications of immobility rather than from the primary brain lesion.
- Akinetic mutism (also called coma vigil), in which patients lie motionless, mindless, and unaware of their surroundings but show sleep-wake cycles and may be capable of reflex movement. Very few survive.
References
- Nordgren RE, Markesbery WR, Fukuda K, Reeves AG. Seven cases of cerebral medullary disconnexion: the locked in syndrome. Neurology 1971;21:1140-8.
- Plum F, Posner JB. The diagnosis of stupor and coma. 2nd ed. Philadelphia: FA Davis,1972.
- Chase TN, Moretti L, Prensky AL. Clinical and electroencephalographic manifestations of vascular lesions of the pons. Neurology 1968;18:357-68.
- Schnetzer L, McCoy M, Bergmann J, Kunz A, Leis S, Trinka E. Locked-in syndrome revisited. Ther Adv Neurol Disord. 2023;16:1-20.
- Zola E. Thérèse Raquin. Translated L Tancock, ed. London, Penguin Classics, 1982. (Published originally in 1868.)
- Pearce JMS. The locked in syndrome. British Medical Journal 1987;1:198-9.
- Jennett B. The Vegetative State: Medical facts, ethical and legal dilemmas. Cambridge University Press, 2002.
JMS PEARCE is a retired neurologist and author with a particular interest in the history of medicine and science.
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