Locked-in Syndrome after Brain Damage

Living within my head

Barbara Wilson, Paul Allen, Anita Rose, Veronika Kubickova

• Publisher: Routledge
• Year: 2018
• ISBN:
  9781351791878
• Edition: 1
• Topic: Psychology
• Subtopic: History & Theory in Psychology
• Index: Psychology

The newest title in the series Survivor Stories, this book tells the story of Paul Allen, a photographer who likes opera and was a good baritone singer. At the age of 56 he sustained a stroke that left him paralysed and speechless. He has Locked-In Syndrome (LIS), a rare consequence of brain damage. Although Paul is fully conscious and his cognitive abilities are intact, he is unable to move or speak due to the paralysis of nearly all his voluntary muscles. However, Paul is keen to communicate and through his eye movements he tells his story, from his early life, career, singing and other interests, to the details of his stroke and the effects it has had on his life. The book also includes contributions from Paul's wife Liz, who tells the story from her point of view, along with Paul's physiotherapists, occupational therapists, speech therapists, psychologists and others from the Raphael Hospital who have assisted in Paul's rehabilitation. In telling of his frustrations, his successes, his views on life and how he sees his future, Paul raises awareness of the quality of life possible for those with LIS.

Combining scientific knowledge with personal narrative, this unique and optimistic book is of huge importance to any professional involved in the care of someone with a brain injury, and to the individuals and families touched by LIS.

1What is Locked-In Syndrome? What causes it? Who is most at risk? Do people recover from this condition?

Locked-In Syndrome (LIS) is a rare consequence of brain damage. Patients are fully conscious but unable to move or speak due to paralysis of nearly all voluntary muscles except the eyes. Communication is with movement of the eyes. Some people can move their eyes up and down (vertically), but not side-to-side (horizontally) (National Organization for Rare Disorders [NORD] 2016). The term ‘‘locked-in syndrome’’ was first used by Plum and Posner (1966). Patients diagnosed with this condition had quadriplegia, lower cranial nerve paralysis, mutism with preservation of consciousness, vertical gaze and upper eyelid movement. According to Bauer et al. (1979), LIS can be subdivided into three types depending on the extent of motor impairment. These are classical LIS, partial LIS and total LIS. Classical LIS is characterised by total immobility except for vertical eye movements or blinking; partial LIS is where some minimal motor activity may be seen, and total LIS is where all mobility is lost, including eye movements, and where consciousness is preserved. Smith and Delargy (2005) point out that because mutism could be interpreted as unwillingness to speak, it was redefined in 1986 as quadriplegia and anarthria with preservation of consciousness.

In 1995, The American Congress of Rehabilitation Medicine noted that LIS had five characteristics, namely (i) sustained eye opening, (ii) preserved basic cognitive abilities, (iii) aphonia or severe hypophonia (loss of voice), (iv) quadriplegia or quadriparesis and (v) vertical or lateral eye movement or blinking of the upper eyelid as the primary means of communication.

LIS is caused by damage to the pons, a part of the brainstem that contains nerve fibres that relay information to other areas of the brain (NORD 2016). Most LIS patients have sustained a stroke in the basilar artery or suffered a pontine haemorrhage (Plum and Posner 1983; Patterson and Grabois 1986). Smart et al. (2008) give six vascular conditions that may give rise to LIS: (i) basis pontis infarct, (ii) pontine haemorrhage, (iii) midbrain infarction, (iv) transient ischaemia, (v) basilar artery occlusion and (vi) hypoxic-ischaemic events. They also suggest that there are six ­non-vascular causes, namely (i) trauma, (ii) central pontine myelinolysis, (iii) tumour, (iv) encephalitis, (v) toxins and (vi) multiple sclerosis affecting the ventral pons. Murphy et al. (1979) also report a case of LIS following a pontine abscess. Nevertheless, according to Schnakers and her colleagues (2008), at least 60 per cent of LIS patients have sustained a basilar artery or pontine haemorrhage.

LIS can affect people of all ages, including children. Males and females appear to be affected in equal numbers (NORD 2016). Beaudoin and De Serres (2010) report that the prevalence rate of LIS is not specifically documented in the literature. They believe, however, that LIS probably represents less than 1 per cent of all strokes. Having said this, they recognise that the incidence rate is probably underestimated. They also believe that the average age of onset of LIS varies between 17 and 52 years old (Doble et al. 2003; Bruno et al. 2008; Beaudoin and De Serres 2010; Casanova et al. 2003). The youngest patients have a better prognosis for survival. In contrast, the website for the National Organisation of Rare Disorders (NORD 2016) state that LIS is seen most often in adults at risk for stroke. This would include people with high blood pressure, those who smoke and those of increased age (Sharma et al. 2016). Because cases of LIS may go unrecognised or misdiagnosed, it is difficult to determine the actual number of individuals who have had the disorder in the general population (NORD 2016).

Although prognosis is generally poor and death can occur through pneumonia or thrombosis, with the right care people with LIS can live for many years, and a few make a good or even a complete recovery. Kate Allatt, for example, was a 40-year-old, very fit, mother of three, who describes her recovery from LIS (Allatt and Stokes 2011). It took Kate eight months to recover from her brain stem stroke. She walked out of hospital by herself to resume her normal life. She still had some difficulties however and continued to improve over the following months. Peter Coghlan, an Australian soldier, was 33 years old when he had his stroke. He documents his recovery over the next six months, when he was able to leave hospital and continue his improvement (Coghlan 2013). Richard Marsh was 60 when he had his stroke and it took him a little over four months to recover to the extent that he could leave hospital (Marsh and Hudson 2014). Partial recovery may also be seen. Kerry Pink, for example, was 35 years old and a mother of two when she became locked-in. After three years she was able to speak again and function, although confined to a wheelchair (Pink 2010). Nick Chisholm, a New Zealand rugby player, sustained his brain injury and LIS on the rugby field. Although he regained a little movement, he remained significantly limited in his independence. Andrew Davies also showed very little recovery. He was only 33 years old when he sustained his stroke. He spent 10 months in hospital and after three years remained profoundly disabled (Davies et al. 2015). More recovery was shown by Allison O’Reilly (2014). She was 49 years old when she became locked-in. She remained in hospital for a year but when she was discharged home she still required 24-hour care. Three years later, however, she was able to walk and to drive. A rare account of a child who sustained LIS is provided by Pistorius and Davies (2011). Written when he was an adult, Martin Pistorius was 12 years old when he became ill with an unknown illness which left him wheelchair bound and unable to speak. He spent 14 years in institutions. In 2001 he learned to communicate via a computer, make friends and change his life. He continued to make small improvements over many years.

Many people, however, remain with LIS for ever. The best-known case is probably that of Jean-Dominique Bauby, a French man who “wrote” The Diving Bell and the Butterfly (Bauby 1997) through painstakingly blinking one eye to his amanuensis. Bauby died just two days after his book was published. An American woman, Julia Tavalaro, published a book called Look Up for Yes with Richard Tayson in 1997. Another French man, Philippe Vigand, together with his wife, wrote an account of their experiences (Vigand and Vigand 2000). A British woman, Tracey (Wilson et al. 2011; Wilson and Okines 2014), became locked-in after sustaining a tear in the basilar artery following a fall in the gym when she was 27 years old. She has remained with LIS ever since. We return to these and other accounts in Chapter Two.

To turn to the academic literature, Katz et al. (1992) looked at the long-term survival and prognosis of 29 patients with LIS. They found that survival ranged from just over two years to a little over 18 years with a five-year survival rate of 81 per cent. One study, Casanova et al. (2003), looked at 14 patients with classic or total LIS who were seen three to six months following neuro-rehabilitation. Of the 14 patients, motor recovery was seen in 21 per cent; return of swallowing in 42 per cent; verbal communication in 28 per cent; bladder and bowel control in 35 per cent; and weaning of the ventilator in 50 per cent of the patients. Early recovery from LIS may be seen after recanalisation (unblocking of an obstructed vessel) of the basilar artery (Al-Raweshidy et al. 2011). It may also occur because of fluctuations in the early stages. Hocker et al. (2015) describe a patient of theirs with “a nearly complete locked-in syndrome” (p. 832) whose recovery was monitored. He was weaned off the ventilator and the tracheostomy tube was removed at three months post stroke, by which time he had a severe dysarthria, some head control but no trunk control. At six months, he was able to sit with support of his hands; at 10 months, he was able to ambulate with a walker; at 17 months, he was able to climb stairs. So, although recovery was slow, he was able to achieve a reasonable degree of independence. The same authors say their clinical experience has shown that patients with the classic LIS from pontine infarction may improve, but rarely to functional independence.

In short, recovery does occur for some patients, but for many the LIS is complete and permanent despite some minor improvements. This was the case for Paul, as we shall see later.

2Accounts of people with LIS¹

One of the world experts in LIS is Steven Laureys from Belgium. In 2005, he and his colleagues wrote an excellent chapter called “The locked-in syndrome: What is it like to be conscious but paralyzed and voiceless?”. The authors state that the earliest report of a patient with LIS in the medical literature comes from Darolles in 1875. Even before that, however, Alexandre Dumas described the condition in The Count of Monte Cristo (1844) and Emile Zola described a case in his novel Thé rè se Raquin (1867). In this chapter we describe accounts of and by people with LIS.

To start with Dumas and The Count of Monte Cristo (1844), the character described is a Monsieur Noirtier de Villefort, said to be “a corpse with living eyes”. M. Noirtier had been in this state for more than six years, and he could only communicate by blinking his eyes: “His helper pointed at words in a dictionary and the monsignor indicated with his eyes the words he wanted” (Laureys et al. 2005, p. 496). Some years later, Emile Zola wrote in his novel Thé rè se Raquin (Zola 1867) about a paralysed woman who “was buried alive in a dead body” and “had language only in her eyes”. Thus, Dumas and Zola highlighted the locked-in condition before the medical community reported it.

The best-known case of LIS is probably that of Jean-Dominique Bauby, who “wrote” The Diving Bell and the Butterfly (Bauby 1997). Bauby had a brain stem stroke in 1995 when he was 43 years old, after which he could only move his left eye. He wrote the book with the help of an amanuensis who used a frequency-ordered alphabet which she recited aloud. Bauby blinked his left eyelid when she reached the correct letter. Unfortunately, Bauby died shortly after the book was published. A highly regarded film has since been made of his story.

Since Bauby’s book, there have been a number of others written by survivors of LIS. One of the first to appear after Bauby is Look Up for Yes by an American woman, Julia Tavalaro (with co-author R. Tayson). This was also written in 1997. Julia Tavalaro had a haemorrhage in 1966; she was comatose for seven months, during which time she was placed in ­long-term care. She gradually regained consciousness, but it was not until six years later that her condition was correctly diagnosed. Her mother and sister believed Julia was aware for years before a speech and language therapist, Arlene Kraat, worked out a way of communicating with her. In the book Tavalaro writes movingly of her pain and distress in the years before Arlene Kraat and an occupational therapist began to change her life. She used her eyes to tell of her terrible years “in captivity”. Eventually, she was able to use a communication device and wrote poetry. Julia died in 2003 at the age of 68 from aspiration pneumonia.

A French couple, Philippe and Stephane Vignand, published Only the Eyes Say Yes in 2000. Philippe Vignand had a stroke at the age of 32 and was in a coma for two months. His wife realised that he was communicating by blinking his eyes in response to her questions, but she had difficulty convincing the staff. One day when the speech therapist was assessing Vigand’s gag reflex, Vigand bit her finger, the therapist yelled and Vigand started to grin. Thereupon, the therapist asked “how much is two plus two?”. Vigand blinked four times. A letter board was then used initially until Vigand went home and was able to use an infrared camera, which was attached to another camera, allowing more sophisticated communication. The pair discuss Philippe’s LIS from their different points of view.

Other books worth mentioning are those by Pistorius and Davies (2011), Allatt and Stokes (2011), Coghlan (2013), Marsh and Hudson (2014), O’Reilly (2014) and Davies et al. (2015). Martin Pistorius (Pistorius and Davies 2011) was a normal, healthy South African boy with a younger brother and sister when he developed an undiagnosed illness at the age of 12. His parents did everything they could to find a diagnosis and a cure, but for many years Martin was “a ghost boy” trapped in a body he could not control, with no voice, no awareness and no memories. He spent his days in a care home but went home each night at his father’s insistence. Eventually he woke up; however, he was so severely disabled and mute that nobody realised he was locked-in until one of the staff at the care home came to believe that Martin was aware and insisted he had an assessment to see if he could use an alternative or augmented communication (AAC) system. This began very simply with Martin just looking at pictures, but over several years developed into a sophisticated AAC. Martin never learnt to speak but became proficient with his augmented communication to such an extent that he was able to gain employment and give lectures through a speaking computer. In addition, he gradually learnt some motor control and was able to marry. His story is certainly inspiring.

Kate Allatt (Allatt and Stokes 2011) was a fit mother of three who worked full-time. While out fell running one day she developed a headache, which she ignored. It turned out to be the onset of a brain stem stroke. She tells of the delay in recognising she was conscious and able to understand and think. She also talks of her slow and gradual recovery and the good rehabilitation care she received. Eventually Kate Allatt was able to return home; she was even able to run again. Early on she imagined that one of the nurses had tried to kill her with a “graphite drip” and was told that many LIS patients have delusions early on. This is reminiscent of Tracey (Wilson and Okines 2014), who thought at first that she was in a computer game and had to hide from the nurses. Kate Allatt showed determination and a fighting spirit. These characteristics, however, are only part of reason for her recovery while others such as Andy Davies and Tracey Okines (see below) with the same characteristics did not achieve so much. Nevertheless, Kate’s story has been inspirational for many people with LIS, including Coghlan (2013) and Davies et al. (2015).

Peter Coghlan (2013), a British ex-soldier and a bricklayer, developed a brain stem stroke in Australia. Although initially believed to be comatose and unaware, his girlfriend (later to become his wife) said that if he could hear her, he should blink, which he did. So she was the first to believe he was aware of what was happening around him. Coghlan talks about his frustration with the spelling board, which some nurses had not the slightest idea how to use. Tracey (see below) felt the same frustration. It is not difficult for those conversing with LIS people to use a spelling or alphabet board, but it does take time. Coghlan talks about his small early improvements such as his thumb twitching and then learning to swallow. He tells us about the dangers of inhalation pneumonia, which can be caused by too much laughter! Within two years he was walking and speaking again as well as working full-time.

O’Reilly (2014) is another who made slow improvements over a long time. She talks about brain plasticity, suggesting, for example, that an undamaged part of the brain can take over the function of a damaged part. This is only partly true and of limited value for most adult stroke survivors. Nevertheless, this is another book that is very positive about rehabilitation.

Andy Davies was a successful dentist who sustained his stroke at the age of 33 years. He wrote a book with his wife and mother (Davies et al. 2015). They hoped he would do as well as Kate Allatt but it was not to be. In chapter 14 he says:

My disability is profound. I have no functional movement in my body below my neck, apart from my right thumb. I can barely speak, and even my eyes do not focus properly. I have no ability to take deep breaths nor clear my throat voluntarily, and my power to select and control an appropriate emotion is almost nonexistent. About the only two functions that still work properly are my hearing and the reasoning part of my mind.

(Davies et al. 2015, p. 153)

Despite his serious physical disabilities, Andy has a good quality of life. He puts this down to his strong religious beliefs, whereas Kate puts hers down more to “bloody mindedness” and not taking “no” for an answer.

Richard Marsh (Marsh and Hudson 2014), at the age of 60, was a little older than the previous people described above when he had his stroke but, like them, he was in good physical shape; he was a fit man who had been a policeman for many years before becoming a teacher. His description of his paralysis leaving him conscious of what was happening while those around believed him comatose is compelling reading. The life support machines were about to be turned off when he managed to blink and tell those ready to notice that he was cognisant of what was happening. He describes his early care, his fears of drowning in his own saliva and the embarrassment of many of the procedures that had to be performed (such as changing his soiled diaper [nappy]). Unlike many people with LIS though, Richard made a reasonably good recovery.

All these people talk about their fears; their concerns with toileting; problems with feeding, communication and pain; and their difficulty controlling emotions.

In addition to bo...

Table of contents

• Cover
• Half-Title
• Series
• Title
• Copyright
• Contents
• Preface
• Foreword
• Acknowledgements
• 1 What is Locked-In Syndrome? What causes it? Who is most at risk? Do people recover from this condition?
• 2 Accounts of people with LIS
• 3 Neuropsychological assessments of patients with LIS
• 4 Is it always easy to diagnose LIS?
• 5 Paul’s journey
• 6 An interview with Liz, Paul’s wife
• 7 Treatment at the Raphael Hospital
• 8 Interviews with Paul’s friends
• 9 Paul’s continuing involvement with music
• 10 Quality of life for people with LIS, and assessing capacity
• 11 Ethical issues in LIS
• 12 Summary and conclusions
• References
• Appendices
• Index