Identity Unknown

How acute brain disease can destroy knowledge of oneself and others

Barbara A. Wilson, Claire Robertson, Joe Mole

• Publisher: Psychology Press
• Year: 2014
• ISBN:
  9781317649113
• Edition: 1
• Topic: Psychology
• Subtopic: History & Theory in Psychology
• Index: Psychology

Imagine being unable to recognise your spouse, your children, or even yourself when you look in the mirror, despite having good eyesight and being able to read well and name objects. This is a condition which, in rare cases, some brain injury survivors experience every day.

Identity Unknown gives an exceptional, poignant and in-depth understanding of what it is like to live with the severe after-effects of brain damage caused by a viral infection of the brain. It tells the story of Claire, a nurse, wife, and mother of four, who having survived encephalitis, was left with an inability to recognise faces – a condition also known as prosopagnosia together with a loss of knowledge of people and more general loss of semantic memory

Part One describes our current knowledge of encephalitis, of perception and memory, and the theoretical aspects of prosopagnosia and semantic memory. Part Two, told in Claire's own words, is an account of her life before her illness, her memories of the early days in hospital, an account of the treatment she received at the Oliver Zangwill Centre, and her description of the long-term consequences of encephalitis. Claire's profound insights, clear writing style, and powerful portrayal of her feelings provide us with a moving insider's view of her condition. These chapters also contain additional commentary from Barbara Wilson, providing further detail about the condition, treatment possibilities, potential outcomes, and follow-up options.

Identity Unknown provides a unique personal insight into a condition which many of us have, for too long, known too little about. It will be of great interest to a broad audience including professionals working in rehabilitation settings, and all those who have sustained a brain injury, their families and carers.

Part I

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Chapter 1

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Encephalitis

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What is encephalitis and how common is it?

Encephalitis simply means an inflammation of the brain (Granerod & Crowcroft, 2007). There are two major types of encephalitis. The first is caused by an infection from a virus, bacteria or a parasite, whereas the second results from an abnormal immune response in which the body attacks itself (Stone & Hawkins, 2007) and can be triggered by a recent infection or vaccination (ibid.). The most common infections, at least in the Western world, are those caused by the Herpes simplex virus (the same virus that causes the common cold sore) and Varicella zoster (the virus that causes chicken pox and shingles). Measles, mumps and cytomegalovirus are examples of other viruses that can cause inflammation of the brain. In other parts of the world, West Nile virus, Japanese virus and viruses from the bites of ticks or mosquitoes may be the cause (Stone & Hawkins, 2007; Stapley, Atkins, & Easton, 2009). Frequently, indeed in more than 50% of cases, the infecting virus cannot be determined (Granerod & Crowcroft, 2007; Stapley et al., 2009).

Of the non-infectious encephalopathies, inflammation of the brain is caused by the central nervous system attacking itself. Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating condition that mainly affects children and young adults. One study showed that it was triggered by an infectious illness or vaccination in 74% of cases (Stone & Hawkins, 2007). This happened to Kate, a young teacher, who developed a sore throat and an influenza-type illness. After a few days she lost consciousness and was later diagnosed with ADEM. Although physically handicapped, Kate was cognitively unimpaired. She describes her journey and her slow but continuing improvement over a 14-year period in a book of survivors’ stories by Wilson and Bainbridge (2013).

There are, of course, other kinds of autoimmune encephalopathies. One rare and recently identified autoimmune form is anti-NMDA receptor encephalitis, where NMDA stands for N-methyl-D-aspartate. This is an acute form of encephalitis, potentially lethal but with a high probability of recovery, which is caused by an autoimmune reaction. An excellent book, written by a survivor of this form of encephalitis, was recently published (Cahalan, 2012). Susannah Cahalan was a successful young reporter (aged 24 years) for the New York Post when she started to become unwell with strange symptoms. She became worse and began to experience hallucinations, paranoia and seizures. One diagnosis from the medical profession concluded that she was having a breakdown caused by stress, while another suggested it was symptoms of withdrawal due to alcoholism. Continuously deteriorating, Susannah became extremely thin, was hospitalised, and came close to being admitted to a long-stay psychiatric unit. She was then fortunate enough to be seen by a Syrian-born psychiatrist, Dr Najjar. He gave her the classic “clock” test, usually administered by neuropsychologists, which indicated that she had unilateral neglect (most often associated with a right hemisphere stroke). In other words, Susannah had organic damage to her brain. She then had to undergo a biopsy, which revealed that her brain was inflamed, caused by cells from her immune system attacking nerve cells in her brain. Four years earlier, Dr Dalmau (from Pennsylvania) had identified a rare type of autoimmune encephalitis, NMDA autoimmune encephalitis. Susannah became the 217th person in the world to be diagnosed with this rare disorder. Treatment began and she slowly improved to the extent that she eventually returned to work, and now continues to be a brilliant journalist and gifted speaker. Most professionals involved in the treatment of Susannah before her final diagnosis thought her illness was due to stress or emotional difficulties, so, sadly, she was not referred for neuropsychological assessment, which would have detected signs of organic brain damage much earlier.

For many people, encephalitis begins with an influenza-type illness or headache, followed, hours or days later, by more serious symptoms, which can include a drop in the level of consciousness (ranging from mild confusion to coma), high temperature, seizures, sensitivity to light and other changes in behaviour. As Stapley and colleagues (2009) state, the types of symptoms seen in encephalitis reflect the specific areas of the brain affected by inflammation. The range of possible symptoms and their rate of development vary widely, thus making it difficult to actually diagnose encephalitis. Granerod and Crowcroft (2007) provide a good review of the epidemiology of encephalitis, while Stone and Hawkins (2007) deliver a comprehensive medical review.

Stone and Hawkins (2007) estimate that, in the USA alone, one in a million people each year contract Herpes simplex viral encephalitis (HSVE), while Leake and colleagues (2004) suggest that the incidence of ADEM is 0.4–0.8 per 100,000 population per year. Looking at all cases, the Patient.co.uk website (www.patient.co.uk) reports that there are around 4000 cases of encephalitis each year in the UK, with infections being seen most frequently and most severely in children and the elderly. Hokkanen and Launes (2007) state that 20,000 cases occur each year in the USA. However, a recent paper by Granerod, Cousens, Davies, Crowcroft, and Thomas (2013) suggests that the incidence is higher than has been thought. The only previous study looking at the incidence in the UK (Davison, Crowcroft, Ramsay, Brown, & Andrews, 2003) reported 1.5 cases per 100,000 population per year for viral encephalitis alone. However, as most cases are of unknown aetiology and an increasing number of viruses are known to cause the condition, this was felt to be a serious underestimate. Furthermore, many cases are not reported, despite the fact that encephalitis is a notifiable disease in the UK.

Granerod and colleagues (2013) carried out an exhaustive investigation of hospital records to estimate the number of encephalitis cases in England “attributable to infectious and noninfectious causes” (p. 1455). They found an incidence rate of 5.23 cases per 100,000 population per year. The incidence rate was highest among patients less than one year of age and over 65 years of age. Females were 8% less likely to contract the disease than males. This paper is recommended for those interested in the incidence of encephalitis.

Compared to other infectious diseases, encephalitis has a high mortality rate. Some 10% of those with encephalitis die from their infections or from complications resulting from a secondary infection. Some forms of encephalitis have more severe outcomes, including Herpes encephalitis, for which the mortality is 15–20% with treatment and 70–80% without treatment (Stapley et al., 2009).

Help for survivors and their families, as well as for those who have lost a loved one, is provided by the Encephalitis Society (see the section “The Encephalitis Society” later in this chapter and www.encephalitis.info). For parents, siblings and grandchildren who have been bereaved, another source of assistance is the support group The Compassionate Friends (www.tcf.org.uk).

The neuropsychological consequences of encephalitis

A host of cognitive, emotional and psychosocial problems are faced by survivors of encephalitis (Kapur et al., 1994; Hokkanen, Salonen, & Launes, 1996; Hokkanen & Launes, 2007; Pewter, Williams, Haslam, & Kay, 2007). Details of the neuropsychological assessments of 19 survivors, with various forms of encephalitis, are presented in the paper by Pewter et al. (2007). For the purposes of this book, however, we focus on the neuropsychological consequences of HSVE, first because it is the most common kind seen in the Western world, and second because it is the viral encephalitis that Claire endured. The virus tends to attack the temporal lobes, the orbitofrontal cortices and the limbic system. For this reason, memory and executive problems are common (Stapley et al., 2009). Some people, of course, suffer very mild effects from the illness, or none at all, while others are left with devastating deficits and handicaps. Pewter et al. (2007) report that memory deficits are the most common cognitive problems experienced, with a high percentage of survivors also showing impairments of executive functioning. These latter issues include difficulties with planning, organisation, problem solving and divided attention. Hooper, Williams, Wall, and Chua (2007) noticed that executive deficits in children are also associated with higher levels of parental stress.

One of the best-known survivors of HSVE in the UK is Clive Wearing (Wilson, Baddeley, & Kapur, 1995; Wilson, 1999; Wearing, 2005; Wilson, Kopelman, & Kapur, 2008). Clive, a professional musician, conductor and world expert on the Renaissance composer Orlando Lassus, became ill with HSVE in March 1985. He has one of the most severe cases of amnesia on record; he cannot retain information for more than a few seconds, he cannot learn new information, and he has lost much of the knowledge about his earlier life. Thus, he has both anterograde and retrograde amnesia. Here, it is debated as to whether it is possible to have one without the other, but most people with anterograde amnesia have some retrograde amnesia. Retrograde amnesia without anterograde amnesia is a much rarer condition. Nevertheless, it has been documented in patients with HSVE (O’Connor, Butters, Militois, Eslinger, & Cermak, 1992; Fujii, Yamadori, Endo, Suzuki, & Fukatsu, 1999; Tanaka, Miyazawa, Hashimoto, Nakano, & Obayashi, 1999). Hokkanen and Launes (2007) provide a more detailed discussion of memory problems following HSVE.

In addition to memory deficits, executive problems are also reported, which include difficulties with planning, organisation, problem solving and divided attention. In one study by Utley et al. (1997), 9 of 22 HSVE patients had persistent executive impairments. Pewter and colleagues (2007) report that up to 84% of their sample showed problems in tests of executive functioning. Language problems, typically anomia (i.e. a naming deficit), are frequently experienced (Benjamin et al., 2007). Category-specific semantic deficits may also be seen, where people are much better able to recognise one category of objects than another. Commonly, they have a disproportionate problem recognising living things compared to non-living things (Warrington & Shallice, 1984). Two of the patients in the Warrington and Shallice study, JBR and SBY, both of whom had sustained HSVE, showed “a striking discrepancy between their ability to identify inanimate objects either with pictures or words and their inability to identify living things. J.B.R. was almost at ceiling level on the visual inanimate object condition, yet he virtually failed to score on the visual living things condition” (p. 837). JBR is the same patient as Jason, described in Wilson (1999), who showed the same discrepancy when asked to draw objects. He was able to produce good drawings of a bicycle and an aeroplane, for example, but could not draw a recognisable fish or bird. The opposite pattern is less often seen but does occur. Sacchett and Humphreys (1992) report a stroke patient who was better with living things than manufactured objects, and Laws and Sartori (2005) describe the same phenomenon in a patient with HSVE.

Acquired disorders of reading are also seen after encephalitis (Kapur et al., 1994). Indeed, Jason, in addition to his problems with recognising living objects, also had a surface dyslexia, which means errors in reading aloud words, with an atypical correspondence between spelling and sound (e.g. pronouncing the written word “sew” to rhyme with “new”, “few”, “chew” and so on). Clive, too, had a mild form of this disorder (Wilson, 1999). Perceptual problems, including agnosia (failure to recognise objects) and prosopagnosia (face blindness) are also seen, but these will be addressed in Chapter 2. Suffice it to say here that Pewter et al. (2007) administered two perceptual tests (the Object Decision and Cube Analysis subtests) from the Visual Object and Space Perception Battery (Warrington & James, 1991) and found that 32% of patients failed the Object Decision subtest and 5% the Cube Analysis subtest.

As well as cognitive problems resulting from HSVE, there can be emotional, psychosocial and behavioural difficulties (Pewter et al., 2007; Stapley et al., 2009). A large-scale survey of members carried out by the Encephalitis Society (Dowell, Easton, & Solomon, 2000) found that 68% reported frustration and anger, 67% anxiety, 59% mood swings and 58% depression. Pewter and colleagues (2007), in order to measure psychiatric distress, administered the Revised Symptom Checklist 90 (Derogatis, 1983) to 37 post-encephalitic patients (not all with HSVE), and found that 10 of the 11 indices were elevated in their sample. Only paranoid ideation remained within normal limits. Participants scored highest on the obsessive-compulsive, depression, phobic anxiety and global severity indices. In children, of course, educational problems are most likely to be part of the picture. Starza-Smith, Talbot, and Grant (2007), working in a service for children, said that 80% of their referrals were for behavioural and educational concerns...

Table of contents

• Cover
• Half Title
• Title Page
• Copyright Page
• Table of Contents
• List of illustrations
• Series preface
• Foreword by Dr Bonnie-Kate Dewar
• Preface
• Acknowledgements
• Part I
• Part II
• Appendix 1 A summary of Claire’s one-day preliminary assessment at the Oliver Zangwill Centre
• Appendix 2 A summary of Claire’s rehabilitation programme
• References
• Index