1 Introduction
When, at 3:30 pm on October 30th 1990, Prime Minister Margaret Thatcher made a statement to the House of Commons on the European Council, which she had attended in Rome a few days earlier, her written statement was factual almost to the point of dryness, contrasting markedly with the robust opposition to the Council’s federalist agenda that she had conveyed in earlier statements to the press. The statement covered agricultural trade negotiations, the Hungarian economy, the situation in the Gulf, and finally the Council’s preparations for forthcoming intergovernmental conferences on economic, monetary and political union. On the latter, Mrs. Thatcher reported that she had “…reserved the United Kingdom’s position on, for example, extension of the Community’s powers into new areas, greater powers for the European Parliament in the legislative sphere, defining European citizenship, and a common foreign and security policy. All these are issues for discussion at the intergovernmental conference itself rather than to be settled in advance.”
In the unscripted debate that followed, her performance took on a strikingly different character, famously described by the Parliamentary sketch-writer Hugo Young (1998) as “…leaping with rage, ringing round the chamber, startling even those who in eleven years had much experience of the Thatcher vocabulary on Europe. ‘No! No! No!’ she bawled, her eyes seemingly directed to the fields and seas, the hills and the landing-grounds, where the island people would never surrender.” Owen (2012), who witnessed the debate from the opposition benches, recalled his impression of Mrs Thatcher as ‘on an emotional high… the adrenalin … pumping round her system as she handbagged every federalist proposal’ and cites the event as marking the emergence of ‘full-blown hubris’.
The events marked the culmination of an increasingly domineering pattern in Thatcher’s leadership style, the onset and time-course of which appears to have been reflected in the language that she used during parliamentary debate (Garrard et al. 2014a, b). They also exposed the deep ideological rifts at the heart of her Cabinet and led within weeks to her decision to resign from office. Against the background of her astonishing political success over the preceding decade, this period of the Thatcher premiership also marked out a paradigm case of what Owen and Davidson (2009) described in clinical terms as ‘Hubris Syndrome’—a distinctive change in personality brought about by the acquisition and prolonged tenure of significant power.
Owen and Davidson (2009) argued that a pattern of exuberant overconfidence, isolation, and narcissism was also associated with the periods in office of three other British Prime Ministers—Tony Blair (1997–2007), Neville Chamberlain (1937–1940) and David Lloyd George (1916–1922), and one US President, George W. Bush (2001–2009). Although the observed Hubris Syndrome changes overlapped to some extent with the personality disorders that are described in the Diagnostic and Statistical Manual of Mental Disorders (DSM), specifically the Antisocial , Histrionic and Narcissistic varieties, there were also distinctive elements, identifiable from historical records, which occurred repeatedly in these individuals. The distinctive features included: a tendency to ‘identify with the nation’; adoption of the ‘royal we’; restlessness and impulsivity; and an unshakeable belief that their decisions and actions are accountable to an authority higher than that of equally well-placed colleagues or the collective voice of public opinion. Perhaps the best known example of the last of these was the comment made by Blair looking back on his Iraq policy during a television interview in March 2006: ‘In the end’ he said, ‘there is a judgement that, I think if you have faith about these things then you realize that judgement is made by other people… and if you believe in God , it’s made by God as well’.
What are we to make of the biological basis for these recurrent and distinctive Hubris Syndrome phenomena? It is widely accepted that cognitive states (such as the formation of memories and the experience of emotions) are not only subjectively felt but can in principle be described at the level of a neurobiological event. John Coates and his co-authors Mark Gurnell and Zoltan Sarnyai (Chap. 2 of this volume) argue persuasively that the event or events in question consist of alterations in the responsiveness of neuronal circuits caused by changes in their hormonal environments. Using the ‘winner effect ’ (the competitive enhancement produced by the experience of success) as a paradigm case, Iain Robertson (Chap. 3) puts forward the related proposal that personality traits may develop as a result of local biological alterations that take place in response to specific types of experience. The changes in marking, colouring and size, as well as behaviour, that accompany any change in the ecological status of a male fish from the family Chichlidae , represent an extreme example of the transformations of which this psycho-biological continuum is capable. Although not explored in depth in this volume, the biology of addiction may also be relevant to a neural account of personality change. When a novel motivational state is encoded by the brain’s dopamine -mediated reward system a new predisposition to act (or ‘trait’) may be said to have emerged, and proponents of the psychometric school regard such predispositions (‘traits’) as the fundamental units of personality (see, e.g. Eysenck (1953), Cattell (1965) and Allport (1937)).
The central thesis of the present chapter, however, is that personality change can also result from changes to the physical integrity of the brain. At first sight this assertion may seem to hark back to simplistic notions of the brain as an interconnected series of functional units (‘modules’), first articulated in the phrenology movement of the late nineteenth century. More informed interpretations of functional architecture at both neural and cognitive levels, however, can fully reconcile ‘lesion based’ accounts of cognitive impairment with state- or system-based accounts put forward in the following two chapters. So these are not competing accounts so much as descriptions of different pathways to a common outcome—the fundamental transformation of one personality into another.
The most celebrated example of personality change following brain injury is that of Phineas Gage , the American railway construction foreman whose prefrontal cortex was destroyed by a metal rod, which he was using to pack gunpowder into a hole. When the gunpowder accidentally ignited, the rod was propelled upwards like a bullet, piercing Gage’s left cheek and travelling up through his brain, before emerging out of the top of his skull. (If you think you have a strong enough stomach, perform a Google image search on ‘Phineas Gage’.) Improbably, the physical effects of this devastating injury were minimal; the surface wounds healed effectively with a minimum of surgical intervention, and there were no impairments to the power or dexterity of the limbs. In consequence, he was before long able to return to work. The damage to the inferior frontal regions of Gage’s brain, however, resulted in dramatic changes to his personality. From one who was ‘…looked on by those who know him as a shrewd, smart business man, very energetic and persistent in executing all his plans of operation’ his behaviour turned into that of a man who was ‘…fitful, irreverent, indulging at times in the grossest profanity… manifesting but little deference from his fellows, impatient of restraint or advice when it conflicts with his desires, at times pertinaciously obstinate, yet capricious and vacillating, devising many plans of future operations, which are not sooner arranged than they are abandoned in turn for others appearing more feasible.’ (Quotations taken from the case report published by John Harlow , the provincial physician who treated Gage’s injuries (Harlow 1868)).
Industrial accidents resulting in focal penetrating injuries such as Gage’s are vanishingly uncommon in the modern era, though are still caused by gunshot wounds sustained by both military and civilian victims, and result in similar changes in judgement, social and ethical propriety, which may remain remarkably isolated from their effects on more traditional aspects of cognition. See, for instance, Damasio ’s case history of a ‘modern day Phineas Gage’ in whom inability to take decisions in his own long-term interest despite preservation of razor-sharp analytical abilities, followed damage to the same, inferior regions of his frontal cortex (Damasio 2008).
Diffuse traumatic brain injuries are more frequently recognised and often give rise to personality change in the context of a more widespread pattern of neurological and physical (orthopaedic) disability. In neurological practice, however, when isolated and insidious personality change is reported the underlying cause is most likely to be some form of neurodegenerative process.
2 The Frontotemporal Dementia Spectrum
There is an unhelpful tendency in the media to refer to Alzheimer’s disease and dementia as if they mean one the same thing. Alzheimer’s disease is, to be sure, a common cause of dementia but is no more synonymous with that clinical state than influenza is with the state of having a fever. Although in both examples the assumption of equivalence would result in a correct diagnosis most of the time, it would also lead to a substantial error rate and (at least in the fever scenario) a great many avoidable deaths from other treatable causes of infection. The stakes are perhaps not quite so high in the case of cognitive impairment, but a good clinician will always consider treatable alternatives before diagnosing a progressive and incurable disease—a piece of clinical common sense that is reflected in recommendations of expert bodies (such as the UK National Institute of Clinical Excellence and the American Academy of Neurology ), that brain imaging and blood tests including for vitamin deficiency, thyroid status and certain forms of chronic infection should be included in the workup of a patient with suspected dementia .
Even when (as is almost always the case) these simple screening tests prove to be negative, the spectrum of possible neurodegenerative causes includes too wide a range of distinct pathological processes for Alzheimer’s to be assumed by default. Again, the organised clinical mind recognises heuristic boundaries marking out the territories of rarer conditions: Creutzfeldt Jakob Disease when progression is unusually rapid; one of a growing number of genetic mutations when a patient has close relatives who are similarly affected; and in someone with symptom onset before the age of 60, one of the frontotemporal dementias.
The clinical spectrum of frontotemporal dementia is broad and encompasses a range of difficulties and disabilities that are distinct from those seen in patients with Alzheimer’s disease. Alzheimer’s typically begins with increasing difficulty forming new memories, leading to a failure to keep track of changes over days, hours, or even smaller intervals. Frontotemporal dementia, on the other hand, is heralded by difficulties in the domains of either the use of language or of ‘social cognition’. These two faculties are broadly dependent on the integrity of n...