Book Review |
Apoplexia and Lethargus
Death casts a shadow over words like stupor and coma. They do not inspire optimism either in literature or in everyday experience. Coma, transcribed directly from the Greek
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, was associated with death in the writings of Hippocrates. Johann Wepfer (1620–93), credited with the first demonstration of the association between apoplexy and cerebral haemorrhage, gave a vivid and oft-quoted account of the coma preceding the death of the first case in his Historiae Apoplecticorum (1658): ‘... the Abbot ... found him prostrate upon the ground, insensible to shouts, to shaking and pinching of the body, the same in the trunk, senseless’. In his novel Clayhanger, Arnold Bennett describes the prolonged illness and slow death of Darius, his Cheyne-Stokes respiration and his withdrawal ‘into some fastness more recondite than sleep’. Stupor, with the same Latin root as stupidity, but usually less persistent, was referred to by Macaulay in recording the death of James II: ‘... James sank into a stupor which indicated the near approach of death’. All is not gloom. In Shakespeare's play, the wife of Pericles dies giving birth to a daughter, aptly named Marina, during a storm at sea. Her body, in a ‘chest ... caulked and bituméd’, is cast overboard in an effort to still the waves and the superstition of the sailors. Washed ashore, it is opened in the presence of the lord of Ephesus who muses that ‘Death may usurp on nature many hours, and yet the fire of life kindle again the o’erpressed spirits’. Sure enough, ‘she ’gins to blow into life's flower again’ and makes a full recovery.
The child of the Shunammite woman also awoke unexpectedly. In the harvest field with his father, he collapsed with the cry ‘my head, my head’ (a favourite quotation of those teaching on subarachnoid haemorrhage). He died at noon on his mother's lap. After some delay he was attended by Elisha who ‘lay upon the child, and put his mouth upon his mouth ...’ (a favourite quotation of those teaching on cardiopulmonary resuscitation). ‘The flesh of the child waxed warm’; he sneezed seven times and opened his eyes.
And again there's Snow White.
The surprises of literature mirror the uncertainties of clinical practice. It was the difficulty of predicting amongst patients in coma who might live and who might die that prompted the young Fred Plum, recently appointed as Head of the Division of Neurology in the University of Washington, to embark on a systematic study of unconscious patients admitted to King County Hospital. The study was to continue in the New York and Bellevue Hospitals after his translation to Cornell University where he succeeded Harold Wolff, under whom he had trained, as the Anne Parrish Titzell Professor of Neurology. His primary aim was to find clinical ways of differentiating those patients who required neurosurgical intervention from those requiring medical treatment. He was assisted by Jerome Posner, who had been his second resident in Seattle and who also moved to New York to appointments at Cornell and the Memorial Sloan-Kettering Cancer Center. Having amassed a wide experience, which they assimilated into a coherent and logical discourse they published together The Diagnosis of Stupor and Coma in 1966.
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There is often something special about books that cut across the conventional boundaries of specialty designation and disease classification. They bring a new perspective to their subject and broaden its scope. Plum and Posner's book, although grounded in neurological science and technique, exceeded those traditional territories and included the study of a wide range of endocrine and metabolic disorders. It combined an informative theoretical text with practical guidance on a systematic approach to the unconscious patient. Its clarity and the originality of its method assured its success. There was no other recent or contemporary volume which was comparable and but scant reference in earlier literature to the assessment and diagnosis of states of impaired consciousness. Whilst there were no diagnostic issues involved in the death of Socrates, his executioner perhaps deserves credit for his objective assessment of the effects of the draught he administered. According to Plato ‘... the man who gave the poison began to examine his feet and legs, from time to time; then he pressed his foot hard, and asked if there was any feeling in it.’ He recorded finally that ‘... his eyes were fixed’.
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A more recent text is something of a landmark in the history of coma and merits pause for consideration. It was acknowledged in the preface to the first edition of Plum and Posner but with the observation that having been written a hundred and fifty years previously it was ‘somewhat out of date’. John Cheyne (1777–1836), his name, as we have already seen, permanently hyphenated with that of William Stokes, practised in Edinburgh, where he was an associate of Charles Bell, and later in Dublin, where he also held the post of Physician-General to the Army in Ireland in which he had earlier served in the field. Among his several publications was one entitled Cases of Apoplexy and Lethargy with Observations upon Comatose Diseases. Many of the clinical descriptions and post mortem illustrations of his cases can be identified within our present classification of cerebrovascular diseases. His use of the word lethargy does not equate with the susceptibility with which many of us are familiar but applies to one of the seven genera believed at the time to belong to the order comata. Apoplexia and lethargus were the two most important but were distinguished by their clinical and pathological features, a distinction lost on Sir John Falstaff (Henry IV, Part 2) who, addressing the Lord Chief Justice on the matter of the King's illness, declares that ‘This apoplexy, as I take it, is a kind of lethargy, an’t please your lordship, a kind of sleeping in the blood, a whoreson tingling’.
Cheyne had the ability to convey in a sentence or two a rounded and vivid account of the predicament of his patients: ‘A florid and corpulent woman of fifty, heart-broken by the misconduct of her children, and secretly a dram-drinker, being, after a fatiguing walk in a warm day, struck with an intense pain in the side of her head, hurried into a shop, sick and alarmed. When her servants came to her assistance she was quite insensible’. Surely a model history. Regarding another patient he observed that ‘the extreme of insensibility from intoxication ... is sometimes, with difficulty, to be distinguished from apoplexy’ and, again in a different context, that ‘... we draw our prognostic from the quantity of sensorial power which still exists in the body. Thus we do not despair until the pupil ceases to contract’. These are but a few examples of a text, elegant in style, which prefigured some of today's wisdom. Cheyne did not hesitate to scorn those with whose opinions he disagreed. On the subject of the anatomy of apoplexy: ‘If I have not misunderstood Morgagni, (which, from his rambling and parenthetical manner, and defective arrangement, is not improbable,) he is not consistent in his commentary upon these opinions’. He castigated Dr Cullen, a President of the Royal College of Physicians of Edinburgh and friend of Adam Smith, for his denial of the existence of catalepsy because he had not himself seen a case: ‘But to believe only such facts as our own observation enables us to verify, would be such an excess in scepticism, as would soon check all enquiry’. He had it in for Cullen and went on to say that ‘lethargic diseases are not mentioned ... an omission very remarkable, when we consider the space they occupy in the writings of those physicians, whose labours Dr Cullen had no hesitation in profiting by on other occasions’. On balance, not of course on principle, one is inclined to side with the President. Cheyne was prejudiced against alcohol. This coloured both his case histories and his aetiological speculations. In the instructions he left about his funeral arrangements he stipulated that there was to be no tolling of bells but that the ringers should be compensated to the usual amount in an order for bread; not in money as they would only spend it in the ale-house, and even in death he ‘... would by no means give occasion for sin’.
Second and third editions of Plum and Posner were published in 1972 and 1980. We now welcome the fourth, which is even better than its predecessors. The delay in its appearance for more than 25 years was, as explained by the authors, occasioned by a number of factors, particularly the steady progress in the development of imaging techniques, which so dramatically enhanced diagnostic procedures; also the changing concepts of the neural basis of consciousness, all of which the senior author, now retired, was anxious to have included. The new edition has expanded its title and its authorship but not to any significant extent its girth, for which, as for much else, it is to be commended. The two new authors write with the same clarity as do their seniors—perhaps not surprising as both trained with Drs Plum and Posner at Cornell. Although the book has been largely rewritten, it retains much of the original organization and structure. An early chapter on the physiology and pathophysiology of consciousness and coma contains new and updated material, but that on the examination of the comatose patient preserves much of Plum's original writing. Many of the figures from the earlier editions, such as those on pupillary changes and ocular reflexes, are also retained but with some modification and enhancement by the use of colour. Reorganization and expansion of the sections dealing with the structural and the multifocal, diffuse and metabolic causes of coma lead to greater clarity and the description of intracranial shifts in the pathogenesis of coma is exemplary.
In the interval since the publication of the third edition there have been many advances and discoveries which are now incorporated. This is reflected in the very high proportion of references of recent date in all sections of the book. The discovery that narcolepsy is associated with a loss of the orexin neurones in the posterior and lateral hypothalamus (Thannickal et al., 2000
), the conservative management of chronic subdural haematoma (Voelker, 2000), the recognition of amyloid angiopathy as a cause of cerebellar haemorrhage (Itoh et al., 1993), the inclusion of prion diseases among the non-structural causes of coma (Johnson, 2005), the use of serum prolactin levels in distinguishing epilepsy from psychogenic unresponsiveness (Chen et al., 2005)—these are but a few examples of the new material introduced in this volume. The most striking advances, and those which have had the greatest impact on the approach to the unconscious patient, have been in the field of imaging. The immense value of CT and MRI scanning in the diagnosis of patients in coma is acknowledged, and they, and the several variants on their basic technologies, are discussed in detail and beautifully illustrated in relation to both routine and experimental investigations. The point is made that in clinico-pathological correlations nowadays ante mortem scanning compensates to some extent for the regrettable decline in post mortem examination. In a chapter on psychogenic unresponsiveness, it is emphasized that psychologically induced symptoms are not wholly imaginary and are often associated with measurable changes in brain function. Studies are quoted illustrating the application of functional imaging in demonstrating abnormalities in a variety of psychogenic disorders. The value of these techniques in studying patients in minimally conscious and vegetative states is also demonstrated and there is fascinating reference to their ability to correlate residual cerebral circuits with the fragments of behaviour sometimes seen in patients in vegetative states.
With the development of these sophisticated imaging techniques and of increasingly refined electrophysiological and biochemical markers, there is a temptation to anticipate their gradual usurpation of the traditional clinical tests that are the mainstay of assessment of patients in coma in the emergency room. The issue was addressed by Bates (2006) in a thoughtful article written in response to a Dutch study (Zandbergen et al., 2006), which postulated the superiority of biological numbers derived from somatosensory evoked potentials (SSEPs) and neuron-specific enolase (NSE) measurements in determining outcomes in patients in coma after anoxic-ischaemic brain damage. It seems likely that for the foreseeable future the ‘approach to management of the unconscious patient’, the title of a chapter which could stand alone as a vade-mecum for emergency physicians and surgeons, will continue to be based essentially on clinical assessment, and that the descriptors we employ, such as the Glasgow Coma Scale (Teasdale and Jennett, 1976), will continue to serve a useful purpose. The challenge must be that training and practice are brought up to the standards which the book promotes.
The sections of the book relating to the vegetative state and brain death are written in the context of US regulatory and legal frameworks. Whilst these vary to some extent in different countries, the definitions and general principles enunciated are with few exceptions generally applicable. For detailed guidance in the UK, we turn to the reports of working parties of the Royal College of Physicians of London, endorsed by its sister Colleges, and to the publications of the General Medical Council. Discussion on brain death and the vegetative state does turn our thoughts to the immensely difficult ethical questions which they raise and the book ends with a section on ethics written by J.J. Fins.
Times have changed since the days when pneumonia, ‘the old man's friend’, was allowed to carry us off without interference and when the hastening of death by pain relieving or sedating medication was unquestioned and but rarely abused. Modern life-support technology has changed things and precipitated questions not only about the definition of death but also about the right of those approaching it to influence its termination. Whilst philosophers from earliest times have been preoccupied with the moral issues attending life and death, the question of whether life must be preserved irrespective of its quality has been increasingly to the fore in recent years. In Who Owns Our Bodies?—an unattractive title but a good question—John Spiers advances the argument that we ourselves own our bodies; that choice is a principal expression of that ownership and, indeed, that choice is the most basic of all freedoms. He moves on to develop the theme of personal autonomy and to the conclusion that we have a right to determine our own end. Not everyone believes in the importance of personal choice in medical matters. On the subject of his own illness Alan Bennett writes: ‘One reason why one goes to a doctor is to try and make another person responsible for one's life’. Today choice is emphasized in the context of reforms of health services; it is to be hoped that one day it will be extended to end of life decisions, not just to choice of hospital or doctor. Doctors cannot press for greater responsibility in decisions on the withdrawal of life support or for legalization of assisted suicide without risk of accusations of arrogance or self-interest. It is to be hoped nevertheless that public awareness and debate on these issues will spread and that enlightenment and clarity will eventually prevail.
These are not the issues with which the book is primarily concerned but they confront all those who are involved with patients suffering profound incapacity whether at the beginning or the end of life.
Fred Plum has always had a reputation for directness. For a short time, about five minutes, I bore him a grudge. In the course of a recruiting mission at the time of his move from Seattle to New York in 1963 he visited us in our modest clapboard house on the outskirts of Rochester, Minnesota. He accepted the offer of a pre-lunch refreshment. Unfortunately our only drinking vessels were superannuated, supermarketed jam jars of surprising elegance and enormous capacity. Such were the clefts and refractive properties of their deeply bevelled base that a normal measure of whisky did no more than question the cleanliness of the glass or the generosity of the host. Handed a ‘shot’ of visible proportions, Fred did not comment other than to proceed to the kitchen and pour half of it down the sink—a spectacle shocking to a frugal Scot. Some years later, in a collaborative study of prognosis in non-traumatic coma involving groups in New York, Glasgow, Newcastle upon Tyne and San Francisco, we were to enjoy the exhilaration, and sometimes the exhaustion, of working with him; to sense the determination in the pursuit of excellence and the qualities of leadership which have inspired so many of his colleagues. There cannot be many books which are dedicated to one of its authors. Fred Plum's name no longer occupies pole position in the authorship, but to a reader of the preface and the dedication, the high regard of his co-authors and their gratitude for his guidance and leadership shine through. His contribution to this latest edition is graciously acknowledged by them and the inclusion of his name in its new title ensures that he will always be remembered as its originator. This is a great book and a fitting tribute to him who inspired it. And I wasn’t really upset about the whisky—it was only bourbon.
Formerly of University of Newcastle upon Tyne
References
Bates D. Can somatosensory evoked potentials and neuron-specific enolase reliably predict outcome in postanoxic coma? Nat Clin Pract Neurol (2006) 2:472–3.[Web of Science][Medline]
Bennett A. Untold stories (2006) London: Faber and Faber Limited.
Chen DK, So YT, Fisher RS. Use of serum prolactin in diagnosing epileptic sseizures: report of the therapeutics and technology assessment subcommittee of the American academy of neurology. Neurology (2005) 65:668–75.
Cheyne J. Cases of Apoplexy and Lethargy with observations upon Comatose diseases (1812) Edinburgh: Adam Black.
Itoh Y, Yamada M, Hayakawa M, et al. Cerebral amyloid angiopathy: a significant cause of cerebellar as well as lobar cerebral haemorrhage in the elderly. J Neurol Sci (1993) 116:135–41.[CrossRef][Web of Science][Medline]
Johnson RT. Prion diseases. Lancet Neurol (2005) 4:635–42.[CrossRef][Web of Science][Medline]
Spiers J. Who owns our bodies? (1997) Oxford: Radcliffe Medical Press.
Teasdale G, Jennett B. Assessment and prognosis of coma after head injury. Acta Neurochir (Wien) (1976) 34:45–55.[CrossRef][Medline]
Thannickal TC, Moore RY, Nienhuis R, et al. Reduced number of hypocretin neurons in human narcolepsy. Neuron (2000) 27:469–74.[CrossRef][Web of Science][Medline]
Voelker JL. Nonoperative treatment of chronic subdural haematoma. Neurosurg Clin N (2000) 11:507–13.
Zandbergen EGJ, Hijdra A, Koelman JHTM, et al. Prediction of poor outcome within the first three days of postanoxic coma. Neurology (2006) 66:62–8.
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