Editorial

When he wrote the fourth in the series of eight ‘Bridgewater Treatises on the Power and Wisdom and Goodness of God as manifested in the Creation’ on The Hand: its mechanisms and vital endowments as evincing design in 1833, Sir Charles Bell explained that his motivation was that of the anatomist wishing to refute the representation of life as the mere physical result of certain combinations and actions of parts; and his text moved from the definition, evolution and comparative anatomy, and structure and function of the hand towards a broader account of sensibility, touch and muscular sense, and the hand as the instrument of complex bodily, social and cultural activities: ‘the hand is not a thing appended, or put on, like an additional movement in a watch; but a thousand intricate relations must be established throughout the body in connection with it … the power of the hand … accommodated [man] to every condition through which his destinies promised accomplish[ment]’. In the same spirit of man as neither divine nor beast but as embodied subject, Raymond Tallis entitled the first volume of his philosophical trilogy on personal identity, The Hand: a philosophical inquiry in human being (2004: reviewed by John Cornwell, Brain 2005: 128; 443–446). Now, in ‘The muddle of embodiment’ James Le Fanu reviews The kingdom of infinite space: a fantastical journey around your head in which Raymond Tallis again adopts a phenomenological philosophical stance, sifting what is obvious from what is not and elevating the currency of the mysterious and metaphysical above the illusion of explanation that lies in materialism (page 1678). In asking ‘who am I?’ Prof. Tallis has moved from the forelimb to the head, and focused first on its physicality. He elevates many bodily functions—salivation, vomiting and yawning, inter alia—from brutish physiology to a status in language, cultural symbolism and social interaction; and, chiming with Sir Charles Bell (Essays on the anatomy and philosophy of expression, 1824), Tallis converts reflex blinking into the language of winking, and facial grimacing into the amused smile that acknowledges the concept of time. But Dr Le Fanu is disappointed by Tallis's decision to duck any account of the guts of the matter—the brain itself—arguing that it is ‘absurdly over-rated’ and necessary but not sufficient for understanding the mystery of personal identity. James Le Fanu considers this to be Hamlet without the Prince of Denmark; the principal guest not invited to the party: ‘halfway through the journey, his investigation of the interdependence of the head and self fizzles out and the further stages are a grievous disappointment’. And whereas Raymond Tallis pauses to contemplate the privacy of thinking when reflecting in the mirror on his stubble, blemishes and facial movements that reveal nothing of ‘the silent cacophony of thought’, James Le Fanu sees the deeply enigmatic colours of mind as simply spiralling out of the creative process of ‘thinking about thoughts’. James Le Fanu studied the humanities before switching to medicine in Cambridge, and trod the specialist corridors of several metropolitan hospitals before settling on his hybrid career as general practitioner in south London and writer of a weekly column in the Sunday and Daily Telegraph newspapers, with occasional contributions to several other learned journals. In the present context, his journalistic titles include: ‘even earwax has its place in the grand order of things’, ‘why our nails are the cutting edge of civilisation’, ‘remembrance of smells past’, ‘how the blind really do see with their fingers’ and much else besides. He has written The rise and fall of modern medicine, winner of the Los Angeles Prize Book (2001), and Why us?: how science rediscovered the mystery of ourselves (2009) in which Le Fanu argues that mystery is the foundation of science, and the natural world far more profound and beautiful than intuition or superstition might suggest; nor is he shy to argue that much of modern imaging and the quest for the holy grail of consciousness reveals a neuroscience emperor claiming new clothes. On all of this, author and reviewer are in full agreement. Several papers in the current issue resonate with the issues that Charles Bell, Raymond Tallis and James Le Fanu address. On page 1645, Georg Goldenberg and Josef Spratt from München (Germany) and Vienna (Austria) explore the efficient use of tools in aphasic patients and demonstrate that the dominant parietal lobe contributes important knowledge on the general principles of grip and manipulation involved in tool use but not detailed awareness of how individual items best work or their interactions with other mechanical objects or materials. The personal and social consequences of losing a hand hardly need elaboration: Paul Marasco, Aimee Schultz and Todd Kuiken from Chicago (USA) describe the sophisticated sensibility acquired by previously rather insensitive skin when nerves that once sensed a now amputated arm are redirected to intact proximal muscles and the chest wall creating the impression that the missing hand is itself being stimulated and providing a theoretical basis for improved design of neural-machine interface-based prostheses (page 1441). A more direct beneficial effect on regenerating nerve provided by the α-2,8 polysialic acid moiety of neural cell adhesion molecule interacting with the endogenous fibroblast growth factor receptor, is described by Ali Mehanna and investigators from Hamburg and Jena (Germany), New Jersey (USA) and Shantou (China) who show that enhanced Schwann cell remyelination rather than neuroprotection underlies the functional improvements seen in mice recovering from transection of the femoral nerve (page 1449). Hillel Aviezer and colleagues from Jerusalem (Israel) and Toronto (Canada) probe the ability of people with Huntington's disease to appreciate differences in facial expression and show that, despite deficits in the appreciation of individual emotions, carriers of the huntintin mutation are no less able to perceive disgust when paradoxically embedded in an expression of sadness, and vice versa, than controls—findings that draw attention to the relevance of context in the processing of emotion (page 1633). Amongst a batch of papers in the current issue having a neurogenetics flavour, Anne Kjersti Erichsen and investigators from Oslo (Norway) calculate and compare the prevalences, on 1 February 2008, of the hereditary ataxias and spastic paraplegias in southeast Norway, noting generally higher rates and similarities and differences in the frequency of each sub-type, by comparison with other surveys but setting a new gold standard for morbidity statistics that combine classical and molecular classifications (page 1577). Cyril Goizet and 25 co-authors from Paris, Bordeaux, Rouen, Nantes and Marseille (France), Sfax (Tunisia), Jena (Germany), London and Newcastle-upon-Tyne (UK) and Lisbon and Santa Maria da Feira (Portugal) take forward a molecular epidemiological account of the spastic paraplegias and highlight the importance of mutations in CYP7B1 in a large sample of index cases suspected in advance as more (6 of 82, 7.3%) or less (3 of 90, 3.3%) likely of harbouring a CYP7B1 mutation based on the family pedigree and clinical phenotype (page 1589). John Vissing and colleagues from Copenhagen (Denmark) and Texas (USA) compare two patients with myophosphorylase deficiency having unusual exercise tolerance with a large number of more typical examples of McArdle's disease and controls showing that plasma lactate, peak workload and oxidative capacities increase paradoxically after exercise in these atypical cases—findings that are attributed to small amounts of residual myophosphorylase activity and which suggest a therapeutic dividend from strategies that achieve even small increases in muscle enzyme levels (page 1545). Writing from the National Institutes of Health (USA), Marinos Dalakas and colleagues set the anti-CD52 humanized monoclonal antibody, alemtuzumab, the triple challenge of depleting lymphocytes both in the circulation and in muscle infiltrates and altering the clinical phenotype of inclusion body myositis in order to prove the principle with respect to the immunological nature of this ambiguous acquired muscle disease; overall, the pre-treatment rate of clinical decline is reduced from 15% to 2% per annum and the systemic lymphopaenia matched by substantial reduction in CD3 endomysial infiltrates and expression of stressor molecules (page 1536). Mahinda Yogarajah and a group of investigators from London and Manchester (UK) and Rome (Italy) bring new tools to an old trade—mapping the anatomy of Meyer's loop and its vulnerability during anterior temporal lobe resection (see also Brain 2005; 128: 1959–1961) to show that, because distance from the tip of Meyer's loop to the temporal pole is variable [range 24–43 (47), mean 34 (35) mm in cases (and controls)], how much brain can safely be removed without risking a disabling visual field defect is unpredictable and requires pre-operative diffusion tensor tractography to establish boundaries in the individual scheduled for epilepsy surgery (page 1656; and see cover). Aurélie Tchoghandjian and colleagues from Marseille and Vitry-sur-Seine (France) tackle the basis for the relatively aggressive behaviour of childhood optic pathway astrocytomas using microarray and immunocytochemistry to show that tumours affecting the chiasm and hypothalamus arise from Notch2 and vimentin- and glial fibrillary acidic protein positive radial glia present in the normally developing chiasm and originating from the floor of the third ventricle—a cell of origin considered distinct from that giving rise to the less aggressive but related pilocytic astrocytoma of the (optic nerve and) cerebellum (page 1523). Previously we reported two series of these tumours, each collected in illustrious North American centres over a period of nearly five decades, in which the poor prognosis of the chaismal lesions, and the glial fibrillary acidic protein signature of the cell of origin, were documented. In From the Archives we review ‘Evaluation and management of gliomas of the anterior visual pathways’ by N. R. Miller, W. J. Iliff and W. R. Green (Brain 1974: 97; 743–754) and ‘The biological and clinical behaviour of pilocytic astrocytomas of the optic pathways’ by Adam Borit and Edward P. Richardson Jr (Brain 1982: 105; 161–187).

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