Editorial
Scientists working in a previous Decade of the Brain (the 1660s) were the first reliably to link function to structure in the nervous system. But not everyone agreed on the physical basis of human behaviour. In Meditationes de Prima Philosophia (1641), René Descartes (1596–1650) had already made the case for a separation of mind and body, distinguishing in ‘Cartesian dualism’ brain and intelligence from self-awareness and consciousness, and formulating the contemporary mind-body debate. In ‘Brain and (Bad) Behaviour’, John Cornwell brings the journalist's ability to probe the vulnerable points in any argument, together with good working knowledge of neuroscience and its implications for society, to debate the neural basis for morality and creativity (page 1339). Mr Cornwell is an affiliated research scholar in the Department of History and Philosophy of Science and director of the Science and Human Dimensions project at Jesus College, Cambridge. He works on the public understanding of science and medicine, and has written The Power to Harm (Viking, 1996), Nature's Imagination (1995), Consciousness and Human Identity (1998) and Explanations (2004). John Cornwell reviews Hardwired Behaviour: What Neuroscience Reveals about Morality by Laurence Tancredi, The Creating Brain: The Neuroscience of Genius by Nancy C. Andreasen and Brain Fiction: Self-deception and the Riddle of Confabulation by William Hirstein. Leaving black-box neuropsychology in the slipstream of contemporary neuroscience that increasingly claims a structural basis for more and more human behaviours, John Cornwell draws attention to some simplistic (we might say silly) reductionist formulations: inter alia, the San Francisco killer's plea of ‘not guilty’ through diminished responsibility as a result of having eaten too many Twinky cup cakes. How far should the cursor be allowed to drift in the direction of ‘physicalism rather than mentalese’ when contemplating the nature of good and bad behaviour? In claiming more brain and less mind, are we devaluing the currency of choice and free will and regarding even our responses to the Ten Commandments and the Seven Deadly Sins as no more ‘than an articulation of responses etched in the biological structure of the brain’? And to reinforce the limitation of failing to understand the roles of imagination and freedom in morality and artistic creativity, Mr Cornwell re-tells the scene from Peter Brook's play based on Oliver Sacks's The Man Who Mistook His Wife for a Hat in which the neurologists and their patients gaze with satisfaction at a brain scan, oblivious to the possibility that, in accepting this depiction of the mind as informative, they have not spotted their own cultural and spiritual agnosias.This month, Oxford University Press adds a third dimension to Brain-Online. All material from the first issue in 1879 through to the end of 1996 is now available as a full text PDF with searchable headers and abstracts, all images and graphics, and links to similar articles, as part of a newly created electronic archive. To retain authenticity, covers, notes, editorial board membership and advertisements are also provided. Abstracts are all on open access. Full text is currently only available through library subscriptions but individual articles may be accessed through our Pay Per View system. Details are at www.oxfordjournals.org/collections/archives. To celebrate their centenary of journals publication, Oxford University Press has created a collection of 100 papers chosen to represent the breadth of material that the journals they now publish have produced over this time. These will be freely available online, and further information is available on the Oxford Journals website. In creating this list, selected journals were invited to identify one article that best illustrates their publication record. Brain was allowed two selections. So, what did we choose from 126 years, during many of which we have been the preferred location for publishing original articles on the nervous system in health and disease? The Associate Editors rated step changes in knowledge, general applicability of the work, an emphasis on clinical neuroscience and the social context in which discoveries were made—the stuff of history. We did not agree, and so the Editor had to apply a touch of autocracy to this otherwise democratic process. We chose the following:
- (Sir) Gordon Holmes. The symptoms of acute cerebellar injuries due to gunshot injuries. Brain 1917; 40: 461–535; doi:10.1093/brain/40.4.461
- Norman Geschwind. Disconnection syndromes in animals and man. Brain 1965; 88: 237–294; doi:10.1093/brain/88.2.237 (part 1): and 1965; 88: 585–644; doi:10.1093/brain/88.3.585 (part 2) [see also Brain 2005: 128; 2217–8 and 2224–39]
Whether any papers in the current issue will make the Hall of Fame when the next birthday at Brain or Oxford University Press is celebrated remains to be seen. On page 1113, Arun Bokde and colleagues from the Ludwig-Maximilian University, Munich, Germany, plot functional connectivity involved in a face-matching task in normal individuals and a group presumed to have early Alzheimer's disease, all with normal functional MRI activation, to show altered connectivity throughout the hemisphere that predicts subsequent loss of cognitive function attributable to abnormalities of the fusiform gyrus. Sarah Hart and a team from Duke University, North Carolina, USA, characterize the neural basis for spatial and verbal working memory during each phase of acquiring and retrieving knowledge in girls with Turner syndrome (page 1125): perhaps the specific impairments in frontoparietal circuitry tell us about influences of the X chromosome on development of these pathways. Kathrin Finke and investigators from the Ludwig-Maximilian University (Munich), Friedrich-Schiller University (Jena) and Huntington Center South, Germany, use an ingenuous set of tasks to show that a lateralization bias in visual attention (to the left) may be a biomarker of cytosine–adenine–guanine (CAG) triplet repeat number in Huntington's disease, whereas reduction in processing speed and storage capacity correlate with progression and severity (page 1137).
Amongst the papers on neurogenetics in the present issue, Hyder Jinnah and an international team from Johns Hopkins Hospital and the University of Maryland (Maryland), Iowa City (Iowa), Peapack (New Jersey) and San Diego (California: specifically, Dr William Nyhan) in the USA; and Nijmegen (the Netherlands), Madrid (Spain), Cordoba (Argentina), Paris (France) and Sofia (Bulgaria) report on motor function in the largest series to date of people with the Lesch–Nyhan syndrome. The experience of these 44 cases and an additional 254 culled from 122 prior papers highlights the characteristic action dystonia superimposed on baseline hypotonia, set in the context of contemporary ideas on pathophysiology in the basal ganglia (page 1201). Francesco Muntoni and colleagues from Imperial College, the Royal Veterinary College, Harefield Hospital and Oswestry (United Kingdom), Pitié-Salpétrière and Hôpital Cochin (Paris, France), and the National Institutes of Health (Bethesda, USA) report the atypical severity and clinical course in two patients with proven mutations leading to Emery Dreifuss muscular dystrophy but harbouring additional mutations in related proteins, thus representing the complex phenotype of digenic effects on different parts of a presumed functional pathway (page 1260). Iris Martínez-Juárez and colleagues from Los Angeles (USA), Mexico City and Guadalajara (Mexico), Tegucigalpa (Honduras), Madrid (Spain), San Miguel (El Salvador) and Lima (Peru) conclude that separation of juvenile myoclonic epilepsy (JME) from other types of idiopathic generalized epilepsy is warranted, by prospectively studying the phenotype and evolution in 257 families (page 1269). They conclude that four subtypes exist: ‘(i) the more frequent classic JME with or without spanioleptic absences, (ii) pyknoleptic childhood absence epilepsy evolving to JME, (iii) JME with adolescent onset pyknoleptic absences, and (iv) the rare JME with astatic seizures’. Pierre Thomas, Luc Valton and Pierre Genton from Nice, Toulouse and Marseilles, France, describe 14 cases in whom absence or myoclonic epileptic status was precipitated by drugs used to treat seizures that had in fact been classified incorrectly (page 1281): the most common error was not to recognize JME and to treat these young people with carbamezapine.
In 2001, a group of experts met at the Royal College of Physicians in London and devised the McDonald criteria for the diagnosis of multiple sclerosis. Published in the Annals of Neurology, the paper is much cited (and will have helped our sister journal to raise its impact factor). Now, those criteria are revised and the new paper is also published in Annals of Neurology (Polman et al. Diagnostic criteria for multiple sclerosis: 2005 revisions to the ‘McDonald Criteria’. Annals of Neurology 2005: 58; 840–6). We reproduce, gratis, an advertisement for these criteria at the request of the National Multiple Sclerosis of the USA, and take this opportunity to welcome the new Editor of the Annals and Dr Stephen Hauser's team of Associate Editors from the University of California, San Francisco. Three papers in this issue of Brain deal with multiple sclerosis. Mariusz Stasiolek from Lodz, Poland, and collaborators in Würzburg and Göttingen, Germany, characterize the phenotype, frequency and responsiveness of plasmacytoid dendritic cells: specifically, the lack of an effect on CD4+Foxp3+ regulatory T cells suggests a mechanism for immune dysregulation in multiple sclerosis (page 1293). Wensheng Lin and colleagues from the University of Chicago, Wake Forest University Baptist Medical Center, Virginia Commonwealth University School of Medicine and New York University School of Medicine in the USA show that conditional expression of IFN-
in transgenic mice suppresses remyelination in two models of demyelination by increasing endoplasmic stress in remyelinating oligodendrocytes, effects that are further enhanced by elimination of a kinase normally responding to endoplasmic stress (page 1306). Joel Black and Stephen Waxman from Yale School of Medicine, USA, and Kenneth Smith from King's College, London, trace the maturation of ion channel profiles on central axons, stably remyelinated by endogenous Schwann cells, and show that this faithfully reproduces the arrangement of Nav1.6 and Kv1.2 seen in normal nodes of Ranvier (page 1319).
The plight of conjoined twins understandably makes the news, as developments in medical technology offer new possibilities for successful separation in increasingly complex anatomical situations. The most famous pair, Chang and Eng Bunker (each 1811–1874), from whom the epithet ‘Siamese’ derives, were xiphopagus—joined by a band of cartilage at the chest, and with one liver. Separation would now be a relatively simple matter. After being paraded as a fairground curiosity, Chang and Eng settled in the USA, and married two sisters; but they fell out and required separate homes, which the twins attended on alternate days eventually siring an estimated 22 children (precise details are vague) between Adelaide and Sarah Anne Bunker (née Yates). Amongst the survivors of these accidents of nature are about 2% of conjoined twins who are craniopagus, with fused skulls and varying degrees of brain distortion. Not until 1952 was success achieved with attempts at separating such twins. Oscar Sugar, from the University of Illinois, Chicago, recognized that gradual isolation of the venous circulations was crucial if either twin was to survive (Grossman et al. Surgical separation in craniopagus. Journal of the American Medical Association 1953: 153; 201–207): of the two Brodies, ‘Rodney’ lived for 11 years, but his sibling had died in the peri-operative period. On page 1084, James Stone and James Goodrich review the literature on 64 sets of craniopagus twins reported from 1919 and propose a new classification updating that introduced by John O'Connell, who studied with Sugar in Chicago and later worked as a neurosurgeon at St Bartholomew's Hospital, London, in the 1970s: O'Connell categorized cases as partial or total vertical, and, following Herbert Grossman and Oscar Sugar, emphasized the crucial issue of shared venous sinuses. Professor Stone assisted Oscar Sugar, to whom their paper is dedicated, at an unsuccessful craniopagus separation in 1980. In suggesting his classification, John O'Connell drew on a personal experience of three separations in which one twin had survived on each occasion. O'Connell's formulation embraced his knowledge of embryology and anatomy. In From the Archives, we review O'Connell's paper on the developmental anatomy of the cerebral veins and sinuses venous from a much earlier period in the career of this eminent but modest British neurosurgeon (O'Connell JEA. Some observations on the cerebral veins. Brain 1934: 57; 484–503).
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