Editorial
In ‘Fashion and cult in neuroscience—the case of hysteria’, Simon Shorvon reviews The Story of Blanche and Marie (by Per Olav Enquist), Freud's Wizard (by Brenda Maddox) and Human Traces (by Sebastian Faulks), illustrating why ‘the scientist as stone mason, chipping away at the rock of ignorance and uncovering the glorious forms of reason’ is an awkward metaphor given that ‘the march of science has an erratic course. and ... a trajectory influenced continuously by personality cult and by public fashion’ (p. 3342). The dominant personality appearing centre-stage or providing a sub-text to both novels and the one biography under review is Jean-Martin Charcot. Professor Shorvon reveals the voyeuristic Parisian neuroscience theatre in which, as ‘circus ringmaster’, Charcot and his troupe mixed art, technology and clinical description ... ‘with lurid clinical descriptions of the subjects’ sexual fantasies (délire erotique) and photographs of women and girls in varied stages of hystero-epilepsy and undress’. In this, the élite of Parisian neurology was undoubtedly aided and abetted by compliant patients, none more-so than Blanche Wittman. Despite the invitation to this literary peep-show, Simon Shorvon reminds us that Charcot's methods were ... ‘absurd and frankly unethical—a mixture of magic and theatrics, served up with a dressing of not-so repressed sexuality’. Enter stage left, Sigmund Freud and his amanuensis, Ernest Jones—no slouch himself, it would seem, on matters of precocious and inappropriate sexuality, activities that earned a reputation sufficient to wreck his application for the post of Clinical Assistant at Queen Square in 1906.Much of the history and flavour of these events that launched neurology and psychiatry on their intermittently intimate 20th century relationship pops up in Human Traces. Readers will know the fates of the two central characters, and the message Sebastian Faulks conveys on the merits and demerits of mind-therapies rooted in mainstream neurology or psychiatry. His exposition of the hypothesis that ‘only man is mad; what makes madness therefore must be what makes man’, and ideas developed during Dr Thomas Midwinter's subsequent expedition to Africa, fuel the rift between Thomas and his brother-in-law, Dr Jacques Rebière, that follows Midwinter's public lecture at the Schloss Seeblick on May 12th 1909. But, reasonably to our reading, Professor Tim Crow claims originality in his writings from 1995 for the interesting concept that psychosis was a formative component in the speciation event that gave rise to Homo Sapiens and a necessary element in the evolution of human language (Lancet 2006: 367; 727). Two papers in the current issue touch on the interface between mind and brain. Lara Menzies and colleagues from Cambridge, Welwyn Garden City, and GlaxoSmithKline (UK) correlate behavioural performance on a response inhibition task with altered structural patterns of grey matter in affected individuals and their 1st degree relatives to define a quantitative cognitive and brain imaging endophenotype for obsessive-compulsive disorder (p. 3223). Bruce Hermann and investigators from Madison and Chicago (USA) study an incident population with childhood onset epilepsy to show a much increased frequency and the social morbidity of co-existing attention deficit hyperactivity disorder (p. 3135), marked by regional variations in brain structure but not attributable to any identifiable risk factor that may predispose to epilepsy.
Three papers in the current issue focus on astrocytes. Adrienne Müller, Thomas Hauk and Dietmar Fischer from Ulm (Germany) prioritize the role of astrocyte-derived ciliary neurotrophic factor, signalling through activators of transcription 3 and cyclic AMP, in enhancing retinal ganglion cell axon regeneration, rather than oncomodulin and molecules produced by activated macrophages, as previously reported (p. 3308; and see cover). Using tissue from cases of neurodegenerative disease or epilepsy and normal individuals, Ronald Verwer and colleagues from Amsterdam (the Netherlands) refine the distribution and cellular architecture of the adult human neocortex to show that, rather than providing a specific marker for neurogenesis, doublecortin (DCX) stains peripheral astrocyte processes that form envelopes around neurones and is therefore better regarded as a marker of glial-neuronal interaction. Carla Jung and a team from Frankfurt and Heidelberg (Germany) evaluate pre-operatively patients with a variety of brain tumours to show that serum glial fibrillary astrocyte protein (at levels >0.05 µg/l) is a sensitive and completely specific biomarker for glioblastoma multiforme, and one that also correlates quantitatively with tumour size and necrosis (p. 3321).
On p. 3297, Elan Louis and colleagues from New York, Kansas and Hawaii (USA) and Saskatoon (Canada) report autopsy findings in 33 cases of essential tremor either showing loss and abnormalities of surviving cerebellar Purkyn
cells and the dendate nuclei in cases with early onset, gait disturbance and a strong family history; or, in a minority of cases older at onset, Lewy bodies in the locus ceruleus. Other papers on neurogenetics include the report by Doreen Fialho and investigators from Queen Square, London, and Birmingham (UK) who have sequenced the entire coding region to catalogue mutations of CLCN1 in 22 families with myotonia congenita as the basis for genotype–phenotype correlations in dominant and recessive pedigrees (p. 3265); taking forward the evidence for a dominant-negative effect of the exon 8 mutation hot-spot, they then screen >300 suspected cases to validate their exon hierarchy analysis strategy and catalogue the distribution and features of the existing and novel mutations now revealed. Ekehard Hewer and a team from Zurich (Switzerland), München, Bad Nauheim and Mainz (Germany), Santiago (Chile) and New Mexico (USA) return to the clinical features and muscle histology in X-linked McLeod neuroacanthocytosis to show in the original patient—Hugh McLeod—and 9 others that the muscle involvement is mainly secondary to a neurogenic defect that primarily affects motor and sensory nerves but also results in psychiatric manifestations, cognitive impairment, movement disorder, epilepsy and systemic features that are often the cause of death—together constituting a syndrome that may be confused with Huntington's disease (p. 3285). Rudolf Kley and colleagues from Bochum, Lübeck, Bonn, Dresden, Freiburg, Erlangen, Halle and München (Germany) and St Gallen (Switzerland) describe the evolution of limb weakness (mimicking limb-girdle muscular dystrophy) with respiratory muscle involvement and prominent cardiac involvement, together with details of muscle appearance on imaging and biopsy, in 31 affected individuals from four families having the same mutation of FLNC, implicating a founder effect, that results in one form of myofibrillar myopathy (p. 3250). Emma Groen and the group of Kate Bushby from Newcastle-upon-Tyne (UK) analyse 85 patients with limb-girdle muscular dystrophy (LGMD2A), many but not all of whom have mutations of CAPN3 (calpain 3), to propose a strategy for diagnosis that prioritises genetic testing in the evaluation of this complex and heterogenous phenotype (p. 3237).
We receive not a few ‘original articles’ that turn out to be systematic reviews or meta-analysis of the existing literature. More often than not, referees share our own view that the value of these analyses—dependent on the quality of the papers on which they are based, a high proportion often not qualifying for inclusion and so raising issues of sampling—rests on their ability to reveal truths buried in the primary literature. Those that we do accept appear as review, not original, articles and the present issue contains two on stroke. Based on 101 publications describing 3353 experimental animals, Hendrik van der Worp and colleagues from Utrecht (the Netherlands), Edinburgh (UK) and Melbourne (Australia) conclude that cooling to temperatures that might be achieved in clinical practice improves outcome after ischaemic stroke in animals by one-third. And Maria Orfei and investigators from Rome (Italy), Iowa City and Phoenix (USA) and Fremantle (Australia) survey the literature from 1990 to 2007 on anosognosia (‘lack of awareness or the underestimation of a specific deficit in sensory, perceptual, motor, affective or cognitive functioning’) after stroke; failing to find a consistent thread in that literature on frequency, features or mechanisms, they propose a way forward that, we can hope, will resolve these issues (p. 3075). Amongst our original articles, Anne Ducros and colleagues from the group of Marie-Germaine Bousser in Paris (France) tackle the nature and consequences of reversible cerebral vasoconstriction syndrome in a series of 67 patients many of whose illnesses occurred in association with exposure to vaso-active substances (p. 3091). In ‘Cerebral vasoconstriction, headache and sometimes stroke: one syndrome or many?’, Jan van Gijn—Associate Editor of Brain and former editor of the Nederlands Tijdschrift voor Geneeskunde brings a clinically experienced and historically alert head to bear on the nosological status of this disorder—lacing his commentary with apt Oslerian aphorisms (p. 3060). At the same time as Sigmund Freud was developing his ideas on psychoanalysis and Josef Breuer was ‘treating’ Anna O, the founder of the Neurologisches Institut at the University of Vienna was deliberating on the interface between normal and abnormal cerebral vessels that we published 123 years ago. In ‘From the Archives’, we return to ‘The cerebral blood-vessels in health and disease’ by Professor Heinrich Obersteiner (Brain 1884: 7; 289–309).
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