Editorial
In October 2006, we published a remarkable personal account of acquired amusia with recovery written by a member of that elite band of neurologists who are also competent musicians. Sadly, that was to be the last of his many Brain papers that Ian McDonald read in print. Commenting on the account, Oliver Sacks drew attention to ‘its candour and depth, its narrative force and its self analysis ... how precious such accounts are when they come from physician-patients’; and Jason Warren writing on the disordered musical emotion Professor McDonald described, suggested that ‘the affective response hints at ... processing that ... depend(s) on ... mechanisms in the parietal lobe’. Now, in ‘Another musical mystery tour’, Jason Warren reviews Musicophilia by Oliver Sacks and This is your brain on music by Daniel Levitin sidestepping the trap that to write ‘about musical experience is like dancing about architecture’ by finessing the new respectability of debating the neurology and neuroscience of the musical brain (page 890). Dr Warren is more attuned to the brain-based reductionist approach taken by Oliver Sacks than the analysis of music as an experience that exists outside any interpretative neural programmes offered by Daniel Levitin. Unable, or unwilling, to find fault with details of the illustrative case-lore, here neuro-musical in flavour, that has been Oliver Sacks's highly successful stock-in-trade, Jason Warren anticipates that Musicophilia will become Sacks's ‘most beloved book’. Orchestrating a veritable cacophony of illustrative material—from the Rolling Stones and Led Zeppelin to music hall burlesque and the contemporary and classical repertoires—Jason Warren takes a reductionist approach to his formulations on the ‘divinity of music’. He builds on the evidence that listening to music activates the ‘ancient subcortical and limbic apparatus of biological drives, rewards and punishments’: music provides an emotional response that is linked to memory and experience; it is quite different from and goes beyond the cognitive pleasure of problem solving. Dr Warren resists any attempt to yoke music to language-based or naturalistic (inter alia bird-song) analyses, although the music of a nation may resonate with the linguistic structures of that native language (Elgar speaks English; and Fauré parle francais). Music allows the brain to encode and represent feeling states at times other than their primary experience, that is ‘off-line’; and it serves to harness and resolve the turbulent experience of shifting emotional states into structures that are, in the main, pleasurable whilst also accommodating related cognitive processes such as memory.Tracing the story from 1944, and giving it special flavour through his own experiences with post-encephalitic parkinsonism from the 1960s, Oliver Sacks is not in doubt concerning the role and benefits across a range of neurological disorders of music therapy. Jason Warren is not so sanguine. On page 866 of this issue, Teppo Särkämö and colleagues from Helsinki, Jyväskylä and Turku (Finland) and Montreal (Canada) exploit the widespread brain activations and engagement of emotional and mnestic processes in music to show—using a single-blind, randomized and controlled design—that mood, confusion, attention and verbal memory are improved at 6 months after stroke in those who listen daily to self-selected music compared with individuals rehabilitating in a soundless environment or those exposed merely to language through listening to audio-books of their choice.
Our editorial policy at Brain is to communicate individually by email with all the corresponding authors and, especially for the disappointed, briefly to explain the basis for our decisions. Although some template letters are used, all correspondence contains text that is exclusive to the paper in question. No one of our associate editors has been more informative, sensitive or courteous in transmitting this information than John Hodges who has handled most of the material submitted in the field of dementia and behavioural neurology since the present editorial team assumed office in 2004. With a change in his professional appointment from Cambridge (UK) to Sydney (Australia), Professor Hodges no longer feels in a position to act as an associate editor. Our readers in general and those with an interest in behavioural neurology in particular will join us in thanking John for his work over the last 3 years. That role is now taken over by Argye Hillis who we welcome to the editorial team. Dr Hillis is qualified in speech-language pathology, neuropsychology, and medicine and is now a professor of neurology at John Hopkins Hospital (Baltimore) and deputy director of the department of neurology with faculty appointments in physical medicine, rehabilitation and cognitive neuroscience: she has served several other journals in an editorial capacity; and her work on stroke, language and dementia syndromes has received prestigious awards from the American Neurological Association and the American Academy of Neurology. We are delighted that Dr Hillis has accepted our invitation to bring these many talents to the editorial process at Brain. Lisa Edwards will also be well known to those who interact with the editorial office in Cambridge. She is leaving to take up freelance work in the publishing world: we are grateful for her skill in managing all practical aspects of manuscript handling over the last 3 years, and often in designing the cover for each issue; and we welcome Joanne Bell as the new Editorial Assistant.
Readers of Brain-and his many friends, colleagues and students in the United Kingdom and throughout the world-will be sorry to learn of the death of Peter (PK) Thomas, after a long period of ill health, on January 25th, 2008, aged 81. ‘PK’ was editor of Brain from 1982 to 1991, during which the range of material published, and hence the scale of the journal, increased substantially-‘PK’ serving as an editor in the real sense by not only managing the review process and selection of material submitted but taking a direct role in improving the precision and language of every paper that eventually was published. Of his own articles, the first in Brain (on Xanthomatous neuropathy in primary biliary cirrhosis) appeared in 1965 and the last (on Clinical, pathological and genetic characterization of hereditary sensory and autonomic neuropathy type 1) in 2006. His death marks the end of a great period in world neurology, in which ‘PK’ and his second wife Anita Harding were such popular and central figures on the stage of international neurology.
Amongst several papers on neurogenetics, Violeta Mihaylova and colleagues from Munich, Bonn, Münster, Berlin and Essen (Germany), Valencia and Barcelona (Spain), Riyadh (Saudi Arabia), Rome (Italy), Belgrade (Serbia), Prague (Czech Republic), Bratislava (Slovakia), Budapest (Hungary) and Curitiba (Brazil) gather together 22 individuals (11 not previously reported) with congenital myasthenia harbouring 20 separate mutations of the acetylcholinesterase collagen-like tail subunit (COLQ) to describe the typical neonatal or infantile presentation, but with some examples having later onset, that may mimic DOK7 limb-girdle congenital myasthenic syndrome, and showing a clinical course not necessarily severe or progressive but with rather little response to anticholinesterase therapy (page 747). Bernt Engelsen and investigators from Bergen and Trondheim (Norway) and Milan (Italy) provide a detailed analysis of the epilepsy that characterizes mitochondrial disease resulting from mutations of POLG1; most typical is occipital epilepsy syndrome – persistent flickering light, post-ictal visual loss, perversions of visual perception and disordered eye movements – but frontal lobe seizures, simple and complex partial epilepsy, myoclonus and generalized seizures with status epilepticus carrying a poor prognosis for survival, all may occur (page 818). Of the three papers dealing with aspects of inflammatory brain disease, Leonora Fisniku and investigators from institutions in London (UK) bring up-to-date the cohort with clinically isolated demyelinating syndromes identified by the group of Ian McDonald between 1984 and 1987 and studied prospectively, now at 20 (range 18–28) years and with much improved ascertainment compared with the previous (14 year) report. The message has not changed materially: MRI abnormalities carry an 82% risk of conversion to multiple sclerosis compared to 21% in the group with normal brain imaging at presentation; and, moreover, the evolving MR profile during the early years reliably predicts the late clinical course and disability (page 808).
Amongst papers on the dementias, Clifford Jack and investigators from Rochester and Pittsburgh (USA) advance the story on molecular imaging of amyloid deposition using the 11C Pittsburgh Compound (PiB) to show that individuals with mild cognitive impairment have a profile that is intermediate between the high global cortical PiB retention with low hippocampal volume that is exactly the opposite of the pattern seen in cognitively intact individuals; regional variations in the ratio of PiB uptake to grey matter loss correlate with cognitive performance and perhaps reflect the temporal evolution of amyloid deposition and neuronal loss in Alzheimer's disease (page 665). Stefan Klöppel and a team from London (UK), Freiburg (Germany), Scottsdale and Rochester (USA), Paris (France) and Rome (Italy) use linear support vector grey matter T1-weighted MRI to show high specificity and sensitivity in the automated computer-based diagnosis of Alzheimer's disease and its distinction from frontotemporal dementia, that is transferable between machines and across different centres (page 681).
The subject of frontotemporal dementia received much attention in 2006 with reports of mutations in progranulin (PGN) as the genetic basis in many cases with ubiquitin-positive, tau-negative inclusions (FTLD-U). Following the initial reports in Nature, we published 5 papers on PGN in a special issue (Brain 2006: 129; 2799–3137) marking the centenary of Alzheimer's description in 1906; and other articles have since appeared in Brain and elsewhere. Now, we publish 3 further papers on the neurology of PGN mutations. Jonathan Beck and investigators from London and Birmingham (UK) identify a 4 basepair insertion mutation in exon 2 as the most prevalent (20/25) cause of familial early onset frontotemporal dementia in the United Kingdom, with pathological confirmation of type 3 TDP-43-positive pathology in those coming to autopsy, and 4 other variations accounting for the remaining five cases (page 706): the distinct phenotype is typically behavioural with apathy but examples of progressive aphasia syndromes are also seen – overall, disease onset is later (especially in those with a modifying APOE-E4 genotype) and dementia more accelerated in individuals with these PGN mutations than in other categories of frontotemporal dementia. Stuart Pickering-Brown and colleagues from Manchester and Birmingham (UK) screened for mutations of PGN and tau (MAPT) in large cohorts (each >200) of cases with frontotemporal dementia and motor neuron disease presenting consecutively, many with pathological confirmation: they catalogue the spectrum of mutations, finding a different set from the selected London series, and with some hitherto undisclosed relatedness between probands (page 721); they reveal genetic influences on disease onset but not the clinical course; and the progressive aphasia phenotype correlates with PGN mutations by comparison with other cases of frontotemporal dementia whereas, in this series, behavioural disorder with apathy implicates MAPT. In a series of >500 cases from Paris, Rouen, Lille, Marseille, Reims and Toulouse (France) and Antwerp (Belgium) Isabelle Le Ber and colleagues document a variety of pathological mutations associated with frontotemporal dementia: no clear-cut genotype-phenotype correlations emerge but the spectrum of features complicating the core syndromes and accumulating over time is associated with gradual evolution of SPECT imaging abnormalities and, presumably therefore, the spreading pathology (page 732). Details of the clinical neuroscience and genetic insights into the frontotemporal dementias are new but the disorder is not. In ‘From the Archives’ we review ‘On aphasia due to atrophy of the cerebral convolutions’ by Dr G(iovanni) Mingazzini, professor of neuropathology at the Royal University of Rome (Brain 1914: 36; 493–524).
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