Editorial
Neurologists woke up to the concept of medical disorders of sleep during the rich descriptive period of the late 19th century. In his classic account of the narcolepsies, based on a lecture given to the Association of Physicians at Belfast on June 3, 1927, later published in Brain (1928; 51: 63–109) and reprinted in Modern Problems in Neurology (1928), SA Kinnier Wilson (1878–1937) traces his own clinical experience to the examples of that disorder, first described by Jean-Baptiste-Édouard Gélineau (1859–1928. De la narcolepsie, Gazette d'Hôpital de Paris, 1880; 53: 626–628 and 635–637), to his appointment as house physician to Sir William Gowers (1845–1915) at the time Gowers was publishing The borderland of epilepsy (1907). Evidently, Kinnier Wilson saw no more cases until the 1920s. But medicine and sleep have an older history. William Shakespeare (1564–1616) makes 13 references to sleep in his plays (see RR Simpson, Shakespeare and Medicine, 1959). Friar Lawrence, a fine example of the medieval priest–physician whose type disappeared with foundation of the Royal College of Physicians by Henry VIII, tells Romeo:‘Young son, it argues a distempered headSo soon to bid good morrow to thy bed,
Care keeps his watch in every old man's eye,
And where care lodges sleep will never lie;
But where unbruised youth with unstuff’d brain
Doth couch his limbs, there golden sleep doth reign’
In the May issue Eldred Parry paraphrased Hamlet (‘To sleep, to die’, Brain 2008; 131: 5) reviewing Peter Kennedy's marvellous account of sleeping too much—‘The Fatal Sleep: Africa's killer disease that went undiscovered for centuries’:
... ... To die, to sleep;To sleep: perchance to dream: aye, there's the rub;
For in that sleep of death what dreams may come,
When we have shuffled off this mortal coil,
Must give us pause: ... .
Now in ‘Come sleep. O sleep, the certain knot of peace’—taken from Sonnet 39 by Shakespeare's contemporary, Sir Philip Sidney (1554–1586: poet, courtier and soldier who succumbed to the ‘The Big Sleep’ a few days after being shot in the thigh at the battle of Zutphen)—James Ironside tackles the problem of dying from too little in reviewing The family that couldn't sleep: a Venetian medical mystery by DT Max (page 1671). Set against the backdrop of a declining Venetian aristocracy, the story unfolds of a family illness that defied diagnosis for 200 years before eventually providing the first neuropathological description of fatal familial insomnia by Pierluigi Gambetti and colleagues (Lugaresi et al., Fatal familial insomnia and dysautonomia with selective degeneration of thalamic nuclei. New Eng J Med 1986; 315: 997–1003). Only later were clues pieced together that led to identification of the D178N mutation and the recognition of familial fatal insomnia as an inherited disorder of the human prion protein gene. We are fortunate in having Professor Ironside as our narrator of this detective story since, as neuropathologist to the National CJD Surveillance Unit in Edinburgh, he is part of the team that has clarified so many aspects of the aetiology, pathogenesis and clinical neurology of prion diseases over a period in which awareness of these disorders and the threat to agronomy and human health loomed and were managed politically, medically and socially in the United Kingdom. James Ironside provides his own commentary on the history of scrapie in merino sheep from Spain; the emergence of Bovine Spongioform Encephalopathy and the role of foodstuffs containing ruminant proteins; Kuru in the Fore Indians of New Guinea and those who beat a path to these remote Highlands; the personalities and achievements of the two individuals (Carleton Gajdusek and Stanley Prusiner) who have received the Nobel Prize for Medicine or Physiology for their work on prion disease, to date; the status of Creutzfeld's original neuropathological description; public and political opprobrium for some of those caught in the social maelstrom of mad cow disease; and the well-intentioned but often uncritical and wide-of-the-mark opinions of armchair commentators on this complex and evolving modern medical mystery. Despite some well argued criticisms, Professor Ironside much admires the literary talents, attention to historical detail, histological reconstructions and scholarship on display in The family that couldn't sleep.
In 2007, we published ‘Characteristics of orthostatic hypotension in Parkinson's disease’ by H Oka, M Yoshioka, K Onouchi, M Morita, S Mochio, M Suzuki, T Hirai, Y Ito and K Inoue from the Department of Neurology, Jikei University School of Medicine, Tokyo, Japan (Brain 2007; 130: 2425–2432). This paper has been withdrawn. We understand that control observations relating to Valsalva manoeuvres and the head-up tilt table tests do not relate to the same individuals in whom cardiac 123I-meta-iodobenzylguanidine scintography was reported. Five other papers, published in other neurological journals, containing similar mis-representations of results are also withdrawn. We are told that an investigative committee of Jikei University School of Medicine considers these to be serious mistakes but concludes that errors in dealing with the data did not occur intentionally.
Two papers in the present issue deal with aspects of multiple sclerosis. Markus Krumbholtz and investigators from Martinsreid, Munich, Berg and Regensburg (Germany) show that those patients showing increased expression of B cell activating factor of the tumour necrosis factor family (BAFF) in response to interferon beta, in vitro and in vivo, have enhanced antibody production and so, in theory, be disadvantaged by generating neutralizing antibodies against beta interferon and self antigens (page 1455). Karen-Amanda Irvine and William (Bill) Blakemore provide support for the view that, over and above the direct effect of inflammation, demyelination contributes per se to axonopathy, presumably through loss of trophic support, thereby emphasizing the need to enhance remyelination as part of a comprehensive strategy for limiting and repairing the damage in multiple sclerosis (page 1464). Amongst four papers on electrical properties of the cortex, Francesco Noè and colleagues from Milan (Italy), Kuopio (Finland), Columbus (Ohio, USA) and Innsbruck (Austria) assess the therapeutic potential of gene therapy in a model of temporal lobe epilepsy and show a remarkable effect on the frequency and progression of seizures in association with increased hippocampal expression of human neuropeptide Y (page 1506). Exploiting the opportunities for modelling complex disorders in invertebrates, Paul Thornhill and colleagues from Newcastle-upon-Tyne show that the fukutin and Pomt1 variants of the glycosyltransferase deficiencies, manifesting as muscular dystrophies, can be reproduced in zebrafish having mutations that downregulate the gene for fukutin-related protein (FKRP) to produce abnormal muscle fibre organization and ocular and neuronal dysmorphology in association with defective 
-dystroglycogan glycosylation and laminin binding (page 1551, and see cover). Amongst five papers on movement disorders, Ludvic Zrinzo and investigators from London (United Kingdom), Umea (Sweden) and Msida (Malta) promote the credentials of the pedunculopontine nucleus as a target for deep brain stimulation and provide an MRI- and atlas-based map for stereotactic localization of its rostral and caudal poles as the basis for reliable evaluation of pedunculopontine nucleus stimulation in the treatment of Parkinson's disease (page 1588). The three papers on neurodegenerative disease include a detailed radiological and histological correlation showing that the Pittsburgh compound-B derivative 6-CN-PiB binds amyloid-ß(Aß)42, Aß40 and vascular Aß but not neurofibrillary tangles in the prefrontal and temporal cortex; pre-mortem positron emission tomography in one individual allows correlation with regional quantitative analyses of substrate-specific binding emphasizing the potential use of PiB-PET imaging for reliable evaluation of Aß plaque burden in life (page 1630).
The East Anglian city of Norwich may seem an unlikely place for a fierce 19th century debate on aphasia. But did it actually take place? On page 1658 Marjorie Lorch from London (UK) traces the background to the 38th meeting of the British Association for the Advancement of Science in 1868 at which Dr Frederick Bateman (senior physician to the Norfolk and Norwich Hospital, former student of Paul Broca, corresponding member of the Academy of Medicine of Paris and author of On Aphasia and the localisation of the faculty of speech: 1870, second edition 1890) exercised his role as a member of the organizing committee by inviting John Hughlings Jackson to speak ‘On the physiology of language, founded on facts supplied by cases of diseases of the brain’ and Paul Broca to present ‘On the seat of the faculty of articulate language’. But far from a fierce debate ensuing between these presenters, Dr Lorch concludes that, although Broca's views on the value of single case studies and theoretical aspects of cerebral localization were not well received by experts in the audience, it is far from clear that neither Hughlings Jackson nor Broca attended each others’ lectures on August 24th and 25th, respectively.
On page 1433 Holly Bridge and colleagues from Oxford use tractography based on diffusion-weighted imaging to characterize the altered occipital cortical connections in GY, a patient with blindsight. In From the Archives we review the first description in detail of that phenomenon: ‘Visual capacity in the hemianopic field following a restricted occipital ablation’ by Larry Weiskrantz (deputy editor of Brain from 1882 to 1991), Elizabeth Warrington, Michael Sanders and John Marshall (Brain 1974: 97; 709–728).
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