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The internet www.jnnp.com,4 as have customised ...... @lerts, collected resources, and emailed tables of contents, with free back issues, rapid responses, and electronic manu- Don’t slip through the net script submission via Bench>Press in- troduced recently. In this issue, the first R Al-Shahi, C Kennard of a series of review articles deals with 5 ...... the internet itself. Subsequent articles will focus on resources for JNNP could help you become a technophile (including neurophysiology and neuro- radiology), neurosurgery (including neuropathology), and psychiatry (in- t is possible to thrive in the modern people across the world (almost 10% of cluding neuropsychiatry). Every review world without recourse to the internet. the global population) were online in will be free at www.jnnp.com, and But only just. If you are a “techno- May 2002 (www.nua.com). Health is I the recommended web sites are available phobe”, deterred by the daunting size probably the largest single category of as downloadable Bookmarks/Favorites and complexity of this continually evolv- information hosted by the world wide at http://jnnp.bmjjournals.com/misc/ ing medium, JNNP could help you web, and over two thirds of the online bookmarks.shtml. If you are already a become a “technophile”. public—known as “cyberchondriacs”— 1 “technophile”, or if we succeed in con- Fewer than four decades ago, the cold seek information about it. verting you, join us in our attempt to war motivated the creation of the inter- Despite its impact, the internet is still keep pace with the internet in the net. Academic inspiration subsequently flawed. Access in the developing world monthly Neuronline filler section.6 harnessed the internet’s potential in its remains limited, but initiatives like Digital two best-known components: email and Island seek to broaden the availability of J Neurol Neurosurg Psychiatry 2002;73:611 the world wide web. Born of a need for material—such as JNNP—and make the communication and maturing through a world a smaller place.2 While freedom to hunger for information, these two publish is part of the essence of the world ...... human desires have made the internet wide web, cultural constraints will pre- Authors’ affiliations indispensable. clude consensus about what constitutes R Al-Shahi, Web Editor, JNNP, Department of The overwhelming size of the internet appropriate censorship.3 The notorious Clinical Neurosciences, Western General makes it a formidable repository of excess of poor quality information— Hospital, Crewe Road, Edinburgh EH4 2XU, largely due to lack of regulation and com- UK; [email protected] knowledge. The figure below shows the C Kennard, Editor, JNNP, Division of Internet Software Consortium’s latest mercial exploitation—has become Neuroscience and Psychological Medicine, internet domain survey (www.isc.org), enough of a priority for the BMJ to devote Imperial College School of Medicine, Charing copyright. which estimates that there are >160 mil- its 9 March 2002 (Evaluating the quality of Cross Hospital, Fulham Place Road, London lion “hosts” and that their greatest prolif- health information on the internet) theme W6 8RF, UK; [email protected] eration has occurred in the last four years. issue to it. A recent survey is cause for It is, of course, impossible to know some optimism; almost half of the how many people use the internet, but “cyberchondriacs” in the USA and France REFERENCES an educated guess is that ∼580 million were found to visit medical journals and 1 Four-nation survey shows widespread but academic or research institutions, closely different levels of Internet use for health followed by commercial health web sites.1 purposes. Health Care News 2002;2:1–4. (www.harrisinteractive.com) Furthermore, the entire contents of two 2 Kennard C. Getting our Journal to online evidence based health resources developing countries. J Neurol Neurosurg (Clinical Evidence and the Cochrane Psychiatry 2001;71:711. 3 Gittings J. Google is back in China but don’t http://jnnp.bmj.com/ Library) were made available to the try asking any difficult questions. The public in September 2002 through the Guardian 14 September 2003: 3. National Electronic Library for Health 4 Kennard C. Same name, new face for JNNP: (www.nelh.nhs.uk). www.jnnp.com. J Neurol Neurosurg Psychiatry 1999;66:415. So how can JNNP help you cope with 5 Al-Shahi R, Sadler M, Rees G, et al.The this immortal yet imperfect (but improv- internet. J Neurol Neurosurg Psychiatry ing) medium? 2002;73:619–628. 6 Macleod M. Neuronline: PubMed:

Figure 1 Internet domain survey host count. Since 15 March 1999 the full text of http://www.pubmed.org. J Neurol Neurosurg on September 29, 2021 by guest. Protected Reproduced with permission. the Journal has been available at Psychiatry 2002;73:764.

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Huntington’s disease Huntington’s disease—expanded CAG ...... repeats within the huntingtin locus— resulted in an explosion of research on the pathogenesis of the disease. Recent Fetal striatal transplantation in advances include the development of several murine genetic models and the Huntington’s disease: time for a pause identification of various molecular proc- esses as potential therapeutic targets. I R L Albin believe the likelihood of an effective treatment emerging from these lines of ...... investigation is considerably higher than The need to look at the rationale for fetal striatal the likelihood of marked improvement with fetal striatal engraftment. transplantation Given the practical and theoretical objections to fetal striatal engraftment for n this issue of the Journal (pp 678– subjects is unacceptable. It is imperative Huntington’s disease, the best approach 685),1 the NEST-UK consortium re- that efficacy trials be controlled appropri- would be for the NEST-UK investigators Iports safety data on four subjects with ately. The recent experience with fetal tis- and other groups in this area to complete Huntington’s disease receiving unilateral sue engraftment for Parkinson disease their safety trials and then pause. revealed the existence of large magnitude intrastriatal transplantation of human J Neurol Neurosurg Psychiatry 2002;73:612 fetal striatal tissue. This well designed and and remarkably long duration placebo carefully executed study adds to the small effects. Some form of sham surgery amount of previous data on fetal striatal control is necessary. The NEST-UK proto- ...... 234 col involves general anaesthesia, the sur- grafting for Huntington’s disease. Authors’ affiliations gery, a week of postoperative hospital Groups in California and France reported R L Albin, Department of Neurology, University a total of eight subjects receiving bilateral care, and immunosuppression with fre- of Michigan, and Geriatrics Research, engraftment without complications and quent blood monitoring. Designing an Education, and Clinical Center, Ann Arbor adequate and safe sham surgery protocol VAMC, room 4412D, Kresge III Building, 200 either no change in clinical outcome will be a challenge. Zina Pitcher Place, Ann Arbor, MI 48109-0585, measures or some improvement.23 In The rationale for the fetal striatal USA another series of seven patients undergo- transplantation is questionable. Success- ing bilateral engraftment, there were four Correspondence to: Dr R Albin; ful striatal engraftment is likely to have [email protected] subdural haematomas (in three subjects), 4 limited effects. The hallmark of Hunting- two requiring neurosurgical drainage. ton’s disease is striatal degeneration, but copyright. These latter subjects may have had more the disorder affects the whole brain. REFERENCES advanced Huntington’s disease with a There is significant whole brain atrophy, 1 Rosser AE, Barker RA, Harrower T, et al. greater degree of brain atrophy, and this Unilateral transplantation of human primary and of the neocortex, fetal tissue in four patients with Huntington’s may be a contraindication to engraft- hippocampal formation, thalamus, sub- disease: NEST-UK safety report ISRCTN ment. The NEST-UK investigators plan a stantia nigra, and cerebellum have all no 36485475. J Neurol Neurosurg Psychiatry second phase safety study examining been documented. Fetal striatal trans- 2002;73:678–685. bilateral engraftment with a total of 10 2 Kopyov OV, Jacques S, Lieberman A, et al. plantation has shown efficacy in animal Safety of intrastriatal neurotransplantation for subjects (including the four in the present models of Huntington’s disease, but Huntington’s disease patients. Exp Neurol report), and a two year follow up to these models use mainly toxin destruc- 1998;149:97–108. generate preliminary efficacy data. This 3 Bachoud-Levi AC, Remy P, Nguyen JP, et al. tion of the striatum and do not mimic Motor and cognitive improvements in patients systematic approach is commendable. the systemic nature of Huntington’s dis- with Huntington’s disease after The accumulated data, however, raise ease. If there were no alternative ap- transplantation. Lancet 2000;356:1975–9. http://jnnp.bmj.com/ real questions about moving forward into proaches, continued pursuit of engraft- 4 Hauser RA, Furtado S, Cimino CR, et al. Bilateral human fetal striatal transplantation in efficacy trials. An incidence of two symp- ment would be rational. However, Huntington’s disease. Neurology tomatic subdural haematomas among 19 identification of the genetic defect in 2002;58:687–94.

Neuroimaging for MRI scanning has become much more ...... available over the last decade in many on September 29, 2021 by guest. Protected community hospitals, to the benefit of the populations they serve. There are Neuroimaging for epilepsy: quality some concerns, however, about quality control, and particularly because of the and not just quantity is important shortage of trained neuroradiologists. This important issue is addressed in the J S Duncan paper by Von Oertzen et al (this issue pp 643–647).2 ...... It was found that MRI scans carried Current resources for neuroimaging could be used more out and reported on in German commu- nity hospitals had a low rate of detection efficiently of focal lesions such as hippocampal sclerosis, tumours, cortical malforma- he value of magnetic resonance the case for those with medically refrac- tions, and vascular anomalies. There was imaging (MRI) of the brain in tory partial , for whom appropri- a modest increase in detection rate by people developing epilepsy has been ate surgical treatment may result in having these scans reviewed by expert T 1 amply demonstrated. This is particularly complete resolution of seizures in 70%. neuroradiologists at the Bonn Epilepsy

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Centre. There was a greater increase in and used for volumetric measurements: J Neurol Neurosurg Psychiatry detection by obtaining epilepsy dedi- proton density, T2 weighted, and fluid 2002;73:612–613 cated MRI in the centre, and focal lesions attenuated inversion recovery (FLAIR) ...... were identified in 85% of patients with acquisitions obtained with contiguous Author’s affiliations unremarkable standard MRI. slices that cover the entire brain with as J S Duncan, Department of Clinical and Does this mean that all patients with thin slices as possible and orientated per- Experimental Epilepsy, National Hospital for refractory epilepsy need to be referred to pendicular to the hippocampus. The ob- Neurology and Neurosurgery, Queen Square, London WC1N 3BG specialist centres for their MRI scans? At lique coronal orientation minimises par- present this would be an appropriate part tial volume effects that otherwise Correspondence to: Professor J S Duncan; of the evaluation for possible surgical commonly obscure hippocampal sclerosis [email protected] treatment of the epilepsy, but it is also and small lesions in the temporal lobe, REFERENCES important to improve the diagnostic accu- and also displays the rest of the brain in a 1 Duncan JS. Imaging and epilepsy. Brain racy of MRI scans obtained at smaller clear and logical fashion. Standardisation 1997;120:339–78. units. There is some impact from im- of acquisition protocols also facilitates 2 von Oertzen J, Urbach H, Jungbluth S, et al. Standard magnetic resonance imaging is proved scanning hardware, but the subsequent review of images at a special- inadequate for patients with refractory focal greater advance is possible from the use of ist centre and may avert the need for epilepsy. J Neurol Neurosurg Psychiatry MRI acquisition protocols for patients repeat scans. 2002;73:643–647. 3 Barkovich AJ, Berkovic SF, Cascino GD, et with epilepsy that may be used by radiog- There is a clear need for an increased al. Recommendations for neuroimaging of raphers without extensive specialist train- number of trained neuroradiologists to patients with epilepsy. Epilepsia 1997;38:1255–6. ing. Protocols have been formulated in direct image acquisition and to report on 4 Berkovic SF, Duncan JS, Barkovich A, et al. guidelines by the International League MRI scans. In the meantime, current ILAE neuroimaging commission against Epilepsy34 that have also consid- resources could be used more efficiently recommendations for neuroimaging of persons with refractory epilepsy. Epilepsia ered the complementary role of func- by the increased use of standardised 1998;39:1375–6. 5 tional imaging with MRI. A typical basic acquisition protocols and effective telere- 5 Duncan JS, Ali R, Barkovich J, et al. epilepsy MRI protocol would comprise a porting in the managed clinical net- Neuroimaging subcommission of ILAE. Recommendations for functional neuroimaging T1 weighted volume acquisition which works of community hospitals and re- of persons with epilepsy. Epilepsia may be reformatted in any orientation gional specialist centres. 2000;41:1350–6.

Creutzfeldt-Jakob disease clinical progression (mean duration: five ...... months). Severe neurological impair-

ment, even mutism, may be present at copyright. first neurological assessment.1 It is there- CJD: are there distinct fore particularly notable that the authors were able to assess their cases, using neuropsychological features? neuropsychological tools previously de- veloped for other . Two par- R Knight ticular findings, namely fluctuations in ...... ability over time and variability of responsiveness will resonate with ordi- Different faces wearing the same expression: is there a core nary clinical observations of cases of neuropsychological deficit in sporadic CJD? sCJD and accord well with comments made by many relatives. http://jnnp.bmj.com/ As the authors admit, these results are may simply reflect the usual function of s with many diseases originally derived from only a small number of the initially affected part of the CNS.1 defined on purely clinicopathologi- slightly atypical cases. One case was Whatever the explanation, the current Acal phenomenological grounds, relatively young, four cases had longer “Creutzfeldt-Jakob disease” (CJD) has view of sCJD is of an illness that may than average durations and at least two been subject to various “lumping” and present in a variety of ways, including were of unusual PRNP genotype. Further “splitting” processes over the years.1 The , cortical blindness, dys- studies are therefore needed to confirm demonstration of laboratory transmissi- phasia, and other deficits but which rap-

these preliminary findings. on September 29, 2021 by guest. Protected bility and the central role of the prion idly evolves into a global with Although sCJD shows us different protein have served to unify the concept features of widespread brain disease. of CJD. Sporadic CJD (sCJD) remains, of While attention has been given to the faces, perhaps there is a single facial course, defined by the absence of a different modes of “physical” neurologi- expression. Recognising this might be known cause, in contradistinction to cal presentation, there has been little helpful in diagnosis, although other genetic, iatrogenic, and variant CJD. comment on the pattern of cognitive diagnostic techniques (such as the estab- Despite this background unification, dysfunction. A paper by Snowden et al lished csf 14–3–3 test and the developing there is notable clinical phenotypic vari- (this issue pp 686–694) describes some role of cerebral magnetic resonance 4 ation in sCJD including age of onset, detailed observations on five cases of imaging) will be more useful. However, duration of illness, and presenting symp- sCJD (and one presumed familial case).3 understanding this may help us to man- tom(s) in particular. Research has at- The authors claim to have found a com- age patients and better explain unusual tempted to correlate these variations mon, distinct neuropsychological profile phenomena to distressed relatives. Un- with molecular factors such as the PRNP despite different neurological presenta- derstanding may also help us to explore genotype and the prion protein tions. further the mechanisms of this terrible structure.2 If sCJD is indeed a “spontane- There are obvious reasons for the pre- disease. Perhaps the most intriguing ous” disease due to a chance protein vious neglect of detailed cognitive as- suggestions made by the authors relate structural change or somatic PRNP mu- sessments, as the authors indicate, in- to the possible anatomic and pathologi- tation, then the different presentations cluding the rarity of sCJD and its rapid cal implications of their findings.

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J Neurol Neurosurg Psychiatry REFERENCES analysis of 300 subjects. Ann Neurol 46 2002;73:613–614 1 RG Will, MP Alpers, D Dormont, et al. 1999; :224–33. 3 Snowden JS, Mann DMA, Neary D. Distinct Infectious and sporadic prion diseases. In: neuropsychological characteristics in ...... Prusiner SB (ed). Prion biology and diseases. Creutzfeldt-Jakob disease. J Neurol Neurosurg New York: Cold Spring Harbour Laboratory Author’s affiliations Psychiatry 2002;73:686–694. Press, 1999:465–507. 4 DA Collie, RJ Sellar, M Zeidler, et al. MRI of R Knight, NCJDSU, Western General Hospital, 2 Parchi P, Giese A, Capellari S, et al. Creutzfeldt-Jakob: imaging features and Crewe Road, Edinburgh EH4 2XU, UK; Classification of sporadic Creutzfeldt-Jakob recommended MRI protocol. Clin Radiol [email protected] disease based on molecular and phenotypic 2001;56:726–39.

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