Search

Elevated tone (hypertonia) is a common problem seen in the paediatric clinic. For most children and young people, hypertonia is just one aspect of a broader disorder of movement and posture. This paper describes a clinical approach to the management of hypertonia in children, considering the contribution of high tone to the functional problems experienced by the child, the potential adverse effects of reducing tone, side effects of the intervention and the importance of setting objectives/goals for intervention which can be measured at follow-up. We describe this as the 'MOTOR' approach and provide some examples of how it can be used in practice. [ABSTRACT FROM AUTHOR]

Morning report is an important clinical learning activity in many neurological institutions. A long experience of these meetings allows identification of several components to enhance its success. Meetings are best if brief (one or two cases) and held regularly, preferably daily and early in the working day, with full in-person team engagement. A senior clinician should lead the meeting and commit to a single interpretation, without fear of being wrong. Although the environment is relaxed (refreshments typically provided), it is a working meeting and with the essential focus on the patient rather than the learners. The rich learning experience is greatly enhanced by a subsequent confidential email summary and interpretation of the case(s) sent to all participants. [ABSTRACT FROM AUTHOR]

The author discusses the modern perspective on a research paper titled "A prospective study of acute cerebrovascular disease in the community: the Oxfordshire Community Stroke Project, 1981—86". He comments on the paper with reference to a scale like the Glasgow Coma Scale or a technique which modified the aspects of clinical neurology like Computed Tomography brain scanning.

The paediatric epilepsies are a challenging group of disorders to assess and manage appropriately. This may be because of factors relating to the epilepsy itself, to the difficulties associated with obtaining an appropriate history and investigations or to factors relating to the individual child or young person such as other health issues. This paper discusses the particular challenges of assessing and managing epilepsies in children with intellectual disabilities (with or without other comorbidities) with a view to raising awareness of potential pitfalls in this field and ultimately improving the standards of epilepsy care that are offered to this complex group of children. [ABSTRACT FROM AUTHOR]

The article discusses the development of the "Journal of Neurology, Neurosurgery and Psychiatry" in the U.S. The authors announce that the staff is preparing the online publication of the journal. They also assure that majority of papers are publishable and interesting. However, they emphasize that they are being selective of papers that will be published.

The article reports on the changes in editorial board of the "Journal of Neurosurgery, Neurosurgery & Psychiatry." Ian Whittle, who have done all hard work and has been replaced by Peter Warnke from Liverpool as new Associate Editor for the journal. The vitality of the journal rests with its reviewers and authors. The journal has listed their reviewers in the December issue and reiterated their thanks to them. The journal wishes to attract papers of general interest to the multi-disciplinary readership of Neurologists, Psychiatrists, and Neuro-surgeons. Papers should be of direct clinical relevance.

This article presents corrections for various articles published in the "Journal of Neurology Neurosurgery & Psychiatry." In the letter by M. Deschauer the order of authorship is incorrect and should be: M. Deschauer, P.F. Chinnery, S. Shanske, S. DiMauro, K. Majamaa, E. Wilichowski, D.R. Thorburn, S. Zierz, A.M. Schaefer, D.M. Turnbull and R.W. Taylor. The second correction focuses on the number of nucleotides of the TREM2 mutation. In the paper D. Soragna, L. Papi and M.T. Rattihad had written that the mutation was at position 191 in exon 2 of the TREM2 gene. The correct mutation is at position 97 in exon 2 of the TREM2 gene.

Background: Cerebellar involvement in spatial data management has been suggested on experimental and clinical grounds.Objective: To attempt a specific analysis of visuospatial abilities in a group of subjects with focal or atrophic cerebellar damage.Methods: Visuospatial performance was tested using the spatial subtests of the WAIS, the Benton line orientation test, and two tests of mental rotation of objects-the Minnesota paper form board test (MIN) and the differential aptitude test (DAT).Results: In the Benton line orientation test, a test of sensory analysis and elementary perception, no deficits were present in subjects with cerebellar damage. In MIN, which analyses the capacity to process bidimensional complex figures mentally, and in the DAT, which is based on mental folding and manipulation of tridimensional stimuli, subjects with cerebellar damage were impaired.Conclusions: The results indicate that lesions of the cerebellar circuits affect visuospatial ability. The ability to rotate objects mentally is a possible functional substrate of the observed deficits. A comparison between visuospatial performance of subjects with focal right and left cerebellar lesions shows side differences in the characteristics of the visuospatial syndrome. Thus cerebellar influences on spatial cognition appear to act on multiple cognitive modules. [ABSTRACT FROM AUTHOR]

Objectives: To develop a Glucocorticoid Toxicity Index (GTI) to assess glucocorticoid (GC)-related morbidity and GC-sparing ability of other therapies.Methods: Nineteen experts on GC use and outcome measures from 11 subspecialties participated. Ten experts were from the USA; nine from Canada, Europe or Australia. Group consensus methods and multicriteria decision analysis (MCDA) were used. A Composite GTI and Specific List comprise the overall GTI. The Composite GTI reflects toxicity likely to change during a clinical trial. The Composite GTI toxicities occur commonly, vary with GC exposure, and are weighted and scored. Relative weights for items in the Composite GTI were derived by group consensus and MCDA. The Specific List is designed to capture GC toxicity not included in the Composite GTI. The Composite GTI was evaluated by application to paper cases by the investigators and an external group of 17 subspecialists.Results: Thirty-one toxicity items were included in the Composite GTI and 23 in the Specific List. Composite GTI evaluation showed high inter-rater agreement (investigators κ 0.88, external raters κ 0.90). To assess the degree to which the Composite GTI corresponds to expert clinical judgement, participants ranked 15 cases by clinical judgement in order of highest to lowest GC toxicity. Expert rankings were then compared with case ranking by the Composite GTI, yielding excellent agreement (investigators weighted κ 0.87, external raters weighted κ 0.77).Conclusions: We describe the development and initial evaluation of a comprehensive instrument for the assessment of GC toxicity. [ABSTRACT FROM AUTHOR]

There is usually great concern over the use of psychiatric patients for clinical research, as it raises the ethical and legal issues of human dignity and autonomy. In this paper the authors describe and evaluate a follow-up neurobiological study of patients who had been discharged from a psychiatric research ward at least ten months earlier. It is pointed out that such studies are rare and that the writers were provided with the unique opportunity to examine attitudinal and motivational dimensions involved in the patients' agreement to participate in the study. [ABSTRACT FROM PUBLISHER]

One of the most fascinating issues in the emerging field of neuroethics is pharmaceutical cognitive enhancement (CE). The three main ethical concerns around CE were identified in a Nature commentary in 2008 as safety, coercion and fairness; debate has largely focused on the potential to help those who are cognitively disabled, and on the issue of 'cosmetic neurology', where people enhance not because of a medical need, but because they want to (as many as 25% of US students already use nootropic cognitive enhancers such as ritalin). However, the potential for CE to improve public health has been neglected. This paper examines the prospect of improving health outcomes through CE among sections of the population where health inequalities are particularly pronounced. I term this enhancement of the public's health through CE 'neuroenhancing health'. It holds great promise, but raises several ethical issues. This paper provides an outline of these issues and related philosophical problems. These include the potential effectiveness of CE in reducing health inequalities; issues concerning autonomy and free will; whether moral enhancement might be more effective than CE in reducing health inequalities; and the problem of how to provide such CE, including the issue of whether to provide targeted or universal coverage., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

This paper explores the relationship between neurology and psychiatry. It marshals evidence that disorders of the brain typically have neurological and psychological--cognitive, affective, behavioural--manifestations, while disorders of the psyche are based in the brain. Given the inseparability of neurological and psychiatric disorders, their disease classifications should eventually fuse, and joint initiatives in training, service and research should be strongly encouraged. [ABSTRACT FROM AUTHOR]

Objectives To develop prognostic models for time to 12-month remission and time to treatment failure after initiating antiepileptic drug monotherapy for generalised and unclassified epilepsy. Methods We analysed data from the Standard and New Antiepileptic Drug (arm B) study, a randomised trial that compared initiating treatment with lamotrigine, topiramate and valproate in patients diagnosed with generalised or unclassified epilepsy. Multivariable regression modelling was used to investigate how clinical factors affect the probability of achieving 12-month remission and treatment failure. Results Significant factors in the multivariable model for time to 12-month remission were having a relative with epilepsy, neurological insult, total number of tonicclonic seizures before randomisation, seizure type and treatment. Significant factors in the multivariable model for time to treatment failure were treatment history (antiepileptic drug treatment prior to randomisation), EEG result, seizure type and treatment. Conclusions The models described within this paper can be used to identify patients most likely to achieve 12-month remission and most likely to have treatment failure, aiding individual patient risk stratification and the design and analysis of future epilepsy trials [ABSTRACT FROM AUTHOR]

Neurocognitive enhancement, or cosmetic neurology, offers the prospect of improving the learning, memory and attention skills of healthy individuals well beyond the normal human range. Much has been written about the ethics of such enhancement, but policy-makers in the USA, the UK and Europe have been reluctant to legislate in this rapidly developing field. However, the possibility of discrimination by employers and insurers against individuals who choose not to engage in such enhancement is a serious threat worthy of legislative intervention. While lawmakers should not prevent individuals from freely pursuing neurocognitive enhancement, they should act to ensure that such enhancement is not coerced. This paper offers one model for such legislation, based upon a proposed US law, the Genetic Information Nondiscrimination Act of 2008, to address precisely this particular pitfall of the impending neuroscience revolution.

The neurological exam can be a challenging part of a newborn's full evaluation. At the same time, the neonatal neurological exam is a useful tool in identifying babies needing closer evaluation for potential problems. The Dubowitz assessment is a standardised approach to the neonatal neurological exam designed for use by paediatricians in routine practice. Evidence has validated this technique and delineated its utility as a screening exam in various populations. This paper reviews clinical application of the Dubowitz assessment of the newborn. [ABSTRACT FROM AUTHOR]

The author describes how a paper, Fisher’s rules by Louis R. Caplan changed his perspective toward medical practice and which has since then, been his guiding principle.

The article comments on a study focusing on measurement, natural history and prognosis of functional abilities after stroke. The study illustrates a general principle of neurology and neurological rehabilitation. It states that the graph of the recovery after stroke can be seen to presage the realisation that stroke is associated with progressive disability.

Acute ischaemic stroke (AIS) is a leading cause of death and disability. Until the 1990s early intervention in AIS was limited to prevention of secondary brain injury. Early thrombolysis has now become standard practice in many areas of the world. Despite the evidence of benefit, the lack of good alternative acute treatments and the recent shift in political focus to stroke management, a thrombolytic strategy for AIS has been very slow to develop in the UK. In this paper, the successful introduction of thrombolysis for AIS through the development of an emergency department-focused process is reported. Obstacles to service development and ways to overcome these are discussed and this therapeutic process is demonstrated to be both possible and self-sustaining in most UK hospitals. [ABSTRACT FROM PUBLISHER]

The article presents abstracts on papers related to neurology including "The Continuing Role of Tensilon (Edrophonium) Tests in the Diagnosis of Childhood Myasthenia," by D. Pore and colleagues, "Leigh Syndrome: A Familiar Phenotype But a Disappearing Disease?," by C. Verity and colleagues, and "Transcranial Doppler and Stroke in South African Children With Human Immunodeficiency Virus," by N. Dlamini and colleagues.

Children and adolescents treated with oral, inhaled, and intravenous corticosteroids (CS) may experience adverse psychological side effects (APSE), including psychotic symptoms. These can occur at any point during treatment, including withdrawal. In this paper the literature on these effects in children and adults is reviewed. From the evidence available, it is not possible to give reliable estimates for incidence or prevalence of APSE, nor clear risk factors. Some evidence is reported to suggest that oral dexamethasone treatment may carry a higher risk of APSE than other CS, but this requires further investigation. There is evidence from the adult literature that higher CS doses increase the risk of APSE. However, the dose response effect is not straightforward or predictable for individuals or groups. This is likely to be a reflection of the complex effects of CS on the central nervous system and the probable interplay between individual susceptibility, disease factors, and external environmental stressors in the emergence of APSE. More research is required to further our understanding of the adverse effects of these clinically valuable agents. [ABSTRACT FROM AUTHOR]

The article provides information on principles of drawing a pedigree during the neurological consultation. The date when the history was obtained and the pedigree drawn must be documented, and the name of the informant and person drawing the pedigree. A blank sheet of paper should be used always. Remember to leave plenty of space between each individual because this allows further information to be added as and when it becomes available. Start with a single individual. Males are denoted by squares, females by circles and persons of unknown gender by diamond shaped symbols.

International comparisons suggest that British neurological services are underdeveloped. Historical factors which have contributed to the current state of neurological services in the United Kingdom are described. Key issues include the dominance of London and the concept of specialised hospitals in the early history of neurology; the subsequent recognition of the needs of other parts of the United Kingdom, of district general hospitals, and of patients with chronic neurological disabilities not necessarily included within the traditional bounds of neurology; and the relationship between neurology and general medicine. The paper concludes with some suggestions as to how neurology services might develop in the future.

The White Paper, "Working for Patients", led to a change in the way in which hospitals were funded from April 1991. The changes will have profound effects on the future shape of health care in the United Kingdom. Neurologists will need to understand the new National Health Service if their patients are to benefit from the changes. If neurology is to survive as a specialty separate from general medicine it will have to show that it can provide quality care which is accessible, relevant, efficient and effective, at a price which Districts can afford.

Health care planning relies on accurate data, yet there are few published data on neurological services in the United Kingdom. This paper describes the number and distribution of consultant neurologists in the UK and is based on a questionnaire completed by Regional Health Authorities and their equivalents, by Special Health Authorities, and by regional representatives of the Association of British Neurologists. The data were published by the ABN in 1988 but have not previously been widely available. The study identified 190 consultant neurologists (152 whole time equivalents). Overall there was one whole time neurologist for 373,000 persons but the ratio varied by a factor of four in different regions. There was a wide variation in the distribution of consultant neurological sessions between Health Districts. One group of neurologists was based in centres. A second group spent most of their time within the Health District, but had an attachment to a Regional Centre.

Innovations in interventional cardiology historically predate those in neuro-intervention. As such, studying trends in interventional cardiology can be useful in exploring avenues to optimise neuro-interventional techniques. One such cardiology innovation has been the steady conversion of arterial puncture sites from transfemoral access (TFA) to transradial access (TRA), a paradigm shift supported by safety benefits for patients. While neuro-intervention has unique anatomical challenges, the access itself is identical. As such, examining the extensive cardiology literature on the radial approach has the potential to offer valuable lessons for the neuro-interventionalist audience who may be unfamiliar with this body of work. Therefore, we present here a report, particularly for neuro-interventionalists, regarding the best practices for TRA by reviewing the relevant cardiology literature. We focused our review on the data most relevant to our audience, namely that surrounding the access itself. By reviewing the cardiology literature on metrics such as safety profiles, cost and patient satisfaction differences between TFA and TRA, as well as examining the technical nuances of the procedure and post-procedural care, we hope to give physicians treating complex cerebrovascular disease a broader data-driven understanding of TRA. [ABSTRACT FROM AUTHOR]

We have recently introduced a new item to our neurology Grand Rounds-the '1-3-5 presentation'. The format comprises a presentation on one topic, using three slides and lasting no more than 5 minutes. This a useful way of covering brief single topics and introducing and sparking discussion on more complex ones. '1-3-5s' have proven popular in our department and we have compiled a library of these presentations that is hosted on a YouTube channel. This article discusses the benefits and prospects for this format and encourages other units to provide similar opportunities for teaching and learning among all clinical grades. [ABSTRACT FROM AUTHOR]

The article offers information on the neurological facilities in Gloucester, England, which has a population of 550 000. As reported, there is a lot of neurological work to be done in the periphery of Gloucestershire Royal Hospital. The white paper presented by the new Government in Great Britain has provided another initiative and a chance to develop the facilities in Gloucester.

Objective To quantify the association between stressful life events and exacerbations of multiple sclerosis. Data sources PubMed,PsychInfo,and Psychological Abstracts searched for empirical papers from 1965 to February 2003 with terms "stress,"trauma,"and "multiple sclerosis." Review methods Three investigators independently reviewed papers for inclusion/exclusion criteria and extracted the relevant data,including methods, sample statistics,and outcomes. Results Of 20 studies identified,14 were included. The meta-analysis showed a significant increase in risk of exacerbation in multiple sclerosis after stressful life events,with a weighted average effect size of d =0.53 (95%confidence interval 0.40 to 0.65),P <0.0001. The studies were homogenous,Q=16.62,P =0.22, I2=21.8%.Neither sampling nor study methods had any effect on study outcomes. Conclusions There is a consistent association between stressful life events and subsequent exacerbation in multiple sclerosis.However these data do not allow the linking of specific stressors to exacerbations nor should they be used to infer that patients are responsible for their exacerbations. Investigation of the psychological,neuroendocrine, and immune mediators of stressful life events on exacerbation may lead to new behavioural and pharmacological strategies targeting potential links between stress and exacerbation. [ABSTRACT FROM AUTHOR]

Good teamwork underpins excellent clinical services; a formal (typically annual) teambuilding event can help to foster a team's sense of purpose and ensure solidity and collaboration between team members. We have held several Epilepsy Unit teambuilding events and use this experience to identify their essential components and suggestions for various workplace- based and leisure activities to include. Other neurology teams might consider similar events to help develop their teamworking. [ABSTRACT FROM AUTHOR]

Adeno-associated virus (AAV) gene therapies are generating much excitement in the rare disease field, particularly for previously untreatable neurological conditions. Efficacy has been claimed for several gene therapy products and the number of trials is rapidly increasing. However, reports of severe treatment-related adverse reactions are emerging, including death. There is still insufficient knowledge about their aetiology, prevention and treatment. We therefore undertook to systematically review publicly available data on AAV gene therapies in order to collate existing information on both safety and efficacy. Here, we review emerging efficacy reports of these novel therapies, many of which show promise. We also collate an increasing number of adverse reactions. Overwhelmingly, these results make a case for unified reporting of adverse events. This is likely to be critical for improving the safety of these promising treatments. [ABSTRACT FROM AUTHOR]

STUDY QUESTION: What is the risk of neuropsychiatric adverse events associated with varenicline compared with placebo in randomised controlled trials? SUMMARY ANSWER: There is no evidence of an increased risk of suicide or attempted suicide, suicidal ideation, depression, or death with varenicline compared with placebo. Varenicline was associated with a higher risk of sleep problems, such as insomnia and abnormal dreams. WHAT IS KNOWN AND WHAT THIS PAPER ADDS: Varenicline is a commonly prescribed and effective drug used in smoking cessation. Safety warnings have been issued regarding suicidal thoughts and depression associated with the use of varenicline, based on reports from spontaneous reporting systems. This new review found no evidence of an increased risk of suicide or attempted suicide, suicidal ideation, depression, or death in varenicline users compared with placebo users in randomised controlled trials. Varenicline was associated with an increased risk of sleep disorders, insomnia, and abnormal dreams; side effects that are already well recognised and included in patient information leaflets for varenicline. [ABSTRACT FROM AUTHOR]

A review of several articles is presented which include one about exome sequencing published in a 2014 issue of "Nature Genetics," a study about the relationship between cognitive ability and cortical surface published in "NeuroImage" and about functional imaging as evidence in legal cases.

The investigation of children presenting with infantile and childhood epileptic encephalopathies (ICEE) is challenging due to diverse aetiologies, overlapping phenotypes and the relatively low diagnostic yield of MRI, electroencephalography (EEG) and biochemical investigations. Careful history and thorough examination remain essential as these may identify an acquired cause or indicate more targeted investigation for a genetic disorder. Whole exome sequencing (WES) with analysis of a panel of candidate epilepsy genes has increased the diagnostic yield. Whole genome sequencing (WGS), particularly as a trio with both parents' DNA, is likely to supersede WES. Modern genomic investigation impacts on the timing and necessity of other testing. We propose a structured approach for children presenting with ICEE where there is diagnostic uncertainty, emphasising the importance of WGS or, if unavailable, WES early in the investigative process. We note the importance of expert review of all investigations, including radiology, neurophysiology and biochemistry, to confirm the technique used was appropriate as well as the results. It is essential to counsel families on the risks associated with the procedures, the yield of the procedures, findings that are difficult to interpret and implication of 'negative' results. Where children remain without a diagnosis despite comprehensive investigation, we note the importance of ongoing multidisciplinary care. [ABSTRACT FROM AUTHOR]

The article presents an essay related to introduction of Neurology as a science by Thomas Laycock, neurologist, at the York Medical School in Yorkshire, England. It discusses the life history of Laycock. It discusses the contribution of James CRICHTON-Browne in establishing a pathology and providing supply of primates, pigeons, guinea pigs and cats to test experimentally. It also discusses the planning and management of Samuel Tuke in introducing West Riding Pauper Lunatic Asylum (WRPLA).

The article offers information on various issues related to practical neurology. It mentions the use of ear biospy and magnetic resonance imaging (MRI) in investigating ataxia. It cites how BBC Radio 4 broadcasts series of programmes regarding the brain in November 2011. Moreover, it features a study on the role of Facebook on having traditional friendship.

BACKGROUND: Data are limited on the proportion of stroke patients nationally appropriate for thrombolysis either within the 3 h time window or the recently tested 4.5 h. This information is important for the redesign of services. METHODS: Data on case mix, eligibility for thrombolysis, treatment and outcomes were extracted from the National Sentinel Stroke 2008 Audit dataset. This contains retrospective data on up to 60 consecutive stroke admissions from each acute hospital in England, Wales and Northern Ireland between 1 April and 30 June 2008. FINDINGS: All relevant hospitals participated, submitting data on 11 262 acute stroke patients. 2118 patients arrived within 2 h and 2596 within 3 h of the onset of symptoms and 587 people were already in hospital. Therefore, 28% (3183) were potentially eligible for thrombolysis based on a 3 h time criterion. Of these, 1914 were under 80 years and 2632 had infarction with 14% (1605) meeting all three National Institute of Neurological Disorders and Stroke study criteria and so being potentially eligible for thrombolysis. If the time window is increased to 4.5 h then only another 2% became eligible. If the age limit was removed for treatment, the percentage potentially appropriate for tissue plasminogen activator increased to 23% within 3 h and 26% within 4.5 h. Overall, 1.4% (160) of patients were thrombolysed. INTERPRETATION: Thrombolysis rates are currently low in the UK. 14% of patients in this sample were potentially suitable for thrombolysis using the 3 h time window. This would only increase marginally if thrombolysis was extended to include those up to 4.5 h. The greatest impact on increasing the proportion of patients suitable for thrombolysis would be to increase the number of patients presenting early and by demonstrating that the treatment is safe and effective in patients over 80 years of age. [ABSTRACT FROM AUTHOR]