Neurological conditions: involvement to improve service delivery

A major, ongoing Public Health Agency led consultation exercise has identified 12 recommendations to improve the lives of the 48,000 people, and their carers, who experience neurological conditions across Northern Ireland. These recommendations will form the basis of an action plan to improve service delivery and support for those experiencing a range of conditions, such as epilepsy, Huntington's Disease, Parkinson's Disease, progressive supranuclear palsy (PSP) and multiple sclerosis (MS).The recommendations cover four areas:accurate information and diagnosis;control and choice, particularly self-management and person-centred services;day-to-day living and independence, including finance, employment, social life and ability to get out and about;emotional and psychological impact on individuals and families, eg the support available to deal with stress, fear, frustration, isolation, loss and vulnerability associated with living with a neurological condition.The report was launched at a regional workshop, held in Cookstown (today) and co-ordinated through the Neurological Conditions Network, which was established to develop this work.Speaking before the workshop, Health Minister Edwin Poots said: "Neurological conditions give rise to complex needs, which require support from a wide range of professionals. They also change lives, both for those directly affected and for their families and carers, and it is so important not to lose sight of this if we are to successfully address the challenges in tackling neurological conditions."Last week, I visited the home of Beth McCune, who suffers from motor neurone disease. I was invited to see for myself the daily challenges faced by Beth and her husband and carer, Arthur, and to hear of their experiences. While I was struck by their courage and patience, this visit underlined again for me the severe life-changing impact of the disease."At present, there are some 48,000 people in Northern Ireland living with neurological conditions. It was in recognition of the needs of men and women like Beth that my department requested the establishment of the Neurological Conditions Network and provided the necessary funding to support it."Michelle Tennyson, PHA Assistant Director and Chair of the Neurological Conditions Network, said: "This detailed engagement exercise was undertaken to get the views and quality of life experiences of those affected by these conditions. We tried to ensure everyone who wanted to contribute could, by providing support through helplines, the internet and face-to-face events. I am honoured that so many people have trusted us with their experiences to help us make a difference and was privileged to be invited into the home of Beth and Arthur McCune for the same reason."The recommendations cover a range of conditions and their implementation will need cooperation and action from professionals, service users, voluntary organizations and others, across many sectors and agencies. The network is looking forward to delivering on these challenging new ways of working to improve the lives of all those affected by neurological conditions."The workshop attracted service users and carers along with delegates from across Northern Ireland's community, voluntary and statutory sectors.If you have a neurological condition, or care for someone who does, and want to share your experiences, please go to: www.publichealth.hscni.net/ncnsurveyYou can also contact Julie Mawhinney, Tel: 028 9032 1313.

Neurological Conditions Network - Engagement exercise summary report

This is the summary report of the Speak out for Change engagement exercise carried out by the Neurological Conditions Network. This engagement undertook to ask people's experiences of living with a neurological condition and caring for someone with a neurological condition. Across the wide range of conditions represented,� the 142 patient experiences gathered to date, have consistently revealed issues around information, choice and control, independence and the emotional impact of neurological conditions.

Autoanticorps en neurologie: implications cliniques [Autoantibodies in neurological diseases: clinical implications]

Autoantibodies are defined as antibodies directed against self antigens, i.e., against a normal antigenic endogenous tissue constituent. They can be the immediate cause of the neurological syndrome or be detected as an epiphenomenon of the pathogenic process. Autoantibodies are often considered useful biomarkers for the improvement of diagnostic accuracy, for the staging of disease progression or for the follow up of a biological response to a therapeutic intervention. The purpose of this article is to review the autoantibodies that are available to investigate immune-mediated neurological conditions. The detection of some of these autoantibodies may help the clinician to establish a definite diagnosis which may further facilitate the therapeutic decision.

A cohort study of early neurological consultation by telemedicine on the care of neurological inpatients

Objectives: To find out the effect of early neurological consultation using a real time video link on the care of patients with neurological symptoms admitted to hospitals without neurologists on site. Methods: A cohort study was performed in two small rural hospitals: Tyrone County Hospital (TCH), Omagh, and Erne Hospital, Enniskillen. All patients over 12 years of age who had been admitted because of neurological symptoms, over a 24 week period, to either hospital were studied. Patients admitted to TCH, in addition to receiving usual care, were offered a neurological consultation with a neurologist 120 km away at the Neurology Department of the Royal Victoria Hospital, Belfast, using a real time video link. The main outcome measure was length of hospital stay; change of diagnosis, mortality at 3 months, inpatient investigation, and transfer rate and use of healthcare resources within 3 months of admission were also studied. Results: Hospital stay was significantly shorter for those admitted to TCH (hazard ratio 1.13; approximate 95% Cl 1.003 to 1.282; p = 0.045). No patients diagnosed by the neurologist using the video link subsequently had their diagnosis changed at follow up. There was no difference in overall mortality between the groups. There were no differences in the use of inpatient hospital resources and medical services in the follow up period between TCH and Erne patients. Conclusions: Early neurological assessment reduces hospital stay for patients with neurological conditions outside of neurological centres. This can be achieved safely at a distance using a real time video link.

Mild cognitive deficits associated to neocortical microgyria in mice with genetic deletion of cellular prion protein

The cellular prion protein (PrP(c)) has been implicated with the modulation of neuronal apoptosis, adhesion, neurite outgrowth and maintenance which are processes involved in the neocortical development. Malformations of cortical development (MCD) are frequently associated with neurological conditions including mental retardation, autism, and epilepsy. Here we investigated the behavioral performance of female adult PrP(c)-null mice (Prnp(%)) and their wild-type controls (Prnp(+/+)) presenting unilateral polymicrogyria, a MCD experimentally induced by neonatal freeze-lesion in the right hemisphere. injured mice from both genotypes presented similar locomotor activity but Prnp(%) mice showed a tendency to increase anxiety-related responses when compared to Prnp(+/+) animals. Additionally, injured Prnp(%) mice have a poorer performance in the social recognition task than sham-operated and Prnp(%) injured ones. Moreover the step-down inhibitory avoidance task was not affected by the procedure or the genotype of the animals. These data suggest that the genetic deletion of PrP(c) confers increased susceptibility to short-term social memory deficits induced by neonatal freezing model of polymicrogyria in mice. (C) 2008 Published by Elsevier B.V.

Qualitative analysis of hippocampal plastic changes in rats with epilepsy supplemented with oral omega-3 fatty acids

Studies have provided evidence of the important effects of omega-3 fatty acid on the brain in neurological conditions, including epilepsy. Previous data have indicated that omega-3 fatty acids lead to prevention of status epilepticus-associated neuropathological changes in the hippocampal formation of rats with epilepsy. Omega-3 fatty acid supplementation has resulted in extensive preservation of GABAergic cells in animals with epilepsy. This study investigated the interplay of these effects with neurogenesis and brain-derived neurotrophic factor (BDNF). The results clearly showed a positive effect of long-term omega-3 fatty acid supplementation on brain plasticity in animals with epilepsy. Enhanced hippocampal neurogenesis and BDNF levels and preservation of interneurons expressing parvalbumin were observed. Parvalbumin-positive cells were identified as surviving instead of newly formed cells. Additional investigations are needed to determine the electrophysiological properties of the newly formed cells and to clarify whether the effects of omega-3 fatty acids on brain plasticity are accompanied by functional gain in animals with epilepsy. (C) 2009 Elsevier Inc. All rights reserved.

Thermal skin reference values in healthy late pregnancy

Digital thermal imaging has been employed in medicine for over 50 years. However, its use has been focused on vascular, musculoskeletal and neurological conditions, while other potential applications,such as obstetrics, have been much less explored. This paper presents a study conducted during 2011 at the Hospital of Braga on a group of healthy pregnant women in the last third of gestation. The analysis focused on characterizing typical pregnant women steady temperature profiles in specific defined regions of interest (ROI), and determining if the thermal symmetry values for late pregnant healthy women are in line with the values for non-pregnant healthy women. A temperature distribution pattern was found in the defined ROI. The obtained thermal symmetry value had a maximum of 0.370.2 1C, and there was no evidence for the influence of age (p40.05) in the observed group. The influence of the BMI requires further investigation since one ROI (P2 right) presented a p¼0.059, close to the threshold of statistical evidence in the influence of BMI. The study group presented symmetry values in line with non-pregnant reference values, but the profiles in temperature distribution are different. Assumptions can therefore now be used with higher confidence when assessing abnormalities in specific pathologic states during pregnancy using the defined ROI. This work represents a first contribution towards establishing guidelines for future research in this specific field of study.

Acetyl-L-Carnitine Prevents Methamphetamine-Induced Structural Damage on Endothelial Cells via ILK-Related MMP-9 Activity

Methamphetamine (METH) is a potent psychostimulant highly used worldwide. Recent studies evidenced the involvement of METH in the breakdown of the blood-brain-barrier (BBB) integrity leading to compromised function. The involvement of the matrix metalloproteinases (MMPs) in the degradation of the neurovascular matrix components and tight junctions (TJs) is one of the most recent findings in METH-induced toxicity. As BBB dysfunction is a pathological feature of many neurological conditions, unveiling new protective agents in this field is of major relevance. AcetylL-carnitine (ALC) has been described to protect the BBB function in different paradigms, but the mechanisms underling its action remain mostly unknown. Here, the immortalized bEnd.3 cell line was used to evaluate the neuroprotective features of ALC in METH-induced damage. Cells were exposed to ranging concentrations of METH, and the protective effect of ALC 1 mM was assessed 24 h after treatment. F-actin rearrangement, TJ expression and distribution, and MMPs activity were evaluated. Integrin-linked kinase (ILK) knockdown cells were used to assess role of ALC in ILK mediated METHtriggered MMPs’ activity. Our results show that METH led to disruption of the actin filaments concomitant with claudin-5 translocation to the cytoplasm. These events were mediated by MMP-9 activation in association with ILK overexpression. Pretreatment with ALC prevented METH-induced activation of MMP-9, preserving claudin-5 location and the structural arrangement of the actin filaments. The present results support the potential of ALC in preserving BBB integrity, highlighting ILK as a new target for the ALC therapeutic use.

Comparison between the spastic paraplegia rating scale, Kurtzke scale, and Osame scale in the tropical spastic paraparesis/myelopathy associated with HTLV

INTRODUCTION:The objective of this study was to compare Osame's scale of motor incapacity and the expanded scale of the state of incapacity of Kurtzke with the spastic paraplegia rating scale for the clinical evaluation of patients with HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP). METHODS: Patients with the diagnosis of infection by HTLV-I/HTLV-II and with the clinical suspicion of HAM/TSP were included in the study. RESULTS: There were 45 patients who were evaluated. When analyzing the results of the scales, the researchers found the following averages of 21.08 points for the spastic paraplegia rating scale, 4.35 points for Osame's scale, and 4.77 points for Kurtzke's scale. The relation between the scale of paraplegia with Osame's was very significant with p < 0.0001, and regarding Kurtzke's scale, there was a similar result of p < 0.0001. When comparing Osame's, Kurtze's, and the spastic paraplegia rating scale with the time of disease, the researchers found a significant result of p = 0.0004 for the scale of spastic paraplegia, p = 0.0018 for Osame's scale, and p < 0.0001 for Kurtzke's scale. CONCLUSION: The spastic paraplegia rating scale has a good relation with Osame's and Kurtzke's scales showing a p index that is very significant that indicates that, although the scale was not initially made to be applied to patients with HAM/TSP because of the infection by HLTV, it showed to be as efficient as Osame's and Kurtzke's scales in evaluating the patients' neurological conditions.

Role of the JNK pathway in NMDA-mediated excitotoxicity of cortical neurons.

Excitotoxic insults induce c-Jun N-terminal kinase (JNK) activation, which leads to neuronal death and contributes to many neurological conditions such as cerebral ischemia and neurodegenerative disorders. The action of JNK can be inhibited by the D-retro-inverso form of JNK inhibitor peptide (D-JNKI1), which totally prevents death induced by N-methyl-D-aspartate (NMDA) in vitro and strongly protects against different in vivo paradigms of excitotoxicity. To obtain optimal neuroprotection, it is imperative to elucidate the prosurvival action of D-JNKI1 and the death pathways that it inhibits. In cortical neuronal cultures, we first investigate the pathways by which NMDA induces JNK activation and show a rapid and selective phosphorylation of mitogen-activated protein kinase kinase 7 (MKK7), whereas the only other known JNK activator, mitogen-activated protein kinase kinase 4 (MKK4), was unaffected. We then analyze the action of D-JNKI1 on four JNK targets containing a JNK-binding domain: MAPK-activating death domain-containing protein/differentially expressed in normal and neoplastic cells (MADD/DENN), MKK7, MKK4 and JNK-interacting protein-1 (IB1/JIP-1).

PHA urges parents to get 'at risk' children vaccinated against seasonal flu

The Public Health Agency is urging Northern Ireland parents to make sure children in 'at risk' groups get their flu vaccine early.The message has been issued to parents and carers of children as the PHA's seasonal flu vaccination programme gets underway for 2011/12.It is very important that children with any condition that puts them more at risk of the complications of flu get the vaccine.These 'at risk' conditions include:chronic lung conditions such as asthma;chest infections that have required hospital admission;chronic heart conditions;chronic liver disease;chronic kidney disease;diabetes;lowered immunity due to disease or treatment such as steroids or cancer therapy;chronic neurological conditions such as stroke, multiple sclerosis or a condition that affects the nervous system, such as cerebral palsy;hereditary and degenerative diseases of the central nervous system or muscles.Children who attend special schools for severe learning or physical disabilities are considered to be particularly at risk, as well as those with other complex health needs.The PHA has written to principals of local special schools, as well as parents of children at these schools, to raise awareness of the importance of getting vaccinated early.Dr Richard Smithson, PHA Flu Vaccination Lead, said: "For many people, flu is a short, unpleasant illness, but it does not usually cause any serious problems. However, for others, it can have very serious complications including, in rare cases, being fatal."We have been particularly reminded over the last two winters that children with chronic neurological problems and other complex health needs are very vulnerable to these complications. We have seen children become very seriously ill and, tragically, there have even been a few deaths in children who attend special schools."For this reason, we recommend that all children who attend special schools for severe learning disability, and special schools for physical disability, are offered the flu vaccine early in the autumn, before the flu viruses start circulating."The vaccine is now available from GP surgeries and the PHA recommends that parents check arrangements with their own GP's surgery so that their child can get the jab.The earlier you get vaccinated the better, as it takes the body about 10-14 days after the jab to develop antibodies. These will then protect you against the same or similar viruses if the body is exposed to them. The vaccine contains three strains of the flu virus, which are considered the most likely to be circulating this winter, including the H1N1 (swine flu) virus."Your child needs to get the flu jab every year - the protection it gives only lasts for one winter, so even if they got it last year, they still need to get it this year," added Dr Smithson."Also, if your child has been diagnosed with flu or swine flu in the past couple of years, they will still need the jab this year as there are different types of flu that the jab will protect against. Getting the flu jab is the best way to protect your child against flu and we would strongly recommend that you arrange for them to have it."Although the vaccine gives good protection, no vaccine gives total protection, so if your child develops flu-like symptoms (such as fever, cough, aches and pains, and sore throat) you should contact your GP for advice. If your child has any of these symptoms, they should be kept at home until they feel better."For more information on seasonal flu, go to www.fluawareni.info and follow us on Facebook and Twitter.

Dépression résistant au traitement: enjeux, thérapie et place des nouvelles approches de neurostimulation [Treatment resistant depression: therapy and novel neuromodulatory treatments].

Unipolar depression is among the leading cause of invalidity and disability-adjusted life-years. Many depressed patients do not respond to several antidepressant treatments. Several treatments have been investigated in resistant depression using electrical or magnetic stimulation of the brain. In this field, electroconvulsivotherapy remains to date the only treatment validated for efficacy and security. Novel neuromodulatory treatments used in neurological conditions are currently under investigation. Vagus nerve stimulation and deep brain stimulation may offer long-term efficacy and therefore justify expensive and highly specialized treatment programs.

Beclin 1-independent autophagy contributes to apoptosis in cortical neurons.

Neuronal autophagy is enhanced in many neurological conditions, such as cerebral ischemia and traumatic brain injury, but its role in associated neuronal death is controversial, especially under conditions of apoptosis. We therefore investigated the role of autophagy in the apoptosis of primary cortical neurons treated with the widely used and potent pro-apoptotic agent, staurosporine (STS). Even before apoptosis, STS enhanced autophagic flux, as shown by increases in autophagosomal (LC3-II level, LC3 punctate labeling) and lysosomal (cathepsin D, LAMP1, acid phosphatase, β-hexasominidase) markers. Inhibition of autophagy by 3-methyladenine, or by lentivirally-delivered shRNAs against Atg5 and Atg7, strongly reduced the STS-induced activation of caspase-3 and nuclear translocation of AIF, and gave partial protection against neuronal death. Pan-caspase inhibition with Q-VD-OPH likewise protected partially against neuronal death, but failed to affect autophagy. Combined inhibition of both autophagy and caspases gave strong synergistic neuroprotection. The autophagy contributing to apoptosis was Beclin 1-independent, as shown by the fact that Beclin 1 knockdown failed to reduce it but efficiently reduced rapamycin-induced autophagy. Moreover the Beclin 1 knockdown sensitized neurons to STS-induced apoptosis, indicating a cytoprotective role of Beclin 1 in cortical neurons. Caspase-3 activation and pyknosis induced by two other pro-apoptotic stimuli, MK801 and etoposide, were likewise found to be associated with Beclin 1-independent autophagy and reduced by the knockdown of Atg7 but not Beclin 1. In conclusion, Beclin 1-independent autophagy is an important contributor to both the caspase-dependent and -independent components of neuronal apoptosis and may be considered as an important therapeutic target in neural conditions involving apoptosis.

Nutrición enteral en el paciente neurológico: ¿es suficiente el contenido en vitamina D en las fórmulas de uso habitual?

INTRODUCTION Vitamin D deficiency produces inadequate bone mineralization, proximal muscle weakness, abnormal gait and increased risk of falls and fractures. Moreover, in epidemiological studies, has been associated with increased risk of cancer, autoimmune diseases, type 1 and 2 diabetes, rheumatoid arthritis, multiple sclerosis, infectious diseases, cardiovascular diseases and depression. When synthesis through the skin by sun exposure is not possible and the patient can not eat by mouth, as in the advanced stages of various neurological diseases, the supply of vitamin D has to be done by enteral nutrition. OBJECTIVES The aim of this study is to review the role of vitamin D in a common group of neurological conditions that often require artificial nutrition and analyze whether the vitamin D of different enteral nutrition formulas is adequate to meet the needs of this group of patients. RESULTS Numerous studies have shown the association between vitamin D deficiency and increased incidence of dementia, stroke and other neurodegenerative diseases. Interventions aimed to increase levels of vit. D and its effects on functional (falls, pain, quality of life) and cardiovascular goals (cardiovascular death, stroke, myocardial infarction, cardiovascular risk factors) have obtained as highlight data a clear reduction of falls and fractures, while the evidence for the other parameters studied is still limited and inconsistent. The content of calcium and vitamin D of enteral formulas is legislated in our country. The total amount of vitamin D for a daily intake of 1,500-2,000 kcal ranges between 300 and 1,600 IU/d (mean ± SD: 32.9 ± 8.5 mg/100 kcal) in the complete formulas for enteral nutrition most commonly used. 50% of the diets studied, for an intake of 2,000 kcal/d, and 90% for an intake of 1,500 kcal/d, provide less than 600 IU/d of vitamin D. DISCUSSION Some revised recently guidelines published recommendations of daily intake of vitamin D. The document published by the U.S. Institute of Medicine recommended for adults between 19 and 70 years, 600 IU/d and up from 70, proposes 800 IU/d of vitamin D. These amounts are deemed insufficient by other scientific societies to state that to achieve blood levels of 25 (OH) D equal or greater than 30 ng/ml may be required a daily intake of 1,500-2,000 IU and a number two or three times higher if previous deficiency exists. CONCLUSIONS Further controlled studies are needed to ascertain which is the appropriate dose of vitamin D in advanced stages of neurological disease, where sun exposure is difficult and unlikely. We suggest that the vitamin D content should probably be reconsidered in enteral nutrition formulas, which, in light of recent publications appear as clearly insufficient for standard energy intakes (1,500-2,000 kcal).

Consensus paper: the role of the cerebellum in perceptual processes.

Various lines of evidence accumulated over the past 30 years indicate that the cerebellum, long recognized as essential for motor control, also has considerable influence on perceptual processes. In this paper, we bring together experts from psychology and neuroscience, with the aim of providing a succinct but comprehensive overview of key findings related to the involvement of the cerebellum in sensory perception. The contributions cover such topics as anatomical and functional connectivity, evolutionary and comparative perspectives, visual and auditory processing, biological motion perception, nociception, self-motion, timing, predictive processing, and perceptual sequencing. While no single explanation has yet emerged concerning the role of the cerebellum in perceptual processes, this consensus paper summarizes the impressive empirical evidence on this problem and highlights diversities as well as commonalities between existing hypotheses. In addition to work with healthy individuals and patients with cerebellar disorders, it is also apparent that several neurological conditions in which perceptual disturbances occur, including autism and schizophrenia, are associated with cerebellar pathology. A better understanding of the involvement of the cerebellum in perceptual processes will thus likely be important for identifying and treating perceptual deficits that may at present go unnoticed and untreated. This paper provides a useful framework for further debate and empirical investigations into the influence of the cerebellum on sensory perception.