Neurological Diseases (neurological + disease)

Distribution by Scientific Domains
Distribution within Medical Sciences

Kinds of Neurological Diseases

  • other neurological disease


  • Selected Abstracts


    Detection and Management of an Outbreak of Equine Herpesvirus Type 1 Infection and Associated Neurological Disease in a Veterinary Teaching Hospital

    JOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 5 2010
    L.S. Goehring
    Background: Because of the serious disease sequelae associated with equine herpesvirus type 1 (EHV-1) infections, awareness and control measures used to control outbreaks are important issues for all horse populations. Objectives: Describe the occurrence and management of an outbreak of EHV-1 infection at a veterinary hospital. Animals: Horses hospitalized at a referral veterinary hospital. Methods: A horse with myeloencephalopathy associated with EHV-1 infection (EHM) was admitted for diagnostic evaluation and treatment under strict infection control procedures. We describe the occurrence and management of a nosocomial outbreak of EHV-1 infections associated with admission of this patient. Results: Despite institution of rigorous biosecurity precautions at the time of admission of the index case, EHV-1 infections spread to 6 other horses that were hospitalized at the James L. Voss Veterinary Teaching Hopsital, including 2 that served as sources of infection for horses on their home premises after discharge. Infection with EHV-1 was confirmed by polymerase chain reaction (PCR) and by seroconversion documented by glycoprotein G ELISA. A voluntary quarantine was imposed and admissions were restricted to prevent additional horses from being exposed. Quarantine duration was abbreviated by serial testing of all horses with PCR. Conclusions and Clinical Importance: These findings illustrate the contagious disease risk that can accompany management of horses with EHM. Horses with active nasal EHV-1 shedding should be isolated in an airspace that is separate from other horses by strictly enforced biosecurity and isolation procedures. Serial testing with PCR may be a useful adjunct to determine when the risk of transmission has been minimized. [source]


    Epilepsy in Colombia: Epidemiologic Profile and Classification of Epileptic Seizures and Syndromes

    EPILEPSIA, Issue 1 2006
    Alberto Velez
    Summary:,Purpose: A national study was performed in Colombia to determine the general and regional prevalence of epilepsy, clinical profiles, seizure types, and clinical syndromes. Methods: Based on the National Epidemiological Study of Neurological Diseases (EPINEURO), we evaluated and followed up for 1 year all the subjects with epilepsy from the National Sample. Clinical profiles were further assessed. Seizure types and epilepsy syndromes were established according to the international classifications. Results: General prevalence was found to be 11.3 per 1,000, with little variation among regions, except the eastern region, where prevalence was 23 per 1,000; prevalence for active epilepsy was 10.1 per 1,000. Women have a slightly greater (not statistically significant) risk. Most seizures are focal (partial), frequently with secondary generalization. The most frequent epilepsy syndrome encountered was partial symptomatic/cryptogenic (80%). Epilepsy onset in Colombia occurs most frequently in childhood. Conclusions: Prevalence rates of epilepsy in Colombia are similar to those reported in nations with comparable developmental status and have diminished over time. The study presents the distribution of seizures and syndromes. The most frequent types are focal syndromes. [source]


    Quantification by real-time PCR of the magnitude and duration of leucocyte-associated viraemia in horses infected with neuropathogenic vs. non-neuropathogenic strains of EHV- 1

    EQUINE VETERINARY JOURNAL, Issue 3 2006
    G. P. Allen
    Summary Reasons for performing study: Neurological disease in horses caused by infection with certain ,paralytic' strains of equine herpesvirus-1 (EHV-1) is a potentially devastating condition the pathogenesis of which is poorly understood. Preliminary observations in both experimentally induced and naturally occurring cases of the central nervous system disease have revealed a more robust cell-associated viraemia in horses infected with paralytic isolates of EHV-1, relative to horses infected with abortigenic isolates. To investigate further this pathogenesis - rdevant question, the present study was performed using a greater number of horses and a more precise method for quantification of EHV-1 DNA present in viraemic leucocytes. Objective: To compare the magnitude and duration of leucocyte-associated viraemia in seronegative, age-matched foals following infection with paralytic vs. abortigenic isolates of EHV-1. Methods: Peripheral blood mononuclear cells (PBMC) were collected from 20 weanling foals at 2, 4, 7, 9, 11, 14 and 21 days after intranasal inoculation with either paralytic or abortigenic isolates of EHV-1. The amount of EHV-1 DNA present in each PBMC sample was measured by real-time quantitative PCR. Results: Foals inoculated with paralytic strains of EHV-1 developed both a greater magnitude and longer duration of PBMC-associated viraemia than foals inoculated with abortigenic strains of the virus. Conclusions: Both the higher magnitude and longer duration of cell-associated viraemia contribute to the risk for development of neurological signs in horses infected with paralytic strains of EHV-1. Potential relevance: Our results provide empirically derived, scientific data that contributes to a better understanding of the pathogenetic basis for the differing abilities of paralytic and abortigenic strains of EHV-1 to cause post infection central nervous system disease in the horse. The findings identify the importance of minimising the quantitative burden of viraemic leucocytes that follows exposure to the virus, by the use of effective therapeutic antiviral drugs and efficacious prophylactic vaccines that stimulate cytotoxic immune responses against EHV-1 infected cells. [source]


    Neurological disease on the global agenda

    ANNALS OF NEUROLOGY, Issue 6 2008
    Farrah Mateen MD
    No abstract is available for this article. [source]


    Cerebrospinal fluid of brain trauma patients inhibits in vitro neuronal network function via NMDA receptors,

    ANNALS OF NEUROLOGY, Issue 4 2009
    Frauke Otto MD
    Neurological diseases frequently induce pathological changes of cerebrospinal fluid (CSF) that might secondarily influence brain activity, as the CSF,brain barrier is partially permeable. However, functional effects of CSF on neuronal network activity have not been specified to date. Here, we report that CSF specimens from patients with reduced Glasgow Coma Scale values caused by severe traumatic brain injury suppress synchronous activity of in vitro-generated neuronal networks in comparison with controls. We present evidence that underlying mechanisms include increased N -methyl- D- aspartate receptor activity mediated by a CSF fraction containing elevated amino acid concentrations. These proof-of-principle data suggest that determining effects of CSF specimens on neuronal network activity might be of diagnostic value. Ann Neurol 2009;66:546,555 [source]


    THE INTERNATIONAL DIMENSIONS OF NEUROETHICS

    DEVELOPING WORLD BIOETHICS, Issue 2 2009
    SOFIA LOMBERA
    ABSTRACT Neuroethics, in its modern form, investigates the impact of brain science in four basic dimensions: the self, social policy, practice and discourse. In this study, we analyzed a set of 461 peer-reviewed articles with neuroethics content, published by authors from 32 countries. We analyzed the data for: (1) trends in the development of international neuroethics over time, and (2) how challenges at the intersection of ethics and neuroscience are viewed in countries that are considered developed by International Monetary Fund (IMF) standards, and in those that are developing. Our results demonstrate a steady increase in global participation in neuroethics from 1989 to 2005, characterized by an increase in numbers of articles published specifically on neuroethics, journals publishing these articles, and countries contributing to the literature. The focus from all countries was on the practice of brain science and the amelioration of neurological disease. Indicators of technology creation and diffusion in developing countries were specifically correlated with increases in publications concerning policy implications of brain science. Neuroethics is an international endeavor and, as such, should be sensitive to the impact that context has on acceptance and use of technological innovation. [source]


    Neuronal plasticity: implications in epilepsy progression and management

    DRUG DEVELOPMENT RESEARCH, Issue 8 2007
    Sherifa A. HamedArticle first published online: 12 FEB 200
    Abstract Epilepsy is a common neurological disease. A growing number of research studies provide evidence regarding the progressive neuronal damage induced by prolonged seizures or status epilepticus (SE), as well as recurrent brief seizures. Importantly, seizure is only one aspect of epilepsy. However, cognitive and behavioral deficits induced by progressive seizures or antiepileptic treatment can be detrimental to individual function. The neurobiology of epilepsy is poorly understood involving complex cellular and molecular mechanisms. The brain undergoes changes in its basic structure and function, e.g., neural plasticity with an increased susceptibility in neuronal synchronization and network circuit alterations. Some of these changes are transient, while others are permanent with an involvement of both glutamatergic and ,-aminobutyric acid (GABA)ergic systems. Recent data suggest that impaired neuronal plasticity may underlie the cognitive impairment and behavioral changes associated with epilepsy. Many neurologists recognize that the prevention or suppression of seizures by the use of antiepileptic drugs (AEDs) alone is insufficient without clear predictions of disease outcome. Hence, it is important to understand the molecular mechanisms underlying epileptogenesis because this may allow the development of innovative strategies to prevent or cure this condition. In addition, this realization would have significant impact in reducing the long-term adverse consequences of the disease, including neurocognitive and behavioral adverse effects. Drug Dev Res 68:498,511, 2007. © 2008 Wiley-Liss, Inc. [source]


    Original Article: Epidemiological features and association with crib-biting in horses with neurological disease associated with temporohyoid osteoarthropathy (1991,2008)

    EQUINE VETERINARY EDUCATION, Issue 9 2010
    N. S. Grenager
    Summary A retrospective study of 43 cases of temporohyoid osteoarthropathy was performed to evaluate the epidemiological features and a possible association with crib-biting. Data collected from records included case details, what diagnostics were utilised, whether medical or surgical treatment was administered, and outcome. Owners were contacted via telephone and asked whether the horse had displayed crib-biting behaviour. Forty-three horses were diagnosed with neurological disease associated with temporohyoid osteoarthropathy, 62.8% of which were Quarter Horse-types. Median age at presentation was 10 years and median duration of neurological signs prior to presentation was 3 days. Skull radiographs and guttural pouch endoscopy were used to definitively diagnose temporohyoid osteoarthropathy in 72% of the cases. Of 43 horses, 21 received medical treatment and 15 surgical treatment, with an overall survival rate of 55.8%. Crib-biting was observed in 31.3% of cases and there was a significant association between being afflicted with THO and likelihood of possessing the behaviour. Horses with neurological disease associated with THO were 8 times more likely to be crib-biters compared to the general population. [source]


    Advanced diagnostic imaging options in horses with neurological disease that localises to the head

    EQUINE VETERINARY EDUCATION, Issue 4 2007
    E. Jose-Cunilleras
    No abstract is available for this article. [source]


    Equine herpesvirus neurological disease

    EQUINE VETERINARY JOURNAL, Issue 2 2008
    J. R. GILKERSON
    No abstract is available for this article. [source]


    Lysosomal storage disease in Sida carpinifolia toxicosis: an induced mannosidosis in horses

    EQUINE VETERINARY JOURNAL, Issue 5 2003
    A. P. LORETTI
    Summary Reasons for performing study: This study reports a neurological disease unrecognised until now in ponies in southern Brazil. Hypothesis: Epidemiological data strongly suggests that the ingestion of Sida carpinifolia is involved in the aetiology. We tested the hypothesis that it is an acquired lyosomal storage disease. Methods: Following the death of 3 ponies, all ponies from the premises were closely monitored; epidemiological data and clinical findings carefully recorded. Fragments of several organs, including CNS, were fixed in neutral formalin and embedded in paraffin-wax. Sections were stained with haematoxylin and eosin. Representative sections of the cerebellum and trigeminal ganglia were submitted to lectin histochemical procedures. Results: The neurological disorder, characterised by stiff gait, muscle tremors, abdominal pain and death, was observed on a farm with 3 hectares of pasture. Three of 11 ponies died 15,20 days after they had been introduced into a new paddock heavily infested by the plant Sida carpinifolia. No significant gross lesions were observed. The main histological findings included multiple cytoplasmatic vacuoles in swollen neurones in the brain, cerebellum, spinal cord, autonomic ganglia (trigeminal and celiac ganglia), and submucosal and myenteric plexus of the intestines. In the kidneys, there was marked vacuolation of the proximal convoluted tubular cells. Sections of cerebellum and trigeminal ganglion were submitted to lectin histochemistry. The vacuoles in different cerebellar and ganglion cells reacted strongly to the following lectins: Concanavalia ensiformis, Triticum vulgaris and succinylated- Triticum vulgaris. Conclusions: The pattern of staining coincides with that of both swainsonine toxicosis and inherited mannosidosis reports. The histopathological changes were similar to those described in S. carpinifolia spontaneous and experimental poisoning in goats. This disease seems to be similar to Swainsona, Oxytropis and Astragalus toxicosis. Potential relevance: S. carpinifolia should be evaluated as a possible cause in the diagnosis of equine neuropathies. [source]


    Neurotoxicity upon infusion of dimethylsulfoxide-cryopreserved peripheral blood stem cells in patients with and without pre-existing cerebral disease

    EUROPEAN JOURNAL OF HAEMATOLOGY, Issue 6 2007
    Lutz P. Mueller
    Abstract Objective:, Toxicity related to the infusion of dimethylsulfoxide (DMSO)-cryopreserved peripheral blood stem cells (DMSO-PBSC) mainly comprises cardiovascular events. Fatal neurotoxicity has been reported in a few cases. DMSO represents the putative causative agent of such rare toxicities and elaborate strategies to replace DMSO would benefit from the identification of predisposing factors for DMSO-related toxicities. Methods:, Here, we report on DMSO-related neurotoxicity in a series of patients (n = 51) receiving DMSO-PBSC. The analyzed patient-series included eight patients with pre-existing cerebral disease, partially with a history of epileptic seizures. Results:, Neurotoxicity was observed in only one patient who suffered from a generalized tonic seizure upon infusion of DMSO-PBSC and for which the clinical course is reported herein. No neurotoxicity was observed in the group of patients with pre-existing neurological disease. Furthermore, no neurotoxicity was observed in patients who received particularly large volumes of DMSO. In all patients, mild non-neurological side effects occurred but besides the reported seizure no other severe adverse events were observed upon PBSC-infusion. Conclusions:, To our knowledge, this is the first report addressing the identification of predisposing factors for DMSO-related neurotoxicty. We conclude that infusion of DMSO-PBSC can be performed safely in patients with pre-existing cerebral disease despite the rare occurrence of severe neurotoxicity. Retrospective multicenter studies are warranted to identify patients who would benefit from elaborate methods of DMSO-replacement. [source]


    How are we doing with the treatment of essential tremor (ET)?

    EUROPEAN JOURNAL OF NEUROLOGY, Issue 6 2010
    Persistence of patients with ET on medication: data from 528 patients in three settings
    Background:, The pharmacological treatment of essential tremor (ET) is not optimal. There are only two first-line medications and troublesome side effects are common. It is not uncommon for patients to simply stop taking medication. Yet, no published data substantiate or quantify this anecdotal impression. Objectives:, To determine, amongst patients with ET who were prescribed medication for tremor, what proportion are still taking medication and what proportion have stopped? Methods:, Five hundred and twenty-eight patients with ET from three distinct study settings (clinical, brain donors, population) were interviewed. Results:, A clear pattern that emerged across settings was that the proportion of patients with ET who had stopped medication was sizable and consistently similar (nearly one-third): 31.4% (clinical), 24.3% (brain donors), 30.0% (population), 29.8% (overall). A similarly high proportion of cases with severe tremor had stopped their medication: 31.9% (clinical), 36.4% (brain donors). For the four most commonly used medications (propranolol, primidone, diazepam, topiramate), one-half or more of the treated patients had stopped the medication; amongst the less commonly used medications, the proportion who stopped was even higher. Conclusions:, Nearly one of every three patients with ET who had been prescribed medication for tremor had discontinued pharmacotherapy. Even more revealing was that a similar proportion of cases with severe tremor had stopped medication. These data make tangibly evident that there is a sizable population of patients with ET who are untreated and disabled, and underscore the inadequacy of current pharmacotherapeutic options for this common neurological disease. [source]


    Increased levels of inflammatory chemokines in amyotrophic lateral sclerosis

    EUROPEAN JOURNAL OF NEUROLOGY, Issue 6 2009
    J. Kuhle
    Background and purpose:, Amyotrophic lateral sclerosis (ALS) is classically assumed to be a neurodegenerative disorder. Inflammation has been observed in CNS tissue in ALS patients. We investigated the expression and prognostic relevance of proinflammatory chemokines in ALS. Methods:, We analyzed nine chemokines, eotaxin, eotaxin-3, IL-8, IP-10, MCP-1, MCP-4, macrophage derived chemokine (MDC), macrophage inflammatory protein-1, (MIP-1,), and serum thymus and activation- regulated chemokine (TARC) in serum and cerebrospinal fluid (CSF) of 20 ALS- and 20 non-inflammatory neurological disease (NIND)-patients. Results:, MCP-1 and IL-8 levels in CSF in ALS were significantly higher than in NIND (1304 pg/ml vs. 1055 pg/ml, P = 0.013 and 22.7 pg/ml vs. 18.6 pg/ml, P = 0.035). The expression of MCP-1 and IL-8 were higher in CSF than in serum (P < 0.001). There was a trend towards higher MCP-1 CSF levels in ALS patients with shorter time between first symptoms and diagnosis (r = ,0.407; P = 0.075). Conclusions:, We confirmed previous findings of increased MCP-1 levels in CSF of ALS patients. Furthermore, increased levels of IL-8 in CSF suggest a stimulation of a proinflammatory cytokine cascade after microglia activation. We found a tendency for higher MCP-1 values in patients with a shorter diagnostic delay, who are known to have also a shorter survival. This may suggest an association of higher MCP-1 levels with rapidly progressing disease. [source]


    Examination of intravenous and intra-CSF protein delivery for treatment of neurological disease

    EUROPEAN JOURNAL OF NEUROSCIENCE, Issue 6 2009
    Kim M. Hemsley
    Abstract Mucopolysaccharidosis type IIIA is a neurodegenerative lysosomal storage disorder characterized by progressive loss of learned skills, sleep disturbance and behavioural problems. Absent or greatly reduced activity of sulphamidase, a lysosomal protein, results in intracellular accumulation of heparan sulphate. Subsequent neuroinflammation and neurodegeneration typify this and many other lysosomal storage disorders. We propose that intra-cerebrospinal fluid protein delivery represents a potential therapeutic avenue for treatment of this and other neurodegenerative conditions; however, technical restraints restrict examination of its use prior to adulthood in mice. We have used a naturally-occurring Mucopolysaccharidosis type IIIA mouse model to determine the effectiveness of combining intravenous protein replacement (1 mg/kg) from birth to 6 weeks of age with intra-cerebrospinal fluid sulphamidase delivery (100 ,g, fortnightly from 6 weeks) on behaviour, the level of heparan sulphate-oligosaccharide storage and other neuropathology. Mice receiving combination treatment exhibited similar clinical improvement and reduction in heparan sulphate storage to those only receiving intra-cerebrospinal fluid enzyme. Reductions in micro- and astrogliosis and delayed development of ubiquitin-positive lesions were seen in both groups. A third group of intravenous-only treated mice did not exhibit clinical or neuropathological improvements. Intra-cerebrospinal fluid injection of sulphamidase effectively, but dose-dependently, treats neurological pathology in Mucopolysaccharidosis type IIIA, even when treatment begins in mice with established disease. [source]


    Tooth loss and associated factors in long-term institutionalised elderly patients

    GERODONTOLOGY, Issue 4 2007
    Paul Tramini
    Objective:, To compare partial and total tooth loss in dependent institutionalised elderly patients and identify any associated factors. Background:, A poor oral health status, together with a reduction of autonomy can seriously affect the general health and increase the risk of death in elderly people. Those with total tooth loss and in need of assistance are the most at risk. Materials and methods:, In 2004, a cross-sectional study of 321 elderly patients was conducted in long-term hospital services provided in Montpellier, France. Socio-demographic, behavioural, medical and oral health information was recorded for each patient. Multivariate logistic regression models were performed to test the relationship between those covariates and partial or total tooth loss. Pearson chi-squared tests were used for bivariate analyses. Results:, The proportion of edentulousness was 26.9%; among these12.6% had no dentures. The factors significantly associated with edentulism were: an age ,older than 87 years' [odds ratio (OR) = 9.4], the presence of a nephropathy (OR = 6.8), and inadequate oral hygiene (OR = 0.1). The factors most significantly associated with partial tooth loss (at least 21 missing teeth) were ,cancerous disease' (OR = 9.9), the presence of a nephropathy (OR = 5.6) and the presence of a neurological disease (OR = 4.1). The factors significantly related to dentate status (20 or more natural teeth retained) were ,hypertension treatment' (OR = 2.4), and ,cortisone treatment' (OR = 0.2). Conclusion:, General health problems as well as a poor oral condition were significant risk indicators for tooth loss among the long-term institutionalised elderly. This suggests that the number of remaining teeth has a strong effect on oral health-related quality of life. [source]


    T-cell reconstitution without T-cell immunopathology in two models of T-cell-mediated tissue destruction

    IMMUNOLOGY, Issue 2 2009
    Pablo Penaloza-MacMaster
    Summary Antigen-specific T cells play a pivotal role in adaptive immune responses. However, they also contribute to the progression of a variety of diseases including autoimmune disorders, graft rejection and graft-versus-host disease (GVHD). Non-specific immune-ablation treatments compromise the ability of the host to respond to infection, whereas the selective removal of epitope-specific T cells could theoretically ameliorate T-cell-mediated pathology while preserving the rest of the host immune function. In this study we investigated whether it is possible to destroy specific unwanted antigen-specific T cells by incubating polyclonal T-cell populations with major histocompatibility complex (MHC) tetramers that are conjugated to the ribosomal-inactivating toxin, saporin. This strategy resulted in a dramatic reduction in the number of targeted antigen (Ag)-specific CD8 T cells with no observable bystander toxicity in vitro. Moreover, in a model of transferable T-cell-dependent neurological disease induced by intracerebral (i.c.) lymphocytic choriomeningitis virus (LCMV) infection, the targeted killing of LCMV-specific CD8 T cells extended the survival of mice or fully prevented their death, depending on the dose of cells transferred. In addition, the tetramer, saporin conjugate also reduced liver damage in a model of donor T-cell-mediated hepatic destruction. These data provide a proof of principle that MHC tetramers could be exploited for the elimination or clinical manipulation of T-cell responses by linking effector molecules (a toxin in this case) to MHC tetramers. Also, the results suggest that it may be feasible to remodel T-cell responses, especially in immunocompromised hosts who receive adoptive cell transfers with many potential alloreactive cells. [source]


    Therapeutic plasma exchange as a nephrological procedure: A single-center experience ,,

    JOURNAL OF CLINICAL APHERESIS, Issue 4 2005
    Fred E. Yeo
    Abstract In the United States, therapeutic plasma exchange (TPE) is both performed and requested by a wide range of services, often on an empiric basis (before a diagnosis is established). Whether empiric therapy is beneficial has not been established. Patients were identified from an electronic procedure log that included those patients who received plasmapheresis at Walter Reed Army Medical Center from 1996 to 2003. The clinical indications, referring service, and outcomes (including deaths) that occurred were tabulated. Between March 1997 and August 2003, 568 TPE treatments were performed in 54 patients. The majority of the diagnoses were either neurologic (48%) or hematologic (37%). Thirty-three patients (61%) received TPE for a Category I indication. Twelve cases were performed empirically (without an established diagnosis) at the request of the referring service, most (7) performed for presumed thrombotic thrombocytopenic purpura (TTP). Almost 80% of patients required central venous catheters for treatment. Twelve patients (22%) experienced a major complication including death, and six patients (11%) died. Of the patients who died, 5 (83%) were treated empirically versus one death (17%) among patients not treated empirically, P < 0.001 by Chi Square. Only one of the seven patients treated empirically for TTP died, however. In logistic regression analysis, empiric treatment was the only factor independently associated with death, adjusted odds ratio, 34.2, 95% CI, 3.4, 334.8, P = 0.003. The most common indication for TPE was neurological disease, which also accounted for the highest proportion of complications. With the exception of presumed TTP, performing TPE in the absence of a confirmed diagnosis was not beneficial. J. Clin. Apheresis Published 2005 Wiley-Liss, Inc. [source]


    Familial cerebral cavernous haemangioma diagnosed in an infant with a rapidly growing cerebral lesion

    JOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 6 2006
    BHK Ng
    Summary Cavernous haemangiomas of the central nervous system are vascular malformations best imaged by MRI. They may present at any age, but to our knowledge only 39 cases in the first year of life have previously been reported. A familial form has been described and some of the underlying genetic mutations have recently been discovered. We present the clinical features and serial MRI findings of an 8-week-old boy who presented with subacute intracranial haemorrhage followed by rapid growth of a surgically proven cavernous haemangioma, mimicking a tumour. He also developed new lesions. A strong family history of neurological disease was elucidated. A familial form of cavernous haemangioma was confirmed by identification of a KRIT 1 gene mutation and cavernous haemangiomas in the patient and other family members. We stress the importance of considering cavernous haemangiomas in the context of intracerebral haemorrhage and in the differential diagnosis of rapidly growing lesions in this age group. The family history is also important in screening for familial disease. [source]


    Multiple sclerosis in a radiosensitive family with low levels of the ATM protein

    JOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 3 2002
    Raymond A Clarke
    SUMMARY Multiple sclerosis (MS) is a chronic neurological disease of the central nervous system (CNS) characterized by demyelination associated with progressive disability. The mechanisms underlying the pathogenesis of MS remain a mystery. The highly pleiotropic syndrome known as ataxia telangiectasia (A-T) overlaps with MS in that it also presents with demyelination in the CNS. Whether demyelination in MS or in A-T is initiated through neuronal degeneration or immune dysfunction is not yet known. However, unlike MS, the underlying cause of A-T is known to result from mutations in the A,T gene (ATM) that often result in the complete loss of ATM protein and loss/gain of function. ATM is implicated in neurological degeneration, particularly in the cerebellum, cellular apoptosis, immunodeficiency, double stranded deoxyribonucleic acid (DNA) rejoining, VDJ antibody recombination, tumour suppression, particularly T-lymphoid malignancies, signal transduction, cell-cycle control and cellular radiohypersensitivity. In this study, we describe a case of MS in a family with cellular radiosensitivity and abnormally low postinduction levels of the ATM protein. Defective DNA repair/rejoining may impact on autoimmunity. [source]


    Humoral immunity in natural infection by tick-borne encephalitis virus

    JOURNAL OF MEDICAL VIROLOGY, Issue 4 2009
    Giulietta Venturi
    Abstract Tick-borne encephalitis (TBE) virus is one of the most important flaviviruses associated with neurological disease in Europe. Cross-reactive antibodies elicited by different flaviviruses can make difficult the interpretation of ELISA and hemagglutination-inhibition (HI) tests for the diagnosis of TBE. Neutralization tests, which are more specific, are not in common use because they are difficult to perform and standardize. A plaque reduction neutralization test (PRNT), optimized previously in vaccinated children, was evaluated in sera from acute cases of TBE, collected for diagnostic purposes, and from healthy human population and wild ruminants, collected for serosurvey purposes. The PRNT results were compared with the results of ELISA and HI tests. In acute TBE disease, most sera were positive for IgM antibodies by ELISA and with high HI antibody titers; neutralizing antibodies were detected in 71.4% of patients, at a very low titer (1:10 NT50) in almost all cases. Seroprevalences of 8% and 6.5% for anti-TBE ELISA antibodies were found in healthy subjects and wild ruminants, respectively. Among anti-TBE positive healthy subjects, a very low 1:10 NT50 titer was detected in 17.4% of cases, while NT80 titers ranging from 1:10 to 1:80 were detected in 65.2% of cases. Among wild ruminants, 90.9% of ELISA and HI positive samples showed a positive, ,1:10 NT80 titer. In conclusion, neutralization assays can be useful for the diagnosis and serosurveys of TBE. J. Med. Virol. 81:665,671, 2009 © 2009 Wiley-Liss, Inc. [source]


    Epidemiology of enterovirus types causing neurological disease in Austria 1999,2007: Detection of clusters of echovirus 30 and enterovirus 71 and analysis of prevalent genotypes

    JOURNAL OF MEDICAL VIROLOGY, Issue 2 2009
    Birgit Ortner
    Abstract Between 1999 and 2007 1,388 stool specimens from patients with acute flaccid paralysis or aseptic meningitis were submitted to the Austrian reference laboratory for poliomyelitis. Samples (201) yielded non-poliovirus enterovirus in culture. One hundred eighty-one viruses were available for typing and 78 isolates which remained serologically untyped were further analyzed by CODEHOP-PCR and sequencing of the VP1 gene and the 5,-untranslated region (5,-UTR). Typing revealed an Echovirus 30 outbreak in northwestern Austria in 2000, which was in accordance with the situation in Europe, and no dramatic seasonal changes of Coxsackie viruses were observed. In 2002/2003 a small outbreak of enterovirus 71 (EV71), affected 12 patients in the province of Styria. This virus was identified as genotype C1 and appeared to be genetically distinct from the isolates observed in 2001/2002 in Vienna. In 2004 two unrelated cases occurred in Lower Austria, which were identified as genotype C4, which has been described associated with high mortality most recently in China. In contrast to the situation in Asia the detected EV71 cases were not associated with hand,foot,mouth disease, but with serous meningitis only. This was surprising as a recent publication suggested a reduced neurovirulence of C1 genotype in children in Norway, presumably due to alterations in 5,-UTR and polymerase gene. However, comparing the 5,-UTR of the Austrian isolates and established virulent reference strains to the Norwegian isolate and an attenuated EV71 laboratory strain we did not find an indication that the genotype C1 possesses a RNA structure in its 5,-UTR leading to reduced neurovirulence. J. Med. Virol. 81:317,324, 2009. © 2008 Wiley-Liss, Inc. [source]


    Presence and expression of JCV early gene large T Antigen in the brains of immunocompromised and immunocompetent individuals

    JOURNAL OF MEDICAL VIROLOGY, Issue 12 2008
    Serena Delbue
    Abstract JC virus (JCV) is a polyomavirus that asymptomatically infects up to 80% of the worldwide human population and establishes latency in the kidney. In the case of host immunodeficiency, it can cause progressive multifocal leukoencephalopathy (PML), which is a fatal demyelinating disease of the central nervous system. In an attempt to understand better PML pathogenesis and JCV infection, the presence of the JCV genome and expression of the early viral protein in the brain of deceased individuals, with and without HIV infection, was investigated. Sixty autopsy samples of brain tissues were collected from 15 HIV-positive PML patients, 15 HIV-positive patients with other neurological diseases, 15 HIV-positive patients without neurological disorders, and 15 HIV-negative individuals who died from diseases unrelated to the central nervous system. By means of specific Real Time Polymerase Chain Reaction, the JCV genome was detected in 14 of 15 PML brains, three of 15 HIV-positive brains (with and without neurological diseases), and 1 of 15 HIV-negative brains. JCV genotyping was also performed. Expression of the early JCV protein T Antigen was verified by a specific immunohistochemistry assay, and it was found in the brain tissues from 12 PML cases and one case with other neurological disease. The data obtained demonstrate that infection of the brain with JCV can also be observed in the brains of HIV-negative individuals, without neurological disorders. However, viral protein expression was limited to PML brains and to one brain from a patient with other neurological disease, suggesting that JCV can also be present in the brains of patients without PML. J. Med. Virol. 80:2147,2152, 2008. © 2008 Wiley-Liss, Inc. [source]


    Identification of a unique BK virus variant in the CNS of a patient with AIDS,

    JOURNAL OF MEDICAL VIROLOGY, Issue 1 2003
    Gunn Eli Kimo Jørgensen
    Abstract Human polyomavirus BK (BKV; GenBank or EMBL or DDBJ accession no. NC001538) is often reactivated in immunosuppressed patients. Reactivation has been associated primarily with excretion of the virus in the urine, and there have been few reports of renal and/or neurological disease caused by BKV in patients with acquired immunodeficiency syndrome (AIDS). Polymerase chain reaction, Southern blotting, and sequencing were used to detect and identify the noncoding control region (NCCR) of BKV in different tissues in an AIDS patient with meningoencephalitis, retinitis, and nephritis. An undescribed reorganized NCCR variant of the virus, completely different from the variants detected in peripheral blood leukocytes (PBLs) and urine, was identified in the cerebrospinal fluid (CSF) and CNS tissues. These results suggest that rearrangements in the NCCR of the virus have resulted in a BKV variant, which is better adapted to the host cell machinery of the cells in CNS tissue. The rearranged variant (BKV CNS) might have been involved in the initiation and/or development of the pathological lesions observed in the CNS-related tissues of this patient. J. Med. Virol. 70: 14,19, 2003. © 2003 Wiley-Liss, Inc. [source]


    Innate and adaptive immune activation in the brain of MPS IIIB mouse model

    JOURNAL OF NEUROSCIENCE RESEARCH, Issue 4 2009
    Julianne DiRosario
    Abstract Mucopolysaccharidosis (MPS) IIIB is a lysosomal storage disease with severe neurological manifestations due to ,- N -acetylglucosaminidase (NaGlu) deficiency. The mechanism of neuropathology in MPS IIIB is unclear. This study investigates the role of immune responses in neurological disease of MPS IIIB in mice. By means of gene expression microarrays and real-time quantitative reverse transcriptase,polymerase chain reaction, we demonstrated significant up-regulation of numerous immune-related genes in MPS IIIB mouse brain involving a broad range of immune cells and molecules, including T cells, B cells, microglia/macrophages, complement, major histocompatibility complex class I, immunoglobulin, Toll-like receptors, and molecules essential for antigen presentation. The significantly enlarged spleen and lymph nodes in MPS IIIB mice were due to an increase in splenocytes/lymphocytes, and functional assays indicated that the T cells were activated. An autoimmune component to the disease was further suggested by the presence of putative autoantigen or autoantigens in brain extracts that reacted specifically with serum IgG from MPS IIIB mice. We also demonstrated for the first time that immunosuppression with prednisolone alone can significantly slow the central nervous system disease progression. Our data indicate that immune responses contribute greatly to the neuropathology of MPS IIIB and should be considered as an adjunct treatment in future therapeutic developments for optimal therapeutic effect. © 2008 Wiley-Liss, Inc. [source]


    The safety profile of anti-tumour necrosis factor therapy in inflammatory bowel disease in clinical practice: analysis of 620 patient-years follow-up

    ALIMENTARY PHARMACOLOGY & THERAPEUTICS, Issue 3 2009
    C. W. LEES
    Summary Background, Anti-TNF agents are now widely used in Crohn's disease (CD), and in ulcerative colitis (UC). Aim, To review the safety profile of anti-TNF agents in all patients treated with infliximab in Edinburgh from 1999 to 2007. Methods, Complete data were available on 202/207 patients comprising 157 CD, 42 UC and three coeliac disease. Median follow-up was 2.4 years (1.0,4.9) with a total of 620 patient-years follow-up. About 19.1% of CD patients were subsequently treated with adalimumab. Results, Seven deaths (3.3%) occurred in follow-up; only one death was <1 year post-infliximab (at day 72, from lung cancer). A total of six malignancies (three haematological, three bronchogenic) and six cases of suspected demyelination (three with confirmed neurological disease) were reported. In the 90 days following infliximab, 95 adverse events (36 serious) occurred in 58/202 (28.7%) patients. In all, 42/202 (20.8%) had an infectious event (22 serious) and 27/202 (13.4%) of patients had an infusion reaction: 19 acute (four serious) and eight delayed (three serious). Conclusions, Serious infections, malignancies and neurological disease complicate anti-TNF use in clinical practice. Although evidence for causality is unclear, potential mechanisms and predisposing factors need to be explored. In individual patients, the risk/benefit analysis needs to be carefully assessed and discussed prior to commencement of therapy. [source]


    Competitive AMPA receptor antagonists

    MEDICINAL RESEARCH REVIEWS, Issue 2 2007
    Daniela Catarzi
    Abstract Glutamic acid (Glu) is the major excitatory neurotransmitter in the mammalian central nervous system (CNS) where it is involved in the physiological regulation of different processes. It has been well established that excessive endogenous Glu is associated with many acute and chronic neurodegenerative disorders such as cerebral ischaemia, epilepsy, amiotrophic lateral sclerosis, Parkinson's, and Alzheimer's disease. These data have consequently added great impetus to the research in this field. In fact, many Glu receptor antagonists acting at the N -methyl- D -aspartic acid (NMDA), 2-amino-3-(3-hydroxy-5-methylisoxazol-4-yl)propionic acid (AMPA), and/or kainic acid (KA) receptors have been developed as research tools and potential therapeutic agents. Ligands showing competitive antagonistic action at the AMPA type of Glu receptors were first reported in 1988, and the systemically active 2,3-dihydroxy-6-nitro-7-sulphamoyl-benzo[f]quinoxaline (NBQX) was first shown to have useful therapeutic effects in animal models of neurological disease in 1990. Since then, the quinoxaline template has represented the backbone of various competitive AMPA receptor antagonists belonging to different classes which had been developed in order to increase potency, selectivity and water solubility, but also to prolong the "in vivo" action. Compounds that present better pharmacokinetic properties and less serious adverse effects with respect to the others previously developed are undergoing clinical evaluation. In the near future, the most important clinical application for the AMPA receptor antagonists will probably be as neuroprotectant in neurodegenerative diseases, such as epilepsy, for the treatment of patients not responding to current therapies. The present review reports the history of competitive AMPA receptor antagonists from 1988 up to today, providing a systematic coverage of both the open and patent literature. © 2006 Wiley Periodicals, Inc. [source]


    Mild Parkinsonian signs: An overview of an emerging concept

    MOVEMENT DISORDERS, Issue 12 2007
    Elan D. Louis MD
    Abstract Mild Parkinsonian signs (MPS) include gait and balance changes, rigidity, bradykinesia, and tremor. MPS can occur commonly during the clinical examination of older people who do not have known neurological disease, with prevalence estimates for MPS as a whole ranging from 15% to 95%. MPS are generally progressive and they are coupled with functional difficulties, impaired gait and balance, and increased risks of mild cognitive impairment, dementia, and mortality. The mechanistic basis for these signs is unclear, but is likely to be multifactorial, with possible factors including an age-associated decline in dopaminergic nigrostriatal activity, the early development of neurodegenerative (Lewy body or Alzheimer's type) pathologies in the basal ganglia, or the accumulation of vascular pathology in the brain. It would be valuable to identify those individuals with MPS who are at increased risk for the development of future Alzheimer's disease, full-blown Parkinson's disease, or strokes, and to develop therapeutic strategies to intervene to lessen the likelihood of MPS-related morbidity and mortality. © 2007 Movement Disorder Society [source]


    Lack of association between progressive supranuclear palsy and arterial hypertension: A clinicopathological study

    MOVEMENT DISORDERS, Issue 6 2003
    Carlo Colosimo MD
    Abstract It has been reported that up to 80% of patients clinically diagnosed as having progressive supranuclear palsy (PSP) may have arterial hypertension (HT). Because previous studies were performed on patients with presumed diagnosis of PSP, we tried to replicate these studies in a series of pathologically confirmed patients. Seventy-three patients with a neuropathological diagnosis of PSP autopsied at the Queen Square Brain Bank for Neurological Disorders in London were collected between 1989 and 1999. For the purpose of this study, patients were considered hypertensive if a blood pressure above 140/90 mm Hg was found in the clinical records. The prevalence of HT in PSP patients at the first and at the last visit during their neurological disease was compared with that found in a series of 21 normal controls who donated their brain to the same institution. Overall, 29 of 73 (39.7%) of the patients were recorded as having HT at the first visit during the disease course; this ratio increased to 42 of 73 (57.5%) at the last visit before death. When these figures were compared to the 21 normal controls (11 of 21 with HT, 52.4%), we were unable to find an increased prevalence of HT in PSP (odds ratio, 0.60; 95% confidence interval, 0.20,1.76). Therefore, HT does not represent an important clinical feature of this neurodegenerative disorder, although cerebrovascular disease can masquerade clinically as PSP. © 2003 Movement Disorder Society [source]


    Validation for tremor quantification of an electromagnetic tracking device

    MOVEMENT DISORDERS, Issue 2 2001
    Padraig E. O'Suilleabhain MB
    Abstract An electromagnetic tracking system was used to record arm motion in subjects with Parkinson's disease (n = 23), essential tremor (n = 28) or without neurological disease (n = 4). Tremor magnitude was calculated by averaging the three-dimensional displacement of individual tremor bursts. Tremor magnitude calculated in this manner was quite closely correlated with a clinician's estimate (r = 0.88 and 0.86 for Parkinsonian and essential tremors, respectively) and was reproducible (r = 0.93 for repeated recordings). The accuracy of the device and algorithm was confirmed by mechanically generating oscillations of known magnitudes and frequencies. This device is adaptable for quantifying different types of tremors and its accuracy is easy to verify. Because position rather than acceleration is tracked, tremor amplitude can be stated in readily comprehensible units. © 2001 Movement Disorder Society. [source]