Abstract:
:Multiple sclerosis is a chronic inflammatory disease of the white and grey matter which results in irrevocable axonal and neuronal damage. Grey matter injury is widespread and reflects disability to a greater extent than do white matter lesions. Growing understanding of the immunopathology of multiple sclerosis is leading the way to the identification and testing of untapped targets that may offer new and more specific ways to treat the disease. For example, data from animal models support a two-step pathological process in multiple sclerosis, whereby T cells initially induce inflammation and open up the blood-brain barrier, which then allows access to antibodies which aggravate tissue damage. Determination of the specificity of the invading T cells and the autoantibodies that cause disease is a major focus of current research. The discovery of anti-aquaporin-4 autoantibodies in patients with neuromyelitis optica and of anti-MOG antibodies in a subset of children with paediatric autoimmune demyelinating disease are promising steps in this direction. Recently, the axoglial antigens neurofascin and contactin-2/TAG-1, which are localised around the node of Ranvier, were identified as targets of an autoimmune response in multiple sclerosis. Such an autoimmune response might induce axonal injury and direct the immunopathological response to the grey matter. It is to be hoped that the outcome of such investigations will lead to the identification of patient subgroups based on their autoreactivity and new ways to treat them safely and effectively.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Meinl Edoi
10.1016/S0022-510X(11)70003-Xsubject
Has Abstractpub_date
2011-12-01 00:00:00pages
S12-5eissn
0022-510Xissn
1878-5883pii
S0022-510X(11)70003-Xjournal_volume
311 Suppl 1pub_type
杂志文章abstract::There have been recent developments in the pathology of sporadic ALS. A new filamentous neuronal inclusion body in ALS detected by immunohistochemical localisation of the protein ubiquitin has been characterised at the light microscopic and ultrastructural level and appears specific for the disease. The molecular comp...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/0022-510x(94)90175-9
更新日期:1994-07-01 00:00:00
abstract:BACKGROUND:Recently, PRRT2 gene mutations have been identified as a causative factor of paroxysmal kinesigenic dyskinesia (PKD). However, evidence is still lacking with respect to the genotype to phenotype correlation in PKD patients. METHODS:We recruited a cohort of PKD patients with or without PRRT2 mutations for th...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.02.034
更新日期:2014-05-15 00:00:00
abstract::A 52-year-old Caucasian male with typical features of myotonic dystrophy (MD) developed a lung abscess and was found to have a mild atypical cyclic neutropenia. Granulocyte function testing revealed a defect in phagocytosis, bactericidal activity and chemotaxis. The defects were less severe at the nadir of the granulo...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(86)90057-2
更新日期:1986-03-01 00:00:00
abstract::Familial hemiplegic migraine type 1, spinocerebellar ataxia type 6 (SCA6) and episodic ataxia type 2 (EA2) are allelic disorders associated with mutations in the CACNA1A gene, which encodes the alpha1 subunit of the P/Q-type calcium channel (Ca(V)2.1). SCA6 and EA2 share a number of clinical features, such as prominen...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2007.01.008
更新日期:2007-03-15 00:00:00
abstract::Paradoxical embolism to the cerebral circulation is often difficult to diagnose clinically. We report a case that illustrates the usefulness of transesophageal echocardiography in the detection of paradoxical embolism. An 84-year-old woman presented with bilateral hemispheric strokes. Despite the presence of atrial fi...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2007.01.006
更新日期:2007-03-15 00:00:00
abstract::Here we report a case with positive serum anti-aquaporin 4 (AQP4) antibody who presented with hypersomnolence, symmetrical hypothalamic lesions and a reduced CSF orexin (hypocretin) level without optic nerve and spinal cord lesions on MRI. All of the symptoms, MRI finding and CSF orexin level improved simultaneously a...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2011.08.031
更新日期:2012-01-15 00:00:00
abstract::In two different studies, monkeys were fed diets with 50% and 30% of calories replaced with alcohol for 5 and 3 years, respectively. Nerves were studied with electrophysiological and quantitative histological methods, but no deleterious effect of alcohol was identified. ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(87)90221-8
更新日期:1987-08-01 00:00:00
abstract::The analysis of human skeletal muscle mitochondria revealed a progressive decline in mitochondrial respiratory chain function with age. The activities affected to the greatest extent were those of complexes I and IV which were decreased by 59% and 47% respectively between the ages of 20-30 years and 60-90 years of age...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(92)90270-u
更新日期:1992-11-01 00:00:00
abstract::The gut microbiome is composed of an enormous number of microorganisms, generally regarded as commensal bacteria. Resident gut bacteria are an important contributor to health and significant evidence suggests that the presence of healthy and diverse gut microbiota is important for normal cognitive and emotional proces...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2019.03.027
更新日期:2019-05-15 00:00:00
abstract::Post-streptococcal neuropsychiatric disorders encompass a broad spectrum of movement disorders, including tics, stereotypies, dystonia and tremor. We report the case of a 15-year-old boy who presented with a relapsing-remitting combination of psychogenic and organic movement disorders. Both relapses occurred after an ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2009.10.004
更新日期:2010-01-15 00:00:00
abstract::Mast cells (MC) have been implicated in the pathogenesis of experimental allergic encephalomyelitis (EAE). In order to further evaluate their role, several morphological and functional studies were performed. Semiquantitative counts of histological sections showed a significant reduction in MC numbers in EAE brains. I...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(94)90300-x
更新日期:1994-04-01 00:00:00
abstract:PURPOSE:We compared clinical characteristics and outcomes of the early-onset seizure post-stroke patients who had seizures occurring at stroke presentation (SSP) with other patients without SSP at a single institution in Eastern China. METHODS:We reviewed 20,947 ischemic stroke patients in our hospital electronic medi...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2018.01.028
更新日期:2018-04-15 00:00:00
abstract::Depression and cognitive impairment are both common conditions in old age, and frequently occur together. However, accurate figures of the co-occurrence are not available. The inter-relationship between the two clinical entities is still complex and not well understood. Clearly depression can be a psychological reacti...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2009.02.346
更新日期:2009-08-15 00:00:00
abstract::Epstein-Barr virus (EBV) is a human DNA herpesvirus infecting more than 90% of the world's population. EBV is the etiological agent of infectious mononucleosis (Pfeiffer's disease). Furthermore, diverse malignancies such as Burkitt and Hodgkin lymphoma have been associated with EBV. More recently, a possible role for ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2009.03.028
更新日期:2009-11-15 00:00:00
abstract::Our objective was to apply the technique of measuring diameters of optic nerve sheath (ONSD) for the intracranial pressure assessment for the cases with traumatic head injury without hemorrhage. In a retrospective study, CT data of 720 adult patients were collected and analyzed. ONSDs were measured at the point where ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2016.07.023
更新日期:2016-09-15 00:00:00
abstract:PURPOSE:Among different seizure types, tonic-clonic seizures are more significant because they are more often associated with morbidity. No prior study has been done to investigate risk factors associated with tonic-clonic seizures in patients with mesial temporal epilepsy. METHODS:In this retrospective study, all dru...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2015.10.022
更新日期:2015-12-15 00:00:00
abstract::The Trembler mouse suffers from a dominantly inherited hypertrophic neuropathy. Electron microscopy, including a quantitative analysis of myelination was performed on the nerves of Trembler mice from birth to senility and compared with the findings in control mice. Axons in adult Trembler nerves were thinly myelinated...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(76)90139-8
更新日期:1976-12-01 00:00:00
abstract::We treated a female patient with West syndrome caused by thiamine-responsive pyruvate dehydrogenase complex (PDHC) deficiency. Infantile spasms occurred in association with elevated blood and CSF lactate concentrations; these symptoms disappeared when lactate concentrations had been lowered by treatment with concomita...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/s0022-510x(99)00250-6
更新日期:1999-12-01 00:00:00
abstract::The role of excitotoxicity on the neuropathology of glutaric acidemia type I (GA I) is still under debate. Therefore, in the present work, we evaluated glutamate uptake by brain slices and glutamate binding to synaptic membranes, as well as glutamine synthetase activity in cerebral cortex and striatum from glutaryl-Co...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.09.003
更新日期:2014-11-15 00:00:00
abstract:BACKGROUND:Both intra-arterial recombinant tissue plasminogen activator (rt-PA) and stent retrieval are effective for treating acute ischemic stroke. The goal of this study was to evaluate the effectiveness of stent retrieval combined with intra-arterial rt-PA administration via micro-catheter (called the complex techn...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2018.01.012
更新日期:2018-03-15 00:00:00
abstract::Morphologic alterations in biopsies of central and peripheral nervous tissue were investigated at the light-and electron-microscopic level in the first cases of lysosomal alpha-N-acetylgalactosaminidase deficiency. Widespread spheroid formation was observed in terminal and preterminal axons. Neocortical and peripheral...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(95)00124-k
更新日期:1995-09-01 00:00:00
abstract:BACKGROUND:Cladribine causes sustained reduction in peripheral T and B cell populations while sparing other immune cells. We determined two populations of dendritic cells (DCs): namely CD1c(+)/CD19(-) (myeloid DCs) and CD303(+)/CD123(+) (plasmacytoid DCs), CD19(+) B lymphocytes, CD3(+) T lymphocytes and CD4(+) or CD8(+...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.06.003
更新日期:2013-09-15 00:00:00
abstract::Vitamin B1- and B-deficient encephalopathy was produced in adult Wistar rats and the effect of methylcobalamin was studied. These experiments were performed in vitamin B1- or B-deficient rats with or without the administration of guanidine, a magnesium antagonist. These encephalopathic rats showed symmetrical lesions ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(87)90210-3
更新日期:1987-01-01 00:00:00
abstract::We present 2 patients in whom the predominant neurological complication following lightning strike was spinal cord injury. One patient, who was followed for 5 years, showed clinical, electrophysiologic and MRI evidence of cervical spinal cord injury. This patient had significant recovery, which in part, may be related...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2008.09.020
更新日期:2009-01-15 00:00:00
abstract::Multiple sclerosis (MS) patients have been considered a higher-risk population for COVID-19 due to the high prevalence of disability and disease-modifying therapy use; however, there is little data identifying clinical characteristics of MS associated with worse COVID-19 outcomes. Therefore, we conducted a multicenter...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,多中心研究
doi:10.1016/j.jns.2020.117147
更新日期:2020-11-15 00:00:00
abstract::Brainstem gliomas constitute 10% of brain tumors in children and less than 2% in adults. Since therapeutic options are limited and brainstem gliomas are associated with a high morbidity and mortality, we sought to analyze the prognostic factors associated with a better outcome. We reviewed the records of 86 patients w...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2009.03.009
更新日期:2009-06-15 00:00:00
abstract::High plasma homocysteine (tHcy) is a risk factor for cardiovascular disease and stroke and Alzheimer's disease (AD). An inverse relationship has been reported between tHcy and plasma B12 and folate levels. Seventy-nine AD patients and 156 controls from three Arab villages in northern Israel participated. Plasma tHcy, ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2004.08.011
更新日期:2004-12-15 00:00:00
abstract::Neurodegenerative disorders are marked by extensive neuronal apoptosis and gliosis. Although several apoptosis-inducing agents have been described, understanding of the regulatory mechanisms underlying modes of cell death is incomplete. A major breakthrough in delineation of the mechanism of cell death came from eluci...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2008.12.010
更新日期:2009-03-15 00:00:00
abstract::Single channel recording patch-clamp technique was used in the mouse to compare the acetylcholine (ACh)-induced channel behaviour between normal and dystrophic myotubes. While open time and slope conductance were equivalent, ACh-induced channel opening frequency was more than 4-fold reduced in dystrophic compared to n...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(88)90176-1
更新日期:1988-03-01 00:00:00
abstract::Genetic linkage studies have provided evidence for a late-onset Alzheimer's disease (AD) susceptibility locus on chromosome 21q. We have tested, in a two-stage association study, whether allelic or haplotype variation of the beta-amyloid cleaving enzyme-2 (BACE2) locus on chromosome 21q affects the risk of late-onset ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,多中心研究
doi:10.1016/j.jns.2005.04.008
更新日期:2005-09-15 00:00:00