Abstract:
:Myasthenia gravis (MG) is the archetypic disorder of both the neuromuscular junction and autoantibody-mediated disease. In most patients, IgG1-dominant antibodies to acetylcholine receptors cause fatigable weakness of skeletal muscles. In the rest, a variable proportion possesses antibodies to muscle-specific tyrosine kinase while the remainder of seronegative MG is being explained through cell-based assays using a receptor-clustering technique and, to a lesser extent, proposed new antigenic targets. The incidence and prevalence of MG are increasing, particularly in the elderly. New treatments are being developed, and results from the randomised controlled trial of thymectomy in non-thymomatous MG, due for release in early 2016, will be of particular clinical value. To help navigate an evidence base of varying quality, practising clinicians may consult new MG guidelines in the fields of pregnancy, ocular and generalised MG (GMG). This review focuses on updates in epidemiology, immunology, therapeutic and clinical aspects of GMG in adults.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Binks S,Vincent A,Palace Jdoi
10.1007/s00415-015-7963-5subject
Has Abstractpub_date
2016-04-01 00:00:00pages
826-34issue
4eissn
0340-5354issn
1432-1459pii
10.1007/s00415-015-7963-5journal_volume
263pub_type
杂志文章,评审abstract:: ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09192-5
更新日期:2019-03-01 00:00:00
abstract:OBJECTIVES:Sporadic (SSP) and hereditary spastic paraplegias (HSP) are clinically and genetically heterogeneous disorders, which are characterised by a slowly progressive spastic paraparesis. Initial symptoms and the rate of progression are variable even among members of the same family. Spastic paraparesis is the majo...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-004-0366-7
更新日期:2004-05-01 00:00:00
abstract:: ...
journal_title:Journal of neurology
pub_type: 评论,杂志文章
doi:10.1007/s00415-018-9098-y
更新日期:2018-12-01 00:00:00
abstract:BACKGROUND:Spontaneous cervical artery dissections (sCAD) are often preceded by infections. However, existing data about inflammatory parameters remained inconsistent. Remarkably, concurrent information about the coagulation system, whose affection seems also reasonable to cause ischaemic events, are still lacking in s...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8696-4
更新日期:2018-02-01 00:00:00
abstract:OBJECTIVES:To estimate the socio-economic impact of multiple sclerosis (MS) in Italy. METHODS:Outpatients with MS were enrolled at 44 centres across Italy. Socio-demographic, clinical and resource utilization data were collected using a validated questionnaire. Each patient completed a weekly diary of expenses due to ...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1007/pl00007858
更新日期:2002-02-01 00:00:00
abstract:BACKGROUND:Ultrafast brain MRI is required for uncooperative patients and time-critical diseases such as stroke because it reduces scan times and motion artifacts. This study investigated the clinical feasibility of a 1-min ultrafast brain MRI protocol for detecting intracranial abnormalities in restless and uncooperat...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9149-4
更新日期:2019-02-01 00:00:00
abstract::This meta-analysis aims to compare percutaneous transluminal angioplasty (PTA) to medical treatment (MT) for symptomatic vertebral artery stenosis (SVAS) treatment. We searched PubMed, Springer, Google Scholar, Clinical Trials, Cochrane Central, Chinese National Knowledge Infrastructure, and China Biological Medicine ...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s00415-016-8267-0
更新日期:2017-05-01 00:00:00
abstract::Disease management is defined as any medical or pharmaceutical intervention designed to improve both outcomes for the patient and overall cost-effectiveness of the health plan. Disease management focuses on the patient throughout the entire course of the disease, involving both health providers and third-party payers....
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/pl00014806
更新日期:1998-08-01 00:00:00
abstract::To disclose the neuropathological progression course of Machado-Joseph disease (MJD), magnetic resonance imaging (MRI) findings of six genetically confirmed MJD cases (four males and two females, including an autopsied female, all unrelated to one another) were further investigated on neurodegeneration. Brain MRI stud...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-5992-2
更新日期:2011-09-01 00:00:00
abstract::Fatigue is a common symptom of multiple sclerosis (MS) even in the early phases of the disease, when neurological disability is usually still not present. To investigate the pathophysiology of fatigue we compared neurophysiological (motor evoked potentials of the four limbs, MEPs) and brain magnetic resonance imaging ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150070148
更新日期:2000-07-01 00:00:00
abstract::Surface antigens on peripheral blood lymphocytes from myasthenia gravis patients were investigated. The expression of DR+ and CD8+/DR+ T lymphocytes was increased and the expression of CD4+ T cells reduced. Neither thymectomy, clinical condition nor anti-acetylcholine receptor antibody titre correlated with any of the...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00863771
更新日期:1994-02-01 00:00:00
abstract::Little is known about the pattern of subacute cognitive domain impairments after ischaemic stroke, nor the temporal evolution of such impairments. Our objective was to investigate the pattern of cognitive impairment in different neuropsychological domains up to a year after ischaemic stroke. We included prospectively ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-012-6625-0
更新日期:2013-01-01 00:00:00
abstract::Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease which was thought to be untreatable. However, recent evidence in both experimental animals and men indicates that antiglutamatergic strategies are the first to have an influence on its pathogenesis and slow down the disease process. Since the eff...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/BF03161074
更新日期:2000-12-01 00:00:00
abstract::Hereditary peripheral neuropathies present a group of clinically and genetically heterogeneous entities. All known forms, including the various forms of Charcot-Marie-Tooth disease (CMT) are characterized as Mendelian traits and over 35 genes have been identified thus far. The mutational mechanism of the most common C...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-009-5401-2
更新日期:2010-05-01 00:00:00
abstract::Pontocerebellar hypoplasia type 2 (PCH2) is a very rare autosomal recessive disorder. We report two unrelated female patients born to consanguineous parents presenting with this condition. Patient 1 showed a classical clinical/neuroradiological phenotype of PCH2 with dyskinesia/dystonia. Patient 2 had a neonatal onset...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s004150200069
更新日期:2002-05-01 00:00:00
abstract::Patients in a minimally conscious state (MCS) show restricted signs of awareness but are unable to communicate. We assessed cerebral glucose metabolism in MCS patients and tested the hypothesis that this entity can be subcategorized into MCS- (i.e., patients only showing nonreflex behavior such as visual pursuit, loca...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6303-7
更新日期:2012-06-01 00:00:00
abstract::Unlike most neurodegenerative disorders, individuals at risk from Huntington's disease can be identified prior to the onset of clinical signs of the disease by virtue of it being an autosomal dominant condition. This provides the hypothetical opportunity to delay disease onset and/or slow down the progression of the d...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-015-7700-0
更新日期:2015-08-01 00:00:00
abstract::Most inflammatory neuropathies, both acute and chronic, probably result from an immune attack against antigens of the peripheral nervous system. Specific antibodies in serum that react with the peripheral nervous system have been described in a number of inflammatory neuropathies. We review the pathophysiological sign...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s004150070170
更新日期:2000-06-01 00:00:00
abstract:OBJECTIVE:To investigate whether intracranial arachnoid cysts (AC) compromise visual attention and if so, whether surgical cyst decompression leads to improvement in visual attention performance. METHODS:The experiments were carried out on 27 patients with temporal (n=21) or frontal (n=6) AC, and 27 healthy control su...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1007/s00415-006-0280-2
更新日期:2007-01-01 00:00:00
abstract::X-linked adrenoleukodystrophy (XALD), a neurological disorder caused by mutations in the peroxisomal membrane protein gene ABCD1, presents as a rapidly progressing, inflammatory cerebral demyelination (cerebral cases) or a slowly progressing, distal axonopathy (non-cerebral cases). Specific ABCD1 defects do not explai...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6371-8
更新日期:2012-07-01 00:00:00
abstract::Clinical and neuropathological data of a 50-year-old woman with an unusual multisystem degeneration are presented. Clinically the illness was characterized by progressive ataxia with ophthalmoplegia and multiple cranial nerve palsies. Neuropathological investigation showed a severe and selective degeneration of the de...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313709
更新日期:1985-01-01 00:00:00
abstract::Global aphasia is described as a unitary syndrome which is characterized by a severe impairment of all linguistic capabilities. Speech production is extremely limited and consists of stereotyped phrases, recurring utterances or a few isolated words which are usually neologistically distorted. The patients are unable t...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF02430346
更新日期:1977-01-13 00:00:00
abstract::The cases of identical twins with ataxia telangiectasia, early intellectual impairment and progressive spasticity are reported. Immunological tests revealed reduced levels of serum and salivary IgA, increased B cells, reduced T cells and raised alpha-fetoprotein. CT scan performed in one of the twins was normal. The p...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314163
更新日期:1986-10-01 00:00:00
abstract:BACKGROUND:Propranolol is recommended as first-line treatment for preventing migraine attacks; acupuncture has not been compared with propranolol in a head-to-head trial. OBJECTIVE:To compare acupuncture with propranolol using indirect treatment comparison meta-analysis. METHOD:We searched MEDLINE, EMBASE, and Cochra...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析
doi:10.1007/s00415-019-09510-x
更新日期:2020-01-01 00:00:00
abstract::Recessive mutations in GJC2, the gene-encoding connexin 47 (Cx47), cause Pelizaeus-Merzbacher-like disease type 1, a severe dysmyelinating disorder. One recessive mutation (p.Ile33Met) has been associated with a much milder phenotype--hereditary spastic paraplegia type 44. Here, we present evidence that a novel Arg98L...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7429-1
更新日期:2014-10-01 00:00:00
abstract:BACKGROUND:Spinocerebellar ataxias (SCAs) are rare dominantly inherited neurodegenerative disorders that lead to severe disability and premature death. OBJECTIVE:To better characterize the natural history of the most common SCAs, SCA1, SCA2, SCA3 and SCA6, we performed a meta-analysis of literature to determine diseas...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-020-09815-2
更新日期:2020-04-07 00:00:00
abstract::Apraxia of lid opening (ALO) is a non-paralytic inability to open the eyes or sustain lid elevation at will. The exact pathophysiological mechanisms underlying the syndrome are still unknown. ALO has been reported in patients with Parkinson's disease (PD) after subthalamic nucleus (STN) deep brain stimulation (DBS), s...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-012-6447-0
更新日期:2012-09-01 00:00:00
abstract::The objective of this study is to determine the current distribution of clinical phenotypes and to estimate future trends of ALS incidence in Western societies. We report on a clinical-epidemiological registry with a capture-recapture rate of >80% and population-based case-control study in ALS patients in South Wester...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8413-3
更新日期:2017-04-01 00:00:00
abstract:BACKGROUND:More research is needed to understand the contribution of comorbidities to MS symptomatology. OBJECTIVES:To examine the dose-response relationship between the number of comorbidities and severity of MS symptoms and to assess the relative contribution of comorbidity groups and individual comorbidities to the...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10192-z
更新日期:2020-09-02 00:00:00
abstract:OBJECTIVE:The variants of primary progressive aphasia (PPA) are predominantly diagnosed on the basis of specific profiles of language impairments. Deficits in other cognitive domains and their evolution over time are less well documented. This study examined the cognitive profiles of the PPA variants over time and dete...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10382-9
更新日期:2021-01-08 00:00:00