Abstract:
BACKGROUND:Important progress has been made in our understanding of the autoimmune neuromuscular transmission (NMT) disorders; myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS) and neuromyotonia (Isaacs' syndrome). METHODS:To prepare consensus guidelines for the treatment of the autoimmune NMT disorders, references retrieved from MEDLINE, EMBASE and the Cochrane Library were considered and statements prepared and agreed on by disease experts. CONCLUSIONS:Anticholinesterase drugs should be given first in the management of MG, but with some caution in patients with MuSK antibodies (good practice point). Plasma exchange is recommended in severe cases to induce remission and in preparation for surgery (recommendation level B). IvIg and plasma exchange are effective for the treatment of MG exacerbations (recommendation level A). For patients with non-thymomatous MG, thymectomy is recommended as an option to increase the probability of remission or improvement (recommendation level B). Once thymoma is diagnosed, thymectomy is indicated irrespective of MG severity (recommendation level A). Oral corticosteroids are first choice drugs when immunosuppressive drugs are necessary (good practice point). When long-term immunosuppression is necessary, azathioprine is recommended to allow tapering the steroids to the lowest possible dose whilst maintaining azathioprine (recommendation level A). 3,4-Diaminopyridine is recommended as symptomatic treatment and IvIG has a positive short-term effect in LEMS (good practice point). Neuromyotonia patients should be treated with an antiepileptic drug that reduces peripheral nerve hyperexcitability (good practice point). For paraneoplastic LEMS and neuromyotonia optimal treatment of the underlying tumour is essential (good practice point). Immunosuppressive treatment of LEMS and neuromyotonia should be similar to MG (good practice point).
journal_name
Eur J Neuroljournal_title
European journal of neurologyauthors
Skeie GO,Apostolski S,Evoli A,Gilhus NE,Illa I,Harms L,Hilton-Jones D,Melms A,Verschuuren J,Horge HW,European Federation of Neurological Societies.doi
10.1111/j.1468-1331.2010.03019.xsubject
Has Abstractpub_date
2010-07-01 00:00:00pages
893-902issue
7eissn
1351-5101issn
1468-1331pii
ENE3019journal_volume
17pub_type
杂志文章,实务指引abstract:BACKGROUND AND OBJECTIVES:The ataxias are a challenging group of neurological diseases due the aetiological heterogeneity and the complexity of the genetic subtypes. This guideline focuses on the heredodegenerative ataxias. The aim is to provide a peer-reviewed evidence-based guideline for clinical neurologists and oth...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.12341
更新日期:2014-04-01 00:00:00
abstract::Recently, a novel protein-interaction partner of alpha-synuclein, designated synphilin-1, is identified as a constituent of Lewy bodies (LB) in Parkinson's disease (PD) brains. To investigate an involvement of genetic variations of synphilin-1 in development of sporadic PD, a possible single nucleotide polymorphism (S...
journal_title:European journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1046/j.1468-1331.2002.00333.x
更新日期:2002-01-01 00:00:00
abstract::Wegener granulomatosis is a systemic vasculitis that mainly affects the upper and lower respiratory tract and the kidneys. The presence of an ischemic stroke in this disease is very rare. A 40-year-old man, smoker with cavitated lesions in both lungs, and inflammation in the nasal mucosa and vocal cords developed an i...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2007.01726.x
更新日期:2007-06-01 00:00:00
abstract::Studies of the cephalic vascular system have markedly contributed towards an understanding of the mechanisms of migraine pain. Whereas cerebral blood flow changes, and thereby changes in the arterioles, correlate poorly with migraine headache, abnormal regulation of the large cranial arteries seems to play a significa...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.1995.tb00149.x
更新日期:1995-11-01 00:00:00
abstract::The aim of this study was to compare the seizure outcome of two different types of epilepsy surgery, selective amygdalohippocampectomy (AHE) and anterior temporal lobectomy (ATLE) in patients with temporal lobe epilepsy. We included 114 patients who had mesio-temporal lobe epilepsy and hippocampal sclerosis or gliosis...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2006.01565.x
更新日期:2007-01-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Clinical outcomes vary substantially among individuals with large vessel occlusion (LVO) stroke. A small infarct core and large imaging mismatch were found to be associated with good recovery. The aim of this study was to investigate whether those imaging variables would improve individual predic...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.14651
更新日期:2020-11-21 00:00:00
abstract::We report a long-term outcome of motor function in a patient with adult-onset adrenoleukodystrophy after bone marrow transplantation (BMT). Clinically motor function gradually improved and became almost normal in 2 years after BMT. Serial transcranial magnetic stimulation showed gradual improvement of central motor co...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2005.01055.x
更新日期:2005-10-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Patients with chronic focal epilepsy may have atrophy of brain structures important for the generation and maintenance of seizures. However, little research has been conducted in patients with newly diagnosed focal epilepsy (NDfE), despite it being a crucial point in time for understanding the un...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.14565
更新日期:2021-02-01 00:00:00
abstract::Intracranial internal carotid artery (ICA) aneurysms are frequently treated either by microsurgical clipping of the aneurysm neck, by endovascular coiling of the aneurysm sac or by balloon occlusion of the parent vessel. For some broad-based aneurysms that may not be suitable for any of these options, microsurgical wr...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1046/j.1468-1331.2003.00546.x
更新日期:2003-03-01 00:00:00
abstract::Among patients who underwent cerebrospinal fluid (CSF) and magnetic resonance imaging (MRI) examination during a 5-year period, we found 18 patients at the multiple sclerosis center of the University of Rome and clinically definite multiple sclerosis, MRI white matter abnormalities, normal CSF examination, a disease d...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.1994.tb00060.x
更新日期:1994-11-01 00:00:00
abstract:BACKGROUND:Although Wernicke encephalopathy (WE) is a preventable and treatable disease it still often remains undiagnosed during life. OBJECTIVES:To create practical guidelines for diagnosis, management and prevention of the disease. METHODS:We searched MEDLINE, EMBASE, LILACS, Cochrane Library. CONCLUSIONS AND REC...
journal_title:European journal of neurology
pub_type: 杂志文章,实务指引
doi:10.1111/j.1468-1331.2010.03153.x
更新日期:2010-12-01 00:00:00
abstract::Twenty-one consecutive women with the antiphospholipid syndrome were investigated with a full cardiological and neurological examination including a semi-structured interview. They were also systematically studied with magnetic resonance imaging (MRI), transcranial Doppler (TCD), duplex sonography of neck vessels and ...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.1995.tb00119.x
更新日期:1995-07-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Subjective cognitive decline (SCD) is a self-experienced decline in cognitive capacity with normal performance on standardized cognitive tests and has been shown to increase the risk of Alzheimer's disease (AD). SCD could also be related to other conditions such as normal aging, psychiatric, neur...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.14167
更新日期:2020-05-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Better understanding the incidence, predictors and mechanisms of early neurological deterioration (END) following intravenous thrombolysis (IVT) for acute stroke with mild symptoms and isolated internal carotid artery occlusion (iICAo) may inform therapeutic decisions. METHODS:From a multicenter...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.14541
更新日期:2021-02-01 00:00:00
abstract::Multiple sclerosis (MS) is a T-cell-mediated autoimmune demyelinating disease of the central nervous system, associated with an altered cytokine network. We previously assayed peripheral blood T-lymphocyte binding for two prototypic cytokines, tumour necrosis factor-alpha (TNF-alpha) and interleukin-6 (IL-6), and foun...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1046/j.1468-1331.2000.00139.x
更新日期:2000-11-01 00:00:00
abstract:BACKGROUND AND PURPOSE:The 'hot cross bun' sign (HCBS), typically seen in the patients with multiple system atrophy, refers to a cruciform hyperintensity in the pons on T2-weighted MRI. Little is known about its pathological basis and prevalence in other degenerative cerebellar diseases and healthy population. We inves...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2008.02524.x
更新日期:2009-04-01 00:00:00
abstract:BACKGROUND:Impaired cortical inhibiton and maladaptive cortical plasticity are functional hallmarks of sporadic focal dystonias. Whether or not these mechanisms translate to generalized dystonias and whether these features reflect state or trait characteristics are topics of research in hereditary dystonias. METHODS:W...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2010.03054.x
更新日期:2010-07-01 00:00:00
abstract:BACKGROUND AND OBJECTIVE:The HLA-DRB1*15 allele is consistently associated with multiple sclerosis (MS) susceptibility in most studied populations. This study investigated the association between HLA-DRB1 alleles and the presence of oligoclonal immunoglobulin G bands (OCB) in the cerebrospinal fluid (CSF) in a Spanish ...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2011.03379.x
更新日期:2011-10-01 00:00:00
abstract:OBJECTIVE:An immune activation response resembling virus or type I interferon responses has been observed in untreated multiple sclerosis (MS), but its pathogenic significance is uncertain. We studied the relationship between a type I interferon-like response in untreated patients with MS and disease activity. METHODS...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2010.03116.x
更新日期:2011-02-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Anosognosia refers to a deficit of self-awareness or impaired insight for cognitive and behavioral problems. Cognitive anosognosia was explored in de novo patients with Parkinson's disease (PD) and its relationship to cognitive function and neuropsychiatric symptoms was investigated. METHODS:The...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.14188
更新日期:2020-06-01 00:00:00
abstract::There are about 25.7 million stroke survivors worldwide. Ischaemic stroke remains the most common type of stroke. Numerous modifiable risk factors have been identified, including behaviors such as cigarette smoking and sedentary lifestyle and treatable medical comorbidities such as hypertension, hyperlipidemia and atr...
journal_title:European journal of neurology
pub_type: 杂志文章,评审
doi:10.1111/ene.13409
更新日期:2018-01-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Headache has been associated with various lifestyle and psychosocial factors, one of which is smoking. The aim of the present study was to investigate whether the association between smoking intensity and headache is likely to be causal. METHOD:A total of 58 316 participants from the Nord-Trønde...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.13675
更新日期:2018-09-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Previous antiplatelet therapy (APT) in cardiovascular prevention is common in patients with first-ever stroke. We aimed to evaluate the prognostic value of APT on early outcome in stroke patients. METHODS:All first-ever strokes from 1985 to 2011 were identified from the population-based Stroke R...
journal_title:European journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1111/ene.12060
更新日期:2013-06-01 00:00:00
abstract::The authors report results of a retrospective pilot study showing a strong association between patients with stroke/TIA and the presence of circulating IgG and IgA antibodies to Chlamydia pneumoniae. These results support the hypothesis that chronic active or persistent infection may play a role in the mechanism of th...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1046/j.1468-1331.2003.00605.x
更新日期:2003-07-01 00:00:00
abstract:BACKGROUND:Multiple mitochondrial DNA (mtDNA) deletions usually have a mendelian inheritance secondary to mutation in nuclear genes. One of these is the Twinkle gene whose mutation is responsible for autosomal dominant progressive external ophthalmoplegia (PEO). The number of reported cases with mainly myopathic sympto...
journal_title:European journal of neurology
pub_type: 杂志文章,评审
doi:10.1111/j.1468-1331.2010.03171.x
更新日期:2011-03-01 00:00:00
abstract::In this paper we report about the Danish Year of the Brain 1997 because it may serve as a model for other countries. It was possible in Denmark to organize a very large scale effort which has been well received by the general public and which has significantly improved the image of the neurological disciplines. The re...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1046/j.1468-1331.1998.560619.x
更新日期:1998-11-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Growth hormone (GH)/insulin-like growth factor 1 (IGF-1) axis abnormalities in multiple sclerosis (MS) suggest their role in its pathogenesis. Interferon β (IFN-β) efficacy could be mediated also by an increase of IGF-1 levels. A 2-year longitudinal study was performed to estimate the prevalence ...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.13207
更新日期:2017-02-01 00:00:00
abstract:OBJECTIVE:We aimed to investigate the prevalence of TOR1A, GNAL and THAP1 variants as the cause of dystonia in a cohort of Spanish patients with isolated dystonia and in the literature. METHODS:A population of 2028 subjects (including 1053 patients with different subtypes of isolated dystonia and 975 healthy controls)...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.14638
更新日期:2020-11-11 00:00:00
abstract:BACKGROUND AND PURPOSE:Improved biomarkers are needed to facilitate clinical decision-making and as surrogate endpoints in clinical trials in multiple sclerosis (MS). We assessed whether neurodegenerative and neuroinflammatory markers in cerebrospinal fluid (CSF) at initial sampling could predict disease activity durin...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/ene.13274
更新日期:2017-05-01 00:00:00
abstract:BACKGROUND AND PURPOSE:Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) is a distinct epileptic syndrome with a broad range of severity even amongst affected members of the same pedigree, and the level of pharmacoresistance may reach 30%, close to that seen in sporadic focal epilepsies. METHODS:To investiga...
journal_title:European journal of neurology
pub_type: 杂志文章
doi:10.1111/j.1468-1331.2012.03839.x
更新日期:2013-03-01 00:00:00