Abstract:
:Using a novel trial design, we prospectively examined the effect of intravenous immunoglobulin in seven patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in a double-blind, placebo-controlled cross-over study. We suggest that the commonly used manual muscle testing and Rankin scale are not sufficiently sensitive to measure changes in CIDP and should not be used as isolated outcome measures. We propose a timed 10-m walk, the Nine-Hole Peg Test, the Hammersmith Motor Ability Score, and myometry as alternative measures which are valid, reliable and sensitive. Our trial design permitted the measurement of a treatment response in three responders despite different patterns of disability typical of the broad clinical picture seen in CIDP.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Thompson N,Choudhary P,Hughes RA,Quinlivan RMdoi
10.1007/BF00868527subject
Has Abstractpub_date
1996-03-01 00:00:00pages
280-5issue
3eissn
0340-5354issn
1432-1459journal_volume
243pub_type
临床试验,杂志文章,随机对照试验abstract::In recent years, it has been widely recognised that modern doctors are not receiving the training they need to provide appropriate and effective palliative care. Deficiencies in care have been reported in several formal studies and, anecdotally, in many professional and popular lay publications. Doctors themselves hav...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/pl00007722
更新日期:1997-10-01 00:00:00
abstract::The influence of portal-systemic shunting on maximal motor nerve conduction velocity (MCV) was analyzed in rats with portacaval shunts. At 3 and 8 weeks after the shunting there was a marked but transient fall in MCV. This was not prevented by colectomy. At 16 weeks the MCV had almost normalized, despite sustained por...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313181
更新日期:1980-01-01 00:00:00
abstract::Leukoencephalopathy with brainstem and spinal cord involvement and elevated lactate (LBSL) is a rare, autosomal recessive disorder caused by mutations in the gene encoding a mitochondrial aspartyl-tRNA synthetase, DARS2. The disease is characterized by progressive spastic ataxia and magnetic resonance imaging (MRI) sh...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6176-9
更新日期:2012-02-01 00:00:00
abstract:OBJECTIVE:To determine the response to treatment and the long-term outcome of patients with the antisynthetase syndrome associated with anti-Jo-1-antibodies. PATIENTS AND METHODS:A total of 12 patients with histologically proven myositis and anti-Jo-1-autoantibodies were evaluated over a mean follow-up period of 66.4 ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-004-0449-5
更新日期:2004-07-01 00:00:00
abstract:BACKGROUNDS:Wake-Up Stroke (WUS) patients are generally excluded from thrombolytic therapy (rTPA) due to the unknown time of stroke onset. This study aimed to investigate the effects of rTPA in WUS patients during every day clinical scenarios, by measuring ischemic lesion volume and functional outcomes compared to non-...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09603-7
更新日期:2020-03-01 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::Untreated sleep disorders may contribute to secondary causes of uncontrolled hypertension, cardiovascular disease (CVD), and stroke. Restless legs syndrome, or Willis-Ekbom Disease (RLS/WED), is a common sensorimotor disorder with a circadian rhythmicity defined by an uncontrollable urge to move the legs that worsens ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-013-7065-1
更新日期:2014-06-01 00:00:00
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...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-015-7963-5
更新日期:2016-04-01 00:00:00
abstract::Secondary worsening of stroke symptoms is described in 13-37% of stroke patients, but the exact mechanism of this phenomenon remains unclear. The aim of this study is to find prognostic variables in correlation to secondary worsening of stroke symptoms in patients with lacunar or cortical strokes. In this prospective ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-010-5577-5
更新日期:2010-09-01 00:00:00
abstract::Four patients with aphasia due to small circumscribed thalamic lesions are presented. A review of the literature on thalamic aphasia revealed 16 similar cases. While the general consensus that only left-sided thalmic lesions are associated with aphasia is confirmed, analysis of the sites of the thalamic infarctions an...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/BF00314212
更新日期:1989-01-01 00:00:00
abstract::Quality of Life (QoL) is the preferred outcome in non-pharmacological trials, but there is little UK population evidence of QoL in epilepsy. In advance of evaluating an epilepsy self-management course we aimed to describe, among UK participants, what clinical and psycho-social characteristics are associated with QoL. ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8512-1
更新日期:2017-06-01 00:00:00
abstract::A 73-year-old woman presented with multifocal cerebral dysfunction of 1 month's duration. Cranial CT scanning revealed unusual widespread abnormalities. Brain biopsy showed amyloid angiopathy affecting vessels in the meninges and cerebral cortex, with associated granulomatous angiitis. There was no clinical evidence o...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314491
更新日期:1988-09-01 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::Urate is a natural antioxidant, and high serum urate levels could be protective against the development of amyotrophic lateral sclerosis (ALS). To determine if serum urate concentrations were lower in ALS patients than in healthy controls, we compared serum urate levels in 132 ALS patients and 337 age/sex-matched cont...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-010-5735-9
更新日期:2011-02-01 00:00:00
abstract:BACKGROUND:Progression of parkinsonian motor impairment is usually rapid and relentless in multiple system atrophy (MSA). However, it may also be subject to considerable variation. Prospective natural history studies using validated rating scales are required to accurately determine the progression of parkinsonism in M...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0617-2
更新日期:2005-01-01 00:00:00
abstract::Sera from 23 patients with acute Guillain Barré syndrome (GBS), 15 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and from 40 age-matched blood donors were analysed for antibodies to acidic glycosphingolipids from human brain and peripheral nerve. Antibodies to ganglioside LM1, the majo...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00315684
更新日期:1991-04-01 00:00:00
abstract:OBJECTIVE:To determine the prevalence and risk factors for paroxysmal atrial fibrillation (PAF) diagnosis in non- cryptogenic ischemic stroke (CIS) patients. METHODS:In this pilot-prospective cohort study of non-CIS patients from September 2014 to September 2017, 53 patients were enrolled. 51/53 patients were implante...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1007/s00415-018-8982-9
更新日期:2018-10-01 00:00:00
abstract:BACKGROUND:Late-onset Pompe disease (LOPD) is a rare, metabolic disease primarily affecting the musculoskeletal and respiratory systems. Forced vital capacity (FVC) is commonly used to measure pulmonary function; however, associations between FVC and other LOPD outcomes remain unclear. METHODS:A systematic literature ...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析
doi:10.1007/s00415-019-09401-1
更新日期:2019-09-01 00:00:00
abstract:BACKGROUND:An upper motor neuron (UMN) lesion in amyotrophic lateral sclerosis (ALS) is often difficult to identify because clinical signs may be discrete or masked by severe simultaneous LMN lesions. We compared the diagnostic sensitivity of transcranial magnetic stimulation (TMS) to cranial muscles and limb muscles i...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1007/s004150170068
更新日期:2001-10-01 00:00:00
abstract::Two previously healthy women are described who in their late thirties suffered transient strokelike episodes, consisting of initial headache and vomiting, with various subsequent neurological signs that were only partially reversible. Investigations revealed elevated serum creatine kinase, lactic acidosis, hypertrigly...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00818708
更新日期:1993-01-01 00:00:00
abstract::The association of pathology and neurological deficit with quality of life (QoL) in multiple sclerosis (MS) is not fully understood. In this study, magnetic resonance imaging (MRI) measures of pathology--T1 and T2 lesion volume and ratio; active T2 lesion number; global and regional brain volume and atrophy; magnetiza...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6151-5
更新日期:2012-02-01 00:00:00
abstract::Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are often clinically confused with each other. Moreover, the discrepancy between clinical and pathological diagnoses of CBD and PSP are still controversial. We report here two atypical cases of PSP and CBD. A 73-year old woman was admitted with r...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0718-y
更新日期:2005-06-01 00:00:00
abstract::KIF1A gene encodes the kinesin 1a protein, an axonal motor protein working in cargo transport along neurites. Variants in KIF1A were identified in different forms of neurodegenerative diseases with dominant and recessive inheritance. Homozygous recessive mutations were found in the hereditary sensory and autonomic neu...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-015-7899-9
更新日期:2015-12-01 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::We examined the influence of alleles at the HLA loci, previously found to be associated with multiple sclerosis (MS) in Sardinia, on the clinical course of the disease in 835 relapsing (R) and 100 primary progressive (PP) patients. Multivariate analysis was carried out on predisposing 0301 or non-associated DPB1 allel...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0957-y
更新日期:2006-02-01 00:00:00
abstract:OBJECTIVE:Children with neuromuscular disorders have been assumed to be a particularly vulnerable population since the beginning of COVID-19. Although this is a plausible hypothesis, there is no evidence that complications or mortality rates in neuromuscular patients are higher than in the general population. The aim o...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10339-y
更新日期:2021-01-02 00:00:00
abstract::Amyotrophic lateral sclerosis (ALS), which is the most serious form of degenerative motor neuron disease in adults, is characterized by upper and lower motor neuron degeneration, skeletal muscle atrophy, paralysis, and death. Some patients with respiratory-dependent ALS die of sudden cardiac arrest or anoxic encephalo...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-7005-0
更新日期:2013-09-01 00:00:00
abstract::A method for the preparation of a potent and specific peroxidase-labelled antihuman IgG antibody is described. CSF cells from patients with various neurological diseases were stained. Only in cases of inflammatory diseases was IgG detectable. The stained cells were so-called lymphoid cells. As the demonstrable IgG can...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313346
更新日期:1980-01-01 00:00:00
abstract:BACKGROUND:Body weight reduction after disease onset is an independent predictor of survival in amyotrophic lateral sclerosis (ALS), but significance of weight variation after diagnosis remains to be established. OBJECTIVE:To investigate weight variation after diagnosis and its prognostic significance in patients with...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09276-2
更新日期:2019-06-01 00:00:00
abstract::We review the neuronal antibodies described in CNS disorders in order to clarify their diagnostic value, emphasize potentials pitfalls and limitations in the diagnosis of paraneoplastic neurological syndromes (PNS), and examine the current evidence for a possible pathogenic role. We propose to classify the neuronal an...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-009-5431-9
更新日期:2010-04-01 00:00:00