Abstract:
:Six children with clinical and pathological features of Batten-Vogt syndrome showed autofluorescent material in bone marrow cells and in 5 of them sea-blue histiocytes were seen. It is suggested that bone marrow examination for sea-blue histiocytes and autofluorescenceis a useful screening test for ceroid-lipofuscinosis.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Gadoth N,O'Croinin P,Butler IJdoi
10.1016/0022-510x(75)90140-9subject
Has Abstractpub_date
1975-06-01 00:00:00pages
197-203issue
2eissn
0022-510Xissn
1878-5883pii
0022-510X(75)90140-9journal_volume
25pub_type
杂志文章abstract:OBJECTIVES:Though much information exists about the diagnosis, treatment, and epidemiology of myasthenia gravis (MG), a comprehensive data registry and biorepository is critical to better understand disease mechanisms, treatment outcomes, and the impact of treatment strategies. We aimed to design and implement the "Exp...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2020.116830
更新日期:2020-07-15 00:00:00
abstract::Autoantibodies to myelin basic protein (anti-MBP) can be detected in the cerebrospinal fluid (CSF) of MS patients using a solid phase radioimmunoassay. Acute relapses of MS are characterized by an elevated, above unity, free (F) to bound (B) anti-MBP ratio; while in contrast, patients with chronic progressing disease ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(88)90216-x
更新日期:1988-12-01 00:00:00
abstract::The authors have shown in a recent paper that survival with amyotrophic lateral sclerosis (ALS) can be increased by the use of non-invasive methods of assisted ventilation (Bipap). However, the progression of muscle weakness was not affected and the quality of life was not positively enhanced. In ALS, reduced physical...
journal_title:Journal of the neurological sciences
pub_type: 临床试验,杂志文章
doi:10.1016/s0022-510x(99)00218-x
更新日期:1999-10-31 00:00:00
abstract::Cannabis use is on the rise both as medical treatment and recreational use. There is evidence that cannabis can cause hallucinations and psychosis especially with heavy and prolonged use. Parkinson's disease (PD) carries an increased risk for development of hallucinations and psychosis. It is possible that cannabis ma...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2020.117206
更新日期:2020-12-15 00:00:00
abstract::Neuronal damage seems to be a major source of disability in multiple sclerosis (MS) patients and at present magnetic resonance imaging (MRI) is a sensitive method to evaluate lesion and disease activity. We studied the potential correlation between changes in MS patients' disability after relapse, the degree of T1 les...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(01)00650-5
更新日期:2002-01-15 00:00:00
abstract::A forty seven year old woman with previously undetected goitre, developed thyroid storm in the immediate postoperative period. Forty eight hours after undergoing nephrolithotomy for renal calculus, she was noted to have flaccid areflexic quadriparesis, most marked in the lower limbs. Electrophysiologic studies reveale...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(97)00313-4
更新日期:1998-03-05 00:00:00
abstract::Migraine is an independent risk factor for ischemic stroke, mainly in the subpopulation of women with migraine with aura who are younger than 45 years, particularly those that use estrogen containing oral contraceptives. Migraine however should be considered a benign condition as the absolute increase of stroke risk i...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2009.02.333
更新日期:2009-08-15 00:00:00
abstract::Two transgenic strains of mutant mice lacking hypoxanthine-guanidine phosphoribosyltransferase (HPRT) activity were examined behaviorally and neurochemically for phenotypic similarity to the human Lesch-Nyhan syndrome. In this syndrome, male children markedly deficient in the enzyme HPRT develop self-mutilation and se...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(88)90099-8
更新日期:1988-09-01 00:00:00
abstract:INTRODUCTION:'No evidence of disease activity' (NEDA) is increasingly used as a treatment target with disease-modifying drugs for relapsing multiple sclerosis. METHODS:This post-hoc analysis of the randomised EVIDENCE trial compared interferon beta-1a injected subcutaneously three times weekly (IFN β-1a SC tiw) with i...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,随机对照试验
doi:10.1016/j.jns.2017.05.052
更新日期:2017-08-15 00:00:00
abstract:BACKGROUND:Antiplatelet therapies for secondary prevention of ischemic stroke or transient ischemic attack (TIA) is a highly active research topic with five critical drugs obtained by visual analysis. We aimed to compare and rank multiple antiplatelet therapies using a network meta-analysis. METHODS:Relevant medical d...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,meta分析
doi:10.1016/j.jns.2019.02.037
更新日期:2019-04-15 00:00:00
abstract::Stroke is a leading cause of global morbidity and mortality. Sub-Saharan Africa (SSA), where an unprecedented rise in stroke burden is currently raging, has the highest age-standardized stroke incidence, stroke prevalence, and stroke mortality rates. This is in sharp contrast to the relative decline in stroke incidenc...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2019.03.026
更新日期:2019-05-15 00:00:00
abstract::The reduction in the effectiveness of the blood-brain and blood-spinal cord barriers, previously seen in rats at the height of the acute episode of experimental allergic encephalomyelitis, has now been measured at various stages in the development of the disease up to 60 days after inoculation with guinea pig spinal c...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(83)90147-8
更新日期:1983-08-01 00:00:00
abstract::PHARC (Polyneuropathy, Hearing loss, Ataxia, Retinitis pigmentosa and Cataracts) (MIM# 612674) is an autosomal recessive neurodegenerative disease caused by mutations in the ABHD12 gene. We evaluated two Spanish siblings affected with pes cavus, sensorimotor neuropathy, hearing loss, retinitis pigmentosa and juvenile ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2018.02.021
更新日期:2018-04-15 00:00:00
abstract::Aceruloplasminemia is a disorder of iron metabolism caused by mutations in the ceruloplasmin gene. It is characterized by progressive neurodegeneration of the retina, basal ganglia, dentate nucleus and cerebral cortex in association with iron accumulation in these tissues. Enzyme activities in the mitochondrial respir...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(00)00452-4
更新日期:2000-12-15 00:00:00
abstract:BACKGROUND:Microglial activation is thought to be a key pathophysiological mechanism underlying disease activity in all forms of MS. Hydroxychloroquine (HCQ) is an antimalarial drug with immunomodulatory properties that is widely used in the treatment of rheumatological diseases. In this series of experiments, we explo...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2015.08.1525
更新日期:2015-11-15 00:00:00
abstract::A method for preparative separation of cerebrospinal fluid specific proteins by affinity chromatography and isoelectric focusing is described. It has been tested on CSF from a group of human patients suffering from TIA. The advantage of the method is the production of a protein pattern easy to survey by eye. The sensi...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(80)90132-x
更新日期:1980-01-01 00:00:00
abstract:OBJECTIVE:Despite the wide utilization of mechanical thrombectomy (MT) for acute ischemic stroke treatment, little is known about the incidence of early post-thrombectomy seizures, its predictors, and association with long-term outcome. METHODS:Using a prospective registry of mechanical thrombectomy in ischemic stroke...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2018.11.033
更新日期:2019-01-15 00:00:00
abstract:INTRODUCTION:Mirror movements (MM) have been previously reported in patients with Parkinson's disease (PD). Despite being potentially relevant in PD, MM as a neurological sign have remained less recognized. In this study we critically evaluated the characteristic features of MM and their attributes among a cohort of PD...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2016.05.026
更新日期:2016-07-15 00:00:00
abstract::Perception of verticality is essential for postural control. On the other hand, postural instability is one of the cardinal features in Parkinson's disease (PD). Thus, the objective of this study was to evaluate the vertical perception using the subjective visual vertical test in PD patients with different degrees of ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.07.057
更新日期:2014-11-15 00:00:00
abstract:BACKGROUND:Headache burden is not adequately explored in Africa. Here, we measured weighted migraine prevalence from community-based studies in Africa. METHODS:PubMed search was employed using terms 'headache in Africa' AND/OR 'migraine in Africa' for published literature from 1970 until January 31, 2014. PRISMA was a...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,meta分析,评审
doi:10.1016/j.jns.2014.04.019
更新日期:2014-07-15 00:00:00
abstract::The production of interleukin-1 (IL-1) and interleukin-2 (IL-2) by peripheral blood mononuclear cells (PBM) was assessed in multiple sclerosis (MS) patients in relapse, chronic progressive MS patients, patients with other neurological diseases (OND) and healthy subjects. Production was defined as the level of IL-1 and...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(88)90036-6
更新日期:1988-05-01 00:00:00
abstract::Healthy brain tissue pulsates with the cardiac cycle, but whether brain tissue pulsations (BTPs) are impaired by tissue ischemia due to ischemic stroke is currently unclear. This study is the first to explore the clinical potential of measuring BTPs using ultrasound in acute ischemic stroke patients. BTPs were measure...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2020.117164
更新日期:2020-12-15 00:00:00
abstract::Epidemiologists have found a decreased risk of developing Alzheimer's disease (AD) in people taking statins (cholesterol biosynthesis inhibitors). We have reported previously that, in cell culture, lovastatin decreases the output of beta-amyloid, a peptide that is toxic to neurones, and is reputably the prime cause of...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2004.11.044
更新日期:2005-03-15 00:00:00
abstract::Mutations in COL4A1, encoding one of the six collagen type IV proteins, cover a wide spectrum of autosomal dominant overlapping phenotypes including porencephaly, small-vessel disease and hemorrhagic stroke, leukoencephalopathy, hereditary angiopathy with nephropathy, aneurysms and muscle cramp (HANAC) syndrome, and W...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2015.03.042
更新日期:2015-05-15 00:00:00
abstract::In our study, mitochondria were isolated from skeletal muscle in 2-, 3-, 4-, 6-, 8-, and 12-week-old normal (C57BL6j dy/+), and 4-, 8-, and 12-week-old dystrophic (C57BL6j dy/dy) mice and in normal subjects and patients with Duchenne or Becker muscular dystrophy. A deficit was observed in a calcium-specific mitochondr...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(89)90116-0
更新日期:1989-05-01 00:00:00
abstract::Ischemic brain lesions were induced in adult Wistar and Fischer rats by temporary occlusion of the bilateral common carotid artery. The severity of ischemic lesions depended on the duration of carotid occlusion. While 2 h occlusion resulted in 15 deaths among 40 rats and the development of ischemic lesions in 16 of 25...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(89)90098-1
更新日期:1989-04-01 00:00:00
abstract::This study explores the severity of depression and anxiety experienced by patients suffering from motor neurone disease and seeks to link this to illness progression, recent life events and personality factors. The 18 patients who participated in the study were assessed for the presence of psychiatric symptoms using t...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(98)00203-2
更新日期:1998-10-01 00:00:00
abstract::Previously, we demonstrated that neuronal nitric oxide synthase (nNOS) is activated and promotes muscle atrophy in skeletal muscle during tail suspension, a model of unloading and denervation. Here, we examined patients with amyotrophic lateral sclerosis (ALS) and mutant (H46R) SOD1 transgenic (Tg) mice model using im...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2010.03.022
更新日期:2010-07-15 00:00:00
abstract:BACKGROUND:A recent pilot study suggested spasticity improvement during glatiramer acetate (GA) treatment in multiple sclerosis (MS) patients who previously received interferon-β (IFN-β). OBJECTIVE:To evaluate changes in spasticity in MS patients switching from IFN-β to GA. METHODS:Observational, multicentre study in...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,多中心研究
doi:10.1016/j.jns.2011.11.010
更新日期:2012-04-15 00:00:00
abstract:BACKGROUND AND PURPOSE:Dementia after first clinical stroke frequently has been found, but the clinical and radiological correlates have not been fully detailed. We examined magnetic resonance imaging (MRI) correlates of dementia in a large well-defined series of patients with first clinical ischemic stroke. METHODS:D...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(00)00437-8
更新日期:2000-12-01 00:00:00