Abstract:
:Based on the development of a transgenic animal model, an increasing number of experimental strategies have revealed the potential to modify the selective degeneration of motor neurons, a feature unique to motor neuron diseases such as amyotrophic lateral sclerosis (ALS). The translation of this success into therapeutic effects in human diseases is a challenge of the future. For this purpose, tools must be developed which serve as diagnostic and surrogate markers for diagnosis and disease progression. Currently, to understand the pathogenesis of the spinal cord disease, the focus remains on more traditional electrodiagnostic techniques. For the characterization of the involvement of brain structures, imaging techniques are increasingly explored. This review focuses on the use of imaging techniques as surrogate markers for the involvement of the brain in motor neuron disorders, but also tries to point out that the final goal will be the development of cheap biochemical markers for the screening of populations at risk.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Karitzky J,Ludolph ACdoi
10.1016/s0022-510x(01)00628-1subject
Has Abstractpub_date
2001-10-15 00:00:00pages
35-41issue
1-2eissn
0022-510Xissn
1878-5883pii
S0022510X01006281journal_volume
191pub_type
杂志文章,评审abstract:BACKGROUND:In the diagnostic work-up of young stroke patients angiography is still considered as mandatory to identify otherwise undetected causes of stroke. We hypothesized that stroke cause is more heterogeneous among patients with a territorial infarct, whereas in lacunar stroke it is generally small vessel disease....
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2004.04.007
更新日期:2004-07-15 00:00:00
abstract::Cultured post-fused human skeletal muscle monolayers exposed to WSN influenza A virus were analyzed by scanning and transmission electron microscopy. At 12-14h post-inoculation (p.i.), affected mononuclear cells retracted from the cell surface, but remained anchored to the substrate by taut filar processes. Retraction...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(78)90162-4
更新日期:1978-03-01 00:00:00
abstract::Syringomyelia is frequently accompanied by an extramedullary lesion at the foramen magnum, particularly a Chiari I malformation. Although syringomyelia associated with foramen magnum obstruction has characteristic clinical, radiological, and neuropathological features, its pathogenesis remains unclear. Currently preva...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2004.01.014
更新日期:2004-05-15 00:00:00
abstract::Axonal degeneration plays an important role in the accumulation of disability in patients with multiple sclerosis (MS). Pathological studies have demonstrated axonal damage, particularly in areas of acute inflammation and demyelination, and in chronic lesions. Axonal loss and its progression, which is associated with ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/s0022-510x(99)00178-1
更新日期:1999-11-15 00:00:00
abstract::Altered functional connectivity has been associated with the influence of epileptic activity. Abnormalities in connectivity, particularly in dorsal attention (DAN), salience (SN) and default mode (DMN) networks, might contribute to the loss of consciousness during seizures and cognitive deficits in patients with child...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2015.04.054
更新日期:2015-07-15 00:00:00
abstract::We have monitored the cell surface phenotypic changes occurring in T, B and NK cells of chronic progressive multiple sclerosis (MS) patients after total lymphoid irradiation (TLI) plus low-dose prednisone (TLI-LDP) therapy in comparison to sham TLI-LDP. TLI-LDP resulted in a marked reduction in the relative and absolu...
journal_title:Journal of the neurological sciences
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1016/s0022-510x(97)00156-1
更新日期:1997-11-25 00:00:00
abstract::We present a patient with Parkinson's disease whose bilateral tremor transiently resolved after a unilateral left ventrolateral thalamotomy. The transient resolution of the bilateral tremor was associated with a focal thalamic lesion and a second lesion in the corpus callosum. The mechanism of this phenomenon may be r...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(94)00236-h
更新日期:1995-03-01 00:00:00
abstract:BACKGROUND:Pseudoxanthoma elasticum (PXE) is a monogenetic disease with progressive calcification of arteries and potential risk of stroke. To gain insights in the cerebral involvement in PXE, we evaluated prevalence and determinants of cerebral disease in our PXE cohort and performed a systematic review of literature....
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2016.12.053
更新日期:2017-02-15 00:00:00
abstract::Sleep disturbance is one of the commonly reported non-motor symptoms in patients with Parkinson's disease (PD) as well as in Parkinson plus disorders such as multiple system atrophy (MSA), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP), and corticobasal syndrome (CBS). Although there is a wealth...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2020.116891
更新日期:2020-08-15 00:00:00
abstract::Frontal gaits (FG) and parkinsonian gaits (PG) are common neurological gait abnormalities in older adults. It may be difficult to distinguish these gaits as they share common clinical characteristics such as unsteadiness, slowing, and shuffling. Of 488 community-residing subjects in an aging study, 11 were diagnosed w...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2006.05.035
更新日期:2006-10-25 00:00:00
abstract::Experience with systemic or selective local administration of thrombolytic agents in pediatric ischemic stroke is limited to sporadic case reports, since patients of age less than 18 years were systematically excluded from randomised controlled trials. We report a case of childhood IS attributable to the terminal inte...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2008.07.018
更新日期:2008-12-15 00:00:00
abstract:BACKGROUND:The association between the angiotensin-converting enzyme insertion/deletion (ACE I/D) polymorphism and risk of ischemic stroke (IS) remains controversial and ambiguous. To clarify this association, a large meta-analysis was performed. METHODS:Electronic databases in both English and Chinese were used to id...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,meta分析,评审
doi:10.1016/j.jns.2014.07.023
更新日期:2014-10-15 00:00:00
abstract::Anti-Yo antibodies are present in the sera and cerebrospinal fluid of some patients with paraneoplastic cerebellar degeneration (PCD), but there is no evidence that the presence of anti-Yo antibodies causes the Purkinje cell loss seen in PCD patients. We examined the level of cytotoxic T lymphocyte (CTL) activity agai...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(99)00163-x
更新日期:1999-09-15 00:00:00
abstract::Benign familial neonatal convulsions (BFNC) are a rare autosomal dominant inherited epilepsy syndrome. Two voltage-gated potassium channel genes, KCNQ2 on chromosome 20q13.3 and KCNQ3 on chromosome 8q24, have been identified as the genes responsible for benign familial neonatal convulsions. By linkage analysis and mut...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2004.03.001
更新日期:2004-06-15 00:00:00
abstract:OBJECTIVE:Despite the strong evidence for the efficacy of surgery to treat temporal lobe epilepsy, the number of such surgeries has plateaued in western countries. This study examined trends in the number of epilepsy cases diagnosed, number of surgeries performed, and certain characteristics of surgery patients in west...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2019.06.026
更新日期:2019-08-15 00:00:00
abstract::Management of myasthenic crisis (MC) requires admission of the patient into a neurological intensive care unit and timely institution of an efficient and safe treatment. Despite the growing clinical experience with disease modifying immunotherapy there is no clinical consensus regarding the use of plasma exchange or h...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2007.04.045
更新日期:2007-10-15 00:00:00
abstract:BACKGROUND AND PURPOSE:Post-conditioning with volatile anesthetics can create ischemic tolerance against cerebral ischemia-reperfusion injury. The present study was designed to determine whether delayed exposure to sevoflurane could induce ischemic tolerance and if this effect was dependent on increasing phosphorylated...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.12.011
更新日期:2015-01-15 00:00:00
abstract:OBJECTIVE:Multiple sclerosis (MS) is a disabling idiopathic inflammatory disorder with evidence of immune dysfunction. Current therapies for MS include preparations of beta-interferon (beta IFN). We studied the gene expression patterns in peripheral blood mononuclear cells from relapsing-remitting MS patients undergoin...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2007.02.034
更新日期:2007-07-15 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::To compare muscle fiber loss in young and old mdx mice, we have blocked regeneration in one leg with a high dose (18 Gy) of X-rays administered at two ages; 16 days, just prior to the onset of the myopathy, and 15 weeks, when the myopathy is considered to be quiescent. Mice were examined 4 days after irradiation to lo...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(99)00061-1
更新日期:1999-04-01 00:00:00
abstract:BACKGROUND:Data on the determinants and outcome of ischemic stroke (IS) from the Arabian Gulf countries (AGC) are still scanty. The aim of this study was to characterize IS in six large stroke centers on the Arabian peninsula. METHODS:IS subtypes were evaluated in four AGC from January 2006 to December 2007 in a large...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2010.08.023
更新日期:2011-01-15 00:00:00
abstract:BACKGROUND:Pontine infarcts can be classified into four regions based on the vascular anatomy: anteromedial, anterolateral, lateral and posterior. The purpose of this study was to determine if different etiological mechanisms are responsible for these four types of pontine infarcts. METHODS:We studied consecutive pati...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.01.039
更新日期:2014-04-15 00:00:00
abstract::Cerebral gliomas may cause a reduction of glucose metabolism in the cerebellum contralateral to the tumor side (crossed cerebellar diaschisis, CCD). We investigated whether CCD is related to tumor localization, histological grade, size and tumor biochemistry. Cerebellar glucose metabolism was measured in 44 glioma pat...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(98)00019-7
更新日期:1998-01-01 00:00:00
abstract::There is significant evidence that the pathogenesis of several neurodegenerative diseases, including Parkinson's disease, Alzheimer's disease, Friedreich's ataxia (FRDA), multiple sclerosis and amyotrophic lateral sclerosis, may involve the generation of reactive oxygen species (ROS) and/or reactive nitrogen species (...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2005.03.012
更新日期:2005-06-15 00:00:00
abstract:IMPORTANCE:Workers exposed to aerosolized brain in a swine-processing plant developed immune-mediated polyradiculoneuropathy (IP) possibly triggered by an immune response. OBJECTIVE:Immunohistochemistry results were correlated with electrophysiological variables to examine the immunopathogenesis of this disorder. DES...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2017.12.005
更新日期:2018-02-15 00:00:00
abstract::The understanding of the genetic basis of neurological disorders has grown rapidly in the last two decades. Despite the genomic heterogeneity within African populations, large-scale candidate gene or linkage and exome studies are lacking. However, current knowledge on neurogenetics in African populations is limited an...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2016.05.006
更新日期:2016-07-15 00:00:00
abstract::A 75-year-old woman developed painful legs and moving toes syndrome (PLMT) 16 months after the onset of herpes zoster (HZ) myelitis. Although the scattered extensive lesions due to HZ myelitis were observed to be eccentric near the posterior horn on MRI, these changes had disappeared upon the development of PLMT. Comb...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2004.01.011
更新日期:2004-04-15 00:00:00
abstract::Duchenne muscular dystrophy (DMD) is an X-linked recessive disease caused by mutations in the dystrophin gene and is characterized by muscle degeneration and death. DMD affects males; females being asymptomatic carriers of mutations. However, some of them manifest symptoms due to a translocation between X chromosome a...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.09.036
更新日期:2014-01-15 00:00:00
abstract::Thalamic damage is associated with a variety of neuropsychological dysfunctions, as well as strategic infarct dementia. However, only a limited number of reports in the medical literature have discussed the correlation between the clinical findings and the specific functional changes observed on images. We investigate...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2007.09.016
更新日期:2008-03-15 00:00:00
abstract::Pelizaeus-Merzbacher-like disease (PMLD) is an autosomal recessive hypomyelinating disorder of the central nervous system characterized by nystagmus, motor developmental delay, ataxia, and progressive spasticity. The gap junction protein gamma-2 gene (GJC2), encoding the gap junction protein connexin 47, is one of the...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.04.017
更新日期:2013-07-15 00:00:00