Abstract:
:In five patients with cerebral manifestation of Wilson's disease, copper was measured in CSF, serum, urine and liver, and ceruloplasmin was determined in CSF and serum. CSF copper was found to be elevated in all cases, especially in the four examined before therapy. Two patients were followed up for a period of 3 years, while undergoing therapy with chelating substances. In case 1, the data and the clinical course are presented in detail: prior to therapy, the daily urinary copper excretion had been elevated, and this increased during the initial treatment stages. The serum copper concentration, which was already low, decreased quickly the during the initial stages of therapy, and remained at a low level during further treatment. In contrast to its level in serum, the copper level in the CSF was up to 3-fold the normal range and fell only very slowly as clinical symptoms improved. These findings suggest transport of copper from the CNS to the CSF. The copper concentration in CSF appears to be a valuable parameter for diagnosis and monitoring therapy in patients with cerebral manifestation of Wilson's disease.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Weisner B,Hartard C,Dieu Cdoi
10.1016/0022-510x(87)90275-9subject
Has Abstractpub_date
1987-06-01 00:00:00pages
229-37issue
1-2eissn
0022-510Xissn
1878-5883pii
0022-510X(87)90275-9journal_volume
79pub_type
杂志文章abstract::We describe a case of a 50-year-old man with bilateral shoulder girdle weakness caused by anterior subdural hygroma secondary to a previous spontaneous CSF leak. The CSF leak occurred and resolved 16 years prior to presenting with a 6-year progressive painless, asymmetric proximal muscle weakness involving both upper ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2011.02.029
更新日期:2011-06-15 00:00:00
abstract:OBJECTIVE:Aneurysm growth is a risk factor for rupture, however the detailed mechanism remains unclear. The present study was performed to identify whether hemodynamic insult could prompt small unruptured aneurysms to grow. METHODS:Six pairs of unruptured small (<5 mm) cerebral aneurysms from patients followed with lo...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2020.116798
更新日期:2020-06-15 00:00:00
abstract::We have analyzed the serum from patients with refractory epilepsy for the presence of autoreactive antibodies to AMPA glutamate receptor subunits. The presence and the level of autoantibodies were assessed using immunoblot and ELISA with synthetic peptides specific for subregions of AMPA glutamate receptor subunits. P...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(97)00150-0
更新日期:1997-11-06 00:00:00
abstract::Spinal cord blood flow (SCBF) has been measured in segments of the thoracolumbar cord of dogs using the hydrogen clearance technique. Clearances were recorded and flows calculated from electrodes placed in grey and white matter. The position of the recording electrodes was marked by diathermy and confirmed in each exp...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(75)90054-4
更新日期:1975-12-01 00:00:00
abstract::This study included 40 consecutive patients with athrothrombotic carotid occlusive disease (A-group) and 13 consecutive patients with moyamoya disease (M-group) who had an internal carotid artery occlusion and underwent EC-IC bypass. Flow velocity and diameter of the operated STA on duplex ultrasonography (STDU), as w...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2012.11.006
更新日期:2013-02-15 00:00:00
abstract::Recent studies have demonstrated that cytokines play an important role in the pathogenesis of intracranial aneurysms (IAs). Interleukin-6 (IL-6) is an important proinflammatory cytokine. In our study, we investigated the association of genetic variants within the gene encoding interleukin-6-572G/C (IL-6-572G/C) with I...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2011.03.036
更新日期:2011-07-15 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:OBJECTIVE:To investigate the effect of mild cognitive impairment (MCI) on balance. METHODS:87 subjects with subjective memory impairment were enrolled, and subdivided into two groups, MCI and non-MCI, according to diagnostic criteria of amnestic subtype of MCI according to the 1999 MCI international panel (Current Con...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2011.02.031
更新日期:2011-06-15 00:00:00
abstract::We studied neurite regeneration in MRL-lpr/lpr mice, a murine model of systemic lupus erythematosus, using a culture system to investigate the influences of immunological abnormalities on neurons. The regeneration of cultured dorsal root ganglion (DRG) neurons from MRL-lpr/lpr mice was delayed compared with control MR...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(96)05216-1
更新日期:1997-07-01 00:00:00
abstract::Peripheral nerve injury results in a series of events culminating in degradation of the axonal cytoskeleton (Wallerian degeneration). In the time period between axotomy and cytoskeletal degradation (24-48 h in rodents), there is calcium entry and activation of calpains within the axon. The precise timing of these even...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(02)00013-8
更新日期:2002-04-15 00:00:00
abstract::The primary objective of this study was to attempt to induce excessive intraglial acidosis during ischemia by subjecting rats to an initial insult which leads to post insult accumulation of glycogen, presumed to accumulate primarily in astrocytes. The initial insults were 15 min of transient forebrain ischemia, 30 min...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(96)82226-x
更新日期:1996-04-01 00:00:00
abstract::An immunocytochemical study of surface and cytoplasmic markers of the monocyte/macrophage lineage was undertaken on peripheral blood (PB) and cerebrospinal fluid (CSF) cells in patients with multiple sclerosis (MS) or other neurological diseases (OND) and in three healthy controls. Size and granularity were assessed b...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(93)90274-3
更新日期:1993-12-15 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::To clarify the current status of treatments and outcomes of patients with myasthenia gravis (MG) in Japan, a total of 470 patients (164 men and 306 women; mean age 41 years) were recruited from 19 Japanese tertiary medical centers in 1999-2000. Thymectomy was performed in 319 (68%) of the patients. Patients who receiv...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,多中心研究
doi:10.1016/j.jns.2003.09.016
更新日期:2004-09-15 00:00:00
abstract::Prolonged-release (PR) fampridine is approved to treat walking impairment in persons with multiple sclerosis (MS); however, treatment benefits may extend beyond walking. MOBILE was a phase 2, 24-week, double-blind, placebo-controlled exploratory study to assess the impact of 10mg PR-fampridine twice daily versus place...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,随机对照试验
doi:10.1016/j.jns.2016.08.052
更新日期:2016-11-15 00:00:00
abstract::The aims of this study were to test the theory that transduction of low-level electromagnetic fields (EMFs) is mediated like other stimuli, and to determine the false-negative rate of the method used to assess the occurrence of transduction (intra-subject comparison of stimulus and non-stimulus states (ICOS)). A light...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(96)00188-8
更新日期:1996-12-01 00:00:00
abstract::A subgroup of limbic encephalitis is associated with antibodies against voltage-gated potassium channels (VGKC), and responds well to immuno-modulating therapies. Anti-VGKC antibodies are also found in Isaacs' syndrome and Morvan's syndrome, both of which are sometimes complicated by thymoma. We describe a 52-years-ol...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2007.12.019
更新日期:2008-06-15 00:00:00
abstract::Recently, TAR DNA-binding protein of 43-kDa (TDP-43) was identified as a major component of ubiquitinated neuronal cytoplasmic inclusions observed in lower motor neurons in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitinated inclusions. We herein investigated the relationship b...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2007.12.016
更新日期:2008-06-15 00:00:00
abstract::This report concerns the expression of ubiquitin in anterior horn cells of various subgroups of adult and infantile motor neuron disease (MNDs); immunohistochemical techniques were employed. Ubiquitin-positive skein-like inclusions (SLIs) were found in all cases of adult-onset amyotrophic lateral sclerosis (ALS), incl...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(93)90226-o
更新日期:1993-04-01 00:00:00
abstract:OBJECT:The purpose of this study was to determine the effects of diazoxide on apoptosis and the relative mechanisms in a model of brain injury induced by cerebral ischemia/reperfusion (I/R) during deep hypothermia. METHODS:Three-week-old Sprague-Dawley male rats were randomly and equitably divided into sham-operated g...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2007.10.029
更新日期:2008-05-15 00:00:00
abstract::REM sleep behavior disorder (RBD) is characterized by vigorous movements during REM sleep. Here, the authors report the case of a patient who presented such a disorder immediately after the implantation of the definitive electrode for left subthalamic stimulation. Interestingly, this was and has remained a unique epis...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2006.11.005
更新日期:2007-02-15 00:00:00
abstract::This is a case report of PCR proven herpes simplex (HSV-1) encephalitis in a 26 years old immunocompetent adult taking an unusual course of acute intracerebral haematoma after successful and complete recovery with acyclovir therapy. This transient late complication was associated with a negative repeat CSF PCR for HSV...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2006.10.020
更新日期:2007-01-31 00:00:00
abstract::The technique of parabiotic reinnervation has been used to test directly the neurogenic theory of the aetiology of muscular dystrophy in mice. Dystrophic muscles contain significantly fewer muscle fibres than their normal controls; they also have a much broader spectrum of fibre size because of a much higher proportio...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(75)90212-9
更新日期:1975-11-01 00:00:00
abstract:PURPOSE:To assess whether clonal IgH genes in CSF of patients with CNS lymphoma correlates with the disease course. BACKGROUND:It has been shown that the PCR technique, which offers a sensitive test for diagnosis of systemic lymphoproliferative malignancies, can be applied to the CSF. METHODS:Seventy-three CSF specim...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2006.03.012
更新日期:2006-08-15 00:00:00
abstract::We ascertained two families in Eastern Canada segregating a form of ataxia consistent with a recessive mode of inheritance. We performed a whole genome scan using dense SNP genotyping, and despite an absence of shared homozygosity in the families we defined linkage to a small region on chromosome 13. Direct DNA resequ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2009.09.034
更新日期:2010-01-15 00:00:00
abstract::Clinical and electrophysiological observations have been carried out on 12 patients with myotonic dystrophy. Neurological examination showed that the tendon reflexes were absent or weak in almost all cases, whereas the cutaneous reflexes were normal. Examination of both deep and superficial sensibility gave normal res...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(76)90136-2
更新日期:1976-12-01 00:00:00
abstract:BACKGROUND:Hypertensive (HT) patients are at higher risk of cognitive decline than normotensive individuals, because high blood pressure is a risk factor for mild cognitive deterioration. In this study cardio- and cerebrovascular reactivity along with cognitive performance was assessed on newly diagnosed HT patients. ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2010.07.022
更新日期:2010-12-15 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::Association of changes in olfactory-related structures with olfactory function in patients with multiple sclerosis (MS) is not well understood. We used a T&T olfactometer test kit to evaluate olfactory function in 26 patients with MS and 26 age- and sex-matched healthy controls (HC). Then, Brain MRI were performed and...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2016.03.045
更新日期:2016-06-15 00:00:00
abstract::Here, we report the observation of extrapontine lesions, in addition to the pontine lesions previously documented in a diagnosed case of hemodialysis-associated osmotic demyelination syndrome due to end-stage renal disease. The patient exhibited lesions on bilateral middle cerebellar peduncles, and had been receiving ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2006.10.012
更新日期:2007-02-15 00:00:00