Abstract:
:The effects of combined treatment with low-density lipoprotein (LDL)-apheresis, chenodeoxycholic acid (CDCA) and 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) reductase inhibitor were studied in 2 patients with cerebrotendinous xanthomatosis. Patient 1 was initially treated with LDL-apheresis alone: serum cholestanol levels decreased by 50% after each apheresis, but returned to their initial levels within 2 weeks. After an addition of CDCA administration, the serum cholestanol levels steadily decreased, resulting in slight improvement of neurological symptoms. Patient 2 received a combined treatment with LDL-apheresis, CDCA and HMG-CoA reductase inhibitor. This combination showed less LDL-apheresis-dependent fluctuation and more rapid decrease of serum cholestanol levels than those in Patient 1, resulting in improvement and stabilization of the symptoms. Our results suggest that LDL-apheresis in combination with CDCA and HMG-CoA reductase inhibitor may have beneficial effects and can be one of the treatment options.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Ito S,Kuwabara S,Sakakibara R,Oki T,Arai H,Oda S,Hattori Tdoi
10.1016/j.jns.2003.07.005subject
Has Abstractpub_date
2003-12-15 00:00:00pages
179-82issue
1eissn
0022-510Xissn
1878-5883pii
S0022510X03002375journal_volume
216pub_type
临床试验,杂志文章abstract::The relationship between Restless Legs Syndrome (RLS) and Parkinson's disease (PD) is still controversial. Most genetic, pathological, and imaging data argue against a close association of these two disorders. Still, many studies reported an increased prevalence of RLS in PD patients. These studies are difficult to in...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2009.08.035
更新日期:2010-02-15 00:00:00
abstract:BACKGROUND:Previous studies to estimate burden of neurological disorders in Africa are limited to inpatients in urban hospitals. The spectrum of neurological conditions in rural Africa remains unclear. OBJECTIVE:To determine the spectrum of neurological presentations in an outpatient setting in rural Zimbabwe. METHOD...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2016.01.065
更新日期:2016-03-15 00:00:00
abstract::Late-onset depression (LOD) could be a very early manifestation of Alzheimer's disease (AD), although contradictory results have been reported. Cerebrovascular disease (CVD) may favor the development of LOD, and that the particular forms of vascular depression should be individualized. The Apolipoprotein E (ApoE) epsi...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2007.01.038
更新日期:2007-06-15 00:00:00
abstract:BACKGROUND:The epidemiology and pathophysiology of iNPH remain unclear. We aimed to investigate the incidence of iNPH in elderly inhabitants of the community, and to identify how ventriculomegaly develops on brain MRIs and how symptoms develop in iNPH patients. METHODS:In 2000, 350 inhabitants, all 70-year-olds living...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.01.033
更新日期:2014-04-15 00:00:00
abstract::Ten patients with myotonic dystrophy (MyD) and excessive daytime sleepiness (EDS) were studied. Daytime sleepiness was assessed by means of a subjective alertness rating scale, multiple sleep latency tests and auditory event-related potentials. In addition, the diurnal pattern of daytime sleepiness and ultradian rhyth...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(93)90051-y
更新日期:1993-01-01 00:00:00
abstract::Isometric contractions of single motor units in the first dorsal interosseous muscle were recorded with an intramuscular microstimulation technique in patients with short- or long-term hemiplegia and compared with controls. In the hemiplegic patients motor units could be classified as in controls, utilizing twitch con...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(82)90203-9
更新日期:1982-06-01 00:00:00
abstract::Deficient activity of human lysosomal hydrolase, acid sphingomyelinase (ASM), results in the neuronopathic (type A) and non-neuronopathic (type B) forms of Niemann-Pick disease (NPD). A deficiency of ASM is known to deprive lymphoblasts of their response to apoptotic induction by X-ray irradiation. To elucidate the ge...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(02)00106-5
更新日期:2002-07-15 00:00:00
abstract::We assessed neuropsychological performances of 22 patients affected by Autosomal Dominant Cerebellar Ataxia type 1. All subjects completed a comprehensive battery of standardized tests requiring a verbal response, without time constraints. In order to verify the hypothesis that disease severity is the major factor in ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(98)00070-7
更新日期:1998-05-07 00:00:00
abstract::An endogenous factor that is able to reduce the fast transient sodium current of excitable cells has been reported to exist in the cerebrospinal fluid (CSF) of multiple sclerosis (MS) patients. This was confirmed with nine clinically definite MS patients in the acute relapse. In order to purify and chemically identify...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(99)00285-3
更新日期:2000-01-01 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:INTRODUCTION:TCS is a well-established technique for diagnosis of Parkinson's disease (PD). Volumetric 3D-TCS is a promising complementary approach for objective acquisition and analysis, in particular for less experienced sonographers. This study provides baselines for Parkinson detection (sensitivity and specificity)...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2018.12.001
更新日期:2019-02-15 00:00:00
abstract:PURPOSE:Among different seizure types, tonic-clonic seizures are more significant because they are more often associated with morbidity. No prior study has been done to investigate risk factors associated with tonic-clonic seizures in patients with mesial temporal epilepsy. METHODS:In this retrospective study, all dru...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2015.10.022
更新日期:2015-12-15 00:00:00
abstract::We recently detected varicella zoster virus (VZV) in the temporal arteries (TA) of 5/24 patients with clinically suspect giant cell arteritis (GCA) whose TAs were GCA-negative pathologically; in those GCA-negative, VZV+TAs, virus antigen predominated in the arterial adventitia, but without medial necrosis and multinuc...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.09.034
更新日期:2013-12-15 00:00:00
abstract:OBJECTIVE:To determine the influence of different factors on test-retest reliability of frequently used transcranial magnetic stimulation (TMS) parameters while controlling for potential confounders in healthy subjects. METHODS:TMS was applied in 93 healthy volunteers (61% male) twice (mean retest interval of 34.0 ± 2...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2016.01.039
更新日期:2016-03-15 00:00:00
abstract::Human leptomeningeal (arachnoid and pia mater) cells in culture have been demonstrated in replicated studies to express typical neuronal proteins such as neurofilament protein and neuron-specific enolase. In addition, they can express glial fibrillary acidic protein. The present study examines the possibility that neu...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(96)00189-x
更新日期:1996-12-01 00:00:00
abstract:BACKGROUND:The study aim was to evaluate the safety and efficacy of endovascular treatment (EVT) versus medical treatment (MT) in patients with symptomatic vertebral artery (VA) stenosis. METHODS:Randomized controlled trials with active and control groups receiving EVT plus MT and MT alone in patients with vertebro-ba...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,meta分析
doi:10.1016/j.jns.2018.05.024
更新日期:2018-08-15 00:00:00
abstract::The purpose of this study was to identify clinical factors which may help in predicting the requirement of support of mechanical ventilation (MV) in patients with stroke. This was a prospective cohort study done at a tertiary referral center of India, from December 2008 to December 2009. All consecutive patients of ac...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.11.007
更新日期:2014-02-15 00:00:00
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::During postnatal development of the higher vertebrate CNS, large populations of oligodendroglia are generated from precursor cells in a very dependable way. In adult lesioned CNS tissues, local populations of oligodendroglia are replenished by proliferation of this replenishment varies from one species to another and ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/0022-510x(86)90019-5
更新日期:1986-02-01 00:00:00
abstract::Over two decades, a 42-year old woman experienced the gradual onset of choreic involuntary movements, dystonia, and tics. Decreased caudate nucleus metabolism on 2-deoxyglucose PET scan and a heterozygous 49-CAG repeat expansion within the HTT gene established the diagnosis of HD, although no other family history was ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2012.08.034
更新日期:2012-12-15 00:00:00
abstract::Systemic inflammatory response syndrome (SIRS) is a medical condition in which the all-organ microcirculation is affected including nervous system. We describe neurological findings in 64 patients with SIRS at Hospital das Clínicas of Sao Paulo University School of Medicine; 45.3% were male and 54.7% female; their age...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2006.08.015
更新日期:2006-12-01 00:00:00
abstract::Mast cells (MC) have been implicated in the pathogenesis of experimental allergic encephalomyelitis (EAE). In order to further evaluate their role, several morphological and functional studies were performed. Semiquantitative counts of histological sections showed a significant reduction in MC numbers in EAE brains. I...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(94)90300-x
更新日期:1994-04-01 00:00:00
abstract::Plasma levels of testosterone, luteinizing hormone (LH) and follicle-stimulating hormone (FSH) after 3 or 6 days of administration of the synthetic androgenic hormone fluoxymesterone (10 mg/day) were measured in 26 patients with X-linked recessive bulbospinal neuronopathy (X-BSNP) and 22 age-matched male controls. The...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(94)90347-6
更新日期:1994-02-01 00:00:00
abstract:BACKGROUND:The novel coronavirus disease 19 (COVID-19) causes multi-system disease including possibly heightened stroke risk. Data from high-income countries (HIC) suggest disruptions to care delivery with reduced stroke admissions and administration of acute stroke reperfusion therapies. We are unaware of any publishe...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2020.117044
更新日期:2020-09-15 00:00:00
abstract::We measured the CSF and plasma levels of glutamate, glutamine, aspartate (only in plasma), asparagine, glutamine, glycine and GABA in 31 patients with Parkinson's disease and in 45 matched controls. We used an ion-exchange chromatography method. When compared to controls, PD patients had similar CSF levels of glutamat...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(96)00115-3
更新日期:1996-09-15 00:00:00
abstract::In the last two decades functional magnetic resonance imaging (fMRI) has dominated research in neuroscience. However, only recently has it taken the first steps in translation to the clinical field. In this paper we describe the advantages of fMRI and DTI and the possible benefits of implementing these methods in clin...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2011.07.040
更新日期:2012-01-15 00:00:00
abstract:OBJECTIVE:To determine the sensitivity and specificity of clonal immunoglobulin heavy chain gene rearrangement (IGHR) analysis in the distinction of benign and malignant lymphoproliferative diseases. METHODS:A retrospective analysis was conducted of patients in whom a malignant lymphoproliferative process was suspecte...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2006.05.044
更新日期:2006-09-25 00:00:00
abstract::This paper describes 2 brothers with increasingly severe exercise-induced muscle pain and stiffness, beginning in adolescence. Histochemical studies showed that myoadenylate deaminase activity was absent in the propositus, but present in his younger brother. Biochemical examination of muscle homogenates confirmed thes...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(82)90086-7
更新日期:1982-01-01 00:00:00
abstract::Water-suppressed proton magnetic resonance spectroscopy which has recently shown diagnostic potential in human stroke victims was performed in two patients who had suffered a hemispheric transient ischemic attack. Localized spectra were targeted from routine MR images. In one patient lactate could be observed 8 weeks ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(92)90185-n
更新日期:1992-03-01 00:00:00
abstract::Adoptive transfer of experimental allergic encephalomyelitis (EAE) was achieved in Lewis rats using spleen cells incubated in vitro with various lectins. When stimulated with concanavalin A (Con A), its derivative succinyl-Con A, Lens culinaris or Pisum sativum agglutinin, spleen cells obtained from rats immunized wit...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(84)90014-5
更新日期:1984-11-01 00:00:00