Abstract:
:We previously described demyelination in the brain and subacute combined degeneration of the spinal cord in a patient with 5,10-methylenetetrahydrofolate reductase deficiency. To assess the role of methionine, S-adenosylmethionine, folate, and neurotransmitter amine metabolism in the demyelination process, we measured these metabolites in CSF from this patient; the findings are compared with those obtained from three patients in whom neurologic deterioration had been halted by the administration of betaine. Folate concentrations were low, and amine and biopterin metabolism were abnormal in all patients. Methionine and S-adenosylmethionine concentrations were undetectable in the first patient. In those receiving betaine, methionine concentrations were proportional to the dose administered and S-adenosylmethionine concentrations were near normal. The results provide the first evidence for an association between defective S-adenosylmethionine metabolism and demyelination in humans.
journal_name
Neurologyjournal_title
Neurologyauthors
Hyland K,Smith I,Bottiglieri T,Perry J,Wendel U,Clayton PT,Leonard JVdoi
10.1212/wnl.38.3.459subject
Has Abstractpub_date
1988-03-01 00:00:00pages
459-62issue
3eissn
0028-3878issn
1526-632Xjournal_volume
38pub_type
杂志文章相关文献
NEUROLOGY文献大全abstract::We used two analytic methods (a multichannel coulometric electrode array with high-performance liquid chromatography, and gas chromatography-mass spectrophotometry) to measure CSF dopamine (DA) and its metabolites in mildly affected, unmedicated subjects with Parkinson's disease (PD). The mean (+/- SD) concentration o...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.42.11.2111
更新日期:1992-11-01 00:00:00
abstract:OBJECTIVE:Small-cell lung carcinoma (SCLC) and limbic encephalitis are recognized γ-aminobutyric acid-B receptor (GABABR) autoantibody accompaniments. We sought to determine in a diagnostic serology laboratory the frequency and accompaniments (neurologic, oncologic, and serologic) of GABABR-immunoglobulin G (IgG). MET...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e3182a35271
更新日期:2013-09-03 00:00:00
abstract:OBJECTIVE:To examine associated demographic and clinical correlates and the development of excessive daytime sleepiness (EDS) over 8 years in a community-based cohort of patients with Parkinson disease (PD). METHODS:A total of 232 patients with PD were included in a population-based prevalence study in 1993. Patients ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000233980.25978.9d
更新日期:2006-09-12 00:00:00
abstract::We present a clinical and morphological study concerning a kindred with slowly progressive X-linked muscular dystrophy (Becker type). Five males were affected; one died of heart failure at age 16. Severe and early cardiac disorder is unusual in this type of muscular dystrophy, and death at such an early age had not be...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.29.8.1144
更新日期:1979-08-01 00:00:00
abstract::To examine animal exposure in ALS patients, a case-control study was undertaken on 40 ALS patients and 40 closely matched controls. Exposure to pets of any kind and to small dogs in the period from birth until 10 years before onset of ALS symptoms was significantly increased. Affected men showed a trend toward increas...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.35.5.717
更新日期:1985-05-01 00:00:00
abstract:OBJECTIVES:There is currently much interest in biomarkers of disease activity in frontotemporal lobar degeneration (FTLD). We assessed MRI and behavioral measures of progression in a longitudinal FTLD cohort. METHODS:Thirty-two patients with FTLD (11 behavioral variant frontotemporal dementia [bvFTD], 11 semantic deme...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e3181d1a879
更新日期:2010-02-23 00:00:00
abstract:BACKGROUND:The neurodegenerative process in Alzheimer's disease (AD) and in the Lewy body variant of AD (LBV) patients is characterized by cholinergic dysfunction and deposition of amyloid beta-peptide (Abeta) 1-40 and 1-42; however, the differential effects of Abeta species on the cholinergic system are not completely...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000073987.79060.4b
更新日期:2003-07-22 00:00:00
abstract::Diffusion-weighted MRI (DWI) was used in three patients with autopsy-proven sporadic Creutzfeldt-Jakob disease (CJD) to provide a rapid noninvasive way to make this sometimes confusing diagnosis. DWI prompted the diagnosis of CJD at an early stage and appears to be particularly useful for monitoring the progression of...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.52.1.205
更新日期:1999-01-01 00:00:00
abstract:OBJECTIVE:To assess the recovery process in patients after an acute optic neuritis (ON) attack, comparing static and dynamic visual functions. METHODS:In this prospective controlled study, 21 patients with unilateral, first-ever ON were followed over the course of 1 year. Standard visual tests, visual evoked potential...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e31821f4602
更新日期:2011-06-14 00:00:00
abstract::Lymphocytic meningoradiculitis (Bannwarth's syndrome) is a radicular neuralgia associated with a chronic lymphocytic pleocytosis in cerebrospinal fluid and frequently with unilateral or bilateral peripheral facial weakness. Most reported cases have occurred during the summer among adults living in central Europe. Lymp...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.33.11.1476
更新日期:1983-11-01 00:00:00
abstract:OBJECTIVES:This study examines the anatomical correlates of naming vs recognizing faces using a novel measure that utilizes culturally relevant and age-appropriate items, the Northwestern University Famous Faces (NUFFACE) Test, in primary progressive aphasia (PPA), a syndrome characterized by progressive language defic...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e3182a08f83
更新日期:2013-08-13 00:00:00
abstract:OBJECTIVES:Homonymous hemianopia (HH) is the most frequent visual-field defect after a stroke. Some of these patients also have visual hallucinations, the origin and frequency of which remain largely unknown. The aims of this work were to determine the occurrence of visual hallucinations among poststroke hemianopic pat...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000009366
更新日期:2020-05-05 00:00:00
abstract::We observed a highly topographically specific and consistent pattern of degeneration in the auditory system of Alzheimer's disease (AD) patients. Senile plaques (SP) and neurofibrillary tangles (NFT) were distributed throughout the ventral nucleus of the medial geniculate body (MGB) and the central nucleus of the infe...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.43.4.779
更新日期:1993-04-01 00:00:00
abstract:OBJECTIVE:To validate the Narcolepsy Severity Scale (NSS), a brief clinical instrument to evaluate the severity and consequences of symptoms in patients with narcolepsy type 1 (NT1). METHODS:A 15-item scale to assess the frequency and severity of excessive daytime sleepiness, cataplexy, hypnagogic hallucinations, slee...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000003787
更新日期:2017-04-04 00:00:00
abstract::We describe a 78-year-old man with right hemiballism as the presenting symptom of a metastatic occult adenocarcinoma of the lung. CT demonstrated two contrast-enhancing lesions: one in the left subthalamic region and the other in the left parietal region. Metastatic tumor was confirmed at autopsy. ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.34.2.204
更新日期:1984-02-01 00:00:00
abstract:OBJECTIVES:Intracerebral hemorrhage (ICH) is a highly lethal disease of the elderly. Use of statins is increasingly widespread among the elderly, and therefore common in patients who develop ICH. Accumulating data suggests that statins have neuroprotective effects, but their association with ICH outcome has been incons...
journal_title:Neurology
pub_type: 杂志文章,meta分析
doi:10.1212/WNL.0b013e3182194be9
更新日期:2011-05-03 00:00:00
abstract:OBJECTIVE:Evaluating the TBI incidence during the COVID-19 pandemic era compared with similar period in previous year. BACKGROUND:The emergence of SARS-CoV2 influenced the evolution of governmental and hospital policy worldwide, which might eventually impact many aspects. The incidence of many diseases in the Emergenc...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000719968.10580.81
更新日期:2020-11-17 00:00:00
abstract::We tested the hypothesis that an anticardiolipin antibody (aCL) titer of > or = 10 IgG phospholipid (GPL) at the time of an index ischemic stroke is associated with an increased risk of subsequent thrombo-occlusive events or death. First-time ischemic stroke patients from the Antiphospholipid Antibodies and Stroke Stu...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.48.1.91
更新日期:1997-01-01 00:00:00
abstract:OBJECTIVE:To revise the 1999 Airlie House consensus guidelines for the design and implementation of preclinical therapeutic studies and clinical trials in amyotrophic lateral sclerosis (ALS). METHODS:A consensus committee comprising 140 key members of the international ALS community (ALS researchers, clinicians, patie...
journal_title:Neurology
pub_type: 共识发展会议,杂志文章
doi:10.1212/WNL.0000000000007242
更新日期:2019-04-02 00:00:00
abstract::Subacute encephalitis, characterized by demyelination, gliosis of the gray and white matter, focal necrosis, microglial nodules, atypical oligodendrocyte nuclei, and multinucleation of cells, was present in 27 of 30 (90%) autopsied patients with acquired immune deficiency syndrome (AIDS) or AIDS-related complex. Subac...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.37.4.562
更新日期:1987-04-01 00:00:00
abstract:OBJECTIVE:In this study, we compared a cognition-specific computer-based cognitive training program with a motion-controlled computer sports game that is not cognition-specific for their ability to enhance cognitive performance in various cognitive domains in patients with Parkinson disease (PD). METHODS:Patients with...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000000287
更新日期:2014-04-08 00:00:00
abstract:OBJECTIVE:To investigate whether vitamin D, smoking, and anti-Epstein-Barr virus (EBV) antibody concentrations predict long-term cognitive status and neuroaxonal injury in multiple sclerosis (MS). METHODS:This study was conducted among 278 patients with clinically isolated syndrome who participated in the clinical tri...
journal_title:Neurology
pub_type: 杂志文章,多中心研究
doi:10.1212/WNL.0000000000009371
更新日期:2020-05-05 00:00:00
abstract::A 61-year-old woman presented with two paraneoplastic neurologic disorders--Lambert-Eaton myasthenic syndrome (LEMS) and subacute cerebellar degeneration (SCD)--that antedated the diagnosis of small-cell carcinoma of the lung by 15 months. Plasmapheresis initiated before the identification of the tumor had a beneficia...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.41.10.1682
更新日期:1991-10-01 00:00:00
abstract:OBJECTIVE:To develop a simple imaging rule to predict neurodevelopmental outcomes at 4.5 years in a cohort of preterm neonates with white matter injury (WMI) based on lesion location and examine whether clinical variables enhance prediction. METHODS:Sixty-eight preterm neonates born 24-32 weeks' gestation (median 27.7...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000008172
更新日期:2019-09-24 00:00:00
abstract::Fasciculations and cramps may occur in motor neuron disease or as part of a more benign syndrome. A man with apparently benign fasciculations and cramps for 4 years developed progressive muscle weakness and wasting. Such a previously undocumented evolution of benign fasciculations and cramps to motor neuron disease ma...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.36.7.997
更新日期:1986-07-01 00:00:00
abstract::Combined infusion of high doses of lidocaine and thiopental in a comatose patient induced major latency and amplitude BAEP changes, which progressed to complete BAEP abolition. Responses returned to normal after drugs were discontinued. EEGs during the episodes showed long-lasting periods of activity suppression, but ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.38.9.1487
更新日期:1988-09-01 00:00:00
abstract:OBJECTIVE:To evaluate the prospective association of long-term intake of vegetables and fruits with late-life subjective cognitive function (SCF). METHODS:Among 27,842 men with a mean age of 51 years in 1986, we used multinomial logistic regression to examine the relation of vegetable and fruit consumption to future S...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000006684
更新日期:2019-01-01 00:00:00
abstract:BACKGROUND:Oculopharyngodistal myopathy (OPDM) has been reported as a rare, adult-onset hereditary muscle disease with putative autosomal dominant and autosomal recessive inheritance. Patients with OPDM present with progressive ocular, pharyngeal, and distal limb muscle involvement. The genetic defect causing OPDM has ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e318207b043
更新日期:2011-01-18 00:00:00
abstract::Nine neurosurgical patients suffering from a variety of brain tumors were studied. Sodium valproate was given for 3 days before surgery. At craniotomy, sections of brain and samples of cerebrospinal fluid (CSF) and plasma were obtained and assayed for sodium valproate concentrations. Brain levels were 6.8 to 27.9% of ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.31.4.486
更新日期:1981-04-01 00:00:00
abstract::It is generally believed that a diet high in carbohydrate improves exercise tolerance in patients with carnitine palmitoyltransferase II (CPT II) deficiency, but it has never been systematically investigated. The authors investigated the effect of a high- vs low-carbohydrate diet on exercise tolerance in four patients...
journal_title:Neurology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1212/01.wnl.0000078195.05396.20
更新日期:2003-08-26 00:00:00