Abstract:
:Homocarnosine, a dipeptide of gamma-aminobutyric acid (GABA) and histidine, is thought to be an inhibitory neuromodulator synthesized in subclasses of GABAergic neurons. Homocarnosine is present in human brain in greater amounts (0.4-1.0 micromol/g) than in other animals. The antiepileptic drug vigabatrin increases human cerebrospinal fluid homocarnosine linearly with daily dose. By using 1H nuclear magnetic resonance spectroscopy, serial occipital lobe GABA and homocarnosine concentrations were measured in 11 patients started on vigabatrin. Daily low-dose (2 g) vigabatrin increased both homocarnosine and GABA. Larger doses of vigabatrin (4 g) further increased homocarnosine but changed GABA levels minimally. Seizure control improved with increasing homocarnosine and GABA concentrations. Patients whose seizure control improved with the addition of vigabatrin had higher mean homocarnosine, but the same mean GABA concentrations, than those whose seizure control did not improve. Increased homocarnosine may contribute to improved seizure control.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Petroff OA,Mattson RH,Behar KL,Hyder F,Rothman DLdoi
10.1002/ana.410440614subject
Has Abstractpub_date
1998-12-01 00:00:00pages
948-52issue
6eissn
0364-5134issn
1531-8249journal_volume
44pub_type
临床试验,杂志文章abstract::Infection by the human immunodeficiency virus type 1 (HIV-1) often results in neurological dysfunction including HIV dementia (HIVD). Alterations in cytokine and redox balance are thought to play important roles in the pathogenesis of HIVD, but the specific mechanisms underlying neuronal dysfunction and death are unkn...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10828
更新日期:2004-02-01 00:00:00
abstract::Sporadic amyotrophic lateral sclerosis is a motor neuron disease of unknown origin. Autoimmunity against voltage-gated calcium channels is one mechanism hypothesized to be the cause of the disease. In support of this hypothesis, it was previously reported that amyotrophic lateral sclerosis IgG specifically blocked the...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410400505
更新日期:1996-11-01 00:00:00
abstract:OBJECTIVE:Cortical spreading depolarizations (CSDs) are intense and ubiquitous depolarization waves relevant for the pathophysiology of migraine and brain injury. CSDs disrupt the blood-brain barrier (BBB), but the mechanisms are unknown. METHODS:A total of six CSDs were evoked over 1 hour by topical application of 30...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25298
更新日期:2018-09-01 00:00:00
abstract:OBJECTIVE:Abnormal cortical excitability is evident in various movement disorders that compromise fine motor control. Here we tested whether skilled finger movements can be restored in musicians with focal hand dystonia through behavioral training assisted by transcranial direct current stimulation to the motor cortex ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24151
更新日期:2014-05-01 00:00:00
abstract::Two family members with hereditary pressure-sensitive neuropathy are reported. One patient presented atypically with acute brachial plexus neuropathy following transaxillary removal of the first rib. Electrophysiological studies showed slowing of motor nerve conduction in clinically affected and unaffected nerves. In ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410080613
更新日期:1980-12-01 00:00:00
abstract::The development of serotonin (5HT1B/1D) agonists as treatments for the acute attack of migraine has resulted in considerable interest in their mechanism of action and, to some extent, renewed interest in the role of serotonin (5-hydroxytryptamine; 5HT) in the disorder. The initial synthesis of this class of compounds ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410430605
更新日期:1998-06-01 00:00:00
abstract:OBJECTIVE:Nonketotic hyperglycinemia is a neurometabolic disorder characterized by intellectual disability, seizures, and spasticity. Patients with attenuated nonketotic hyperglycinemia make variable developmental progress. Predictive factors have not been systematically assessed. METHODS:We reviewed 124 patients stra...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24485
更新日期:2015-10-01 00:00:00
abstract::Eighty-three patients with epidural spinal cord compression, from metastatic cancer were treated with high-dose adrenocorticosteroids and a new radiation fractionation protocol. Only those patients were included who had complete or almost complete block on myelography and who had not received prior radiation therapy t...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410080404
更新日期:1980-10-01 00:00:00
abstract::Dichloroacetate (DCA) stimulates pyruvate dehydrogenase complex (PDHC) activity and lowers cerebral lactate concentrations. In the R6/2 and N171-82Q transgenic mouse models of Huntington's disease (HD), DCA significantly increased survival, improved motor function, delayed loss of body weight, attenuated the developme...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.1085
更新日期:2001-07-01 00:00:00
abstract::A patient developed conjugate horizontal eye deviation to the side opposite a large frontal-perisylvian hemorrhage from a ruptured aneurysm. Autopsy examination confirmed the hemispheric locus of the hemorrhage and failed to disclose any lesion of the brainstem or thalamus to explain the "wrong-way" eye deviation. ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410090115
更新日期:1981-01-01 00:00:00
abstract::The mechanism underlying mirrored activity/movements in normal individuals is unknown. To investigate this, we studied 11 adults and 39 children who performed sequential finger-thumb opposition or repetitive index finger abduction. Surface electromyographic (EMG) activity recorded from the left and right first dorsal ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/1531-8249(199905)45:5<583::aid-ana6>3.0.co
更新日期:1999-05-01 00:00:00
abstract::alpha 1-Antichymotrypsin (ACT) is a serine protease inhibitor that is markedly elevated in the serum and cerebrospinal fluid of patients with Alzheimer's disease (AD). Patients with Down's syndrome are known to develop neuropathological changes of AD by age 40 years and many become demented. Therefore, in the present ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410320211
更新日期:1992-08-01 00:00:00
abstract::Regular, evidence-based assignment of patients to etiologic stroke categories is essential to enable valid comparison among studies. We designed an algorithm (SSS-TOAST) that incorporated recent advances in stroke imaging and epidemiology to identify the most probable TOAST category in the presence of evidence for mul...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20617
更新日期:2005-11-01 00:00:00
abstract::The phenotype of severe congenital hydrocephalus secondary to neural cell adhesion molecule L1 (L1CAM) gene mutations includes the distinct finding of brainstem corticospinal tract hypoplasia. Using diffusion-weighted imaging (DWI), we failed to demonstrate anisotropy in the corticospinal tracts of the basis pontis in...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:
更新日期:2000-01-01 00:00:00
abstract::We performed magnetic resonance imaging and magnetic resonance spectroscopic imaging on 28 patients with multiple sclerosis stratified for disability and clinical course (relapsing with at least partial remissions or secondary progressive disease). Lesions were segmented on the conventional proton density and T2-weigh...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章
doi:10.1002/ana.410410314
更新日期:1997-03-01 00:00:00
abstract::Bilateral ptosis is reported with unilateral hemispheric lesions, suggesting partial lateralization of the control of the levator palpebrae superioris. There is a tight synkinesis between vertical eye and eyelid movements, but a similar, lateralized control of vertical gaze has not been previously described. We report...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410400320
更新日期:1996-09-01 00:00:00
abstract::Positron emission tomography was used to study the effect of supratentorial infarction on cerebellar metabolic rate for oxygen and cerebellar blood flow. In a control group of patients, the mean cerebellar metabolic rate for oxygen was 2.97 +/- 0.11 (standard error of the mean [SEM] ) ml-1 . min-1 . hg-1 and mean cere...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410140203
更新日期:1983-08-01 00:00:00
abstract::The nosological status of multifocal motor neuropathy remains controversial. The clinical and electrodiagnostic hallmarks suggest selective motor fiber involvement. In this study, we asked to what extent sensory nerves might be involved pathologically in multifocal motor neuropathy. Examination of sensory nerve biopsy...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410390308
更新日期:1996-03-01 00:00:00
abstract::There is increasing interest in the potential of dopamine agonists to provide a neuroprotective effect and to alter the natural course of levodopa-treated Parkinson's disease (PD). Theoretically, such a protective effect might derive from (a) a levodopa sparing effect, (b) stimulation dopamine autoreceptors resulting ...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.410440725
更新日期:1998-09-01 00:00:00
abstract::Neural tube defects (NTDs) are among the most common birth defects in humans and yet their molecular etiology remains poorly understood. NTDs are believed to result from the complex interaction of environmental factors with a multitude of genetic risk factors in a classical multifactorial disease model. Mendelian form...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25152
更新日期:2018-02-01 00:00:00
abstract::Volumetric magnetic resonance imaging analyses of 30 subjects were undertaken to quantify the global and temporal lobe atrophy in semantic dementia and Alzheimer's disease. Three groups of 10 subjects were studied: semantic dementia patients, Alzheimer's disease patients, and control subjects. The temporal lobe struct...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:
更新日期:2001-04-01 00:00:00
abstract:OBJECTIVE:Gut microbiome alterations in Parkinson disease (PD) have been reported repeatedly, but their functional relevance remains unclear. Fecal metabolomics, which provide a functional readout of microbial activity, have scarcely been investigated. We investigated fecal microbiome and metabolome alterations in PD, ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25982
更新日期:2020-12-03 00:00:00
abstract::We recently reported that systemic administration of a proteasome inhibitor induced a progressive levodopa-responsive, bradykinetic syndrome in rats with imaging, pathological, and biochemical features that strikingly resemble what is found in PD. This model has the potential to be a useful tool for studying the mecha...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20936
更新日期:2006-08-01 00:00:00
abstract:OBJECTIVE:To examine age-related changes in the neural systems for reading in nonimpaired and dyslexic children and adolescents. METHODS:Functional magnetic resonance imaging was used to study age-related changes in the neural systems for reading in a cross-sectional sample of 232 right-handed children 7 to 18 years o...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21093
更新日期:2007-04-01 00:00:00
abstract::Changes in endoneurial fluid pressure (EFP) and morphology were studied in rat sciatic nerves frozen for 60 seconds with a cryoprobe designed for human cryoanalgesia. The onset of increased EFP was rapid, and a peak of 23 cm H2O was reached within 90 minutes after injury. EFP levels returned to normal 32 days after fr...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410100512
更新日期:1981-11-01 00:00:00
abstract::Apolipoprotein E has been implicated in modifying neurological outcome after traumatic brain injury, although the mechanisms by which this occurs remain poorly defined. To investigate the role of endogenous apolipoprotein E following acute brain injury, noninvasive magnetic resonance imaging was performed on anestheti...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10098
更新日期:2002-01-01 00:00:00
abstract::Regional patterns of cerebral glucose utilization (rCMRglc) and blood flow (rCBF) were examined in the early recovery period following transient forebrain ischemia in order to correlate early postischemic physiological events with regionally selective patterns of ischemic neuropathology. Wistar rats were subjected to ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410180410
更新日期:1985-10-01 00:00:00
abstract::Three of six rabbits immunized with purified GD1b developed ataxic sensory neuropathy. They laid on the floor with their limbs splayed out, and their movements were awkward; but muscle power, tonus, and superficial sensation appeared to be intact. Sciatic nerve motor conduction studies were normal. Axonal degeneration...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410390404
更新日期:1996-04-01 00:00:00
abstract::The amygdala and its subnuclei undergo severe volumetric atrophy in Alzheimer's disease (AD). To determine whether this atrophy is due to loss of neuropil, specific neuronal populations, or both, we evaluated the number, size, and packing density of neurons and glia in the cortical and magnocellular basal amygdaloid s...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410320412
更新日期:1992-10-01 00:00:00
abstract::We investigated a family with a new type of autosomal dominant cerebellar ataxia (ADCA) in which pure cerebellar ataxia is often accompanied with epilepsy. No CAG repeat expansions were detected at the spinocerebellar ataxia (SCA) type 1, 2, 3, 6, or 7 locus, and SCAs 4 and 5 were excluded by linkage analysis. We foun...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/1531-8249(199903)45:3<407::aid-ana21>3.0.c
更新日期:1999-03-01 00:00:00