Abstract:
:To evaluate reports of abnormal levels of free amino acids (AA) in patients with amyotrophic lateral sclerosis (ALS), we studied serum, cerebrospinal fluid, and urine AA in 12 patients with ALS and 12 controls matched for age, sex, and severity of disability. ALS patients had statistically significant elevations in serum levels of tyrosine, total aromatic AA, and total basic AA. ALS patients also had statistically significant elevations in cerebrospinal fluid of total basic AA, lysine, essential AA, and leucine. The severity of ALS correlated inversely with acidic AA (glutamate and aspartate) and O-phosphoserine in cerebrospinal fluid. Activity of ALS correlated directly with serum aspartate and cerebrospinal fluid alanine. We conclude that subtle abnormalities of AA levels are present in ALS and that these are not due to age, sex, or disability. The pattern of distribution of AA levels differs from that in hepatic or renal disease and suggests defective membrane transport or poor cellular utilization of basic and essential AA in the central nervous system.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Patten BM,Harati Y,Acosta L,Jung SS,Felmus MTdoi
10.1002/ana.410030405subject
Has Abstractpub_date
1978-04-01 00:00:00pages
305-9issue
4eissn
0364-5134issn
1531-8249journal_volume
3pub_type
杂志文章abstract::Twenty-five patients with recently diagnosed leptomeningeal metastases underwent a prospective clinical and electro-physiological study to assess the mode and extent of peripheral nerve and spinal root involvement. Motor conduction velocities and F wave latencies in the legs were found frequently to be disturbed compa...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410170611
更新日期:1985-06-01 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
abstract::The rate of local cerebral glucose utilization was determined for quantification of energy metabolism in macrostructures of the sensorimotor system during propagation of focal motor seizures in 24-month-old monkeys. The rate was measured in 4 control animals and in 4 monkeys each with seizures limited to the contralat...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410070304
更新日期:1980-03-01 00:00:00
abstract:OBJECTIVE:The risk for symptomatic intracerebral hemorrhage (sICH) associated with thrombolytic treatment has not been evaluated in large studies using diffusion-weighted imaging (DWI). Here, we investigated the relation between pretreatment DWI lesion size and the risk for sICH after thrombolysis. METHODS:In this ret...
journal_title:Annals of neurology
pub_type: 杂志文章,多中心研究
doi:10.1002/ana.21222
更新日期:2008-01-01 00:00:00
abstract::It has been proposed that early-onset familial Alzheimer's disease (FAD) and sporadic Alzheimer's disease (AD) have different causes, with FAD due to a single dominant gene with disease onset before the sixth decade, whereas sporadic AD has a later onset and is not associated with a dominant pattern of inheritance. Gi...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410320513
更新日期:1992-11-01 00:00:00
abstract::In a double-blinded, placebo-controlled, crossover study in seven mitochondrial myopathy patients (MM), we investigated whether lowering of lactate with dichloroacetate (DCA) can improve exercise tolerance and oxidative capacity in MM. DCA lowered plasma lactate at rest and during exercise (from 10.5 +/- 2.0 to 5.0 +/...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章,随机对照试验
doi:
更新日期:2001-05-01 00:00:00
abstract::Using the technique of transcranial magnetic stimulation over the motor areas of cortex and recording electromyographic (EMG) responses from the first dorsal interosseous muscle, we measured the excitability of corticocortical inhibitory circuits at rest using a double pulse paradigm, in 11 patients with Parkinson's d...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章
doi:10.1002/ana.410370208
更新日期:1995-02-01 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::The characteristic pathological features of Huntington's disease (HD) are neostriatal atrophy and neuronal loss. Although neuroradiological studies often show caudate atrophy in patients with moderate HD, frequently no caudate atrophy is found early in the illness. There have been no quantitative reports to date on in...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410310113
更新日期:1992-01-01 00:00:00
abstract:OBJECTIVE:Faster time from onset to recanalization (OTR) in acute ischemic stroke using endovascular therapy (ET) has been associated with better outcome. However, previous studies were based on less-effective first-generation devices, and analyzed only dichotomized disability outcomes, which may underestimate the full...
journal_title:Annals of neurology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1002/ana.24474
更新日期:2015-10-01 00:00:00
abstract:OBJECTIVE:To examine changes in the response properties of meningeal nociceptors that might lead to migraine pain and examine endogenous processes that could play a role in mediating them using a clinically relevant model of migraine triggering, namely infusion of the nitric oxide (NO) donor nitroglycerin (NTG). METHO...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.23873
更新日期:2013-06-01 00:00:00
abstract:OBJECTIVE:Intracellular amyloid β-protein (Aβ) contributes to neurodegeneration in Alzheimer disease (AD). Apomorphine (APO) is a dopamine receptor agonist for Parkinson disease and also protects against oxidative stress. Efficacy of APO for an AD mouse model and effects of APO on cell cultures are studied. METHODS:Th...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.22319
更新日期:2011-02-01 00:00:00
abstract::Measurement of conduction velocity along the H reflex arc was used to study sensorimotor peripheral nerve function in diabetic patients during short- and long-term improvement of hyperglycemia. In ten type I diabetics a slight (p less than 0.05) conduction increase occurred after 6 hours of normal glycemia induced by ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410160204
更新日期:1984-08-01 00:00:00
abstract::Recent neuroepidemiological studies of endemic tropical spastic paraparesis (TSP) have confirmed the existence of high-prevalence foci in several tropical islands, including Jamaica and Martinique in the Caribbean, Tumaco off the Pacific coast of Colombia, and the Seychelles in the Indian Ocean. There is a net prepond...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410230728
更新日期:1988-01-01 00:00:00
abstract::Six polypeptides resolved by two-dimensional electrophoresis of homogenates from human skeletal muscle have been identified as tropomyosin by electrophoretic and immunochemical methods. The 6 proteins are consistently present in approximately the same abundance in normal biceps, deltoid, gastrocnemius, and quadriceps ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410180211
更新日期:1985-08-01 00:00:00
abstract:OBJECTIVE:The aim of the study was to evaluate whether the visual analysis of muscle magnetic resonance imaging scans can identify specific patterns of muscle involvement. METHODS:We assessed scans from 83 patients with muscle disorders characterized by rigidity of the spine secondary to mutations in 4 different genes...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21846
更新日期:2010-02-01 00:00:00
abstract::Stereotaxic injection of kainic acid into rat striatum produces neuronal degeneration and neurochemical alterations resembling Huntington's disease (HD). Since correction of the deficiency in gamma-aminobutyric acid (GABA) in HD may be of therapeutic value, the efficacy of drugs that inhibit the GABA-degrading enzyme ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410020407
更新日期:1977-10-01 00:00:00
abstract::The site of autonomic deficit was investigated in 4 patients with loss of thermoregulatory flushing and sweating on one side of the face but no associated miosis (harlequin syndrome). In 2 patients the sudomotor deficit included the hand. Signs of postganglionic sympathetic deficit included pathological lacrimal sweat...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410340610
更新日期:1993-12-01 00:00:00
abstract::A 51-year-old man presented with a six-year history of gradually progressive exertional dyspnea. His complaint was attributed to increasing age, but evaluation finally led to a diagnosis of diaphragmatic paralysis. Neurological examination disclosed only minimal evidence of weakness of limb muscles. Electromyographic ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410090618
更新日期:1981-06-01 00:00:00
abstract::Fourteen patients with cauda equina syndrome secondary to long-standing ankylosing spondylitis are described. The roughly symmetrical neurological deficits were very slowly progressive and began long after the onset of the spondylitis, usually well after the rheumatological symptoms had stopped. Eventually every patie...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410140610
更新日期:1983-12-01 00:00:00
abstract::We report the clinical, imaging, and laboratory features of 8 patients with Devic's neuromyelitis optica. All patients had severe myelopathy and optic neuritis. In no patient was the brain, the brainstem, or the cerebellum affected, even after several years of disease. Various immunosuppressive treatments failed to be...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410340211
更新日期:1993-08-01 00:00:00
abstract::We present our approach to the diagnosis of pediatric neurotransmitter diseases exemplified by the differential diagnosis of children presenting with dystonia. This approach is based upon the primary aim of early diagnosis of treatable conditions and the need for a logical series of investigations. We have tried to be...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.10628
更新日期:2003-01-01 00:00:00
abstract::Protease-resistant prion protein, total prion protein, and glial fibrillary acidic protein were measured in various brain regions from 9 subjects with fatal familial insomnia. Six were homozygotes methionine/methionine at codon 129 (mean duration, 10.7 +/- 4 months) and 3 were heterozygotes methionine/valine (mean dur...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410380107
更新日期:1995-07-01 00:00:00
abstract::Brainstem auditory evoked responses (BAERs) were recorded from 202 patients with definite, probable, or possible multiple sclerosis (MS). Definitions of abnormality were based only on interwave separations and the wave I/wave V amplitude ratio. Thirty-two percent of the patients had abnormal BAERs, and the presence of...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410070208
更新日期:1980-02-01 00:00:00
abstract::To evaluate the proportion of cases of myoglobinuria that can be ascribed to specific metabolic defects, we have studied eight enzymes--phosphorylase, phosphorylase kinase, phosphofructokinase (PFK), phosphoglycerate kinase (PGK), phosphoglycerate mutase (PGAM), lactate dehydrogenase (LDH), carnitine palmitoyltransfer...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410270214
更新日期:1990-02-01 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::In vivo phosphorus nuclear magnetic resonance spectroscopy (31P NMR) was used to evaluate the pattern of phosphate compounds in seven newborn babies (mean gestational age, 32 weeks; birth weight, 1,430 gm; age, 37 days) with a history of perinatal asphyxia. Spectra were collected in a 1.9 Tesla superconductive magnet ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410160509
更新日期:1984-11-01 00:00:00
abstract::Magnetic resonance imaging is a highly sensitive method for the detection of the lesions of multiple sclerosis and renders possible the study and the evolution of early lesions. Previous reports on magnetic resonance imaging following gadolinium-diethylenetriamine pentaacetic acid (Gd-DTPA) injection demonstrated that...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410340507
更新日期:1993-11-01 00:00:00
abstract::Intravenous bolus administration of oxygen 15-labeled water and positron emission tomography were used to measure changes in brain blood flow induced by two modes of photic stimuli over a wide range of repetition rates. These stimuli (patterned-flash and reversing checkerboard) were chosen in order to determine whethe...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410170315
更新日期:1985-03-01 00:00:00
abstract:OBJECTIVE:Accumulation of mitochondrial DNA (mtDNA) damage has been associated with aging and abnormal oxidative metabolism. We hypothesized that in human immunodeficiency virus-associated sensory neuropathy (HIV-SN), damaged mtDNA accumulates in distal nerve segments, and that a spatial pattern of mitochondrial dysfun...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.22150
更新日期:2011-01-01 00:00:00