bluuebunny/crossref_metadata_2025_split huggingface dataset:
Common T‐cell receptor Vβ usage in oligoclonal T lymphocytes derived from cerebrospinal fluid and blood of patients with multiple sclerosis
Authors: Lee Soon Jin , Wucherpfennig Kai W. , Brod Staley A. , Benjamin Deborah , Weiner Howard L. , Hafler David A. 
Published: January 2005

AbstractT‐cell populations were investigated in the blood and cerebrospinal fluid of patients with multiple sclerosis and other neurological diseases. Individual T cells were directly cloned from the cerebrospinal fluid and blood before in vitro expansion, and their clonotypes were compared by Southern blot analysis of the rearrangement patterns of their T‐cell receptor β chain and γ chain genes. This allowed the determination of whether two T cell clones shared the same T‐cell receptor and thus arose from identical, clonally expanded (oligoclonal) progenitor T cells. As an extension of previous studies, oligoclonal T‐cell clones were identified in both cerebrospinal fluid and blood populations in 5 of 9 patients with inflammatory demyelinating disease among a total of 486 blood and cerebrospinal fluid T‐cell clones. In contrast, no clonally expanded T‐cell populations were found among a total of 424 clones derived from either blood of 4 normal control subjects or blood and cerebrospinal fluid of 8 patients with other neurological diseases. Analysis of T‐cell receptor Vβ genes among 4 oligoclonal T‐cell populations derived from 3 patients with multiple sclerosis demonstrated common usage of the Vβ12 gene segment. These data suggest that oligoclonal T cells share similar specificities and that clonal expansion may have resulted from specific stimulation by an antigen. Moreover, identical clones between blood and cerebrospinal fluid were observed in 3 of 9 patients with demyelinating disease, thus providing further evidence of an equilibrium between peripheral and central nervous system immune compartments.
Major differences in the dynamics of primary and secondary progressive multiple sclerosis
Authors: Thompson Alan J. , Kermode A. G. , Wicks D. , MacManus D. G. , Kendall B. E. , Kingsley D. P. E. , McDonald W. I. 
Published: January 2005

AbstractIn patients with primary and secondary progressive multiple sclerosis (MS), major differences in the pattern and extent of abnormality on cerebral magnetic resonance imaging (MRI) between the two groups have recently been demonstrated. In the present study, 24 patients, matched for age, sex, duration of disease, and disability, had serial gadolinium diethylenetriaminepentaacetic acid–enhanced MRI over a 6‐month period. The 12 patients in the secondary progressive group had a total of 109 new lesions over this time (18.2 lesions per patient per year) and 87% of these enhanced. Enhancement also occurred within and at the edge of preexisting lesions. In contrast, only 20 new lesions were seen in the primary progressive group (3.3 lesions per patient per year) and only one of these enhanced. There was no difference in the degree of clinical deterioration between the two groups over the 6‐month period. These findings may indicate a difference in the dynamics of disease activity between the two forms of progressive MS, particularly in relation to the inflammatory component of the lesions, and have important implications for the selection of patients and the monitoring of disease activity in therapeutic trials.
Loss of chromosome 22 alleles in human sporadic spinal schwannomas
Authors: Fontaine Bertrand , Hanson Mark P. , Von Sattel Jean Paul , Martuza Robert L. , Gusella James F. 
Published: January 2005

AbstractAcoustic neuromas occur either as sporadic solitary tumors in the general population or as inherited bilateral tumors typically in patients with neurofibromatosis type 2. Loss of heterozygosity for markers on the long arm of chromosome 22 has been reported in both instances, and neurofibromatosis type 2 has been genetically linked to a marker on the long arm of this autosome, suggesting that a unique locus on chromosome 22 is implicated in tumorigenesis of both sporadic and inherited acoustic neuromas. To determine whether the locus for neurofibromatosis type 2 might also be responsible for tumorigenesis of those schwannomas distinct from acoustic neuromas in people without neurofibromatosis type 2, we studied the DNA content of three sporadic spinal schwannomas. In all three, we found loss of heterozygosity for at least three markers on the long arm of chromosome 22, indicating a partial or total monosomy 22 in the tumor. Our results suggest that a locus on chromosome 22 is responsible for tumorigenesis in schwann cells regardless of their location in the central nervous system, and that some other mechanism (genetic or nongenetic) might account for the relative high proportion of schwannomas developing from the eighth cranial nerve.
Heterogeneity of the T‐cell receptor β gene rearrangements generated in myelin basic protein‐specific T‐cell clones isolated from a patient with multiple sclerosis
Authors: Richert John R. , Robinson Bs Eve D. , Johnson Armead H. , Bergman Cynthia A. , Dragovic Ljubo J. , Reinsmoen Nancy L. , Hurley Carolyn Katovich 
Published: January 2005

AbstractSeventeen T‐cell clones derived from the peripheral blood of a patient with multiple sclerosis and reactive with a synthetic peptide corresponding to residues 152–170 of the human myelin basic protein molecule were previously shown to be cytotoxic for myelin basic protein‐coated target cells. Genetic restriction studies have now demonstrated that these clones recognize myelin basic protein in association with human leukocyte antigen DRw13. Studies of the T‐cell receptor β gene rearrangements generated by these clones demonstrated 12 different patterns, as evaluated by Southern blot analysis. Thus, the human T‐cell response to myelin basic protein is exceedingly heterogeneous, even among T cells that recognize the same small fragment of the molecule in association with the same class II restriction element.
BN rats do not reject F344 brain allografts even after systemic sensitization
Authors: Poltorak Maciej , Freed PhD William J. 
Published: January 2005

AbstractEmbryonic brain tissue allografts under many circumstances survive transplantation into the brain. It is generally believed that such grafts will not survive if the host animal is systemically sensitized, by skin grafting or other means, to major histocompatibility complex (MHC) antigens of the donor animal. We have found that F344 brain grafts survive in BN hosts even when the host is systemically sensitized to F344 tissue. Embryonic cerebral neocortex from F344 donors was transplanted into BN host rats (n = 95). Subsequently, the host rats were systemically sensitized with donor skin (n = 25), brain tissue (n = 41), or spleen cells (n = 6) and compared with a control group of rats consisting of allografts with no sensitization or sham procedures (n = 23). Rejection of the transplants in BN rat hosts was not provoked by any of the sensitization methods tested. Minor immunological responses that did not result in rejection were, however, present in many host animals. We did not observe infiltration of W3/13+ T cells and OX8+ cytotoxic lymphocytes in any of the groups. Nevertheless, substantial infiltrations of OX6+ antigen‐presenting cells and W3/25+ helper T cells were present. There was also an extensive enhancement of MHC class I immunoreactivity in parts of the grafted tissue developing within the third ventricle, but not for the same type of graft in the lateral ventricle. This increase of MHC class I expression was not accompanied by infiltration of cytotoxic T cells. Our findings thus suggest that neural graft rejection depends on general genetic susceptibility to immune reactions, particularly experimental allergic encephalomyelitis and not only on disparity between donor and host antigens encoded by the MHC. Moreover, enhancement of MHC class I and class II expression within transplanted tissue does not predict graft rejection.
Putative chromaffin cell survival and enhanced host‐derived TH‐Fiber innervation following a functional adrenal medulla autograft for Parkinson's disease
Authors: Kordower Jeffrey H. , Cochran Elizabeth , Penn Richard D. , Goetz Christopher G. 
Published: January 2005

AbstractWe report heretofore undescribed clinical and histological features of a patient with idiopathic Parkinson's disease who showed marked and persistent motoric benefit from an adrenal medulla autograft for 18 months following grafting. The patient returned to the preoperative level of disability prior to his death 30 months after implantation, the longest survival to date for an adrenal transplant. The graft site was primarily necrotic and large numbers of macrophages were still present at the time of death. A few tyrosine hydroxylase‐immunoreactive cells were found within the graft, providing the first evidence that adrenal medullary cells may be viable and produce catecholamines following human transplantation, A dense network of tyrosine hydroxylase‐immunoreactive terminals and processes was also seen ventral to the implant site and on both of its lateral borders. This response may represent sprouting by residual host dopaminergic neurons mediated by the vigorous response to injury displayed by the host striatum The relative paucity of surviving chromaffin cells, however, provides further evidence that adrenal medulla grafts survive poorly following intracerebral implantation, and factors other than dopamine replacement may be responsible for most of the functional effects observed following chromaffin cell transplantation.
Carbamazepine and phenytoin inhibit somatostatin release from dispersed cerebral cells in culture
Authors: Reichlin Seymour , Mothon Stella 
Published: January 2005

AbstractTo elucidate the mechanism by which carbamazepine lowers somatostatin concentration in cerebrospinal fluid of humans, the effect of carbamazepine on secretion of this peptide was studied in rat cerebral cell cultures. Concentrations of carbamazepine within the therapeutic range (4 × 10−5 M) inhibited spontaneous release of somatostatin and blocked secretory responses to the epileptogen, picrotoxin, and to the cyclic cAMP stimulator forskolin. One of the proposed mechanisms of carbamazepine action is that it binds to adenosine receptors, but in this study, aminophylline, an adenosine antagonist, in a concentration as high as 2.4 × 10−4 M, did not reverse carbamazepine effects. Carbamazepine suppression of picrotoxin, however, was overcome by exposure to veratridine, a sodium channel‐active compound. This finding supports the hypothesis that carbamazepine acts by binding to sodium channels. Phenytoin, another anticonvulsant with many similar properties, also blocked picrotoxin‐induced somatostatin release at a concentration of 10−4 M, and its effects were also reversed by veratridine at a concentration of 10−5 M. These findings clarify the mechanism by which carbamazepine and phenytoin act in epilepsy and trigeminal neuralgia.
In vivo magnetic resonance spectroscopy of brain and muscle in a type of mitochondrial encephalomyopathy (MERRF)
Authors: Matthews P. M. , Berkovic S. F. , Shoubridge E. A. , Andermann F. , Karpati G. , Carpenter S. , Arnold D. L. 
Published: January 2005

AbstractPhosphorus magnetic resonance spectroscopy allows noninvasive measurement of the intracellular phosphate‐containing metabolites and intracellular pH in localized volumes of human muscle and brain in vivo. This technique was used to study 8 patients with a mitochondrial cytopathy (myoclonus epilepsy with ragged red fibers). Phosphorus magnetic resonance spectroscopy of resting gastrocnemius muscle demonstrated significantly increased relative intracellular inorganic phosphate concentrations (p < 0.0005) and decreased phosphocreatine to inorganic phosphate concentration ratios (p < 0.01) in the patients, although only 3 had myopathic signs or symptoms. We propose, therefore, that phosphorus magnetic resonance spectroscopy of resting skeletal muscle is a useful clinical test in evaluation of progressive myoclonus epilepsy. In contrast to results from muscle, however, the relative phosphate metabolite concentrations and intracellular pH in central volumes of the brains of these patients were normal, despite evidence from our previous positron emission tomography studies suggesting that there is diffuse impairment of cerebral oxidative metabolism.
Attention modulates somatosensory cerebral blood flow response to vibrotactile stimulation as measured by positron emission tomography
Authors: Meyer E. , Ferguson S. S. G. , Zatorre R. J. , Alivisatos B. , Marrett S. , Evans A. C. , Hakim A. M. 
Published: January 2005

AbstractIn human primary somatosensory cortex, the cerebral blood flow response to vibrotactile stimulation of the fingers (110 Hz), as measured by positron emission tomography and H215O, was 13% higher (p < 0.025) when the subjects attended to the stimulus, compared to when they were simultaneously engaged in a distraction task. This suggests that the physiological response of a primary cortical area can be modulated by the attentive behavior of the subject.
The erythrocyte‐type glucose transporter in blood vessels of primary and metastatic brain tumors
Authors: Harik Sami I. , Roessmann Uros 
Published: January 2005

AbstractWe previously demonstrated that brain microvessels have a high density of the erythrocyte‐type glucose transporter and suggested this could be used as a marker of cells with “occluding” junctions. Now, we have studied by immunocytochemistry the tissue distribution of the glucose transporter in a variety of primary and metastatic human brain tumors with a monoclonal antibody to the glucose transporter of human erythrocytes. Microvessels in normal brain tissue distant from the tumor, and in brain surrounding the tumor, immunostained strongly for the glucose transporter. Microvessels in primary and metastatic tumors, however, lacked glucose transporter immunoreactivity. Only in the relatively benign juvenile cerebellar astrocytomas was glucose transporter immunoreactivity retained in some of the tumor microvessels. Because a high glucose transporter density is a marker of vessels with barrier properties, our results indicate that the vast majority of brain tumor microvessels do not have an intact blood‐brain barrier.
Epileptogenic effect of hypoxia in the immature rodent brain
Authors: Jensen F. E. , Applegate C. D. , Holtzman D. , Belin T. R. , Burchfiel J. L. 
Published: January 2005

AbstractThe response to cerebral hypoxia/ischemia may be different in the neonate compared to other age groups. An in vivo model was developed in the rat to determine whether there are age‐dependent differences in the effects of hypoxia on electroencephalographic (EEG) activity. EEG recordings were obtained from Long Evans hooded rats deprived of oxygen at five ages: postnatal days 5 to 7, 10 to 12, 15 to 17, 25 to 27, and 50 to 60. Oxygen concentration was varied from 0,2,3, and 4% between animals. EEGs were recorded in all animals before, during, and at 1 hour after exposure to the hypoxic condition and at 1 to 7 days afterward in a subset of animals. All animals were deprived of oxygen until the onset of apnea and bradycardia to 20 to 40percnt; of baseline heart rate values. Hypoxia resulted in isoelectric EEG significantly more frequently in the animals deprived of oxygen at postnatal days 25 to 27 and 50 to 60 than in the younger age groups. A highly significant effect was that the animals deprived at postnatal days 5 to 17 revealed a high incidence of epileptiform EEG activity during hypoxia. In contrast, the older animals exhibited only rare isolated EEG spikes before reaching an isoelectric EEG. The severity of hypoxia‐induced epileptiform EEG changes was highest in the animals subjected to moderately hyoxic conditions (3% and 4percnt; oxygen) at postnatal days 10 to 12. Furthermore, epileptiform changes persisted for hours to days following prolonged episodes of hypoxia in the younger animals. This study demonstrates a unique response of the immature brain to exhibit epileptiform activity during hypoxia.
The time course and magnitude of spontaneous recovery of parkinsonism produced by intracarotid administration of 1‐Methyl‐4‐Phenyl‐1,2,3,6‐tetrahydropyridine to monkeys
Authors: Kurlan Roger , Kim Michael H. , Gash Don M. 
Published: January 2005

AbstractWe studied rhesus monkeys with hemiparkinsonism or bilateral parkinsonism produced by unilateral or bilateral intracarotid administration of 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine. Using a standardized clinical rating scale, 4 hemiparkinsonian monkeys showed a 13 to 56% (mean, 36%) spontaneous improvement during an obsevation period of up to 25 weeks. Generally, recovery leveled off after 14 weeks. Four bilaterally parkinsonian monkeys showed a 5 to 42%(mean, 22percnt;) improvement over a period of up to 30 weeks. Our findings emphasize that spontaneous recovery is a potentially confounding characteristic of this monkey model when used for assessing novel antiparkinsonian therapies.
Fatal disseminated hemorrhagic toxoplasmic encephalitis as the initial manifestation of AIDS
Authors: Wijdicks Eelco F. M. , Borleffs Jan C. C. , Hoepelman Andy I. M. , Jansen Gerard H. 
Published: January 2005

AbstractA 28‐year‐old woman presented with progressive coma after being asymptomatic for 1 year after the demonstration of seropositivity for human immunodeficiency virus and high serum immunoglobulin G toxoplasma titers. Computed tomographic scanning showed multiple rounded cerebral hemorrhages and massive cerebral edema. Postmortem examination disclosed Toxoplasma gondii cysts scattered throughout the brain. An overwhelming fatal toxoplasmosis associated with multiple cerebral hemorrhages may be the first presentation of the acquired immunodeficiency syndrome. We raise the possibility that frequent neuroradiological monitoring may be indicated in asymptomatic human immunodeficiency virus–infected patients with high immunoglobulin G toxoplasma titers.
A controlled trial of propoxyphene and naltrexone in patients with tourette's syndrome
Authors: Kurlan Roger , Majumdar Lisa , Deeley Cheryl , Mudholkar Govind S. , Plumb Sandra , Como Peter G. 
Published: January 2005

AbstractTo investigate the effect of drugs acting on the endogenous opioid system, we studied 10 adults with Tourette's syndrome who received propoxyphene hydrochloride (260 mg/day), naltrexone hydrochloride (50 mg/day), and placebo in a double‐blinded, randomized clinical trial. Using a self‐report scale (Tourette's Syndrome Symptom List), subjects noted a significant (p < 0.04) lessening of tics after treatment with naltrexone when compared with placebo. An improvement in performance on the Trail Making B test, a measure of attention and visuomotor sequencing and planning, occurred after receiving naltrexone when compared with placebo (p < 0.08) or propoxyphene (p < 0.02). The Trail Making B test best discriminated the treatments (p < 0.02, analysis of variance). No other treatment effects were observed for several other measures of tic severity, attentional ability, or obsessive‐compulsive symptoms. Our findings indicate that pharmacological manipulation of the endogenous opioid system does influence symptoms of Tourette's syndrome.
Genetic linkage analysis of nerve growth factor (beta) in familial alzheimer's disease
Authors: Alberts Marchk J. , Pericak‐Vance Margaret A. , Royal Vernice , Bebout Jackie , Gaskell Peter , Thomas John , Hung Wu‐Yen , Clark Chris , Earl Nancy , Roses Allen D. 
Published: January 2005

AbstractRecent studies have not shown linkage of late‐onset (mean age, >60 years) familial Alzheimer's disease (FAD) to the chromosome 21 locus reported linked to earlyonset FAD. Beta nerve growth factor (β‐NGF) has been considered a candidate gene in the pathogenesis and therapy of FAD, based on its localization in the cortex and hippocampus and its ability to enhance the growth and survival of cholinergic neurons. A 1.5‐kb fragment of the β‐NGF gene was used to detect a Bgl II restriction fragment length polymorphism, which was then used for linkage analysis. A total of 30 families (27 late onset) with 147 affected members were studied. Close linkage (θ < 0.03, z < −2.00) of late‐onset FAD with β‐NGF was excluded. Two apparent obligate crossovers between affected members were detected in different autopsy‐confirmed families. Based on these results, β‐NGF is not the gene responsible for late‐onset FAD in the families analyzed.
Na+‐Ca2+ exchanger mediates Ca2+ influx during anoxia in mammalian central nervous system white matter
Authors: Stys Peter K. , Waxman Stephen G. , Ransom Bruce R. 
Published: January 2005

AbstractWhite matter of the mammalian central nervous system suffers irreversible injury after prolonged anoxia, which can result in severe neurological impairment. This type of injury is critically dependent on Ca2+ influx into cells. We present evidence that the Na+, Ca2+ exchanger mediates the majority of the damaging Ca2+ influx into cells during anoxia in white matter. Anoxic injury was studied in the isolated rat optic nerve, and functional recovery was monitored using the compound action potential. Blockers of voltage‐gated Na+ channels (tetrodotoxin and saxitoxin) significantly improved recovery, as did perfusion with zeroNa+ solution; both maneuvers would prevent intracellular [Na+] from rising and thus prevent Ca2+ influx by inhibiting reverse operation of the Na+, Ca2+ exchanger. Direct pharmacological blockade of the Na+, Ca2+ exchanger during anoxia with bepridil or benzamil also significantly improved recovery. These findings suggest that reverse operation of the Na+, Ca2+ exchanger during anoxia is a critical mechanism of Ca2+ influx and subsequent white matter injury.
Short‐chainL‐3‐hydroxyacyl‐CoA dehydrogenase deficiency in muscle: A new cause for recurrent myoglobinuria and encephalopathy
Authors: Tein I. , De Vivo D. C. , Hale D. E. , Clarke J. T. R. , Zinman H. , Laxer R. , Shore A. , Dimauro S. 
Published: January 2005

AbstractWe report on a 16‐year‐old girl with short‐chainL‐3‐hydroxyacyl‐coenzyme A (CoA) dehydrogenase deficiency resulting in juvenile‐onset recurrent myoglobinuria, hypoketotic hypoglycemic encephalopathy, and hypertrophic/dilatative cardiomyopathy. Urinary organic acids showed traces of 3‐hydroxy‐dodecanedioic acids and small amounts of suberic, sebacic, and adipic acids. There was a marked decrease inL‐3‐hydroxyacyl‐CoA dehydrogenase activity in muscle with acetoacetyl‐CoA as substrate (2.48 μmol/min/gm; normal = 6.90 ± 1.80 μmol/min/gm of tissue; n = 11), contrasting with normalL‐3‐hydroxyacyl‐CoA dehydrogenase activity with 3‐ketooctanoyl‐CoA and 3‐ketopalmitoyl‐CoA as substrates. Short‐chainL‐3‐hydroxyacyl‐CoA dehydrogenase activity was normal in fibroblasts, suggesting a tissue‐specific defect.
Presynaptic parkinsonism in olivopontocerebellar atrophy: Clinical, pathological, and neurochemical evidence
Authors: Pascual Julio , Pazos Angel , del Olmo Elena , Figols Javier , Leno Carlos , Berciano José 
Published: January 2005

AbstractThe substrate for olivopontocerebellar atrophy parkinsonism is obscure due to the lack of clinical and pathological reports and the absence of studies on dopamine receptors in this entity. We describe a patient with olivopontocerebellar atrophy whose clinical presentation was levodopa‐responsive parkinsonism in whom pathological examination disclosed pronounced nigral cell loss with no striatal damage. Autoradiographic labeling with 3H‐spiperone showed normal densities of D2 dopamine striatal receptors. These data show that indistinguishable nigral, presynaptic parkinsonism occurs in patients with idiopathic Parkinson's disease and in patients with olivopontocerebellar atrophy, and also how a favorable response to levodopa is neither synonymous with idiopathic Parkinson's disease, nor does it exclude multiple‐system, atrophy‐related parkinsonism.