bluuebunny/crossref_metadata_2025_split huggingface dataset:
The trends in incidence of primary brain tumors in the population of rochester, minnesota
Authors: Radhakrishnan Kurupath , Mokri Bahram , Parisi Joseph E. , O'Fallon W. Michael , Sunku Jayasimha , Kurland Leonard T. 
Published: January 2005

AbstractA number of reports have suggested an increasing incidence of primary brain tumors, especially malignant astrocytomas, in the elderly population. To investigate this issue, we analyzed the incidence and temporal trends of primary intracranial neoplasms diagnosed in the population of Rochester, Minnesota, over the 40 years between 1950 and 1990. The incidence of symptomatic primary brain tumors (excluding patients diagnosed incidentally at autopsy and by neuroimaging studies) increased from 9.5 per 100,000 population per year in 1950 to 1969 to 12.5 per 100,000 per year in 1970 to 1989; this change was not statistically significant (χ2 trend, 1.89; p = 0.17). While the incidence of pituitary adenomas increased significantly between the two periods (χ2 trend, 4.44;p = 0.04), the incidence trends of all gliomas, malignant astrocytomas, and meningiomas showed no change among persons younger than 65 years as well as those 65 years and older. The number of patients incidentally found to have neoplasms by neuroimaging studies increased in the recent 20‐year period (χ2 trend, 4.08;p = 0.04). The average age‐ and sex‐adjusted incidence rates per 100,000 per year during the study period in the population of Roshester, Minnesota, for symptomatic tumors were 5.0 for all gliomas, 3.3 for malignant astrocytomas, 2.0 for meningiomas, and 2.4 for pituitary adenomas. In conclusion, our data indicate that the reported increase in the incidence of primary brain tumors is an artifact of improvement in diagnostic technology and practice.
Regional cerebral blood flow changes in motor cortical areas after transient anesthesia of the forearm
Authors: Sadato Norihiro , Zeffiro Thomas A. , Campbell Gregory , Konishi Junji , Shibasaki Hiroshi , Hallett Mark 
Published: January 2005

AbstractTo study the effect of deafferentation on cortical areas activated by movement of the proximal muscles, we measured regional cerebral blood flow with positron emission tomography and 15O‐labeled water. Flexion‐extension movements of the right elbow before deafferentation were associated with an increase of regional cerebral blood flow in the primary sensorimotor area bilaterally, posterior supplementary motor area bilaterally, ipsilateral cerebellum, contralateral putamen, premotor area, anterior cingulate area, and posterior parietal region. Transient anesthesia of the right forearm induced by ischemic block caused an increase of regional cerebral blood flow in the primary sensorimotor area bilaterally at rest, but there was no change of regional cerebral blood flow with movement, indicating that the movement‐related change in cerebral blood flow was reduced. These findings are consistent with increased excitability of neurons as a result of deafferentation. In the supplementary motor area, anesthesia did not induce any change in regional cerebral blood flow at rest, but there was a decline with movement, again indicating a reduction of the change in cerebral blood flow related to movement. This might be due to a reduction in sensory feedback because of the anesthesia.
Comparative study of selegiline plus L‐dopa–carbidopa versus L‐dopa–carbidopa alone in the treatment of parkinson's disease
Authors: Brannan Timothy , Yahr Melvin D. 
Published: January 2005

AbstractThe long‐term effect of selegiline (L‐deprenyl) in the treatment of Parkinson's disease has not been clearly delineated. We report on a group of patients whose treatment was initiated with selegiline (n = 43) and then subsequently included L‐dopa‐carbidopa (Sinemet) and in whom an extended period of observation was carried out; they are compared to a group of patients whose treatment consisted of L‐dopa‐carbidopa alone (n ' 39). In each, serial observations of the parkinsonian state and the response to treatment on a yearly basis for a period of 5 years were performed. No significant difference in the Hoehn‐Yahr stage or in the motor subscores of tremor, rigidity, bradykinesia, and gait‐posture was found between the two groups, nor was there a significant difference in the incidence of fluctuating responses or dyskinesias. The group that received combination therapy required less L‐dopa than did the group that received L‐dopa‐carbidopa alone during the first 3 years of treatment and a similar trend was evident in years 4 to 5. We conclude that minimal benefits accrued to the parkinsonian patients from long‐term use of selegiline. No clinical evidence to support the claim of “neuroprotective” properties was found. Selegiline's major usefulness is to modify the fluctuating therapeutic response seen with L‐dopa‐carbiodopa.
Detection of human T‐lymphotropic virus type I (HTLV‐I) tax RNA in the central nervous system of HTLV‐I–associated myelopathy/tropical spastic paraparesis patients by in situ hybridization
Authors: Lehky Tanya J. , Levin Michael C. , Flerlage Nick , Jacobson Steven , Fox Cecil H. , Koenig Scott , Izumo Shuji , Sato Elichi , Osame Mitsuhiro , Raine Cedric S. 
Published: January 2005

AbstractAutopsy specimens from 3 patients with human T‐lymphotropic virus (HTLV‐I)–associated myelopathy/tropical spastic paraparesis (HAM/TSP) were examined for the presence of HTLV‐I in the central nervous system (CNS). In situ hybridization using an HTLV‐I tax RNA probe detected cells containing HTLV‐I RNA in spinal cord and cerebellar sections. HTLV‐I infected cells were located within the white matter and, in particular, within the anterior and lateral funiculi of the spinal cord. Consistent with previously described HAM/TSP pathology, there were perivascular infiltrates in these CNS specimens. Significantly, HTLV‐I RNA was not localized to these infiltrates but was detected deeper within the neural tissue. Furthermore, phenotypic analysis demonstrated that at least some of the infected cells were astrocytes. While previous polymerase chain reaction studies have demonstrated the presence of proviral HTLV‐I in CNS specimens, here we provide evidence for the in situ expression of HTLV‐I RNA in the CNS of HAM/TSP patients.
Experimental pneumococcal meningitis: Cerebrovascular alterations, brain edema, and meningeal inflammation are linked to the production of nitric oxide
Authors: Koedel Uwe , Bernatowicz Andrea , Paul Robert , Frei Karl , Fontana Adriano , Pfister Hans‐Walter 
Published: January 2005

AbstractWe investigated whether treatment with the nitric oxide synthase inhibitor N‐nitro‐L‐arginine (L‐NA) and the free radical scavenger superoxide dismutase influences cerebral blood flow changes, brain edema, and cerebrospinal fluid pleocytosis in early experimental pneumococcal meningitis. Compared to untreated infected rats, superoxide dismutase given 3 hours after infection significantly attenuated the increase of brain water content, intracranial pressure, and cerebrospinal fluid white blood cell count, but did not modulate the increase in regional cerebral blood flow. N‐Nitro‐L‐arginine treatment (5 mg/kg intravenously, followed by 5 mg/kg/hour) reversed the increase in regional cerebral blood flow; prevented an increase in brain water content, intracranial pressure, and cerebrospinal fluid nitrite concentrations; and reduced cerebrospinal fluid white blood cell count. With a closed cranial window preparation, N‐nitro‐L‐arginine prevented pneumococci‐induced dilatation of pial arterioles. When the effective dose was increased twofold, the effects of N‐nitro‐Lvarginine became more pronounced but resulted in the death of 4 of 5 rats, probably due to hemodynamic side effects. In primary cultures of rat cerebral endothelial cells, nitrite concentrations increased after pneumococcal stimulation, which could be prevented by NvnitrovLvarginine and cycloheximide. These data suggest that (a) nitric oxide accounts for regional cerebral blood flow changes and pial arteriolar dilatation in the early phase of experimental pneumococcal meningitis; (b) both superoxide radical and nitric oxide are involved as mediators of brain edema and meningeal inflammation; and (c) cerebral endothelial cells can be stimulated by pneumococci to release nitric oxide presumably via the inducible nitric oxide synthase.
Positron emission tomography and magnetic resonance spectroscopy of cerebral glycolysis in children with congenital lactic acidosis
Authors: Duncan D. B. , Herholz K. , Kugel H. , Roth B. , Ruitenbeek W. , Heindel W. , Wienhard K. , Heiss W.‐D. 
Published: January 2005

AbstractCongenital lactic acidosis with neurological symptoms may be due to a variety of disorders of energy metabolism. We investigated whether positron emission tomography (PET) and proton magnetic resonance spectroscopy (1H MRS) are capable of demonstrating specific changes to facilitate diagnosis. A corresponding increase of cerebral lactate (with MRS) and rate of glycolysis (with PET) was observed in 2 children with biochemical evidence of defective mitochondrial respiration. No such increase was noted in a child with lactic acidosis due to stress and exercise but normal respiratory chain activity, and in a control case with an epilepsy syndrome without evidence of primary changes of energy metabolism. The results suggest that defects of oxidative phosphorylation may cause a massive increase of glycolysis to cover energy requirements, with corresponding accumulation of lactate in brain tissue. This mechanism can now be demonstrated in vivo and, with further experience, may potentially be used as a diagnostic marker of respiratory chain disorders in brain tissue.
Early‐onset ataxia with cardiomyopathy and retained tendon reflexes maps to the friedreich's ataxia locus on chromosome 9q
Authors: Palau Francisco , de Michele Giuseppe , Vilchez Juan J. , Pandolfo Massimo , Monrós Eugègnia , Cocozza Sergio , Smeyers Patricia , Lopez‐Arlandis José , Campanella Giuseppe , di Donato Stefano , Filla Alessandro 
Published: January 2005

AbstractAbsence of lower limb tendon reflexes has been considered an essential diagnostic criterion for Friedreich's ataxia (FA). However, preservation of knee and ankle jerks has been reported in a few patients. Linkage analysis to FA locus (FRDA) on chromosome 9q13‐21.1 was performed in 11 patients from 6 families with FA phenotype, including cardiomyopathy, but retained reflexes (FARR). A maximal lod score of 3.38 at recombination fraction theta equal to 0.00 was obtained demonstrating that FARR maps to the FRDA locus. These results suggest that FARR is a variant phenotype of FA.
Human immunodeficiency virus type 1 tat activates non—N‐methyl‐D‐aspartate excitatory amino acid receptors and causes neurotoxicity
Authors: Magnuson David S. K. , Knudsen Bodo E. , Geiger Jonathan D. , Brownstone Robert M. , Nath Avindra 
Published: January 2005

AbstractThe human immunodeficiency virus type 1 (HIV‐1) protein Tat is known to be released from HIV‐1‐infected cells. We show that micromolar concentrations of Tat depolarized young rat and adult human neurons. In addition, Tat, at similar concentrations, was toxic to human fetal neurons in culture. Tat‐induced responses were insensitive to the Na+ channel blocker tetrodotoxin, suggesting a direct effect of Tat on neurons. Tat‐induced depolarizations and cytotoxicity were blocked by the excitatory amino acid antagonist kynurenate. The N‐methylvD‐aspartate receptor antagonist Dv2vaminov5‐phosphonovalerate had little effect on Tatvinduced depolarizations but did provide protection from Tat neurotoxicity. These results suggest that Tat, released from HIVv1vinfected cells, may be an important mediator of neurotoxicity observed in HIV1 encephalopathy.
The gene for hereditary progressive dystonia with marked diurnal fluctuation maps to chromosome 14q
Authors: Tanaka H. , Endo K. , Tsuji S. , Nygaard T. G. , Weeks D. E. , Nomura Y. , Segawa M. 
Published: January 2005

AbstractHereditary progressive dystonia with marked diurnal fluctuation (HPD) is a childhood‐onset, postural dystonia that is characterized by marked diurnal fluctuation and a dramatic response to levodopa. Recently, the gene for dopa‐responsive dystonia (DRD), an autosomal dominant dystonia showing similarly marked response to levodopa, has been mapped to chromosome 14q. Since HPD and DRD share many clinical characteristics, we have analyzed microsatellite polymorphisms in the region of the DRD locus and obtained a maximal lod score of 2.0 at D14S52 without obligate recombination events in the affected individuals. The results strongly suggest that HPD and DRD are to be caused by mutations in the same gene on the long arm of chromosome 14.
The adhesion molecule and cytokine profile of multiple sclerosis lesions
Authors: Cannella Barbara , Raine Cedric S. 
Published: January 2005

AbstractThe expression of the adhesion molecules, vascular cell adhesion molecule‐1 (VCAM‐1) and intercellular adhesion molecule‐1 (ICAM‐1), and their respective receptors on leukocytes, very late activation antigen‐4 (VLA‐4) and lymphocyte function–associated antigen‐1 (LFA‐1), together with a selection of proinflammatory and immunomodulatory cytokines (interleukin [IL]‐1, IL‐2, IL‐4, IL‐10, tumor necrosis factor‐α [TNF‐α], transforming growth factor‐β [TGF‐β], and interferon‐γ [IFN‐γ]) was examined by immunocytochemistry in multiple sclerosis (MS) lesions of different ages and compared with central nervous system (CNS) tissue from other neurological diseases, both inflammatory and noninflammatory, and normal CNS tissue. These molecules play key roles in lymphocytic infiltration and interactions during tissue inflammation and are in large part normally not expressed by CNS cells. High levels of expression of all the molecules tested were found in MS, particularly in chronic active lesions. Positivity for all molecules was also seen in other neurological diseases, even in noninflammatory conditions. There was some suggestion that the VCAM‐1/VLA‐4 adhesion pathway was expressed at higher levels in chronic MS lesions, while ICAM‐1/LFA‐1 was used more uniformly in lesions of all ages. Of the cytokines examined, there was increased expression of TNF‐α and IL‐4 in MS; this was found to be statistically significant when compared with noninflammatory neurological diseases. The expression of most adhesion molecules and some cytokines was negligible in normal CNS tissue although low‐level reactivity for ICAM‐1 TGF‐β, IL‐4, TNF‐α, and IL‐10 was detected, perhaps indicative of immunoregulatory mechanisms. Microglial cells and astrocytes were the major CNS cell types expressing cytokines. The results indicate a potential in the CNS for widespread induced expression of molecules involved in the inflammatory cascade. No adhesion or cytokine molecule or pattern of expression unusual for MS was apparent.
Basement membrane reduplication and pericyte degeneration precede development of diabetic polyneuropathy and are associated with its severity
Authors: Giannini Caterina , Dyck Peter J. 
Published: January 2005

AbstractIn a recent paper, we showed that the number of endoneurial microvessels per square millimeter and the average luminal area and size distribution of these microvessels are not significantly different in sural nerves of patients with diabetes mellitus as compared to control subjects. Mural area, especially the component due to basement membrane reduplication and cellular debris, was unequivocally increased in diabetes mellitus. Because these latter changes are associated with a decrease in periendothelial cell area, we hypothesized that cellular degeneration, especially of pericytes, may account for basement membrane reduplication and increased frequency of cellular debris. In the present study, we showed that endoneurial microvessels undergo a statistically significant increase in basement membrane area, mural area, and frequency of cellular debris in diabetics without polyneuropathy and an even greater increase in diabetics with polyneuropathy. We also found that duration of diabetes mellitus was significantly associated with area occupied by reduplicated basement membrane and cellular debris, but not with mural and periendothelial area. None of the examined measurements was associated with age. Since the microvessel abnormalities we describe are already present before the development of polyneuropathy and increase with severity of polyneuropathy, it is likely that they reflect functional derangements of pericytes and microvessel function which precede and might be implicated in fiber degeneration.
Cerebrospinal fluid levels of amyloid β‐protein in alzheimer's disease: Inverse correlation with severity of dementia and effect of apolipoprotein e genotype
Authors: Nitsch Roger M. , Rebeck G. William , Deng Meihua , Richardson U. Ingrid , Tennis Marsha , Schenk Dale B. , Vigo‐Pelfrey Carmen , Lieberburg Ivan , Wurtman Richard J. , Hyman Bradley T. , Growdon John H. 
Published: January 2005

AbstractAlzheimer's disease (AD) is characterized by formation in brain of neurofibrillary tangles and of amyloid deposits. The major protein component of the former is τ, while the latter are composed of amyloid β‐peptides (Aβ), which are derived by proteolytic cleavage of the amyloid β‐protein precursor (APP). Both β and various secretory APP derivatives including Aβ and APPs are present in human cerebrospinal fluid (CSF). To investigate whether clinical signs of AD are paralleled by changes in CSF levels of these proteins, we correlated quantitative measures of dementia severity with CSF concentrations of Aβ, of APPs, and of τ We found that levels of Aβ in CSF of AD patients were inversely correlated both to cognitive and to functional measures of dementia severity. In contrast, levels of APPs and of τ did not correlate with dementia severity. Apolipoprotein E (apoE) genotype did not influence CSF levels of Aβ, APPs, or τ, which were similar among AD patients with Apo E ε3/3, ε3/4, and ε4/4 alleles. These data indicate that CSF levels of Aβ decrease with advancing severity of dementia in AD and suggest that they are independent of a patient's Apo E genotype.
Cortical control of saccades
Authors: Pierrot‐Deseilligny Charles , Rivaud Sophie , Gaymard Bertrand , Müri René , Vermersch Anne‐Isabelle 
Published: January 2005

AbstractA scheme for the cortical control of saccadic eye movements is proposed based partly on defects revealed by specific test paradigms in human with discrete lesions. Three different cortical areas are capable of triggering saccades. The frontal eye field disengages fixation, and triggers intentional saccades to visible targets, to remembered target locations, or to the location where it is predicted that the target will reappear (i.e., saccades concerned with intentional exploration of the visual environment). The parietal eye field triggers saccades made reflexively on the sudden appearance of visual targets (i.e., saccades concerned with reflexive exploration of the visual environment). The supplementary eye field is important for triggering sequences of saccades and in controlling saccades made during head or body movement (i.e., saccades concerned with complex motor programming). Three other areas contribute to the preparation of certain types of saccades. The prefrontal cortex (area 46 of Brodmann) plays a crucial role for planning saccades to remembered target locations. The inferior parietal lobule is involved in the visuospatial integration used for calculating saccade amplitude. The hippocampus appears to control the temporal working memory required for memorization of the chronological order of sequences of saccades.
The apolipoprotein E ε2 allele is associated with an increased risk of early‐onset alzheimer's disease and a reduced survival
Authors: van Duijn Cornelia M. , de Knijff Peter , Wehnert Anita , De Voecht Joke , Bronzova Juliana B. , Havekes Louis M. , Hofman Albert , van Broeckhoven Christine 
Published: January 2005

AbstractIt was suggested that in contrast to the E4 allele, the E2 allele of the apolipoprotein E gene (APOE *2) has a protective effect for late‐onset Alzheimer's disease and early‐onset Alzheimer's disease (EOAD). We studied the role of the APOE*2 allele in the pathogenesis of EOAD in a Dutch population‐based study of 175 probable EOAD patients with onset age at or before 65 years and 532 age‐matched controls. In our population, there was no evidence for a protective effect of the APOE*2 allele on the risk of EOAD. However, our data show that among EOAD patients, survival for APOE*2 carriers was significantly reduced. When restricting the analysis to patients ascertained early after diagnosis at a stage of disease when mortality is low, our data suggest an increased risk of EOAD for subjects with APOE2E2, APOE2E3, APOE3E4, and APOE4E4 genotypes.
Hereditary ceruloplasmin deficiency with hemosiderosis: A clinicopathological study of a japanese family
Authors: Morita Hiroshi , Ikeda Shu‐Ichi , Yamamoto Kanji , Morita Sayuri , Yoshida Kunihiro , Nomoto Shozo , Kato Masahiro , Yanagisawa Nobuo 
Published: January 2005

AbstractA hereditary ceruloplasmin deficiency associated with severe iron deposition in visceral organ and brain tissues found on histopathological examination at autopsy is discussed. Three siblings of consanguineous Japanese parents were studied. Their clinical symptoms were progressive dementia, extrapyramidal disorders, cerebellar ataxia, and diabetes mellitus, all of which appeared when they were between 30 and 50 years old. All had serum ceruloplasmin deficiencies and increased serum ferritin concentrations. The dentate nucleus, thalamus, putamen, caudate nucleus, and liver of each one showed low signal intensities on T1‐ and T2‐weighted magnetic resonance images. Examination of the central nervous system revealed severe destruction of the basal ganglia and dentate nucleus, with considerable iron deposition in neuronal and glial cells, whereas the cerebral cortex showed mild iron deposition in glial cells without neuronal involvement. An electron microscopic study with energy‐dispersive x‐ray analysis showed iron depositions in the hepatocytes, of both the neural and glial cells of the brain. We consider this a new disease entity because of the primary ceruloplasmin deficiency.
Cognitive scores in carriers of huntington's disease gene compared to noncarriers
Authors: Foroud Tatiana , Siemers Eric , Kleindorfer Dawn , Bill Danield J. , Hodes M. E. , Norton James A. , Conneally P. Michael , Christian Joe C. 
Published: January 2005

AbstractHuntington's disease (HD) is progressive neurodegenerative disorder recently shown to be due to an excess number of CAG trinucleotide repeats in the 5' translated region of chromosome 4. One of the cardinal features of HD is cognitive decline. While mental deterioration is obvious later in the disease course, the time of its onset is difficult to determine precisely. A sample of at‐risk individuals without signs or symptoms of HD by self‐report was studied. The Wechsler Adult Intelligence Test—Revised and a neurological rating scale were administered. The genotypes of 394 individual were then determined by polymerase chain reaction testing. On all portions of the WAIS‐R test, the mean score of the HD gene carriers was lower than that of the noncarriers. Scores on two of the performance subtests, the digit symbol and the picture arrangement, were significantly different in the two groups, even after the scores from all gene carriers who were diagnosed as affected based on their neurological motor examination were removed. The scores for the gene carriers on the various subtests were negatively correlated with the number of CAG repeats in the expanded HD allele. Such a relationship was not seen with the normal alleles of the noncarriers. Taken together, our results suggest that a deficit in cognitive function is an early finding of HD and that in this patient population, the degree of cognitive deficit is proportional to the number of CAG repeats in the HD allele.