bluuebunny/crossref_metadata_2025_split huggingface dataset:
Validation of clinical diagnostic criteria for Alzheimer's disease
Authors: Morris John C. , McKeel Daniel W. , Fulling Keith , Torack Richard M. , Berg Leonard 
Published: January 2005

AbstractClinical investigations of Alzheimer's disease (AD) have been limited by diagnostic inaccuracy. We employed explicit clinical inclusion and exclusion criteria to identify subjects with senile dementia of the Alzheimer type (SDAT). In a consecutive series of 26 postmortem examinations from this sample, AD was histologically verified in all subjects and was the priMarchy dementing illness. Seventeen of the 26 SDAT subjects had been diagnosed when only mildly demented. Two control subjects were examined neuropathologically; AD was absent in both. We conclude that research clinical diagnostic criteria for SDAT, even in its mild stage, are valid.
Neurological basis of respiratory complications in achondroplasia
Authors: Nelson F. William , Hecht Jacqueline T. , Horton William A. , Butler Ian J. , Goldie William D. , Miner Michael 
Published: January 2005

AbstractEvaluation of 32 individuals with achondroplasia revealed that 28% had a history of apnea and 22% had respiratory abnormalities on polysomnography. In those patients requiring posterior fossa Decemberompressive surgery, improvement was noted in follow‐up polysomnograms. Multimodality studies suggested that brainstem compression was common in achondroplasia and could account in part for the abnormal respiratory function in this disorder, including obstructive apnea, central apnea, and hypoxemia.
Prediction of intracerebral hemorrhage survival
Authors: Tuhrim Stanley , Dambrosia James M. , Price Thomas R. , Mohr Jay P. , Wolf Philip A. , Heyman Albert , Kase Carlos S. 
Published: January 2005

AbstractThe Pilot Stroke Data Bank obtained information on 94 patients with intracerebral hemorrhage. These data were used to identify factors predictive of 30‐day outcome from among 85 demographic, historical, clinical, and laboratory variables generally available to clinicians on the day of admission. The 9 univariate factors statistically associated with outcome were Glasgow Coma Scale score, systolic blood pressure, pulse pressure, horizontal and vertical gaze palsies, severity of weakness, presence of brainstem‐cerebellar deficits, interval stroke course, and parenchymal hemorrhage size. Beginning with these factors, a step‐down variable selection procedure was used to derive a logistic regression model, containing only Glasgow Coma Scale score, pulse pressure, and hemorrhage size, that could be used to categorize correctly 92% of the patients as alive or dead at 30 days after onset.
Activated t‐lymphocytes with polyclonal gammopathy in patients with human t‐lymphotropic virus type i—associated myelopathy
Authors: Mori Masataka , Kinoshita Kenichiro , Ban Nobutaro , Yamada Yasuaki , Shiku Hiroshi 
Published: January 2005

AbstractHuman T‐lymphotropic virus type I (HTLV‐1)‐associated myelopathy (HAM) has been shown to be closely related to HTLV‐I infection. However, the mechanism by which this disease occurs after infection with HTLV‐I is still obscure. We found that HAM patients have unusually high proportions of CD4+HLADR+ cells, CD8+HLA‐DR+ cells, OKT9+ cells, and CD38(0KTlO)+ cells in their peripheral blood, all of which suggest the presence of activated T‐lymphocytes. Antibody titer against HTLV‐I was much higher in HAM patients than in HTLV‐I carriers without evident neurological disease (p < 0.01). Polyclonal gammopathy was also observed in most HAM patients, and 6 of 10 patients were positive for rheumatoid factor. These observations, coupled with the previous observation that corticosteroid therapy improves clinical symptoms in some patients, make it likely that continuous activation of the immune system, initiated by HTLV‐I infection, plays a key role in the pathogenesis of HAM.
The corpus callosum is larger with right‐hemisphere cerebral speech dominance
Authors: O'Kusky John , Strauss Esther , Kosaka Brenda , Wada Juhn , Li David , Druhan Marchgaret , Petrie Julie 
Published: January 2005

AbstractVariations in the size of the human corpus callosum were examined as a possible morphological substrate of functional asymmetries of the cerebral hemispheres, such as cerebral speech dominance. The midsagittal surface area of the corpus callosum, obtained by magnetic resonance imaging, was measured in 50 patients with epilepsy and 50 neurologically normal control subjects. The mean callosal area did not differ significantly between patients and control subjects, between left‐handed and right‐handed subjects, or between men and women. When measurements were compared among 44 patients, whose cerebral speech dominance had been determined by the intracarotid injection of sodium amytal, the area of the corpus callosum was significantly greater in patients with right‐hemisphere cerebral speech dominance. The mean callosal area was greater by 109 to 159 square millimeters (18–28%) when compared to that of patients with either left‐hemisphere speech dominance or bilateral speech representation. This difference in midsagital surface area could represent as many as 37 to 54 million additional callosal axons in subjects with right‐hemisphere celebral speech dominance.
Alz 50 recognizes abnormal filaments in Alzheimer's disease and progressive supranuclear palsy
Authors: Tabatonm Massimo , Whitehouse Peter J. , Perry George , Davies Peter , Autilio‐Gambetti Lucila , Gambetti Pierluigi 
Published: January 2005

AbstractThe monoclonal antibody Alz 50 recognizes a 68‐kDa protein present in large amounts in cerebral tissue of patients with Alzheimer's disease (AD). We used light and electron microscopic as well as quantitative immunochemical techniques to compare distribution, characteristics, and amount of Alz 50 antigen in the tegmental nuclei of the pons of subjects with progressive supranuclear palsy (PSP) and subjects with AD. In both condtions Alz 50 antigen was found in neurons with and without neurofibrillary tangles (NFT). In NFT‐bearing neurons the antigen was located in the filamentous components of NFT in both diseases, i.e., in straight filaments forming the NFT in PSP and in paired helical filaments components of the NFT in AD. In neurons lacking NFT the antigen was associated with straight filaments of various sizes, but the straight filaments were generally thinner than those of PSP. On immunoblots of both PSP and AD tissues, Alz 50 recognized a set of bands between 68 and 35 kDa which were not present in control tissue. The amount of Alz 50 antigen in AD tissue was estimated to be 10 times higher than that present in PSP tissue. The higher amount of Alz 50 ancigen in AD tissue correlates with the more severe fibrillary pathology in this disease than in PSP. We propose that the Alz 50 antigen, although possibly related to tau proteins, distinguishes neurons that are forming abnormal filaments.
Evidence for retrograde degeneration of epinephrine neurons in Alzheimer's disease
Authors: Burke William J. , Chung Hyung D. , Huang Jung S. , Huang Shuan S. , Haring John H. , Strong Randy , Marchshall Gary L. , Joh Tong H. 
Published: January 2005

AbstractAlzheimer's disease (AD) is associated with a progressive loss of locus ceruleus neurons. These noradrenergic neurons receive a major afferent projection from epinephrine neurons in epinephrine cell groups in the brainstem. The epinephrine neurons have a specific enzymatic Marchker, phenylethanolamine N‐methyltransferase (PNMT), which allows them to be identified chemically and immunohistochemically. We have previously reported a Decemberrease in PNMT in brains of patients with AD. We now report that the Decemberrease in PNMT activity in projections to the locus ceruleus is not due to the loss of epinephrine neurons, although up to 33% of these neurons are atrophic. The Decemberrease in presynaptic PNMT does, however, correlate with the loss of postsynaptic locus ceruleus neurons in brains from AD patients. The percentage of degenerating neurons in the epinephrine nuclei also correlates significantly with the amount of loss of locus ceruleus neurons in AD. In addition, there is a 55% Decemberrease in mitogen activity, a nonspecific measure of growth or maintenance factors, in dialysed locus ceruleus extracts from the AD patients compared to those from control subjects. The mitogen activity in the locus ceruleus was significantly correlated with PNMT activity and with the density of locus ceruleus neurons in all cases examined. These findings provide evidence for the hypothesis that retrograde degeneration is a mechanism of neuronal degeneration in AD and suggest that trophic factors may play a role in this process.
Immunohistochemical verification of senile and kuru plaques in Creutzfeldt‐Jakob disease and the allied disease
Authors: Kitamoto Tetsuyuki , Tateishi June , Sato Yuji 
Published: January 2005

AbstractWe investigated paraffin‐embedded brain sections from 41 patients with Creutzfeldt‐Jakob disease (CJD) and from 9 with Gerstmann‐Sträussler syndrome (GSS) using anti—human prion protein (PrP) antisera (anti—GSS kuru plaque cores and anti—PrP synthetic peptide) and anti—β protein antiserum. The anti—human PrP antiserum reacted with the plaques in CJD and GSS, with or without degenerative neurites (neuritic components). In addition, the anti—β protein antiserum immunolabeled kuru plaque—like compact plaques in some cases of CJD. Therefore, previous morphological classifications of the plaques may not always be valid. Senile plaques labeled with anti—β protein antiserum were evident in 65% of the CJD brains and 50% of GSS brains from patients in their 60s, and in 73% of brains from CJD patients in their 70s, but not in brains from patients under 60 years of age. The incidence of the senile plaques was compatible with the normal aging process and was apparently not accelerated by the disease process of CJD or GSS. These immunostaining approaches using anti—human PrP and anti—β protein antisera allow classification of plaque types and increase the reliability of the pathological diagnosis in persons with dementia.
Rate of cell death in parkinsonism indicates active neuropathological process
Authors: McGeer Patrick L. , Itagaki Shigeru , Akiyama Haruhiko , McGeer Edith G. 
Published: January 2005

AbstractIt has been hypothesized that idiopathic parkinsonism might be due to age‐related attrition of dopamine neurons occurring long after an initial acute episode. We present evidence against this hypothesis, based on our finding of at least six times as many HLA‐DR—positive microglia phagocytosing dopamine neurons in parkinsonian brains as in control brains. This difference indicates an active pathological process.
Hypertrophic brachial plexus neuritis: A pathological study of two cases
Authors: Cusimano Michael D. , Bilbao Juan M. , Cohen Sandra M. 
Published: January 2005

AbstractTwo patients with brachial plexus neuropathy had recurrent symptoms for years that culminated in the development of a Painful lower cervical mass. On macroscopic examination the affected nerves had a fusiform segmental enlargement, suggestive of a nerve sheath tumor, that was excised. Histologically, Marchked endoneurial edema, florid focal chronic inflammation, extensive onion bulb formation, and perineurial sparing were seen. Electron microscopic studies on one patient revealed microvasculitis; frequent tubuloreticular inclusions in endothelial cells, histiocytes, and lymphocytes; and cylindrical confronting cisternae in lymphocytes. We conclude that some cases of recurrent brachial plexus neuropathy are due to a localized chronic inflammation that may be related to autoimmunity or to a viral infection.
Abnormalities of peripheral nerve in patients with human immunodeficiency virus infection
Authors: Mah Vei , Vartavarian Luiza M. , Akers Marchi‐Anne , Vinters Harry V. 
Published: January 2005

AbstractThe purpose of this study was to assess systematically morphology of peripheral nerves from patients with human immunodeficiency virus infection (acquired immunodeficiency syndrome [AIDS] and AIDS‐related complex) examined at autopsy. Sural nerve specimens were taken from 25 patients (mean age 44 years) and evaluated by routine procedures used in our laboratory. In 13 cases no detectable abnormality was seen. Twelve patients (48%) showed loss of myelinated fibers with disproportionately greater loss of large myelinated fibers. Three of these patients showed severe myelinated fiber loss; 2 had no documented symptoms and no other known predisposing factors for a peripheral neuropathy. Changes suggestive of wallerian degeneration were occasionally seen, as were epineurial and endoneurial inflammatory infiltrates. Segmental demyelination was not identified in any nerve examined. Electron microscopy revealed thickened basement membranes around small blood vessels, Schwann cells, and fibroblasts. Peripheral nerve abnormalities in patients with AIDS or ARC are frequent and their pathogenesis remains unclear.
The glucose transporter of the human brain and blood‐brain barrier
Authors: Kalaria Rajesh N. , Gravina Stephen A. , Schmidley James W. , Perry George , Harik Sami I. 
Published: January 2005

AbstractWe identified and characterized the glucose transporter in the human cerebral cortex, cerebral microvessels, and choroid plexus by specific D‐glucose‐displaceable [3H]cytochalasin B binding. The binding was saturable, with a dissociation constant < 1 μM. Maximal binding capacity was ∽ 7 pmol/mg protein in the cerebral cortex, ∽ 42 pmol/ mg protein in brain microvessels, and ∽ 27 pmol/mg protein in the choroid plexus. Several hexoses displaced specific [3H]cytochalasin B binding to microvessels in a rank‐order that correlated well with their known ability to cross the blood‐brain barrier; the only exception was 2‐deoxy‐D‐glucose, which had much higher affinity for the glucose transporter than the natural substrate, D‐glucose. Irreversible photoaffinity labeling of the glucose transporter of microvessels with [3H]cytochalasin B, followed by solubilization and polyacrylamide gel electrophoresis, labeled a protein band with an average molecular weight of ∽ 55,000. Monoclonal and polyclonal antibodies specific to the human erythrocyte glucose transporter immunocytochemically stained brain blood vessels and the few trapped erythrocytes in situ, with minimal staining of the neuropil. In the choroid plexus, blood vessels did not stain, but the epithelium reacted positively. We conclude that human brain microvessels are richly endowed with a glucose transport moiety similar in molecular weight and antigenic characteristics to that of human erythrocytes and brain microvessels of other mammalian species.
Acetylcholinesterase‐rich pyramidal neurons in the human neocortex and hippocampus: Absence at birth, development during the life span, and dissolution in Alzheimer's disease
Authors: Mesulam M.‐Marchsel , Geula Changiz 
Published: January 2005

AbstractAcetylcholinesterase‐rich pyramidal neurons in the human association neocortex and hippocampal formation are virtually absent early in life, become established by adolescence, and appear to increase in density during adulthood and perhaps even senescence. Analogous neurons are not detectable in the adult monkey brain. This Novemberel class of neurons may represent a uniquely human adaptation in primate evolution and may provide a neuroanatomical substrate for the mental development that occurs during the adult stages of life. These phylogenetically and ontogenetically progressive neurons are also Marchkedly vulnerable to degeneration in Alzheimer's disease.
Transverse sinus thrombosis in newborns: Clinical and magnetic resonance imaging findings
Authors: Baram Tallie Z. , Butler Ian J. , Nelson Marchvin D. , McArdle Craig B. 
Published: January 2005

AbstractNeonatal transverse sinus thrombosis (TST) is considered a rare and severe sequela of birth injury. Clinical descriptions of this entity are few since most published series are postmortem studies. The advent of magnetic resonance imaging (MRI) allows recognition of TST ante mortem. We describe 4 full‐term infants with distinct clinical and neuroradiological features indicative of TST, which we suggest may be relatively common, with a wide spectrum of severity, including favorable outcome.
Serial magnetic resonance scanning in multiple sclerosis: A second prospective study in relapsing patients
Authors: Willoughby E. W. , Grochowski E. , Li D. K. B. , Oger J. , Kastrukoff L. F. , Paty D. W. 
Published: January 2005

AbstractA prospective study of serial magnetic resonance (MR) scans of the brain was carried out every 2 weeks for 4 to 6 months in 9 patients with mild, clinically definite, relapsing/remitting multiple sclerosis (MS). Six of the 9 patients developed a total of 12 asymptomatic new lesions in various parts of the brain. In none of the patients were the changes on MR scan accompanied by relevant new neurological symptoms or signs. New MR lesions had a characteristic temporal profile, reaching a maximum size in approximately 4 weeks before gradually shrinking, usually leaving a small residual abnormality indistinguishable from chronic MS lesions. The frequent occurrence of new asymptomatic lesions indicates that MS may be a more active process even in mildly affected asymptomatic patients than has been previously realized. The results emphasize the potential importance of using MR scanning to measure disease activity in laboratory studies of MS and in the assessment of treatment, particularly in asymptomatic patients in the early stages. We suggest that the expanding and contracting new lesions are the basic or primary lesion in MS, that the characteristic demyelinated plaque is represented by the small residual area that these lesions shrink down to, and that the typical collection of scattered white matter lesions in chronic MS may represent the accumulated residua of dozens or more of these active lesions occurring over many years.
Cerebrospinal fluid biogenic amines and biopterin in rett syndrome
Authors: Zoghbi Huda Y. , Milstien Sheldon , Butler Ian J. , Smith E. O'Brian , Kaufman Seymour , Glaze Daniel G. , Percy Alan K. 
Published: January 2005

AbstractWe evaluated cerebrospinal fluid biogenic amine metabolites in 32 patients with Rett syndrome. Significant reductions were noted in the metabolites of norepinephrine, dopamine, and serotonin, as compared with controls of similar age (p < 0.00l, p < 0.001, and p < 0.05, respectively). Tetrahydrobiopterin is an essential cofactor and may be rate‐limiting for the synthesis of catecholamines and serotonin. Accordingly, total cerebrospinal fluid biopterin was measured and found to be elevated in patients compared with controls (p < 0.002). These findings constitute the only biochemical changes detected in Rett syndrome so far.
Bilateral opercular polymicrogyria
Authors: Becker P. Scott , Dixon Ann M. , Troncoso Juan C. 
Published: January 2005

AbstractFoix‐Chavany‐Marie syndrome (FCMS), or faciopharyngoglossomasticatory diplegia, is an uncommon syndrome that can result from vascular or developmental lesions of the anterior opercula bilaterally. We report the first pathological documentation of the developmental form of this disorder. Pathological examination revealed bilateral failure of opercular closure, opercular polymicrogyria, periventricular gray‐matter heterotopias, and absence of the septum pellucidum.
Carbamazepine and its epoxide: Relation of plasma levels to toxicity and seizure control
Authors: Theodore William H. , Narang P. K. , Holmes Mark D. , Reeves Pat , Nice Frank J. 
Published: January 2005

AbstractWe studied the relation of plasma levels of carbamazepine (CBZ) and carbamazepine 10,11 epoxide (CBZ‐E), and their ratio to drug toxicity and seizure control in 7 patients with complex partial seizures. CBZ‐E/CBZ increased with increasing CBZ levels and was higher when patients were taking phenytoin or valproic acid. There were weak correlations between CBZ, CBZ‐E levels, toxicity scores, and seizure control when patients were taking CBZ alone, but not when other drugs were given as well. There were no significant differences in the correlation with toxicity score or seizure frequency between CBZ, CBZ‐E, or their sum. Measurement of CBZ‐E levels did not provide additional information useful for monitoring clinical response to CBZ therapy.
The effects of a new tissue plasminogen activator analogue, Fb‐Fb‐CF, on cerebral reperfusion in a rabbit embolic stroke model
Authors: Phillips David A. , Fisher Marc , Smith Thomas W. , Davis Michael A. , Pang Roy H. L. 
Published: January 2005

AbstractEarly fibrinolytic therapy with full molecular tissue plasminogen activator (t‐PA) has been observed to be both angiographically and clinically effective when employed in animal stroke models. Preliminary clinical trials with t‐PA are in progress. It is possible to refine t‐PA by developing fragments or analogues of the drug. Using recombinant DNA technology in the Escherichia coli system, a t‐PA analogue consisting of the catalytic fragment of t‐PA and a dimer of the B fragment of staphylococcal protein A (Fb‐Fb‐CF) has been produced. Because this analogue has a long serum half‐life of 90 minutes, we employed Fb‐Fb‐CF in a rabbit cerebral embolic stroke model to assess its efficacy as a reperfusion agent. When given as a bolus to 10 animals 15 minutes after embolization, Fb‐Fb‐CF produced angiographic cerebral reperfusion in 48 ± 21 minutes (± SD), while in 8 saline‐treated controls, reperfusion was not observed at 180 minutes in any animal (p < 0.01). In another experiment reperfusion was demonstrated at 66 ± 32 minutes in 11 animals treated with Fb‐Fb‐CF 90 minutes after embolization as compared with 100 ± 25 minutes in 12 saline‐treated controls (p < 0.01). A small macroscopic hemorrhage within an infarct was seen in 1 Fb‐Fb‐CF‐treated animal in the 15‐minute experiment and in none of the controls. In the 90‐minute experiment, macroscopic hemorrhagic infarction was seen in 4 Fb‐Fb‐CF‐treated animals and in 3 controls. Plasma fibrinogen levels measured in the 15‐minute Fb‐Fb‐CF treatment group declined by 16% immediately after the Fb‐Fb‐CF bolus was administered and by 19% after 180 minutes. These data demonstrate that Fb‐Fb‐CF can rapidly reestablish cerebral perfusion with only modest reductions in plasma fibrinogen when given either 15 or 90 minutes after cerebral embolization.
Peripheral nerve vasculitis: Immune characterization of the vascular lesions
Authors: Kissel John T. , Riethman Joseph L. , Omerza Jennifer , Rammohan Kottil W. , Mendell Jerry R. 
Published: January 2005

AbstractQuantitative immunohistochemical analyses were performed on 22 nerve biopsy specimens from patients with systemic vasculitis (n = 14) or isolated vasculitis of peripheral nerve (n = 8). In the vascular lesions the cellular infiltrates were composed primarily of T cells (71 ± 18%; mean ± SD) and macrophages (27 ± 17%), and the majority of the T cells (65 ± 20%) were cytotoxic/suppressor CD8 cells. B cells were seen in only 4 cases and constituted less than 2% of all cells. Natural killer cells and polymorphonuclear leukocytes were rare, and a leukocytoclastic response was not observed. Fourteen biopsy specimens had vascular deposits of immunoglobulins G and M and complement components C3 and C5b‐9 membrane attack complex, while 4 had only the latter. The fact that the immunoglobulin and complement deposits were seen only in vessels that had corresponding intense cellular infiltrates suggests an important, but perhaps not primary, role for immune complexes in causing the vascular lesions. Statistical analysis revealed striking similarities in the lesions of patients with isolated nerve vasculitis and those with systemic vasculitides, suggesting a common pathogenic mechanism. Collectively, our observations suggest an important role for a T‐cell‐dependent cell‐mediated process as a primary mechanism of vessel injury in peripheral nerve vasculitis.