bluuebunny/crossref_metadata_2025_split huggingface dataset:
Tau antisera recognize neurofibrillary tangles in a range of neurodegenerative disorders
Authors: Joachim Catharine L. , Morris James H. , Kosik Kenneth S. , Selkoe Dennis J. 
Published: January 2005

AbstractNeurofibrillary tangles occur in a number of apparently distinct neurodegenerative diseases and in normal aging of the human brain. Antibodies raised against Alzheimer's disease paired helical filaments immunolabel the tangles seen in all other tangle‐associated disorders examined to date. The neuronal microtubule‐associated protein, tau, has recently been identified as an antigenic component of neurofibrillary tangels and senile plaque neurites in Alzheimer's disease. Three different polyclonal antibodies with strong tau immunoreactivity are examined in this study. These antibodies were found to immunostain tangles in normal aged brain and in brains affected by a range of neurodegenerative disorders, including Down's syndrome, Alzheimer's disease plus Parkinson's disease, progressive supranuclear palsy, and the parkinsonism‐dementia complex of Guam, as well as Pick bodies in Pick's disease. The findings further illustrate the relative nonspecificity of neurofibrillary lesions in neurodegenerative disorders.
Progressive aphasia without dementia: Two cases with focal spongiform degeneration
Authors: Kirshner Howard S. , Tanridag Oguz , Thurman Lawrence , Whetsell William O. 
Published: January 2005

AbstractTwo patients with the syndrome of progressive aphasia without evidence of generalized dementia underwent postmortem neuropathological examinations. In both patients, characteristic changes of Alzheimer's disease, Pick's disease, or Creutzfeldt—Jakob disease were absent. Both patients showed a focal spongiform change involving primarily layer 2 of the left inferior frontal gyrus (and temporal cortex in Patient 1) and a mild astrocytosis in layer 2 and deeper cortical layers. This focal, spongiform cortical degeneration in patients with progressive aphasia does not appear to duplicate any known central nervous system degenerative disease.
Dysmyelination in transgenic mice containing JC virus early region
Authors: Trapp Bruce D. , Small Judy A. , Pulley Michael , Khoury George , Scangos George A. 
Published: January 2005

AbstractJC virus (JCV) causes the chronic human demyelinating disease progressive multifocal leukoencephalopathy. Because of host range restrictions, experimental models of JCV‐induced demyelination have not been available. The restricted tropism of JCV infectivity has recently been overcome by the production of transgenic mice that contain the early region of JCV in all cells. This portion of the DNA encodes JCV T‐antigens. These mice display a dysmyelinating phenotype, the severity of which is related to the level of JCV early region expression in brain. With the use of immunocytochemistry and in situ hybridization, we characterized morphologically myelin‐specific and JCV gene expression in a severely affected strain of these mice. Our results suggest that expression of JCV T‐antigens occurs predominantly in oligodendrocytes and is the primary cause of dysmyelination. Affected oligodendrocytes do not myelinate axons properly. However, they express myelin‐specific genes and display some of the morphological phenotypes of early stages of myelination. A decreased ratio between levels of transcriptional and translational products of genes encoding the major structural proteins of central nervous system myelin was apparent. These results suggest that JCV T‐antigens arrest the maturation of oligodendrocytes and inhibit the production of myelin. These results also demonstrate that JCV transgenic mice are a good model for investigating mechanisms of JCV‐induced demyelinating lesions in progressive multifocal leukoencephalopathy.
Multifocal cytomegalovirus encephalitis in AIDS
Authors: Masdeu J. C. , Small C. B. , Weiss L. , Elkin C. M. , Llena J. , Mesa‐Tejada R. 
Published: January 2005

AbstractA 43‐year‐old man with the acquired immunodeficiency syndrome had clinical evidence of multifocal disease of the brain, but computed tomography was negative. Magnetic resonance imaging revealed multifocal lesions, histologically proven to be caused by cytomegalovirus. Therapy with 9{2‐hydroxy‐1‐(hydroxymethyl) ethoxymethyl} guanine (BW B759U) resulted in stabilization of the patient's clinical disease and radiographic improvement of the lesions.
Brain infarction in sickle cell anemia: Magnetic resonance imaging correlates
Authors: Pavlakis Steven G. , Bello Jacqueline , Prohovnik Isak , Sutton Millicent , Ince Carol , Mohr Jay P. , Piomelli Sergio , Hilal Sadek , De Vivo Darryl C. 
Published: January 2005

AbstractBrain infarction is a well‐known but poorly understood complication of sickle cell disease. Seventy‐three sickle cell disease patients underwent neurological examinations and high‐field, heavily T2‐weighted axial cranial magnetic resonance image (MRI) scanning. Eighteen of the 73 had a history of stroke, defined as an acute, focal neurological sign lasting longer than 1 hour; in the event of a convulsive onset, an MRI abnormality as a correlate was necessary. Sixteen of the 18 stroke patients demonstrated focal MRI abnormalities consistent with arterial borderzone infarctions. Fifty‐five of the 73 patients had no history of stroke. Six of the 55 (11%) had focal MRI abnormalities suggesting previous subclinical stroke. Five of these lesions were in borderzone regions. The distinguishing feature in 21 of the 22 patients with MRI abnormalities was the predilection for lesions in the high cortical convexity, in the general regions of arterial borderzones between the major cerebral arteries and adjacent deep white matter. The pattern of MRI lesions suggests two pathogenetic mechanisms: (1) proximal large‐vessel disease with inadequate cerebral perfusion (distal field insufficiency syndrome) and (2) distal small‐vessel disease (sludging syndrome).
Spectral power of human limbic evoked potentials: Relationship to seizure onset
Authors: Meador K. J. , Loring D. W. , King D. W. , Gallagher B. B. , Rogers O. L. , Smith J. R. , Flanigin H. F. 
Published: January 2005

AbstractLimbic evoked potentials were recorded from intracerebral electrodes bilaterally implanted in the hippocampi of 16 epileptic patients undergoing evaluation for surgical resection of a seizure focus. Spectral analysis of the limbic evoked potentials revealed consistently lower spectral power of both low‐band (1–12.6 Hz) and high‐band (13–20 Hz) components recorded from the hippocampus that was associated with the electrographically confirmed seizure focus as compared to the contralateral side. Limbic evoked potential recordings appear to be useful in the determination of a focal functional deficit and thus in identifying the site of epileptogenic pathology. Further, the results support the hypothesis of a local generator for limbic evoked potentials and demonstrate that limbic evoked potentials in humans include not only low‐frequency, high‐amplitude electric field changes, but also a high‐frequency component.
Focal cortical myoclonus and rolandic cortical dysplasia: Clarification by magnetic resonance imaging
Authors: Kuzniecky Ruben , Berkovic Samuel , Andermann Frederick , Melanson Denis , Olivier André , Robitaille Yves 
Published: January 2005

AbstractFocal cortical myoclonus is rare. Obvious causes include tumor or atrophy involving the motor strip, but in some cases no cause is apparent. We present 4 patients who started to have focal myoclonus in childhood. All had focal motor seizures as well, and one had recurrent focal motor status epilepticus. All 4 had a mild progressive hemiparesis. Electrographic investigations showed focal epileptic discharges in the contralateral rolandic areas. Radiological studies were unrevealing, but magnetic resonance showed rolandic lesions in 3 patients. At surgery, abnormally wide gyri were found in the distribution demonstrated by magnetic resonance. The pathological substrate was focal cortical dysplasia. All patients have improved considerably following surgery. These findings suggest that focal myoclonus may be due to a rolandic neuronal migration disorder. Visualization of these lesions by magnetic resonance permits development of a surgical strategy leading to optimal treatment of these medically intractable epileptic disorders.
Successful chemotherapy for recurrent malignant oligodendroglioma
Authors: Cairncross J. Gregory , Macdonald David R. 
Published: January 2005

AbstractEight consecutive patients with recurrent malignant oligodendroglioma were treated with systemic chemotherapy. Six patients received a combination of procarbazine, lomustine (CCNU), and vincristine; 1 received carmustine (BCNU), and 1 diaziquone. All responded by clinical and computed tomographic scan criteria. One patient had a complete response for 78 weeks, and 7 patients had unequivocal partial responses lasting 30+ to 68+ weeks. Two partial responders had complete control of systemic metastases. Malignant oligodendroglioma is a uniquely chemosensitive glial tumor.
Seizures following myelography with iopamidol
Authors: Levey Allan I. , Weiss Howard , Yu Robin , Wang Henry , Krumholz Allan 
Published: January 2005

AbstractIopamidol, a water‐soluble contrast medium, has been rarely associated with seizures. We describe 3 patients (from a series of 785) who had generalized tonic‐clonic seizures after iopamidol myelography. Two of the patients underwent lumbar and one cervical myelography. There was a history of seizures in 2 patients, who convulsed was high, ranging from 3,000 to 4,500 mg iodine. Despite the reported low incidence of complications with iopamidol, seizures may occur, especially in patients with previous seizures and also in those receiving higher doses of iopamidol.
Serum antibodies to gangliosides in guillain‐barré syndrome
Authors: Ilyas A. A. , Willison H. J. , Quarles R. H. , Jungalwala F. B. , Cornblath D. R. , Trapp B. D. , Griffin D. E. , Griffin J. W. , McKhann G. M. 
Published: January 2005

AbstractTo determine whether antibodies to acidic glycolipids of nervous tissue are present in patients with Guillain‐Barré syndrome (GBS), sera from patients with GBS and appropriate control subjects were tested by a thin‐layer chromatogram overlay technique. Chromatograms on which the whole ganglioside fractions from peripheral nerve and brain had been separated were overlaid with appropriate dilutions of the patients' sera (1:100 or greater), and antibody binding was revealed with a radiolabeled or peroxidase‐labeled second antibody. Antibodies to ganglioside antigens were detected in 5 of 26 patients with GBS. IgG antibodies in 1 patient reacted strongly with LM1 (sialosyl Para‐globoside), the major ganglioside of human peripheral nervous system myelin, and its hexaose analog (sialosyl lactosaminyl paragloboside), a minor ganglioside of human peripheral nervous system myelin. The antibody titer in this patient fell 8‐fold over 6 weeks coincident with clinical improvement. IgG from 2 other patients with GBS reacted with GD1b ganglioside, and the antibody titers in these patients also decreased substantially with clinical improvement. IgM antibodies in the sera from 2 other patients reacted with GD1a and GT1b gangliosides, which have a shared terminal carbohydrate sequence. Antibodies to gangliosides were not detected in the sera from 19 patients with other neurological diseases or from 10 normal subjects, and the frequency with which antiganglioside antibodies occurred in the patients with GBS was significantly greater than that in the combined control subjects (p < 0.01). The results demonstrate relatively high levels of antibodies to gangliosides in some GBS patients.
Fluctuations of CD4+ T‐cell subsets in remitting‐relapsing multiple sclerosis
Authors: Rose Lynn Massman , Ginsberg Arthur H. , Rothstein Ted L. , Ledbetter Jeffrey A. , Clark Edward A. 
Published: January 2005

AbstractPatients with multiple sclerosis (MS) frequently have selective depletion of the CD45R+CD4+ T‐cell subset during active phases of disease. To study the relationship between changes in this subset and the onset of objective clinical exacerbations of disease, a longitudinal study was undertaken. Two CD4+ T‐cell subsets and two CD8+ T‐cell subsets were monitored by two‐color immunofluorescence using a fluorescence‐activated cell sorter. These subsets of peripheral blood lymphocytes were monitored monthly for one year in a group of 9 patients with remitting‐relapsing MS and in 11 healthy age‐matched control subjects. Significant changes in the ratio of two CD4+ T‐cell subsets (CD45R−/ CD45R+) were detected in 7 of 9 patients with MS, but not in any of the control subjects. Of those 7 persons, 4 suffered major clinical relapses substantiated by alterations in the neurological examination. The other 3 suffered minor relapses with subjective clinical abnormalities. All 7 had increased CD4+ T‐cell subset ratios (%CD4+CD45R−/%CD4+CD45R+) within the month that new symptoms were reported. Most such increases resulted from a simultaneous depletion in the number of CD45R+CD4+ T cells and an increase in the number of CD45R−CD4+ T cells. One patient suffered a major relapse with no change in the ratio of CD4+ subsets but had a depletion of all CD4+ T cells. There were no consistent changes in any of the other subsets measured. These results indicate that a subgroup of patients with MS have abnormal fluctuations of two CD4+ T‐cell subsets, which may correlate with increased disease activity.
Age effects on properties of motor unit action potentials: ADEMG analysis
Authors: Howard Januarye E. , McGill Kevin C. , Dorfman Leslie J. 
Published: January 2005

AbstractWe have measured the configurational and firing properties of 13,206 motor unit action potentials (MUAPs) from the brachial biceps, brachial triceps, and anterior tibial muscles in 10 young (20–40 years), 10 middle‐aged (40–60 years), and 10 elderly (60–80 years) normal individuals, using an automatic method for Decemberomposition of the electromyo‐graphic (EMG) interference pattern (ADEMG). Recordings were made during stable isometric contractions at threshold, 10%, and 30% of maximum voluntary contraction using standard concentric needle electrodes. At supra‐threshold forces, an average of 5.9 simultaneously active MUAPs were identified at each recording site. Mean amplitudes, durations, and numbers of turns all increased linearly with age in both low‐threshold and high‐threshold MUAPs (p < 0.01), suggesting an ongoing process of progressive denervation and compensatory reinnervation. Mean MUAP firing rates Decemberreased with age (p = 0.01) when force was measured proportionately, but not when measured absolutely. In a subgroup of 12 age‐matched gender pairs, men had larger mean MUAP amplitudes, rise rates, and numbers of turns (p < 0.05), probably reflecting larger muscle fiber diameters. These findings amplify previous observations from traditional analysis of lowest‐threshold single MUAPs; establish a base of normative adult data for ADEMG; and further validate the clinical applicability of rapid, automatic EMG Decemberomposition.
Brain acetylcholinesterase after acute parathion poisoning: A comparative quantitative histochemical analysis post mortem
Authors: Finkelstein Y. , Wolff M. , Biegon A. 
Published: January 2005

AbstractThe postmortem distribution of acetylcholinesterase (AChE) inhibition was studied in the brains of 2 victims of lethal parathion intoxication and 2 control brains matched for age and sex. AChE activity in discrete brain regions was studied by quantitative histochemistry of 40‐μm‐thick sagittal or coronal cryostat sections from the 4 brains. Inhibition of human brain AChE by parathion is regionally selective. The biggest Decemberreases (60–85%) were observed in the cerebellum, some thalamic nuclei, and the cortex. Only a moderate Decemberrease (10–30%) was observed in the substanda nigra and basal ganglia, and no effect was seen in white matter. Detailed knowledge of the brain regions affected bf parathion poisoning may explain some of the clinical manifestations of organophosphate poisoning.
Torsional nystagmus in the lateral medullary syndrome
Authors: Morrow Marchk J. , Sharpe James A. 
Published: January 2005

AbstractTorsional nystagmus was recorded in 3 patients with the lateral medullary syndrome. Magnetic search coil oculog‐raphy demonstrated slow phases of nystagmus of increasing, Decemberreasing, and constant velocity. Neural integration of torsional eye velocity commands to position commands is impaired by lateral medullary infarction. Torsional pulsion of saccades, consisting of torsional fast eye movements induced during saccades downward or away from the side of infarction, was recorded in 2 patients. All patients had skew deviation with hypotropia on the side of brainstem damage. The torsional nystagmus beat away from the side of infarction in each patient, but in one it alternated direction as the eyes drifted about a neutral position of torsion. We attribute the torsional nystagmus to an imbalance of central projections from the anterior and posterior semicircular canals and the otolith receptors that mediate ocular counterroll.
Brain neurotransmitters in glycine encephalopathy
Authors: Kish Stephen J. , Dixon Lori M. , Burnham W. McLntyre , Perry Thomas L. , Becker Lawrence , Cheng Jerome , Chang Li‐Jan , Rebbetoy Michelle 
Published: January 2005

AbstractWe measured neurotransmitter Marchkers in autopsied brain of infants with glycine encephalopathy (GE). Because patients with GE develop intractable seizures, special attention was devoted to those neurotransmitter systems implicated in human epilepsy. Mean levels of glycine in the frontal cortex of GE patients were three times higher than control values. No abnormalities were observed for concentrations of gamma‐aminobutyrici acid (and related receptors), other major neurotransmitter amino compounds, or activities of cholineacetyl‐transferase and aspartate aminotransferase. Mean acetylcholinesterase activity was significantly elevated by 46%. As experimental data suggest, glycine Marchkedly potentiates the action of the excitatory neurotransmitter glutamic acid. To the extent that the brain seizures in patients with GE can be explained by this mechanism, pharmacotherapy with excitatory amino acid antagonists may represent a new approach to the treatment of GE.
Efficacy of adjuvant chemotherapy for patients with poor‐risk medulloblastoma: A preliminary report
Authors: Packer Roger J. , Siegel Kathy R. , Sutton Leslie N. , Evans Audrey E. , D'angio Giulio , Rorke Lucy B. , Bunin Greta R. , Schut Luis 
Published: January 2005

AbstractRecent studies have demonstrated that preoperative and postoperative factors can broadly stratify patients with medulloblastoma/primitive neuroectodermal tumors (MB/PNET) into risk groups. For children with factors that suggest poor outcome after treatment with surgery and radiotherapy, the addition of chemotherapy can improve survival. Since 1983, 26 children with poor‐risk posterior fossa MB/PNET have been treated at our institution with craniospinal radiation therapy and adjuvant chemotherapy. Chemotherapy consisted of vincristine during radiotherapy and eight 6‐week cycles of vincristine, cis‐platinum, and l‐(2‐chloroethyl)‐3‐cyclohexyl‐1‐nitrosourea (CCNU). Twenty‐five of 26 patients (96%) who have been entered on this protocol remain alive and free of disease at a median of 24 months from diagnosis (range 6 to 50 months). Twenty patients have completed all therapy and are at a median of 32 months from initial diagnosis with no evidence of disease. These patients were compared to a group of children with similar prognostic features treated at our institution between 1975 and 1983. Actuarial disease‐free survival was statistically significantly better for protocol patients than for historical control subjects (p < 0.002). This difference was most Marchked in patients who had received radiation therapy alone (p < 0.0003). Actuarial 2‐year disease‐free survival was 96% for patients on protocol as compared to 59% for historical control patients who had been treated with radiotherapy alone. The chemotherapy given in this protocol was well tolerated. The results of this study, although preliminary, suggest that adjuvant chemotherapy is at least transiently effective in improving the rate of disease‐free survival for children with poor‐risk MB/PNET.
Lambert‐eaton myasthenic syndrome IgG depletes presynaptic membrane active zone particles by antigenic modulation
Authors: Nagel Alexandre , Engel Andrew G. , Lang Bethan , Newsom‐Davis John , Fukuoka Tadahiro 
Published: January 2005

AbstractThe Lambert‐Eaton myasthenic syndrome (LEMS) is an autoimmune disease that can be transmitted from human to mouse with immunoglobulin G (IgG). Electrophysiological studies indicate that LEMS IgG acts on presynaptic voltage‐sensitive calcium channels, probably reducing their number, and freeze‐fracture electron microscopy demonstrates that LEMS IgG has an effect on the presynaptic active zone paticles, which represent putative voltage‐sensitive calcium channels. The active zone particles, normally arranged in double parallel rows, move closer together, form clusters, and are reduced in number. The morphological data suggest modulation of the active zone particles cross‐linked by LEMS IgG. If this were the case, then only divalent LEMS IgG and F(ab')2 should alter the deployment of active zone particles and moNovemberalent Fab should be without effect. To test this hypothesis, mouse diaphragms were exposed to control and LEMS IgG and IgG fragments in organ culture for 24 hours and then studied by quantitative freeze‐fracture electron microscopy. Divalent LEMS IgG and F(ab')2 aggregated and depleted the active zone particles, whereas moNovemberalent Fab had no effect. The findings reconfirm that the active zone particles are targets of LEMS IgG and are direct evidence for modulation of the particles by LEMS IgG. The findings are in harmony with parallel electrophysiological studies of the effects of LEMS IgG fragments on transmitter release in the same diaphragm muscles (Lang et al, J Physiol 1987;390:173P).
Biochemical analysis of caudate nucleus biopsy samples from parkinsonian patients
Authors: Goldstein Menek , Lieberman Abraham N. , Helmer Elizabeth , Koslow Max , Ransohoff Joseptemberh , Elsworth John D. , Roth Robert H. , Deutch Ariel Y. 
Published: January 2005

AbstractBiochemical analyses of caudate nucleus biopsy samples from patients with Parkinson's disease undergoing autologous adrenal transplantation were performed. Activity of the dopamine biosynthetic enzyme tyrosine hydroxylase, and concentrations of dopamine and its priMarchy metabolite homovanillic acid were significantly greater than anticipated on the basis of previously published postmortem values. These data suggest that postmortem changes in various biochemical parameters of dopamine function are more rapid than has been generally appreciated. Further analysis of striatal biopsy samples may reveal predictive relationships between striatal indices of dopamine function and therapeutic response to adrenal transplantation.