bluuebunny/crossref_metadata_2025_split huggingface dataset:
Increases in both cerebral glucose utilization and blood flow during execution of a somatosensory task
Authors: Ginsberg Myron D. , Chang Jen Y. , Kelley Roger E. , Yoshii Fumihito , Barker Warren W. , Ingenito Gary , Boothe Thomas E. 
Published: January 2005

AbstractTo investigate local metabolic and hemodynamic interrelationships during functional activation of the brain, paired studies of local cerebral glucose utilization (lCMRGlc) and blood flow (lCBF) were carried out in 10 normal subjects (9 right‐handed, 1 ambidextrous) at rest and during a unilateral discriminative somatosensory/motor task—palpation and sorting of mah‐jongg tiles by engraved design. The extent of activation was assessed on the basis of percentage difference images following normalization to compensate for global shifts. The somatosensory stimulus elevated lCMRGlc by 16.9 ± 3.5% (mean ± standard deviation) and lCBF by 26.5 ± 5.1% in the contralateral sensorimotor cortical focus; smaller increments were noted in the homologous ipsilateral site. The increments of lCMRGlc and lCBF correlated poorly with one another in individual subjects. Stimulation of the right hand resulted in significantly higher contralateral lCMRGlc activation (19.6%) than did stimulation of the left hand (14.1%) (p < 0.005), whereas the lCBF response was independent of the hand stimulated. Our results indicate that both glycolytic metabolism and blood flow increase locally with the execution of an active sensorimotor task and suggest that both measures may serve as reliable markers of functional activation of the normal brain.
Alternating skew on lateral gaze (Bilateral abducting hypertropia)
Authors: Moster Mark L. , Schatz Norman J. , Savino Peter J. , Benes Susan , Bosley Thomas M. , Sergott Robert C. 
Published: January 2005

AbstractWe report thirty‐three patients with alternating skew deviation on lateral gaze. The right eye was hypertropic in right gaze, and the left eye was hypertropic in left gaze. Most patients had associated downbeat nystagmus and ataxia and were diagnosed as having lesions of the cerebellar pathways or the cervicomedullary junction. This contrasts with a previous report in which alternating skew was seen mainly in lesions of the midbrain pretectum.
Release of vasoactive peptides in the extracerebral circulation of humans and the cat during activation of the trigeminovascular system
Authors: Goadsby P. J. , Edvinsson L. , Ekman R. 
Published: January 2005

AbstractThe trigeminal ganglion was activated, in humans by thermocoagulation as part of the treatment of trigeminal neuralgia and in cats by electrical stimulation, and blood samples were taken from the external jugular vein for estimates of plasma levels of substance P and calcitonin gene‐related peptide (CGRP). In those patients who were noted at the time of coagulation to have flushed there were marked elevations of the local (cranial) levels of both peptides. However, in the nonflushing patients no changes in the peptide levels were observed. Parallel experiments in the cat revealed that the levels of substance P—like and CGRP‐like immunoreactivity were increased during electrical stimulation of the trigeminal ganglion. The observation of elevation of substance P—like and CGRP‐like immunoreactivity after activation of the nociceptive afferent system of the head provides new insights into a putative role of peptides in the pathophysiology of migraine and cluster headache, and suggests new areas of possible therapeutic intervention.
Cortisol induction by poly ICLC: Implications for clinical trials of interferon
Authors: Bever Christopher T. , McFarland Henry F. , Levy Hilton B. , McFarlin Dale E. 
Published: January 2005

AbstractBecause interferon injections have recently been reported to induce cortisol in cancer patients, we retrospectively reviewed cortisol levels obtained during a preliminary trial of the interferon inducer polyriboinosinic polyribocytidylic acid in poly‐L‐lysine and carboxymethylcellulose (poly ICLC) in multiple sclerosis patients to determine if significant cortisol induction occurred. Analysis of data from 51 poly ICLC infusions in 6 men and 4 women showed elevated cortisol levels 4 to 16 hours after infusion, with hematological changes consistent with steroid effect. The highest cortisol levels observed were in 2 patients who improved during the treatment period, but there was no clear relationship between cortisol levels and clinical outcome in the group as a whole.
Infantile gaucher's disease: A case with neuronal storage
Authors: Grafe Marjorie , Thomas Carol , Schneider Jerry , Katz Barrett , Wiley Clayton 
Published: January 2005

AbstractThe brain of a 17‐month‐old boy with infantile Gaucher's disease and oculomotor apraxia was studied by light and electron microscopy. Light microscopic examination showed large perivascular accumulations of Gaucher's cells in frontal lobe white matter, severe neuronal loss in the calcarine cortex and dentate nucleus of the cerebellum, and neuronophagia and microglial nodules in the brainstem. Electron microscopy demonstrated intraneuronal cytoplasmic inclusions containing twisted tubules characteristic of Gaucher's disease in both the oculomotor nucleus and a random sample of cortex.
Alz‐50 Antibody recognizes alzheimer‐related neuronal changes
Authors: Hyman B. T. , Van Hoesen G. W. , Wolozin B. L. , Davies P. , Kromer L. J. , Damasio A. R. 
Published: January 2005

AbstractAlz‐50 is a monoclonal antibody directed against an antigen present in Alzheimer's disease. Unlike conventional pathological stains that reveal only neurons with neurofibrillary tangles (NFTs), Alz‐50 recognizes neurons that contain NFTs and additional neurons that do not. Our investigation of the topographical distribution of both NFT‐containing and non‐NFT‐containing neurons recognized by Alz‐50 reveals that they are both found primarily in certain cytoarchitectural areas and lamina that have been established as consistent sites for NFT pathology. Some of the neurons recognized by Alz‐50 appear histopathologically normal. We suggest that Alz‐50 recognizes an antigen in neurons that both precedes and accompanies NFT formation. Thus, it may mark a point early in cellular pathology before irreversible cytoskeletal and degenerative changes occur.
Monoclonal antibody analysis of mononuclear cells in myopathies. V: Identification and quantitation of T8+ cytotoxic and T8+ suppressor cells
Authors: Arahata Kiichi , Engel Andrew G. 
Published: January 2005

AbstractIn polymyositis (PM) and inclusion body myositis (IBM), but not in dermatomyositis there is evidence of cell‐mediated cytotoxicity: T8+ cells accompanied by macrophages focally surround, invade, and destroy nonnecrotic muscle fibers. However, the T8 marker appears on both cytotoxic (Tc) and suppressor (Ts) cells. The Leu‐15 marker appears on Ts but not on Tc cells, but it also appears on macrophages and on some killer/natural killer cells. To obviate this problem, the T8, Leu‐15, and Leu‐7 markers were demonstrated by sequential paired immunofluorescence in single cryostat sections. Using this approach, we reliably differentiated for the first time between Tc and Ts cells in tissue sections. Six cell phenotypes were identified: T8+Leu‐15− Leu‐7− Tc cells, T8+Leu‐15+Leu‐7− Ts cells, three types of Leu‐7+ killer/natural killer cells, and T8−Leu‐15+Leu‐7− macrophages. Muscle specimens from 5 patients with PM and 5 with IBM were studied. In each case, 6 nonnecrotic muscle fibers focally surrounded and invaded by mononuclear cells were selected randomly. A total of 2,022 mononuclear cells were analyzed, 870 from patients with PM and 1,152 from those with IBM. When counts of the identified cell phenotypes in individual patients were pooled, there were four times as many T8+Leu‐15−Leu‐7− Ts cells as T8+Leu‐15+Leu‐7− Ts cells in either PM or IBM samples. However, when the relative frequencies of the Tc and Ts cells were examined in individual patients, the Tc cells tended to become more abundant, and the Ts cells correspondingly less abundant, with the duration of symptoms. These data indicate that the T8+ Tc cell is the predominant phenotype in cell‐mediated cytotoxicity in PM and IBM.
Atlantoaxial subluxation in psoriatic arthropathy
Authors: Kaplan Jerry G. , Rosenberg Richard S. , DeSouza Trevor , Post Kalmon D. , Freilich Mark D. , Salamon Oscar , Lantos George , Reinitz Elizabeth 
Published: January 2005

AbstractSymptomatic atlantoaxial dislocation occurs rarely in psoriatic arthropathy and has previously been reported only as a late complication in this disorder. We report severe upward axial dislocation and acquired basilar impression as a presenting manifestation of psoriatic arthropathy. Magnetic resonance imaging is useful in evaluating this condition.
The metabolic anatomy of paraneoplastic cerebellar degeneration
Authors: Anderson N. E. , Posner J. B. , Sidtis J. J. , Moeller J. R. , Strother S. C. , Dhawan V. , Rottenberg D. A. 
Published: January 2005

AbstractEleven patients with acquired cerebellar degeneration (10 of whom had paraneoplastic cerebellar degeneration {PCD}) were evaluated using neuropsychological tests and 18F‐fluorodeoxyglucose/positron emission tomography to (1) quantify motor, cognitive, and metabolic abnormalities; (2) determine if characteristic alterations in the regional cerebral metabolic rate for glucose (rCMRGlc) are associated with PCD; and (3) correlate behavioral and metabolic measures of disease severity. Eighteen volunteer subjects served as normal controls. Although some PCD neuropsychological test scores were abnormal, these results could not, in general, be dissociated from the effects of dysarthria and ataxia, rCMRGlc was reduced in patients with PCD (versus normal control subjects) in all regions except the brainstem. Analysis of patient and control rCMRGlc data using a mathematical model of regional metabolic interactions revealed two metabolic pattern descriptors, SSF1 and SSF2, which distinguished patients with PCD from normal control subjects; SSF2, which described a metabolic coupling between cerebellum, cuneus, and posterior temporal, lateral frontal, and paracentral cortex, correlated with quantitative indices of cerebellar dysfunction. Our inability to document substantial intellectual impairment in 7 of 10 patients with PCD contrasts with the 50% incidence of dementia in PCD reported by previous investigators. Widespread reduction in PCD rCMRGlc may result from the loss of cerebellar efferents to thalamus and forebrain structures, a “reverse cerebellar diaschisis”.
Spinocerebellar ataxia: Variable age of onset and linkage to human leukocyte antigen in a large kindred
Authors: Zoghbi Huda Y. , Pollack Marilyn S. , Lyons Leslie A. , Ferrell Robert E. , Daiger Stephen P. , Beaudet Arthur L. 
Published: January 2005

AbstractWe studied a seven‐generation kindred with autosomal dominant spinocerebellar ataxia (SCA) to assess linkage relationships to multiple human leukocyte antigen (HLA) loci on the short arm of chromosome 6. Age at onset, clinical features, and course of the disease are described. Although the mean age of onset was 34 years in this family, in 6 of 41 affected individuals onset was below 15 years of age and was accompanied by the unique clinical features of mental retardation and rapid progression of disease. Linkage studies were performed on 93 individuals, and the results show strong evidence for linkage of the SCA locus to the HLA loci. A maximum logarithm of the odds score of 5.83 was found at a recombination fraction of 0.12. This is the first documentation of childhood onset in the HLA‐linked form of SCA.
Influence of neuronal location on antigenic properties of neurofibrillary tangles
Authors: Tabaton Massimo , Perry George , Autilio‐Gambetti Lucila , Manetto Valeria , Gambetti Pierluigi 
Published: January 2005

AbstractWe quantitatively assessed the antigenic properties of the neurofibrillary tangles (NFT) located in neurons of the tegmental nuclei of the pontine raphe in progressive supranuclear palsy (PSP) and Alzheimer's disease (AD). These properties were then compared with those of NFT of AD located in hippocampal neurons. Antibodies known to react with cortical NFT of AD were used to stain sections from PSP, AD, and control cases. The reaction with the straight filaments of NET of PSP and with the paired helical filaments of pontine NFT of AD was ascertained by immunoelectron microscopy. The results show that, despite the ultrastructural difference, straight filaments in NFT of PSP and paired helical filaments in NFT of AD share antigenic properties when they are located in the same neuronal population. In contrast, paired helical filaments located in the cerebral cortex are antigenically different from those in pontine nuclei. Location, more than structure, may play a role as determinant of antigenic properties in straight filaments of PSP and paired helical filaments of AD.
Toluene abuse causes diffuse central nervous system white matter changes
Authors: Rosenberg Neil L. , Kleinschmidt‐DeMasters B. K. , Davis Kathleen A. , Dreisbach James N. , Hormes Joseph T. , Filley Christopher M. 
Published: January 2005

AbstractWe describe the findings of magnetic resonance imaging (MRI) of the brain in 6 chronic toluene vapor abusers and the neuropathological findings in 1 abuser not studied by MRI. MRI in 6 chronic toluene abusers revealed the following abnormalities: (1) diffuse cerebral, cerebellar, and brainstem atrophy; (2) loss of differentiation between the gray and white matter throughout the central nervous system; and (3) increased periventricular white matter signal intensity on T2‐weighted images. Another chronic toluene abuser (MRI not performed) died as a result of acute toluene overdose. The brain displayed diffuse, ill‐defined myelin pallor, maximal in cerebellar, periventricular, and deep cerebral white matter. Neurons were preserved throughout, axonal swelling or beading was not seen, gliosis was minimal, and occasional, scant perivascular macrophage collections were seen. Taken in concert, these findings suggest that the pathological and MRI abnormalities are due to either increased water content of the white matter or subtle toluene‐induced metabolic changes in myelin.
Chronic unilateral optic neuropathy: A magnetic resonance study
Authors: Eidelberg D. , Newton M. R. , Johnson G. , MaCmanus D. G. , McDonald W. I. , Miller D. H. , Halliday A. M. , Moseley I. F. , Heng G. S. , Wright J. 
Published: January 2005

AbstractWe studied the clinical, electrophysiological, and magnetic resonance imaging (MRI) features of 20 patients with chronic unilateral optic neuropathy (CUON): progressive unilateral visual failure lasting a minimum of 6 months. The patients, 10 male and 10 female, ranged in age from 12 to 77 years (mean 44) and had a mean duration of symptoms of 22 months. All had signs of optic nerve dysfunction. Each patient was studied with MRI using a short TI inversion recovery (STIR) sequence to delineate the optic nerve from surrounding orbital tissue. Three distinct groups of patients with CUON were identified using MRI. In the first group (8/20) the optic nerve was compressed by an extrinsic mass, whereas in the second group (5/20) CUON resulted from an intrinsic tumor of the optic nerve or sheath. In both groups STIR sequences compared favorably with computed tomography in identifying mass lesions. MRI was superior in delineating distortion of the optic nerve by mass or tumor extension beyond the orbit. In the third group (7/20) no mass was evident on MRI. However, STIR sequences revealed altered signal (long Tl) in clinically symptomatic nerves. In 4 of the patients T2‐weighted cerebral MRI disclosed periventricular lesions suggestive of disseminated white matter disease. We conclude that MRI complements clinical and electrophysiological testing in the assessment of CUON.
Borrelia burgdorferi—specific and autoreactive T‐cell lines from cerebrospinal fluid in lyme radiculomyelitis
Authors: Martin Roland , Ortlauf Johannes , Sticht‐Groh Veronika , Bogdahn Ulrich , Goldmann Shraga F. , Mertens Hans Georg 
Published: January 2005

AbstractIn 3 patients with Lyme radiculomyelitis, cellular immune reactions of cerebrospinal fluid (CSF) lymphocytes were analyzed. Phenotypic analysis of CSF cells demonstrated that the majority were T cells (CD3 +) of the helper/inducer subset (CD4 +). These T cells were directly expanded from the CSF by limiting dilution. A total of 505 T‐cell lines were tested for Borrelia burgdorferi (Bb)—specific proliferation and also partly tested for reactivity to a panel of central and peripheral nervous system antigens. Proliferative assays revealed 33 of them to be Bb specific, 16 to be specific for myelin basic protein, 16 to be specific for peripheral myelin, 1 to be specific for cardiolipin, and 2 to be specific for galactocerebrosides. The antigen‐specific proliferation was restricted by autologous human leukocyte antigen (HLA) class II molecules. The majority of CSF‐derived T‐cell lines stained positively for CD3, CD4, and HLA class II antigens and negatively for CD8 (cytotoxic/suppressor subset). One T‐cell line provided help for the production of specific IgG by autologous B cells and secreted gamma‐interferon upon stimulation with Bb antigen in the presence of autologous antigen‐presenting cells. These data show that in patients with severe neurological manifestations of late Lyme disease, not only Bb‐specific T‐cell lines but also T cells reactive to central or peripheral nervous system autoantigens can be found.
Infantile and fetal globoid cell leukodystrophy: Analysis of galactosylceramide and galactosylsphingosine
Authors: Kobayashi Takuro , Goto Ikuo , Yamanaka Tatsuhiro , Suzuki Yoshiyuki , Nakano Takeshi , Suzuki Kunihiko 
Published: January 2005

AbstractGalactosylceramide and galactosylsphingosine (psychosine) were assayed in tissues from infants and fetuses with globoid cell leukodystrophy (GLD). Galactosylceramide concentrations were not increased in nervous tissues or other organs. Using a sensitive assay method, we found galactosylsphingosine accumulations in GLD tissues, both infantile and fetal, which suggests that GLD is a generalized galactosylsphingosine storage disease. High galactosylsphingosine levels were observed in the brain, spinal cord, and sciatic nerve of infants with GLD and in the spinal cord of a fetus with GLD, where lesions characteristic to GLD were noted. In tissues without morphological changes, such as somatic organs and the brain in fetal GLD, galactosylsphingosine concentrations were low. These results suggest that a close relationship exists between galactosylsphingosine accumulation and the pathogenesis of GLD. The finding that galactosylsphingosine, but not galactosylceramide, accumulates in the tissue of GLD can be explained by our previous observation that galactosylceramide, but not galactosylsphingosine, is readily hydrolyzed by an intact galactosylceramidase II, which is genetically distinct from galactosylceramidase I.
The relationship between interictal and ictal paroxysms in an in vitro model of focal hippocampal epilepsy
Authors: Jensen Morten S. , Yaari Yoel 
Published: January 2005

AbstractIn studies of focal epilepsy it is frequently assumed that the interictal spike is the elementary form of epileptic activity and that seizure, or ictal, episodes evolve by temporal summation and spatial expansion of interictal paroxysms. We examined this hypothesis in an in vitro model of acute focal epilepsy produced by perfusing rat hippocampal slices with solutions containing moderately elevated concentrations of K+. Some of the preparations treated in this way displayed recurring electrical seizures in the CA1 field. Each seizure episode typically evolved by a seemingly smooth progression of brief interictal bursts into sustained ictal discharge. However, exposure of preparations showing electrical seizures to blockers of synaptic transmission or to cholinergic agonists abolished interictal spiking in all hippocampal fields but did not impede the initiation of ictal episodes in area CA1. Likewise, severing the connections between areas CA3 and CA1 abolished interictal spiking in area CA1 without disrupting the initiation of seizures in this region. These data clearly show that in this model, focal seizures arise independent of interictal spikes and through different cellular mechanisms. While interictal electrogenesis requires chemical synaptic excitation, ictal episodes can be initiated and maintained by nonsynaptic neuronal interactions.
Selective sparing of NADPH‐diaphorase neurons in neonatal hypoxia‐ischemia
Authors: Ferriero Donna M. , Arcavi Lidia J. , Sagar Stephen M. , McIntosh Tracy K. , Simon Roger P. 
Published: January 2005

AbstractExcitatory amino acids have been implicated in ischemic neuronal injury. To test this hypothesis in neonatal hypoxia‐ischemia, lesions of the cortex and striatum were induced in 7‐day‐old rats by unilaterally ligating their carotid arteries and subjecting them to hypoxic conditions for 2 hours. Brains examined 1 week later demonstrated, within the regions of ischemic damage, a striking preservation of neurons that stained histochemically for nicotinamide adenine dinu‐cleotide phosphate diaphorase (NADPH‐d) activity. Concentrations of the neuropeptides somatostatin and neuropeptide Y, which colocalize in neurons containing NADPH‐d, were unaffected in the areas of ischemic damage. The same pattern of injury with sparing of NADPH‐d‐reactive neurons was reproduced by focal microinfusion of the excitotoxin quinolinic acid, an endogenous N‐methyl‐d‐aspartate (NMDA) agonist, into the striatum. These results support the hypothesis that neonatal hypoxic‐ischemic injury is mediated through excitatory transmitters acting at the NMDA receptor and that the NADPH‐d‐reactive neurons in the neonate are resistant to excitotoxic damage. This pattern of cell vulnerability is unique to the developing striatum and may relate to the distinct pathological appearance of the basal ganglia that follows neonatal asphyxia.
Findings in muscle in complex I (NADH coenzyme Q reductase) deficiency
Authors: Koga Yasutoshi , Nonaka Ikuya , Kobayashi Masanori , Tojyo Megumu , Nihei Kenji 
Published: January 2005

AbstractThirteen of 15 patients with complex I deficiency had the multisystemic form, with strokelike episodes and other symptoms that fulfilled the diagnostic requirements for MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes), and 2 had only muscle fatigability and weakness, having the purely myopathic form. In the multisystemic form, 12 patients had ragged‐red fibers. All multisystemic patients had myopathic histochemical abnormalities that consisted of mild to moderate variation in fiber size (13), disorganized intermyofibrillar networks (12), type 2 fiber atrophy (10), and an increased number of type 2C fibers (9). Five of 13 multisystemic patients had Decemberreased cytochromec oxidase (CCO) activity in extrafusal fibers, with sparing of intrafusal muscle fibers. In the myopathic form, pathological findings were similar to those in the multisystemic form. In addition to complex I and NADH dehydrogenase activities being Decemberreased, the CCO activity was significantly Decemberreased (less than 50% of control value) in 8 patients, especially when the disease was in its advanced stages, suggesting that CCO enzyme might be secondarily affected as the disease progresses.