bluuebunny/crossref_metadata_2025_split huggingface dataset:
The clinical correlates of high‐titer IgG anti‐GM1 antibodies
Authors: Kornberg Andrew J. , Pestronk Alan , Bieser Karen , Ho T. W. , McKhann G. M. , Wu H. S. , Jiang Z. 
Published: January 2005

AbstractSerum IgG anti‐GM1 antibodies have been reported to occur in a variety of disorders, including Guillain‐Barré syndrome and chronic polyneuropathies. Of over 5,000 serums tested in our laboratory, high titers of selective IgG anti‐GM1 antibodies (>1: 1,000) and without binding to sulfatide were found in 35 patients. Clinical correlation revealed that almost all patients had axonal, motor neuropathies. One subgroup was comprised of individuals with an acute motor neuropathy, described either as an acute axonal Guillain‐Barré–like syndrome that was occasionally associated with a prodrome of Campylobacter jejuni enteritis or as Chinese paralysis syndrome. A second group of patients had chronic asymmetric lower motor neuron (LMN) syndromes with no conduction block or other evidence of demyelination. The presence of selective high‐titer IgG anti‐GM1 antibody reactivity in serum is uncommon but when present is strongly associated with acute axonal motor neuropathies or chronic asymmetric LMN syndromes.
Prognostic value of myoclonus status in comatose survivors of cardiac arrest
Authors: Wijdicks Eelco F. M. , Parisi Joseph E. , Sharbrough Frank W. 
Published: January 2005

AbstractGeneralized myoclonus status is common in comatose patients after cardiac resuscitation, but its prognostic value is uncertain. We studied the clinical, radiologic, and pathologic findings in 107 consecutive patients who remained comatose after cardiac resuscitation. Myoclonus status was present in 40 patients (37%). Features more prevalent in patients with myoclonus status were burst suppression on electroencephalograms, cerebral edema or cerebral infarcts on computed tomography scans, and acute ischemic neuronal change in all cortical laminae. All patients with myoclonus status died. Of 67 patients without myoclonus, 20 awakened. We conclude that myoclonus status in postanoxic coma should be considered an agonal phenomenon that indicates devastating neocortical damage. Its presence in comatose patients after cardiac arrest must strongly influence the decision to withdraw life support.
Rapidly evolving myopathy with myosin‐deficient muscle fibers
Authors: Al‐Lozi M. T. , Pestronk A. , Yee W. C. , Flaris N. , Cooper J. 
Published: January 2005

AbstractFive patients with rapidly evolving, severe weakness had an unusual myopathy with virtually complete loss of myosin in 5 to 40% of muscle fibers. Three of the 5 patients began to develop weakness 1 to 2 weeks after lung transplantation. The fourth became weak after a febrile illness. The fifth presented with diabetic ketoacidosis and weakness. All patients had received corticosteroid therapy. In all cases the weakness was progressive and led to severe disability, with respiratory failure in 4 patients. Initial diagnostic testing did not localize an underlying cause for the weakness. Creatine kinase was normal or minimally elevated. Electromyography generally showed mildly myopathic or nondiagnostic changes. However, muscle biopsy revealed numerous small angular fibers with no myosin ATPase staining at any pH. Immunocytochemical staining and ultrastructural studies confirmed a severe loss of myosin in many fibers. This rapidly evolving myopathy with myosin‐deficient muscle fibers appears to be different clinically and pathologically from previously described syndromes involving rapidly progressive weakness. Slow recovery over a period of months is the most common outcome.
Muscarinic antagonists in the treatment of acquired pendular and downbeat nystagmus: A double‐blind, randomized trial of three intravenous drugs
Authors: Barton Jason J. S. , Huaman Ana G. , Sharpe James A. 
Published: January 2005

AbstractWe performed a double‐blind, randomized trial of intravenous scopolamine, benztropine, and glycopyrrolate in 7 patients with acquired nystagmus and oscillopsia. Five patients had pendular nystagmus and 2, downbeat nystagmus. We recorded eye movements with a magnetic search coil technique and tested visual acuity and motion perception before and after administration of each drug. Scopolamine reduced nystagmus in all patients. Benztropine was moderately effective and glycopyrrolate had a negligible impact. Visual acuity improved only with scopolamine; motion discrimination and oscillopsia improved significantly with scopolamine and benztropine. Pendular and downbeat nystagmus respond to intravenous antagonists of central muscarinic receptors.
Magnetic resonance spectroscopy of N‐acetylaspartate in hypoxic–ischemic encephalopathy
Authors: Graham S. H. , Meyerhoff D. J. , Bayne L. , Sharp F. R. , Weiner M. W. 
Published: January 2005

AbstractMagnetic resonance imaging and water‐suppressed proton magnetic resonance spectroscopic imaging were used to study N‐acetylaspartate and other metabolites in a patient with severe hypoxic–ischemic encephalopathy. The N‐acetylaspartate signal, a putative marker of neuronal density, was markedly reduced in the forebrain. The relative signal intensity of choline‐containing metabolites, which are more abundant in astrocytes than neurons, was increased. These results support the hypothesis that water‐suppressed proton magnetic resonance spectroscopic imaging measurements of N‐acetylaspartate may be useful for noninvasive detection of selective neuronal loss in a variety of disease states in the human brain.
Neurophysiological properties of pallidal neurons in Parkinson's disease
Authors: Sterio Djordje , Berić Aleksandar , Dogali Michael , Fazzini Enrico , Alfaro George , Devinsky Orrin 
Published: January 2005

AbstractNeuronal properties of the human globus pallidus (GP) are not known. Since GP is the major output of the basal ganglia, it may be involved in the pathophysiology of Parkinson's disease. We studied 12 patients with medically resistant Parkinson's disease by using single cell recording of the GP during stereotaxic pallidotomy to define neuronal firing rate and its modulation during active and passive movements. Different frequency and pattern of single cell activity was found in globus pallidus externus compared with globus pallidus internus. Discharge rates of 19% of GP cells were modulated by passive contralateral movements. Pallidal units were most often related solely to single joint movement. Different patterns of activity in relation to the two different movements of the same joint were often observed. We identified somatotopically arranged cell clusters that alter discharge rate with related movements. These findings suggest at least a partial somatotopic organization of the human GP and similarity with experimental results in both healthy and MPTP monkeys, providing a rationale for surgical or pharmacological targeting of GP for treating Parkinson's disease.
Functional magnetic resonance imaging of human auditory cortex
Authors: Binder J. R. , Rao S. M. , Hammeke T. A. , Yetkin F. Z. , Jesmanowicz A. , Bandettini P. A. , Wong E. C. , Estkowski L. D. , Goldstein M. D. , Haughton V. M. , Hyde J. S. 
Published: January 2005

AbstractMagnetic resonance imaging methods recently demonstrated regional cerebral signal changes in response to limb movement and visual stimulation, attributed to blood flow enhancement. We studied 5 normal subjects scanned while listening to auditory stimuli including nonspeech noise, meaningless speech sounds, single words, and narrative text. Imaged regions included the lateral aspects of both hemispheres. Signal changes in the superior temporal gyrus and superior temporal sulcus were observed bilaterally in all subjects. Speech stimuli were associated with significantly more widespread signal changes than was the noise stimulus, while no consistent differences were observed between responses to different speech stimuli. Considerable intersubject variability in the topography of signal changes was observed. These observations confirm the utility of magnetic resonance imaging in the study of human brain structure‐function relationships and emphasize the role of the superior temporal gyrus in perception of acoustic‐phonetic features of speech, rather than processing of semantic features.
Movement‐ and task‐related activations of motor cortical areas: A positron emission tomographic study
Authors: Remy Philippe , Zilbovicius Monica , Leroy‐Willig Anne , Syrota André , Samson Yves 
Published: January 2005

AbstractUsing repeated measurements of regional cerebral blood flow with positron emission tomography, we investigated the regional cortical activations induced in 10 normal subjects, by two different finger motor tasks, i.e., a repeated flexion–extension of all fingers and a repeated flexion–extension of the middle finger. The all‐finger movement only activated the primary sensorimotor cortex (SM) and the supplementary motor area (SMA) contralateral to the movement. However, the activation of the SMA was clearly task related during this motor task, because it was only observed when the movement was triggered by an auditory cue but not when it was self‐paced. The middle finger movement was performed during self‐paced conditions. It induced a much more complex pattern of activation than the all‐finger movement, characterized by a high degree of SM and SMA activation contralateral to the side of the movement, as well as a slight ipsilateral activation of these areas. We suggest that this pattern of cortical activation may reflect the process of individuating finger movement or the early stages of motor learning of this unusual and technically difficult movement. Our data also confirm that the SM activation is closely linked to the intrinsic parameters of the movement; while the SMA may be activated by different aspects of the movement realization and preparation.
Suppression of motor cortical excitability by electrical stimulation over the cerebellum in ataxia
Authors: Ugawa Yoshikazu , Genba‐Shimizu Kieko , Rothwell John C. , Iwata Makoto , Kanazawa Ichiro 
Published: January 2005

AbstractWe studied the effect of electrical stimulation over the cerebellum on electromyographic responses evoked by magnetic stimulation over the cerebral motor cortex in 14 normal volunteers and 32 patients with ataxia due to various disorders. In all the normal subjects, stimulation over the cerebellum significantly reduced the size of electromyographic response in the first dorsal interosseous muscle evoked by magnetic cortical stimulation, when the cerebellar stimulus preceded the cortical stimulus by 5, 6, and 7 msec. This suppression was absent or reduced in ataxic patients who had atrophy of the cerebellar hemispheres as demonstrated by magnetic resonance imaging and in patients with dysfunction of the cerebellothalamocortical pathway who had lesions in the superior cerebellar peduncle or in the motor thalamus. In contrast, suppression was normal in ataxic patients who had pontine lesions that affected the pontocerebellar afferent pathway to the cerebellum. Results were also normal in patients without cerebellar ataxia, such as those with Parkinson's disease, sensory ataxia, and cerebrovascular disease without ataxia. We conclude that electrical stimulation activates cerebellar structures that suppress motor cortical excitability through a cerebellothalamocortical pathway and that the afferent systems to the cerebellum make no or little contribution to the effect. The technique described here would be useful for distinguishing ataxia due to lesions of cerebellar afferent pathway from other types of cerebellar ataxia.
The effects of ciliary neurotrophic factor on motor dysfunction in wobbler mouse motor neuron disease
Authors: Mitsumoto Hiroshi , Ikeda Ken , Holmlund Tomas , Greene Tom , Cedarbaum Jesse M. , Wong Vivien , Lindsay Ronald M. 
Published: January 2005

AbstractCiliary neurotrophic factor is the first neurotrophic factor to show survival‐promoting effects in developing motor neurons in vitro, in ovo, and in vivo. In the present study we tested the effects of recombinant rat or human ciliary neurotrophic factor in the wobbler mouse model of motor neuron disease. Mice received 1 mg/kg of the factor or a vehicle solution subcutaneously three times a week for 4 weeks, after the disease was diagnosed between the ages of 3 and 4 weeks. Although treatment with rat ciliary neurotrophic factor (n = 6) resulted in delayed weight gain (p <0.001), grip strength normalized to body weight in the factor‐treated mice was significantly greater (p <0.02) and declined at a slower rate (p < 0.05) compared to that in vehicle‐treated animals. Human ciliary neurotrophic factor (n = 27) produced no change in body weight and reduced paw position and walking pattern abnormalities (p < 0.001 and p < 0.02, respectively). After 4 weeks of treatment, the mean grip strength of human ciliary neurotrophic factor‐treated animals was twice as great (p < 0.001) and declined at a much slower rate (p <0.005) than that of control mice. The time required to run 2.5 ft was less (p < 0.005) and muscle twitch tension was greater (p < 0.002) in ciliary neurotrophic factor‐treated animals. Thus, ciliary neurotrophic factor retarded the disease progression and improved muscle strength in this motor neuron disease model.
Immune complex deposition in the choroid plexus of patients with acquired immunodeficiency syndrome
Authors: Falangola M. F. , Castro‐Filho B. G. , Petito C. K. 
Published: January 2005

AbstractWe identified immune complex deposits in the choroid plexus of approximately 75% of patients with acquired immunodeficiency syndrome (AIDS) who had either normal brains or human immunodeficiency virus encephalitis. Since circulating immune complexes are common in AIDS patients, and since local choroid plexus pathology usually was absent, their likely origin is from the bloodstream. Choroid plexus deposits of immune complexes have been implicated in altering the function of this structure or in enhancing its vulnerability to infection. Therefore, immune complex deposition in the choroid plexus of AIDS patients may be responsible for some of the common alterations in the cerebrospinal fluid and for the frequency of opportunistic infections in this region.
Trinucleotide repeat length and rate of progression of Huntington's disease
Authors: Illarioshkin Sergei N. , Igarashi Shuichi , Onodera Osamu , Markova Elena D. , Nikolskaya Natalya N. , Tanaka Hajime , Chabrashwili Tina Z. , Insarova Nina G. , Endo Kotaro , Ivanova‐Smolenskaya Irina A. , Tsuji Shoji 
Published: January 2005

AbstractThe Huntington's disease gene contains an expanded unstable (CAG)n repeat, and the repeat lengths have been shown to correlate with the age of onset. Using detailed clinical scales, we evaluated the rate of progression of Huntington's disease and its relationship to the number of triplet repeats. We found significant positive correlation between the rate of progression of clinical symptoms (both neurological and psychiatric) and CAG repeat length. These data suggest an important role of expanded trinucleotide repeat length in affecting the pathological process during the enitre course of Huntington's disease.
Dose‐dependent association of apolipoprotein e allele ε4 with late‐onset, sporadic Alzheimer's disease
Authors: Yoshizawa Toshihiro , Yamakawa‐Kobayashi Kimiko , Komatsuzaki Yasuko , Arinami Tadao , Oguni Ei'ichi , Mizusawa Hidehiro , Shoji Shin'ichi , Hamaguchi Hideo 
Published: January 2005

AbstractWe examined the apolipoprotein E (apo E) genotypes in 47 patients with late‐onset sporadic Alzheimer's disease (mean age at onset ± standard deviation, 72.2 ± 6.4 years), 8 with late‐onset familial Alzheimer's disease (75.5 ± 5.1 years), 18 with early‐onset sporadic Alzheimer's disease (52.8 ± 4.7 years), and 10 with early‐onset familial Alzhemer's disease (52.0 ± 6.8 years) in Japan and compared them with genotypes in control subjects. In late‐onset sporadic Alzheimer's disease, apo E‐ε4 frequency increased significantly (ε4 frequency: 0.34 vs 0.095 in controls, p < 0.0001), and the odds ratio, which represents the strength of association between Alzheimer's disease and apo E‐ε4 gene (3 [95% confidence interval, 2–6] in one dose; 43 [95% confidence interval, 12–154] in two doses). This study also suggested that apo E‐ε4 is associated with both late‐onset (ε4: 0.31) and early‐onset familial Alzheimer's disease (ε4: 0.35). In contrast, we found no association between apo E‐ε4 and early‐onset sporadic Alzheimer's disease (ε4: 0.08). These results indicate that the risk of developing late‐onset sporadic Alzheimer's disease is markedly dependent on the dose of apo E‐ε4, while apo E‐ε4 does not appear to be a major risk factor for early‐onset sporadic Alzheimer's disease.
In situ polymerase chain reaction demonstration of JC virus in progressive multifocal leukoencephalopathy, including an index case
Authors: Ueki Keisuke , Richardson Edward P. , Henson John W. , Louis David N. 
Published: January 2005

AbstractProgressive multifocal leukoencephalopathy (PML) is a demyelinating disease that is caused by JC papovavirus. The virus can be demonstrated in brains with PML using a variety of techniques. In situ polymerase chain reaction (PCR) is a new method that combines the sensitivity of PCR with the histological localization of in situ hybridization. We here show that in situ PCR can detect JC virus in archival tissue sections of 3 cases of PML, including 1 of the original cases described in 1958 and 1 case that was fixed in formalin for 7 weeks. JC virus DNA was amplified directly on tissue and demonstrated in the characteristic enlarged oligodendrocytes and bizarre astrocytes. These data illustrate the utility of the in situ PCR technique in detecting JC virus in archival sections, including those fixed for prolonged periods, and provide a historical footnote to the original report of PML.
Oxidative damage to mitochondrial DNA is increased in Alzheimer's disease
Authors: Mecocci Patrizia , MacGarvey Usha , Beal M. Flint 
Published: January 2005

AbstractOxidative damage to DNA may play a role in both normal aging and in neurodegenerative diseases. We examined whether Alzheimer's disease (AD) is associated with increased oxidative damage to both nDNA and mtDNA in postmortem brain tissue. We measured the oxidized nucleoside, 8‐hydroxy‐2′‐deoxyguanosine (OH8dG), in DNA isolated from three regions of cerebral cortex and cerebellum in 13 AD and 13 age‐matched controls. There was a significant threefold increase in the amount of OH8dG in mtDNA in parietal cortex of AD patients compared with controls. In the entire group of samples there was a small significant increase in oxidative damage to nDNA and a highly significant threefold increase in oxidative damage to mtDNA in AD compared with age‐matched controls. These results confirm that mitochondrial DNA is particularly sensitive to oxidative damage, and they show that there is increased oxidative damage to DNA in AD, which may contribute to the neurodegenerative process.
Extracellular human T‐cell lymphotropic virus type I tax protein induces cytokine production in adult human microglial cells
Authors: Dhib‐Jalbut Suhayl , Hoffman Paul M. , Yamabe Toshio , Sun Di , Xia Jane , Eisenberg Howard , Bergey Gregory , Ruscetti Francis W. 
Published: January 2005

AbstractTropical spastic paraparesis (HAM/TSP) is caused by human T‐cell lymphotropic virus type I (HTLV‐I) infection. Although the virus infects T cells in vivo and is capable of infecting microglia in vitro, the inflammatory demyelination has not been linked to virus in central nervous system tissue. Thus, indirect mechanisms (e.g., cytokines) could be involved in demyelination and inflammation. The ability of HTLV‐I Tax protein to induce tumor necrosis factor‐α (TNF‐α), interleukin‐1 β (IL‐1β), and IL‐6 in primary adult human microglia and peripheral blood macrophages (PBMs) was examined by enzyme‐linked immunosorbent assay and reverse transcription–polymerase chain reaction (RT‐PCR). Tax (20 ng/ml) induced TNF‐α in microglia (from undetectable or low basal levels to 215–1,075 pg/ml, mean 576 ± 375 pg/ml, n = 4) and in PBMs (70‐1,900, mean 646 ± 844 pg/ml, n = 4). This induction was dose dependent, Tax specific, and maximal at 8 hours after stimulation. IL‐6 levels in microglia increased from a basal level of 368 ± 194 to 664 ± 270 pg/ml 24 hours after Tax stimulation. In contrast, IL‐1β levels were modestly induced (≤26 pg/ml). An increase in mRNA levels of the three cytokines was observed by semiquantitative RT‐PCR (TNF‐α = 28‐fold; IL‐6 = 5.6‐fold; IL‐1β = 3.6‐fold). Thus, in HAM/TSP, extracellular Tax released from infiltrating T cells could induce cytokine release by microglia and contribute to demyelination and inflammation in the absence of detectable virus.
Molecular mimicry between GQ1b ganglioside and lipopolysaccharides of Campylobacter jejuni isolated from patients with Fisher's syndrome
Authors: Yuki N. , Taki T. , Takahashi M. , Saito K. , Yoshino H. , Tai T. , Handa S. , Miyatake T. 
Published: January 2005

AbstractWe isolated Campylobacter jejuni from 2 patients with Fisher's syndrome subsequent to enteritis. Crude lipopolysaccharide fractions were extracted from the bacteria and separated by thin‐layer chromatography. Monoclonal antibodies to GQ1b ganglioside (GMR 13 and 7F5) reacted with both lipopolysaccharide fractions, indicating that the lipopolysaccharides bear the GQ1b epitope. This is the first report of molecular mimicry between neural tissue components and the antecedent infectious agents of Fisher's syndrome.
Apolipoprotein E ε4 allele distribution in alcoholic dementia and in Alzheimer's disease in Japan
Authors: Muramatsu Taro , Higuchi Susumu , Arai Hiroyuki , Sasaki Hidetada , Yamada Koichi , Hayashida Motoi , Trojanowski John Q. 
Published: January 2005

AbstractThe apolipoprotein E ε4 allele has been associated with both familial and sporadic Alzheimer's disease (AD). Given its possible role in nerve repair and growth, it is plausible that apolipoprotein E may be a common denominator in the pathogenesis of several dementing diseases. Therefore, we investigated ε4 frequencies in demented and nondemented alcoholics, as well as in patients with sporadic AD and controls in Japan. No significant difference in allele frequencies was found between demented and nondemented alcoholics and controls, while a significant association was demonstrated between AD and the ε4 allele. These results support a specific role of ε4 in the pathogenesis of AD, rather than a more general role for ε4 in dementing illnesses.
Amyotrophic lateral sclerosis immunoglobulins increase Ca2+ currents in a motoneuron cell line
Authors: Mosier Dennis R. , Baldelli Pietro , Delbono Osvaldo , Smith R. Glenn , Alexianu Maria E. , Appel Stanley H. , Stefani Enrico 
Published: January 2005

AbstractThe sporadic from of amyotrophic lateral sclerosis (ALS) is an idiopathic and eventually lethal disorder causing progressive degeneration of cortical and spinal motoneurons. Recent studies have shown that the majority of patients with sporadic ALS have serum antibodies that bind to purified L‐type voltage‐gated calcium channels and that antibody titer correlates with the rate of disease progression. Furthermore, antibodies purified from ALS patient sera have been found to alter the physiologic function of voltage‐gated calcium channels in nonmotoneuron cell types. Using wholecell patch‐clamp techniques, immunoglobulins purified from sera of 5 of 6 patients with sporadic ALS are now shown to increase calcium currents in a hybrid motoneuron cell line, VSC4.1. These calcium currents are blocked by the polyamine funnel‐web spider toxin FTX, which has previously been shown to block Ca2+ currents and evoked transmitter release at mammalian motoneuron terminals. These data provide additional evidence linking ALS to an autoimmune process and suggest that antibody‐induced increases in calcium entry through voltage‐gated calcium channels may occur in motoneurons in this disease, with possible deleterious effects in susceptible neurons.