bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neurology research: A journal survey, 1980–1990
Authors: Jamieson Patrick W. 
Published: January 2005

AbstractThere have been major changes in neurology research during the past decade, but no systematic examination of the content, methods, funding, and research personnel. All scientific articles in the journals Annals of Neurology, Archives of Neurology, and Neurology were categorized by article type, number of authors, highest degree of first author, institution, disease area, reseach field, and source of support for the years 1980 and 1990. Original articles grew by 54% over the decade. The average number of authors increased by 1.3 to 5 (p > 0.0001). Groups of MDs and PhDs wrote 39% of articles in 1990 (24% in 1980). Contributions with a diagnostic or therapeutic focus rose from 7 to 14%, whereas there was a pronounced drop in case reports from nearly 40% to just under 20%. Articles on movement disorders and neurodegenerative diseases increased more than 100% and now together comprise more than 25% of original articles. Molecular biology increased by 1,600%, and there was significant growth in epidemiology, neuroimaging, neurochemistry, and neuropsychology. The top 20 publishing institutions accounted for 40% of all original articles and 60% of US original articles. Foreign contributions doubled over the decade. The National Institutes of Health funded 46% of US articles in 1990 (35.5% in 1980). More than 80% of articles in 1990 on neurodegenerative disease were supported by the National Institutes of Health. The complexity of neurological research has driven greater collaboration between basic and clinical scientists. New areas of clinical and basic knowledge are rapidly emerging in neurology.
Plasma exchange in Guillain‐Barré syndrome: One‐year follow‐up
Authors:   French Cooperative Group on Plasma Exchange in Guillain‐Barré Syndrome
Published: January 2005

AbstractWe report the one‐year assessment of plasma exchange (PE) versus control in Guillain‐Barré syndrome (GBS), based on a randomized multicenter clinical trial of 220 patients with GBS. Treated patients received four PEs, with either albumin or fresh frozen plasma (FFP) as replacement fluid. This study completes the short‐term analysis previously published (Ann Neurol 1987;22:753–761).Long‐term benefit from PE was observed, as demonstrated by full muscular strength recovery at one year: 71% in the PE group versus 52% in the control group (p = 0.007), confirmed after adjustment for four prognostic factors. FFP showed no additional benefit compared with albumin (77% full recovery in the FFP group versus 65% in the albumin group; p = 0.22). PE did not affect the incidence of severe motor disability (11% in both groups).
Hypodensity of the cerebral white matter in patients with transient ischemic attack or minor stroke: Influence on the rate of subsequent stroke
Authors: van Swieten J. C. , Kappelle L. J. , Algra A. , van Latum J. C. , Koudstaal P. J. , van Gijn J. 
Published: January 2005

AbstractIn a prospective study of 3,017 patients with transient ischemic attack or minor ischemic stroke from the Dutch Transient Ischemic Attack Trial, the presence or absence of diffuse hypodensity of the white matter on a baseline computed tomography (CT) scan of the brain was related to the occurrence of subsequent stroke. On entry, 337 patients were judged to have diffuse hypodensity of the white matter on CT; they were older (71.4 ± 7.4 years versus 64.4 ± 9.9 years), more often had hypertension (50% versus 41%), and more often had lacunar infarcts on CT scan (40% versus 26%) than did patients with normal white matter. Strokes, fatal or nonfatal, occurred in 51 (15%) of the patients with diffuse hypodensity of the cerebral white matter, compared to 217 (8%) in the group with normal white matter (crude hazard ratio, 2.0; 95% confidence interval, 1.4–2.7). After adjustment for age and other relevant entry variables, the hazard ratio was 1.6 (95% confidence interval, 1.2–2.2). In patients younger than 70 years the crude hazard ratio was 2.7 (95% confidence interval, 1.7–4.2). The distribution between the main subtypes of stroke was similar for patients with and those without diffuse hypodensity of the cerebral white matter: Intracerebral hemorrhage occurred in 6 and 9%, cortical infarction in 47 and 45%, and lacunar infarction in 34 and 29%, respectively. We conclude that hypodensity of the cerebral white matter in patients with transient ischemic attack or minor stroke is associated with an extra risk of future stroke, from large as well as from small vessels, and particularly in patients under 70 years old; this increase of risk is independent of other risk factors for stroke.
A novel neuronal messenger molecule in brain: The free radical, nitric oxide
Authors: Dawson Ted M. , Dawson Valina L. , Snyder Solomon H. 
Published: January 2005

AbstractUnderstanding of the organization and function of a newly identified neuronal messenger molecule, nitric oxide, has progressed rapidly. Nitric oxide synthase has been purified and molecularly cloned from brain. Its localization is exclusively neuronal and endothelial. The catalytic activity of nitric oxide synthase accounts for the NADPH diaphorase staining of neurons that are uniquely resistant ot toxic insults and neurodegenerative disorders. Nitric oxide has diverse functions. In platelets it inhibits their aggregation, in macrophages it mediates cytotoxicity, and in blood vessels it acts as a vasodilator. In the nervous system nitric oxide may be the retrograde transmitter in long‐term potentiation. It is the “neurotransmitter” of cerebral vasodilator nerves and the inhibitory “neurotransmitter” of the motor neurons of the intestines. Nitric oxide in situations of excessive production may function as neurotoxin, suggesting a role for nitric oxide in neurodegenerative disorders.
Motor inhibition and excitation are independent effects of magnetic cortical stimulation
Authors: Triggs William J. , Macdonell Richard A. L. , Cros Didier , Chiappa Keith H. , Shahani Bhagwan T. , Day Bruce J. 
Published: January 2005

AbstractWe administered magnetic cortical stimulation (MCS) during voluntary contraction of intrinsic hand muscles to 8 patients with motor neuron disease (MND), 5 patients with pure lower motor neuron syndromes (LMN), a patient with severe subacute sensory neuropathy (SSN), and 10 healthy volunteers. Patients with MND had clinical evidence of upper MND and elevated thresholds for (3 patients) or absence of (5 patients) motor evoked potentials (MEPs). MCS during sustained contraction inhibited electromyographic activity in 6 of 8 patients with MND, without preceding MEPs. MCS had no effect on the electromyogram (EMG) of the other 2 patients with MND. In normal subjects and patients with LMN inhibition of EMG was never seen without a preceding MEP, regardless of stimulus intensity. In the patient with SSN, MCS elicited normal MEPs and inhibited the EMG in a pattern similar to normal subjects, whereas supramaximal electrical stimulation of median and ulnar nerves failed to inhibit the EMG despite normal M and F responses. Our findings indicate that the inhibitory effects of MCS on EMG are not dependent solely on changes in afferent feedback caused by the muscle twitch produced by the MEP, or on Renshaw cell inhibition. We suggest that some of the inhibitory and excitatory effects of MCS on the motor system are mediated by distinct cortical elements, which may have different susceptibilities to pathophysiological processes in MND.
Felbamate monotherapy: Controlled trial in patients with partial onset seizures
Authors: Sachdeo Rajesh , Kramer Lynn D. , Rosenberg Alberto , Sachdeo Satinder 
Published: January 2005

AbstractFelbamate (FBM) monotherapy was evaluated in 44 patients with uncontrolled partial onset seizures in a unique, single‐center, randomized, double‐blind, parallel‐group trial. During the 56‐day baseline period, patients were required to have at least 8 seizures and to be receiving only one standard antiepileptic drug (AED) at a therapeutic level; a second AED was allowed if at a subtherapeutic level. Patients were randomized to valproate (VPA), 15 mg/kg, or to FBM, 3,600 mg/day. In the treatment phase, previous AEDs were discontinued by study day 28 (by one‐third decrements on study days 1, 14, and 28). Study end points were completion of 112 study days or the fulfilling of escape criteria. Criteria for escape relative to baseline were: two‐fold increase in monthly seizure frequency, two‐fold increase in highest 2‐day seizure frequency, single generalized tonic‐clonic seizure (GTC) if none occurred during baseline, or significant prolongation of GTCs. The predetermined primary efficacy variable was the number of patients escaping in each treatment group. Nineteen patients on VPA and 3 on FBM met escape criteria (p < 0.001, chi‐square test). When overall seizure frequency among study completers was compared with baseline, the FBM group had a 50 to 65% reduction in seizure frequency. FBM adverse experiences were all mild or moderate in severity, and the incidence of adverse experiences was lower in monotherapy. FBM monotherapy was effective in the treatment of partial onset seizures with or without generalization and demonstrated a favorable safety profile.
Carnitine acetyltransferase activity in the human brain and its microvessels is decreased in Alzheimer's disease
Authors: Kalaria Rajesh N. , Harik Sami I. 
Published: January 2005

AbstractL‐Carnitine and acetyl‐L‐carnitine facilitate mitochondrial β‐oxidation of fatty acids. In the brain, they may also have a role in acetylcholine synthesis. Carnitine acetyltransferase catalyzes the interchange between L‐carnitine and acetyl‐L‐carnitine. Recently, acetyl‐L‐carnitine was reported to have a beneficial effect in patients with Alzheimer's disease. We therefore assessed carnitine acetyltransferease activity in selected brain regions and in isolated cerebral microvessels obtained at autopsy from patients with Alzheimer's disease and from age‐matched control subjects. We found a 25 to 40% decrease in carnitine acetyltransferase activity in patients with Alzheimer's disease, which attained statistical significance in most brain regions and in cerebral microvessels. These findings document another neurochemical abnormality in patients with Alzheimer's disease and provide a rationale for the use of acetyl‐L‐carnitine in the treatment of patients with Alzheimer's disease.
Electron transfer complex I defect in idiopathic dystonia
Authors: Benecke Reiner , Strümper Petra , Weiss Hanns 
Published: January 2005

AbstractIt has been suggested that dystonia is caused by an autosomal gene with reduced penetrance and a consequent biochemical abnormality affecting cell activity within the basal ganglia. No consistent biochemical disturbance has been identified. In the present study, activities of the mitochondrial electron transfer complexes were measured in platelets of 31 patients with idiopathic dystonia. Enzyme assays of these patients were compared to measurements in 28 control subjects. A significant decrease of complex I activity was observed in the majority of the patients, whereas the activities of other electron transfer complexes were normal. The severity of the complex I defect was more pronounced in patients with the segmental or generalized form than in those with focal dystonia. Complex I activity was not age dependent in the patients or control subjects. Although the electron pathway in complex I is disturbed in patients with idiopathic dystonia, complex I protein content seems to be normal. Whether abnormalities of complex I activity play a role in the pathogenesis of idiopathic dystonia remains to be determined.
The antiparkinson efficacy of deprenyl derives from transient improvement that is likely to be symptomatic
Authors: Schulzer M. , Mak E. , Calne D. B. 
Published: January 2005

AbstractWe undertook an analysis of the hazard functions derived from results published by the Parkinson Study Group following their investigation of deprenyl. Our findings indicate that the action of deprenyl is transient rather than sustained. We also infer that this effect may be mediated through alleviation of symptoms rather than by neuroprotection.
Cytochrome c oxidase activity in single muscle fibers: Assay techniques and diagnostic applications
Authors: Johnson Margaret A. , Bindoff Laurence A. , Turnbull Douglass M. 
Published: January 2005

AbstractMicrophotometric enzyme assay was used to study cytochrome c oxidase activity in single human skeletal muscle fibers. The assay techniques combine the precise localization of enzyme activity provided by histochemical methodology with the precise quantitation of a sensitive assay system. Abnormalities of cytochrome c oxidase were investigated using microphotometric enzyme assay in 12 patients with Kearns‐Sayre syndrome, chronic progressive external ophthalmoplegia, or Leigh's syndrome. Control values were obtained using muscle biopsy specimens from 20 juvenile and 18 adult subjects with no evidence of neuromuscular disease. In the patients with Leigh's syndrome due to cytochrome c oxidase deficiency, the abnormality was found to be expressed uniformly throughout the muscle fiber population. In contrast, patients with Kearns‐Sayre syndrome or chronic progressive external ophthalmoplegia showed abnormal heterogeneity of cytochrome c oxidase activity. In many cases, extreme degrees of variability were seen, with fibers containing high activity adjacent to fibers with no detectable activity. Mitochondrial DNA analysis showed that most of the patients with Kearns‐Sayre syndrome and chronic progressive external ophthalmoplegia had major rearrangements of mitochondrial DNA. It was concluded that the extreme variability of cytochrome c oxidase activity detected using microphotometric enzyme assay was an indicator of a probable abnormality of mitochondrial DNA. Conversely, cytochrome c oxidase defects in muscle which show a homogeneous distribution are more likely to be associated with defects of the nuclear genome.
Rapidly progressive aphasic dementia and motor neuron disease
Authors: Caselli Richard J. , Windebank Anthony J. , Petersen Ronald C. , Komori Takashi , Parisi Joseph E. , Okazaki Haruo , Kokmen Emre , Iverson Rita , Dinapoli Robert P. , Graff‐Radford Neill R. , Stein Steven D. 
Published: January 2005

AbstractArticulatory and language impairment heralded rapidly progressive motor neuron disease in 7 patients aged 54 to 77 years. One patient had a family history of a similar disorder. Severe nonfluent aphasia developed in all 7 patients and 4 were anarthric within a year. Other cognitive domains were impaired, yet 2 patients lived alone until 1 month before their deaths. Four died within 2 years. Abnormalities were found on electromyography, computed tomography, magnetic resonance imaging, single‐photon emission computed tomography, and electroencephalography. Neuropathological examination in 3 patients showed bilateral hemispheric atrophy with neuronal loss and gliosis predominantly of superficial cortical layers. Pigmented and hypoglossal nuclei were relatively preserved. At all spinal levels there was degeneration of corticospinal tracts and loss of anterior horn cells with gliosis. Rapidly progressive aphasic dementia and motor neuron disease are a distinctive clinical entity whose nosology is poorly understood.
Human class I major histocompatibility complex transgene prevents virus‐induced demyelination in susceptible mutant B 10.d2dml mice
Authors: Rodriguez M. , Pedrinaci S. , David C. S. 
Published: January 2005

AbstractTheiler's murine encephalomyelitis virus (TMEV) induces immune‐mediated demyelination in susceptible strains of mice, providing an excellent model for multiple sclerosis. Class I genes within the major histocompatibility complex locus (H‐2D region)play a major role in determining whether strains of mice develop chronic in determining whether strains of mice develop chronic demyelination and TMEV persistence. B 10.D2dml mice with deletion in the 3′ end of Dd and the 5′ end of Ld genes develop the most prominent demyelination in comparison with resistant B10. D2 mice normal complementation of H‐2D region genes. We tested whether expression of a class I human transgene (HLA‐B27) would modulate virus‐induced demyelination in mutant B10.D2dml mice. Transgenic B10.D2dml (HLA‐B27+) mice infected with virus showed dramatic decrease in the extent of demyelination (p < 0.0001) and virus antigen expression in spinal cord compared with littermate controls without the human class I transgene. These experiments demonstrate that transgenic expression of a human class I major histocompatibility complex locus molecule can prevent demyelination induced by a virus in mutant mice.
Cerebral lymphoma presenting as a nonenhancing lesion on computed tomographic/ magnetic resonance scan
Authors: DeAngelis Lisa M. 
Published: January 2005

AbstractCerebral lymphoma is usually indentified on computered tomographic/magnetic resonance scan as a homogeneously contrast‐enhancing periventricular mass lession. We present 10 patients with cerebrllymphoma who had nonenhancing tumor on computed tomographic (2 patients) or magnetic resonance scan (8 patients) either at the time of diagnosis or recurrence. The absence of enhancement caused diagnostic delay in all patients and suggests that tumor resides behind an intact blood brain barrier, raising important therapeutic implications in the design of chemotherapeutic regimens.
A controlled trial of lazabemide (RO19–6327) in untreated Parkinson's disease
Authors:   Parkinson Study Group, Kieburtz Karl 
Published: January 2005

AbstractThe monoamine oxidase type B inhibitor deprenyl (selegiline) has been demonstrated to delay the emergence of disability in early untreated Parkinson's disease. Lazabemide (RO19–6327) is a short‐acting, reversible, highly selective inhibitor of monoamine oxidase type B which, unlike deprenyl, is not metabolized to active compounds. We conducted a randomized, double‐blinded clinical trial to assess the short‐term tolerability of lazabemide in subjects who had early, untreated Parkinson's disease. Two hundred and one patients were enrolled at 14 centers and randomized to receive 100 mg/day, 200 mg/day, or 400 mg/day of lazabemide or matching placebo. Subjects were followed for 8 weeks including a randomized, double‐blinded withdrawal of lazabemide for 2 or 4 weeks. The primary measure of tolerability was the proportion of treated subjects who were able to complete the study on their originally assigned treatment. Clinical features were assessed by the Unified Parkinson's Disease Rating Scale (UPDRS). Lazabemide treatment was as well tolerated as placebo and was not attended by serious adverse experiences. A significant improvement in the activities of daily living component of the rating scale was found after 4 weeks of lazabemide treatment, although other subscale scores did not change significantly. The overall safety and benefits of lazabemide observed in this short‐term study justify further long‐term investigations to determine if this monoamine oxidase type B inhibitor can slow the clinical progression of Parkinson's disease.
Callosal apraxia without agraphia
Authors: Kazui Seiji , Sawada Tohru 
Published: January 2005

AbstractWe describe a patient with left unilateral ideomotor apraxia without left‐sided agraphia caused by a callosal lesion that was demonstrated by magnetic resonance imaging. The clinical features, together with data in the literature, suggest that the callosal fibers for writing are concentrated in the posterior corpus callosum, while those for praxis cross in the more rostral part of the posterior half of the callosum.
Delayed administration of memantine prevents N‐methyl‐D‐aspartate receptor‐mediated neurotoxicity
Authors: Pellegrini James W. , Lipton Stuart A. 
Published: January 2005

AbstractIncreasing evidence supports the hypothesis that escalating levels of excitatory amino acids (EAAs) are responsible for neuronal cell death in a variety of acute neurological conditions including hypoxia/ischemia, trauma, seizures, and hypoglycemia. EAAs may also contribute to several chronic neurodegenerative diseases including Huntington's disease, parkinsonism, and acquired immunodeficiency syndrome dementia. A predominant form of neurotoxicity appears to be mediated by excessive activation of the N‐methyl‐D‐aspartate subtype of glutamate receptor. This laboratory recently reported that memantine, an antiparkinsonian drug, is a potent N‐methyl‐D‐aspartate antagonist capable of preventing the death of central neurons both in vitro and in vivo when given coincident to an EAA insult. In the present study, we found that 12 μM memantine prevented the death of neonatal rat retinal ganglion cells in primary culture when administered up to 4 hours after the initiation of N‐methyl‐D‐aspartate receptor‐mediated neurotoxicity.
Nonepileptic seizures during apparent sleep
Authors: Thacker Kimberley , Devinsky Orrin , Perrine Kenneth , Alper Kenneth , Luciano Daniel 
Published: January 2005

AbstractWe report 12 patients with nonepileptic seizures(NESs) documented with video‐electroencephalographic monitoring during apparent sleep. There were 7 females and 5 males, ages 11 to 67 years(mean, 33 yr). In all patients there was a well‐organized posterior α rhythm present immediately before onset of clinical changes despite the appearance of sleep. Patients later “confirmed” that these attacks occured during sleep. Features of these episodes were typical of NESs and none were associated with ictal or postictal electroencephalographic changes other than muscle and movement artifact. Provocative testing with suggestion confirmed the nonepileptic nature of these episodes in 9 patients. Three patients had epileptiform discharges during prolonged interictal recordings and 1 had a documented complex partial seizure. Our findings indicate that reports of seizures during sleep are not uncommon among patients with NESs.
Spinal fluid lymphocytes from a subgroup of multiple sclerosis patients respond to mycobacterial antigens
Authors: Birnbaum Gary , Kotilinek Linda , Albrecht Lisa 
Published: January 2005

AbstractImmune responses to heat shock or stress proteins are observed in several chronic autoimmune diseases. Such proteins are major antigens of many bacteria, especially mycobacteria. To determine whether immune responses to stress proteins occur in chronic inflammatory diseases of the central nervous system such as multiple sclerosis (MS) we measured proliferative responses of lymphocytes from spinal fluids and bloods of patients with MS and other neurological diseases to a sonicate of M. tuberculosis, an acetone extract of M. tuberculosis, a recombinant 65‐kd heat shock protein of M. leprae, and tetanus toxoid as a control recall antigen. Significantly increased spinal fluid lymphocyte responses to mycobacterial sonicate, relative to responses from paired peripheral blood lymphocytes, were present in 14 of 20 specimens from patients with MS (p < 0.025) and 2 of 9 specimens from patients with other neurological diseases. Spinal fluid lymphocytes also responded to tetanus toxoid, but differences between blood and spinal fluid were not statistically significant. Lymphocytes from 1 patient with MS responded only to M. leprae. There were no proliferative responses to the M. tuberculosis acetone extract. When patients with MS were classified according to duration of disease (<2‐ or >2‐yr duration) 9 of 10 patients with recent onset had cerebrospinal fluid cells that responded to M. tuberculosis compared with 5 of 10 with longer duration symptoms (p < 0.012). Our data suggest a selective recruitment and/or expansion of mycobacterial reactive cells to the central nervous system of a subpopulation of patients with MS. Immune responses to antigens present in mycobacteria, possibly stress proteins, may be important in perpetuating and amplifying chronic inflammatory diseases of the central nervous system such as MS.