bluuebunny/crossref_metadata_2025_split huggingface dataset:
Immunocytochemical and virological characteristics of hiv‐associated inflammatory myopathies: Similarities with seronegative polymyositis
Authors: Illa Isabel , Nath Avindra , Dalakas Marinos 
Published: January 2005

AbstractWe performed an immunoperoxidase study on muscle biopsy specimens from 19 patients with polymyositis who were seropositive for human immunodeficiency virus (HIV) (21 specimens) and 5 HIV‐seronegative patients with polymyositis and compared the findings. A quantitative analysis of T cells and T‐cell subsets, B cells, natural killer cells, interleukin‐2 receptor–positive cells, and macrophages was performed on serial sections from all the specimens. Localization of major histocompatibility complex (MHC)‐I and ‐II antigens, alpha and gamma interferon, and HIV antigens (p24, gp120, and gp41) was performed using specific antisera. In specimens from HIV‐positive and seronegative patients, the predominant cell population was CD8+s cells and macrophages invading or surrounding healthy muscle fibers that expressed MHC‐I antigen on their surface. The endomysial infiltrates in specimens from HIV‐positive patients differed from those seen in specimens from the seronegative patients only by a significant reduction of the CD4+ cells (12.6 ± 3.2% versus 21.1 ± 4.2%). HIV antigens were seen in occasional interstitial mononuclear cells (but not in muscle fibers) in 6 of the 21 specimens from HIV‐positive patients. Interferon was not localized. We conclude that the development of HIV‐associated polymyositis does not appear to be related to direct infection of the muscle fibers by HIV but rather is due to a T‐cell–mediated and MHC‐I–restricted cytotoxic process, perhaps triggered by HIV. Because this immunopathological mechanism is common in both HIV‐associated polymyositis and polymyositis alone, it is suggested that viruses may also be responsible in triggering polymyositis.
Fabry disease: Detection of 13‐bp deletion in α‐galactosidase a gene and its application to gene diagnosis of heterozygotes
Authors: Ishii Satoshi , Sakuraba Hitoshi , Shimmoto Michie , Minamikawa‐Tachino Reiko , Suzuki Tadashi , Suzuki Yoshiyuki 
Published: January 2005

AbstractPolymerase chain reaction amplification of reversetranscribed messenger RNA from a patient with Fabry disease revealed a 13‐base pair deletion in the 5′ region (exon 1) of α‐galactosidase A complementary DNA. This gene rearrangement was not detected by Southern or Northern analysis. Short direct repeats were present around the breakpoints, and considered to be of pathogenetic significance. Gene diagnosis of the mother and a female cousin was successfully achieved by polymerase chain reaction amplification of genomic DNA; the former as a Fabry disease heterozygote and the latter as a normal homozygote.
Zidovudine myopathy: A distinctive disorder associated with mitochondrial dysfunction
Authors: Mhiri C. , Baudrimont M. , Bonne G. , Geny C. , Degoul F. , Marsac C. , Roullet E. , Gherardi R. 
Published: January 2005

AbstractMuscle biopsy specimens were obtained from 48 human immunodeficiency virus–infected patients suffering from various neuromuscular symptoms. Microscopic examination by conventional and electron microscopy revealed a characteristic structural myopathy associated with mitochondrial changes in 13 patients, all of whom had received longterm zidovudine therapy. The mean cumulative dose they had received (498 ± 145 gm) was significantly higher than that of the other 14 zidovudine recipients of the study. They suffered from a progressive, usually painful, proximal myopathy with pronounced wasting, normal‐to‐moderately elevated creatine kinase levels, and a myopathic electromyographic pattern. The condition usually improved after withdrawal of the drug. Assay of mitochondrial enzymes, including succinate–cytochrome c reductase, cytochrome c oxidase, and citrate synthase, showed a decline in respiratory chain capacity. Southern blot analysis of mitochondrial DNA showed no abnormality. It is likely that mitochondrial dysfunction, probably resulting from drug‐induced inhibition of the mitochondrial DNA polymerase, is implicated in the pathogenesis of this complication of zidovudine therapy.
Double infarction in one cerebral hemisphere
Authors: Bogousslavsky Julien 
Published: January 2005

AbstractThirty‐two patients whose first stroke was due to double infarct in one cerebral hemisphere were identified among 1,911 consecutive patients from the Lausanne Stroke Registry. The double infarct involved territories of the superficial middle cerebral artery, superficial posterior cerebral artery, lenticulostriate, anterior choroidal artery, or borderzone. The most common combination involved territories of the anterior middle cerebral artery plus the posterior middle cerebral artery. In the patients with the double infarct, the prevalence of potential cardiac sources of embolism (19%) was similar to that found in the registry in general, but the double infarct was closely associated with tight (≥ 90% of the lumen diameter) stenosis or occlusion (75%) of the internal carotid artery. The most common neurological picture mimicked large infarction in the middle cerebral artery territory, but nearly half of the patients with double infarct in one cerebral hemisphere had a specific clinical syndrome, which was not found in the 1,879 remaining patients from the registry, including hemianopia‐hemiplegia (in 6), acute conduction aphasia‐hemiparesis (in 2), and acute transcortical mixed aphasia (in 6), in relation to characteristic combinations of infarcts. These unique clinical and etiological correlates warrant the recognition of double infarct in one cerebral hemisphere from other acute ischemic strokes.
Dopa‐responsive dystonia: [18F]dopa positron emission tomography
Authors: Sawle G. V. , Leenders K. L. , Brooks D. J. , Harwood G. , Lees A. J. , Frackowiak R. S. J. , Marsden C. D. 
Published: January 2005

AbstractThe syndrome of dopa‐responsive dystonia comprises a minority of patients with dystonia, yet it is of considerable diagnostic importance because patients respond dramatically to L‐dopa therapy. Benefits from this treatment are lasting, and the problems associated with long‐term L‐dopa therapy in patients with Parkinson's disease are generally absent. It has been suggested that this condition is due to a defect in the dopamine synthetic pathway, which is bypassed when patients are treated with L‐dopa. We have studied [18F]dopa uptake in 6 patients with classic doparesponsive dystonia (5 familial patients and 1 sporadic patient), aged 18 to 66 years. Data have been analyzed according to a graphic approach, calculating an influx constant for each region studied. We have also studied a seventh, clinically atypical, patient with juvenile dystonia‐parkinsonism. Similar data have been calculated for a group of 10 healthy control subjects and 10 patients with Parkinson's disease. The 6 patients with typical dopa‐responsive dystonia had a modest but significant reduction in the uptake of tracer into both caudate and putamen, which indicates a defect in the decarboxylation, vesicular uptake, and storage of [18F]dopa. This argues against the proposition that doparesponsive dystonia is due to an inherited defect of tyrosine hydroxylase alone. In the atypical patient, however, we found a greater reduction of [18F]dopa uptake into both caudate and putamen, comparable with that in patients with Parkinson's disease.
Treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulin
Authors: Cornblath David R. , Chaudhry Vinay , Griffin John W. 
Published: January 2005

AbstractChronic inflammatory demyelinating polyneuropathy is an immune‐mediated demyelinating peripheral neuropathy usually treated with immunosuppressants. We reviewed our experience treating 15 patients (9 men, 6 women) with intravenous immunoglobulin. Six patients were on other therapies at the time of intravenous immunoglobulin infusions (4, prednisone; 2, prednisone and azathioprine). The dose of intravenous immunoglobulin was either 0.3 or 0.4 gm/kg/day for 4 to 5 days. Transient fever occurred in 1 patient. Subjective improvement in sensory symptoms was reported by almost all patients. Objective improvements in strength or functional tasks occurred in only 3 patients, a man with human immunodeficiency virus infection, a 14‐year‐old girl, and a woman with an immunoglobulin G kappa paraprotein. Our results suggest that individual patients may respond to intravenous immunoglobulin therapy. A multicenter controlled trial is needed to assess properly the role of intravenous immunoglobulin therapy in patients with chronic inflammatory demyelinating polyneuropathy.
Use of computed tomography, magnetic resonance imaging, and localized 1H magnetic resonance spectroscopy in canavan's disease: A case Report
Authors: Marks Harold G. , Caro Pilar A. , Wang Zhiyue , Detre John A. , Bogdan Andrew R. , Gusnard Debra A. , Zimmerman Robert A. 
Published: January 2005

AbstractThe neuroradiological evaluation of Canavan's disease in a 38‐month‐old girl is discussed. Computed tomography showed diffuse symmetrical low attenuation values of the subcortical and deep cerebral white matter. Magnetic resonance imaging demonstrated symmetrical diffuse low signal intensity of T1‐weighted images and high signal intensity on T2‐weighted images. With the use of 1H magnetic resonance spectroscopy, we were able to show elevated levels of N‐acetylaspartic acid in the occipital lobe of our patient. The in vivo measurement of N‐acetylaspartic acid in the brain by 1H magnetic resonance spectroscopy offers an additional noninvasive diagnostic test for establishing the diagnosis of Canavan's disease. With the increasing availability of magnetic resonance spectroscopy, clinicians may be able to confirm the diagnosis of Canavan's disease immediately after magnetic resonance imaging reveals the typical abnormalities of the white matter.
Does tremor pace repetitive voluntary motor behavior in parkinson's disease?
Authors: Logigian Eric , Hefter Harald , Reiners Karlheinz , Freund Hans‐Joachim 
Published: January 2005

AbstractIn patients with Parkinson's disease and in normal subjects, the influence of tremor on repetitive voluntary movement was investigated in the index figer by comparing frequency of isometric force tremor with frequency of voluntary alternating isometric contractions. Tremor frequency, measured over the range from 0 to 70% maximum voluntary force, usually increased with force. The tremor frequency bands was lower and more often overlapped with the upper voluntary frequency range in patients than in normal subjects. Normal subjects could accurately produce voluntary contractions at all cue frequencies from 1 to 5 Hz. Patients could produce auditory‐paced frequencies of 1 and 2 Hz, but at higher cue frequencies, their voluntary contractions were often faster or slower than the cue. The faster or “hastened” voluntary frequencies were within the tremor frequency band, whereas the slowed voluntary frequencies were below t. Maximal voluntary frequency was often greater than the lowest but always less than the highest tremor frequency. It is concluded that parkinsonian tremor may pace voluntary repetitive movements to go faster than intended with the highest tremor frequency being an upper limit for voluntary frequency. Similar mechanisms may underlie the hastened repetitive vocal responses that wee also observed in the parkinsonian patients.
Lyme neuroborreliosis: A new sensitive diagnostic assay for intrathecal synthesis of borrelia burgdorferi–specific immunoglobulin G, A, and M
Authors: Hansen Klaus , Lebech Anne‐Mette 
Published: January 2005

AbstractAn antibody capture enzyme‐linked immunosorbent assay was developed to measure directly intrathecal immunoglobulin (Ig) G, A, and M synthesis specific for Borrelia burgdorferi. Purified, biotin‐avidin‐peroxidase‐labeled B. burgdorferi flagella was used as test antigen. Paired cerebrospinal fluid and serum specimens from 100 patients with clinically definite neuroborreliosis and 35 control subjects with neurological diseases were examined. Significant B. burgdorferi‐specific intrathecal IgG, A, and M production was found in 89%, 65%, and 67% of patients with neuroborreliosis. Local synthesis of specific IgA was only seen in patients with significant local IgG synthesis. Antibody production in cerebrospinal fluid began by 2 weeks after onset of neurological symptoms. At the end of the second week specific IgM, IgG, or both, was detected in 88% of the patients. Specific IgG synthesis was present in all patients by 6 weeks after onset. Specific local IgM synthesis usually disappeared by 3 to 6 months after therapy, whereas specific IgG synthesis persisted after recovery. Even in patients with a severely altered blood‐brain barrier, the assay discriminated between intrathecal antibody synthesis and antibody leakage from serum. The assay makes diagnostic measurement of B. burgdorferi–specific intrathecal antibody synthesis reliable, rapid, and accessible as a routine serological test.
Asymmetrical ocular pursuit with posterior fossa tumors
Authors: Furman Joseph M. R. , Hurtt Mark R. , Hirsch William L. 
Published: January 2005

AbstractWe report two patients with posterior fossa neoplasms who demonstrated asymmetrically impaired horizontal ocular pursuit documented with electrooculography. One patient had impaired pursuit contralateral to a pontomedullary lesion, whereas the second patient had impaired pursuit ipsilateral to a pontocerebellar lesion. These patients demonstrate that posterior fossa lesions may impair ocular pursuit either contralaterally or ipsilaterally unlike cerebral hemispheric lesions, which impair ocular pursuit ipsilaterally or bilaterally.
Recurrent attacks of amaurosis fugax treated with calcium channel blocker
Authors: Winterkorn Jacqueline M. S. , Teman Allen Jay 
Published: January 2005

AbstractVasospasm may be one of the causes of amaurosis fugax. A patient is reported who daily experienced multiple brief episodes of amaurosis fugax. The absence of physical, laboratory, or radiographic evidence for thromboembolism, hemodynamic compromise, or vasculitis, suggested that the amaurosis might be caused by vasospasm. This hypothesis was supported by cessation of the attacks of amaurosis when the patient was treated with a calcium channel blocker.
The serotonin antagonist mianserin improves functional recovery following experimental spinal trauma
Authors: Salzman Steven K. , Puniak Michael A. , Liu Zhong‐jun , Maitland‐Heriot Richard P. , Freeman Gina M. , Agresta Cynthia A. 
Published: January 2005

AbstractThe ability of the serotonin antagonist mianserin to improve neurological recovery after graded impact trauma to the thoracic region of the spinal cord was compared to that of cyproheptadine and ketanserin in pentobarbital‐anesthetized rats. Spinal cord injury was produced at T‐10 by the weight‐drop method and confirmed by the disappearance of the somatosensory‐evoked response during the subsequent 15 minutes. In all experiments, drug or vehicle treatments were randomly administered as a single intravenous bolus 15 minutes after injury. Functional outcome was blindly assessed for 2 weeks after injury using a modified Tarlov scale, and in some cases, the Rivlin‐Tator angleboard test. The survival of descending raphe‐spinal axons was determined by the measurement of serotonin in postmortem spinal tissues located above and below the site of injury. In separate acute experiments, the physiological and hemodynamic correlates of a 50 gm cm injury and either mianserin or vehicle injection were examined, as were the effects on serotonin content and metabolism in spinal tissues harvested 30 minutes after injury. All doses of mainserin were associated with some index of improved recovery following a 50 gm cm injury, with a 1‐mg/kg dose being clearly superior. Both ketanserin (0.1 mg/kg) and cyproheptadine (2 mg/kg) displayed marginal therapeutic actions for 50 gm cm injuries. In acute studies, mianserin at 1 mg/kg was associated with the preservation of posttraumatic spinal cord blood flow at T‐12 as well as a pronounced alteration in postmortem spinal serotonin content and metabolism, in contrast to vehicle control treatments. These data lend further support to a serotonergic hypothesis of secondary spinal cord injury and suggest the potential use of mianserin for the acute treatment of spinal cord injury.
Physical basis of cognitive alterations in alzheimer's disease: Synapse loss is the major correlate of cognitive impairment
Authors: Terry Robert D. , Masliah Eliezer , Salmon David P. , Butters Nelson , DeTeresa Richard , Hill Robert , Hansen Lawrence A. , Katzman Robert 
Published: January 2005

AbstractWe present here both linear regressions and multivariate analyses correlating three global neuropsychological tests with a number of structural and neurochemical measurements performed on a prospective series of 15 patients with Alzheimer's disease and 9 neuropathologically normal subjects. The statistical data show only weak correlations between psychometric indices and plaques and tangles, but the density of neocortical synapses measured by a new immunocytochemical/densitometric technique reveals very powerful correlations with all three psychological assays. Multivariate analysis by stepwise regression produced a model including midfrontal and inferior parietal synapse density, plus inferior parietal plaque counts with a correlation coefficient of 0.96 for Mattis's Dementia Rating Scale. Plaque density contributed only 26% of that strength.
Somatostatin replacement therapy for alzheimer dementia
Authors: Mouradian M. Maral , Blin Jerome , Giuffra Marianne , Heuser Isabella J. E. , Baronti Fabio , Ownby John , Chase Thomas N. 
Published: January 2005

AbstractSomatostatin is consistently diminished in brains of patients with Alzheimer's disease. To evaluate whether pharmacological restoration of this transmitter deficit has therapeutic value, the synthetic analogue octreotide was administered intravenously to 14 Alzheimer patients under double‐blind, placebo‐controlled conditions. At the highest dose administered, spinal fluid concentrations approximated those found in brains of experimental animals receiving behaviorally effective amounts of the drug. Neuropsychological testing, however, showed no clinically significant improvement. Coadministration of octreotide and physostigmine to 1 patient also failed to improve cognition. Positron emission tomographic studies in 6 patients revealed a generalized decrease in glucose metabolism as a result of octreotide infusion. These findings suggest that stimulation of the somatostatin system has no value in the symptomatic treatment of Alzheimer dementia.
The use of hospital discharge data for public health surveillance of guillain‐barré syndrome
Authors: Koobatian Thomas J. , Birkhead Guthrie S. , Schramm Margaret M. , Vogt Richard L. 
Published: January 2005

AbstractThe sensitivity of passive reporting of Guillain‐Barré syndrome (GBS) to the Vermont Department of Health from 1980 to 1985 was compared to that of computerized hospital discharge abstract data. Written hospital discharge summaries were reviewed for clinical data to validate the computerized abstracts. In all, 51 definite and probable cases of GBS were identified from hospital data during a period when only 4 cases (8%) had been reported to the health department through passive physician reporting. Based on the hospital data, the incidence of this syndrome in Vermont was 1.6/100,000 population/year. The incidence rate for males was 1.5 times that for females. No geographical or seasonal clustering of cases was found. These epidemiological features are consistent with previously published data on the syndrome and suggest that the incidence has not changed significantly in the past 10 years. Incidence rates for GBS based on passively reported cases markedly underestimate the true incidence rate. Although limited by the lack of timeliness for public health surveillance, computerized hospital discharge data are readily available in many states and may be more sensitive in detecting cases, compared to passive surveillance. They may be a useful tool for establishing baseline rates and examining longterm trends for selected acute diseases like GBS for which there are well‐established diagnostic criteria and that usually result in hospitalization.
Neurotensin receptors in human meningiomas
Authors: Przedborski Serge , Levivier Marc , Cadet Jean Lud 
Published: January 2005

AbstractWe have investigated the presence of neurotensin receptors in human meningiomas by in vitro binding autoradiography. Ten of the 12 meningiomas tested displayed specific {3H}neurotensin binding. All meningothelial (n = 3) and transitional (n = 5) meningiomas were positive, whereas only 2 of the 4 fibroblastic meningiomas showed measurable concentrations of neurotensin binding sites. Within the tumors, {3H}neurotensin binding was preferentially observed in syncytial areas. Saturation experiments showed that the maximal binding capacity (Bmax) greatly varied among tumors, ranging from low values to more than 290 fmol/mg of protein. All positive tumors had neurotensin binding sites with a dissociation constant (kd) within the nanomolar range and a pharmacological specificity for {3H}neurotensin similar to neurotensin receptors.
Visual attentional disturbance with unilateral lesions in the basal ganglia and deep white matter
Authors: Sakashita Yasuo 
Published: January 2005

AbstractTo elucidate the role of the basal ganglia and deep white matter in the visual attention mechanism, a new visual attention task was carried out by 15 patients, 9 with left‐side and 6 with right‐side basal ganglia and/or deep white matter damage without visual field defects, and by 12 normal subjects. Their reaction times were recorded in response to a random visual stimulation by pushing a button with the hand ipsilateral to the side of the lesion. All the patients with damage to the right side of the brain had a longer reaction time in the left space than in the right or middle space. With conventional tests, only one of them showed left unilateral spatial neglect. Seven of the 9 patients with a left lesion had a significantly longer reaction time in the right space than in the left. None had unilateral spatial neglect. Both the right and left brain‐damaged groups showed longer reaction times in both spaces, compared to the normal groups. There was no significant difference in reaction time among the control subjects. These findings suggest that the basal ganglia and deep white matter in each hemisphere play some role in directing visual attentional factors into both spaces. Visual attentional disturbance was highly evident even with left‐side brain damage, and this kind of disturbance is not usually revealed with the current tasks used for testing unilateral spatial neglect.