bluuebunny/crossref_metadata_2025_split huggingface dataset:
Increased lymphocyte beta‐adrenergic receptor density in progressive multiple sclerosis is specific for the CD8+, CD28− suppressor cell
Authors: Karaszewski Joseph W. , Reder Anthony T. , Anlar Banu , Kim Woo Chan , Arnason Barry G. W. 
Published: January 2005

AbstractBeta‐adrenergic receptor density on T cells from healthy humans is greatest on suppressor cells (CD8+, CD28−) and the effect of catecholamines, secreted by the sympathetic nervous system, predominates on this subset. The sympathetic skin response, a measure of sympathetic nervous system function, is absent in most patients with chronic progressive multiple sclerosis (MS). We measured beta‐adrenergic receptor density on suppressor cells, cytotoxic cells, and monocytes from patients with chronic progressive MS and healthy control subjects. Control receptor density on suppressor cells was 2.8 ± 0.3 fmol/106 cells versus a density of 5.1 ± 0.7 fmol/106 cells for patients. Cytotoxic cell (CD8+, CD28+) receptor density was 1.4 ± 0.4 fmol/106 cells in control subjects and 0.9 ± 0.3 fmol/106 cells in the patients. Monocytes displayed beta‐adrenergic receptor densities of 2.6 ± 0.4 fmol/106 cells in normal individuals and 2.7 ± 0.4 fmol/106 cells in the patient group. CD8 lymphocyte beta‐adrenergic receptor densities in patients with relapsing‐remitting and those with stable MS were not different from control values, yet were significantly less than the values for patients with chronic progressive MS. We find that mononuclear cells from healthy control subjects and patients with chronic progressive MS proliferate in response to 200 units/ml of recombinant human interleukin‐2 (IL‐2) similarly. However, IL‐2 treatment increased beta‐adrenergic receptor density on normal mononuclear cells, but failed to increase it on mononuclear cells from patients with chronic progressive MS. We conclude that decreased sympathetic skin responses, increased CD8+, CD28− cell beta‐adrenergic receptor density, impaired suppressor cell function, and increased IL‐2 production may be interrelated, and that increased beta‐adrenergic receptor density is confined to the chronic progressive form of MS.
Serum interleukin‐2 concentrations in Guillain‐Barré syndrome and chronic idiopathic demyelinating polyradiculoneuropathy: Comparison with other neurological diseases of presumed immunopathogenesis
Authors: Hartung Hans‐Peter , Reiners Karlheinz , Schmidt Beate , Stoll Guido , Toyka Klaus V. 
Published: January 2005

AbstractSerum concentrations of the cytokine interleukin‐2 (IL‐2) were quantitated by enzyme‐linked immunosorbent assay in 42 patients with Guillain‐Barré syndrome, 15 patients with chronic idiopathic demyelinating polyradiculoneuropathy, 37 patients with other neuropathies, 54 patients with other noninflammatory, nondemyelinating neurological disorders, and 26 healthy control subjects. We found markedly increased serum levels of IL‐2 in patients with Guillain‐Barré syndrome and to a much lesser extent, in patients with chronic idiopathic demyelinating polyradiculoneuropathy. Increased serum concentrations of IL‐2 in patients with Guillain‐Barré syndrome returned to normal in parallel with recovery from the disease. These findings suggest ongoing T‐cell proliferation in patients with Guillain‐Barré syndrome and some patients with chronic idiopathic demyelinating polyradiculoneuropathy. IL‐2 levels were also raised in patients with active multiple sclerosis, myasthenia gravis, and herpes simplex encephalitis, and some patients with polymyositis, invoking T cells in the pathogenesis of these diseases.
Auditory evoked magnetic fields after ischemic brain lesions
Authors: Mäkelä J. P. , Hari R. , Valanne L. , Ahonen A. 
Published: January 2005

AbstractAuditory evoked magnetic fields to noise/square‐wave sequences, presented once every 2.2 seconds, were recorded in 8 patients who had ischemic lesions in the auditory cortex or in its vicinity. In 2 patients with large temporoparietal lesions, the magnetic 100‐msec deflection (N100m) was absent over the damaged side. In 1 patient with a large but less deep frontotemporal lesion, a small N100m could be discerned on the defective side. Frontal lesions or small lesions in the vicinity of the supratemporal plane had no effect on N100m. Auditory evoked magnetic field recordings may be useful in clinical studies of auditory cortical functions.
Phosphorus magnetic resonance spectroscopy (31P MRS) in neuromuscular disorders
Authors: Argov Zohar , Bank William J. 
Published: January 2005

AbstractPhosphorus magnetic resonance spectroscopy monitors muscle energy metabolism by recording the ratio of phosphocreatine to inorganic phosphate at rest, during exercise, and during recovery from exercise. In mitochondrial diseases, abnormalities may appear during some or all these phases. Low phosphocreatine—inorganic phosphate ratios at rest are not disease‐specific, but can be increased by drug therapy in several myopathies. Phosphorus magnetic resonance spectroscopy can also record intracellular pH and thus identify disorders of glycogen metabolism in which the production of lactic acid is blocked during ischemic exercise. The measurements of accumulated sugar phosphate intermediates further delineate glycolytic muscle defects. Myophosphorylase deficiency responds to intravenous glucose administration with improved exercise bioenergetics, but no such response is seen in phosphofructokinase deficiency. The muscular dystrophies show no specific bioenergetic abnormality; however, elevation of phospholipids metabolites and phosphodiesters was detected in some cases. While phosphorus magnetic resonance spectroscopy remains primarily a research tool in metabolic myopathies, it will be clinically useful in identifying new therapies and monitoring their effects in a variety of neuromuscular disorders.
Neuritic pathology and dementia in alzheimer's disease
Authors: McKee A. C. , Kosik K. S. , Kowall N. W. 
Published: January 2005

AbstractPrevious studies of Alzheimer's disease (AD) have correlated the severity of dementia with either the number of senile plaques or neurofibrillary tangles. We used antibodies raised against amyloid β/A4 protein of senile plaque cores and tau protein as well as thioflavine S and the Campbell‐Switzer modification of the Hicks silver method to examine the hippocampal formation and five neocortical regions from 22 nondemented elderly control subjects and 34 demented patients with cerebral senile plaques and neurofibrillary tangles, without complicating disease processes. Ten control subjects (46%) had no β/A4 protein deposition. Twelve control subjects (54%) had widespread β/A4 protein deposition but no neocortical neuritic pathology. Of the 34 patients with AD‐type changes, 27 (79%) had widespread senile plaques and neurofibrillary tangles, while 7 (21%) had neocortical senile plaques with few neurofibrillary tangles. All demented patients had widespread β/A4 protein deposition and neocortical tau‐immunoreactive, Hicks silver‐positive dystrophic neurites. The neurites were found both free in the neuropil as well as surrounding senile plaques. Quantitative analysis showed that dystrophic neurites were significantly increased in patients with AD compared with control subjects and the number of dystrophic neurites and neurofibrillary tangles correlated with the clinical severity of dementia. Widespread cerebral β/A4 protein deposition may be necessary but by itself is insufficient for the development of dementia in AD.
Impaired critical flicker frequency in recovered optic neuritis
Authors: Jacobson Daniel M. , Olson Kurt A. 
Published: January 2005

AbstractThirteen patients with optic neuritis who recovered normal visual acuity and kinetic perimetry all showed impairment of foveal critical flicker frequency, a psychophysical test of visual temporal resolution. Because the magnocellular visual system demonstrates higher temporal resolution than the parvocellular system, this finding implicates involvement of axonal projections of magnocellular retinal ganglion cells in recovered optic neuritis.
Mitochondrial oxidative phosphorylation defects in parkinson's disease
Authors: Shoffner John M. , Watts Ray L. , Juncos Jorge L. , Torroni Antonio , Wallace Douglas C. 
Published: January 2005

AbstractParkinson's disease has been associated with defects in oxidative phosphorylation (Oxphos). We analyzed mitochondria isolated from muscle biopsies of 6 patients with Parkinson's disease for deficiencies in Oxphos enzymes and for mutations in the mitochondrial DNA. Oxphos enzyme assays were compared to the 5 to 95% confidence intervals from 16 control subjects. Four patients had complex I defects, whereas 1 patient had a complex IV defect. A genetic basis for Parkinson's disease was suggested by the presence of affected relatives of 2 patients with Parkinson's disease. Known pathological mitochondrial DNA mutations (insertion‐deletions or point mutations) were not found. We conclude that Parkinson's disease is a systemic disorder of Oxphos, probably of a complex genetic etiology. Premature cell death in the nigrostriatal dopamine pathway could be due to energetic impairment and accentuated free radical generation caused by an Oxphos defect.
Anterior callosotomy in the treatment of medically intractable epilepsies: A study of 43 patients with a mean follow‐up of 39 months
Authors: Oguni Hirokazu , Olivier André , Andermann Frederick , Comair Joseph 
Published: January 2005

AbstractWe studied the effectiveness of anterior callosotomy in 43 patients whose follow‐up period averaged 39 months. These patients had intractable generalized seizures, characterized by a combination of seizure patterns, most frequently drop attacks with generalized tonic‐clonic, generalized tonic, and absence seizures. Overall, drop attacks were the most frequent (31/43 or 72%) and the most disabling seizure pattern (27/43 or 63%); they were also the most likely to benefit from anterior callosotomy (70%). Patients with lateralized changes tended to have a better result than did those without lateralization, but patients with synchronous and symmetrical spike and wave discharges also benefited. The preoperative intelligence quotient and the etiological factors were not predictors of outcome. There was a correlation between the extent of section and the results; patients with section of the anterior two thirds had a better result, compared with those who had section of the anterior half.
Immunosuppressive treatment in multifocal motor neuropathy
Authors: Feldman Eva L. , Bromberg Mark B. , Albers James W. , Pestronk Alan 
Published: January 2005

AbstractWe report the results of immunosuppressive treatments of 13 patients with multifocal motor neuropathy and elevated titers of serum antibodies to the GM1 ganglioside. All patients failed to respond to oral prednisone. There was no clinical response in 4 patients treated with plasma exchange. Nine patients received cyclophosphamide, with clinical improvement and fall in antibody titers in 8. In 3 patients, cyclophosphamide was discontinued with ensuing clinical relapse and rise in the titers of serum anti‐GM1 antibodies. These patients provide further evidence for the efficacy of cyclophosphamide therapy in patients with multifocal motor neuropathy.
Segmental spinal muscular atrophy and dermatological findings in a patient with chromosome 18q deletion
Authors: Weiss B. J. , Kamholz J. , Ritter A. , Zackai E. H. , McDonald‐McGinn D. M. , Emanuel B. , Fischbeck K. H. 
Published: January 2005

AbstractWe have evaluated a young woman with segmental spinal muscular atrophy, who has a deletion of a portion of the long arm of chromosome 18. She also has vitiligo and lichen sclerosis et atrophicus. She has neither the facial dysmorphism nor the mental deficit usually associated with the 18q− syndrome. Magnetic resonance imaging scan of her brain demonstrates high signal intensity consistent with abnormal myelination. Southern blot analysis of her DNA demonstrates that the deletion includes the gene for human myelin basic protein. Neither spinal muscular atrophy nor this patient's skin manifestations have been previously reported in association with 18q−.
Stroke prevention in nonvalvular atrial fibrillation: A review of prospective randomized trials
Authors: Albers Gregory W. , Sherman David G. , Gress Daryl R. , Paulseth J. E. , Petersen Palle 
Published: January 2005

AbstractPatients with atrial fibrillation are at risk for cerebral embolism; however, the roles of chronic anticoagulation or antiplatelet therapy for stroke prevention in patients with nonvalvular atrial fibrillation have been controversial. Recently, the results of three large prospective randomized trials that examined the risks and benefits of warfarin or aspirin for stroke prophylaxis in patients with nonvalular atrial fibrillation were reported. All three studies revealed a reduction in the stroke rate for patients treated with warfarin and a small incidence of major bleeding. One of the studies also reported a reduced stroke rate in aspirin‐treated patients. The reduction of thromboembolic events associated with chronic warfarin therapy appears to outweigh the risks of significant bleeding for most patients with nonvalvular atrial fibrillation. Aspirin may offer an alternative for subgroups of patients who are at low risk for stroke or those who are not good candidates for anticoagulation.
Seronegative myasthenia gravis: A plasma factor inhibiting agonist‐induced acetylcholine receptor function copurifies with IgM
Authors: Yamamoto Takeshi , Vincent Angela , Ciulla Thomas A. , Lang Bethan , Johnston Irene , Newsom‐Davis John 
Published: January 2005

AbstractAnti—acetylcholine receptor antibodies cannot be detected by standard radioimmunoassay in 10 to 15% of patients with generalized myasthenia gravis (seronegative myasthenia gravis). We investigated the effect of seronegative myasthenia gravis plasma on 22Na+ flux through acetylcholine receptors and voltage‐gated sodium channels in the human rhabdomyosarcoma cell line, TE671. Fourteen of 19 seronegative MG plasmas inhibited acetylcholine receptor 22Na+ flux; none inhibited voltage‐gated sodium channel flux. The inhibitory activity was found in the IgG‐depleted fraction, and copurified with IgM after gel‐filtration chromatography. Inhibitory activity was absent from the plasma of 8 healthy control subjects and of 6 patients with the Lambert‐Eaton myasthenic syndrome, but was present in the IgG‐depleted plasma fraction of 4 of 6 seropositive patients with myasthenia gravis and all 5 patients with demyelinating polyneuropathy. We conclude that a low‐affinity serum factor, probably an IgM antibody, found in a high proportion in patients with seronegative and those with seropositive myasthenia gravis may contribute to the muscle weakness in myasthenia gravis, but its role in these and other neuroimmunological disorders requires further investigation.
Immunohistochemical studies on complexes I, II, III, and IV of mitochondria in parkinson's disease
Authors: Hattori Nobutaka , Tanaka Masashi , Ozawa Takayuki , Mizuno Yoshikuni 
Published: January 2005

AbstractWe examined the substantia nigra of 8 patients with Parkinson's disease immunohistochemically using antisera against complexes I, II, III, and IV of the mitochondrial electron transport system. In the patients with Parkinson's disease, a fair proportion of the nigral neurons showed reduced staining against the complex I antibody. The proportion of the neurons with reduced staining ranged from 12.7 to 74.1% of the melanized nigral neurons. Although neurons with reduced immunostaining for complex I were also observed in control subjects, the proportion among the nigral neurons was significantly smaller than in parkinsonian patients. Staining for complexes III and IV appeared normal. Staining of substantia nigra for complex II was decreased in 3 parkinsonian patients. These results are consistent with our findings that there is a deletion of gene coding for the four subunits in the mitochondrial DNA located in the striata of parkinsonian patients.
Quantitative measurements of capillary transport in human brain tumors by computed tomography
Authors: Groothuis Dennis R. , Vriesendorp Francine J. , Kupfer Barry , Warnke Peter C. , Lapin Gregory D. , Kuruvilla Abraham , Vick Nicholas A. , Mikhael Michael A. , Patlak Clifford S. 
Published: January 2005

AbstractThe rate at which water‐soluble chemotherapeutic drugs enter brain tumors can be extremely variable. The ability to measure or predict the rate of drug entry may have an important role in treatment. We have developed a method that uses information from contrast‐enhanced computed tomographic scans to measure quantitatively the rate of transcapillary transport of iodinated compounds in brain tumors. In a group of 10 patients with brain tumors, we obtained serial measurements of tissue (Am) and arterial plasma (Cp) iodine concentration from timed computed tomographic scans done over 30 minutes, after intravenous infusion of meglumine iothalamate (Conray‐60). These measurements were analyzed with a two‐compartment pharmacokinetic model and nonlinear least‐squares regression methods to obtain K1, a blood‐to‐tissue transfer constant; k2, a tissue‐to‐blood rate constant; and Vp, tissue plasma vascular volume. Images of K1, k2, and Vp were reconstructed after calculating these values for each 0.8 × 0.8 × 5‐mm volume element of the original data. Mean whole tumor K1 values varied from 2.0 μl gm−1 min−1 in a thalamic astrocytoma to 33.9 μ1 gm−1 min−1 in a glioblastoma multiforme. The value of k2 varied from 0.034 to 0.108 min−1, and Vp varied from 2.4 to 7.9 ml 100 gm−1. In tumor‐free brain, the K1 of meglumine iothalamate was 2.9 μ1 gm−1 min−1; k2 was 0.058 min−1; and Vp was 2.1 ml 100 gm−1. There was pronounced variability in transcapillary transport values between different malignant brain tumors, as well as regional variability within individual tumors, with focal increases in K1 up to 50‐fold normal brain values. This approach provides a quantitative method to measure the rate of transcapillary transport of passively distributed iodinated compounds in human brain tumors. These types of measurements can be used to study serial changes of capillary permeability in individual patients that occur as a result of therapeutic manipulation and may, potentially, be used to individualize chemotherapy.
Epileptiform activity induced by low extracellular magnesium in the human cortex maintained in vitro
Authors: Avoli M. , Drapeau C. , Louvel J. , Pumain R. , Olivier A. , Villemure J.‐G. 
Published: January 2005

AbstractExtracellular field potentials and [K+]o were recorded in slices of human epileptogenic neocortex maintained in vitro during perfusion with Mg2+‐free artificial cerebrospinal fluid (ACSF). The human neocortex was obtained during neurosurgical procedures for the relief of seizures that were resistant to medical treatment. Spontaneous epileptiform activity and episodes of spreading depression appeared within 1.5 to 2 hours of perfusion with Mg2+‐free ACSF. The epileptiform discharges consisted of negative field potential shifts (amplitude, 0.8–10 mV) that lasted 2.5 to 80 seconds and recurred at intervals ranging between 4 and 160 seconds. Both duration and frequency occurrence of epileptiform events were not significantly different when measured in slices obtained from spiking tissue compared with those gathered from nonspiking neocortical areas. Transient increases in [K+]o of up to 10.5 mM were associated with each epileptiform discharge; these changes were maximal and fastest in the middle neocortical layers. Spreading depression episodes were characterized by 20 to 30‐mV negative shifts that lasted up to 200 seconds and were accompanied by increases in [K+]o of approximately 100 mM. Epileptiform discharges and spreading depressions did not occur during perfusion with Mg2+‐free ACSF that contained either competitive or noncompetitive antagonists of the N‐methyl‐D‐aspartate (NMDA) receptor subtype. In contrast, pharmacological blockade of non‐NMDA receptors did not influence the epileptiform activity observed in Mg2+‐free ACSF. These findings demonstrate that decreasing [Mg2+]o leads to the appearance of both spontaneous epileptiform discharges and spreading depression in the human epileptogenic neocortex. Both activities are solely dependent on NMDA‐activated conductances, suggesting that removal of the voltage‐dependent gating effect exerted by extracellular Mg2+ on the NMDA ionophore is a condition sufficient for neurons in the human neocortex to generate spontaneous, synchronous epileptiform activity. We propose that this experimental paradigm might represent a model suitable for analyzing the cellular mechanisms underlying human neocortical epileptogenesis in vitro as well as for preliminary screening of antiepileptic drugs.
Pleuropulmonary disease associated with dopamine agonist therapy
Authors: Bhatt M. H. , Keenan S. P. , Fleetham J. A. , Calne D. B. 
Published: January 2005

AbstractArtificial dopamine agonists are widely employed for the treatment of idiopathic parkinsonism. Pleuropulmonary disease has previously been reported to occur with the use of bromocriptine and mesulergine. We report similar adverse effects induced by the newer agonists lisuride and cabergoline. All these agents are tetracyclic ergot derivatives. This suggests a causal link between ergot‐derived dopamine agonists and pleuropulmonary disease.
Nighttime levodopa infusions to treat motor fluctuations in advanced parkinson's disease: Preliminary observations
Authors: Sage Jacob I. , Mark Margery H. 
Published: January 2005

AbstractWe report the clinical response of a parkinsonian patient with severe motor fluctuations who was begun on continuous, nighttime‐only levodopa infusions accompanied by oral carbidopa/levodopa use during the day. Sleep disturbances improved immediately. There was a gradual reduction in “off” time and severity of dyskinesias during the waking day. Nighttime‐only levodopa infusions have a carryover benefit to the waking hours and may be suitable for long‐term therapy to reduce daily motor fluctuations in some patients with Parkinson's disease.
Hereditary branching enzyme dysfunction in adult polyglucosan body disease: A possible metabolic cause in two patients
Authors: Lossos A. , Barash V. , Soffer D. , Argov Z. , Gomori M. , Ben‐Nariah Z. , Abramsky O. , Steiner I. 
Published: January 2005

AbstractWe describe 2 unrelated patients with adult polyglucosan body disease (APBD) diagnosed by sural nerve biopsy. Both patients were offspring of consanguineous marriages. They presented clinically with late onset pyramidal tetraparesis, micturition difficulties, peripheral neuropathy, and mild cognitive impairment. Magnetic resonance imaging of the brain revealed extensive white matter abnormalities in both. In search of a possible metabolic defect, we evaluated glycogen metabolism in these patients and their clinically unaffected children. Branching enzyme activity in the patients' polymorphonuclear leukocytes was about 15% of control values, whereas their children displayed values of 50 to 60%, suggesting a possible autosomal recessive mode of transmission. This is the first report of an inherited metabolic defect in patients with adult polyglucosan body disease. We suggest that branching enzyme dysfunction may be implicated in the pathogenesis of some patients with adult polyglucosan body disease.