bluuebunny/crossref_metadata_2025_split huggingface dataset:
Mini‐review: Defense strategies and immunity‐related genes
Authors: Trowsdale John , Parham Peter 
Published: February 2004

AbstractThe immune system is of crucial importance in defense against infection. It has to cope with a large number of different pathogens that relentlessly develop new ways to avoid recognition or elimination. Yet most infections are cleared. Immune‐system genes must evolve to keep pace with increasingly sophisticated evasion by pathogens. In this article we examine features of human defense genes that reflect the demands imposed by such intense selection. Key examples are MHC and KIR genes, where features such as polygeny and polymorphism facilitate the comprehensive logistics needed to counteract infection.
Kinetics of Epoxy–Amine Curing Accompanied by the Formation of Liquid Crystalline Structure
Authors: Vyazovkin Sergey , Mititelu Alice , Sbirrazzuoli Nicolas 
Published: February 2004

AbstractThe curing of a mesomorphic epoxy has been studied by polarized light microscopy (PLM) and differential scanning calorimetry. PLM in combination with wide‐angle X‐ray diffraction proves the formation of a liquid crystalline (LC) structure. An advanced isoconversional method reveals that the formation of the LC structure is accompanied by a dramatic decrease in the effective activation from ∼60 to ∼10 kJ · mol−1. A kinetic model of the phenomenon has been discussed.The dependence of the activation energy on the extent of conversion for isothermal curing of the diglycidyl ether of 4,4′‐dihydroxybiphenyl/2,6‐diaminopyridine (diamonds) and DGEBA/2,6‐diaminopyridine (circles) systems.magnified imageThe dependence of the activation energy on the extent of conversion for isothermal curing of the diglycidyl ether of 4,4′‐dihydroxybiphenyl/2,6‐diaminopyridine (diamonds) and DGEBA/2,6‐diaminopyridine (circles) systems.
Initiator‐Grafted Silica Particles for Controlled Free Radical Polymerization: Influence of the Initiator Structure on the Grafting Density
Authors: Parvole Julien , Laruelle Gaël , Guimon Claude , Francois Jeanne , Billon Laurent 
Published: February 2004

AbstractA series of organic‐inorganic hybrid particles were synthesized by a self‐assembled layer of different initiators, immobilized on silica particles and used for controlled radical polymerization. We use three different initiator systems for atom‐transfer radical polymerization (ATRP), unimolecular nitroxide mediated polymerization (NMP), and bimolecular NMP, for the development of the hybrid inorganic/organic particles. After preliminary qualitative characterization by X‐ray spectroscopy (XPS) and Fourier‐transformed infrared (FT‐IR) measurements, the hybrid nanoparticles were studied by thermogravimetric analysis (TGA) to determine and discuss the initiator graft density in terms of steric hindrance.The coupling agents employed for the various approaches used here: a) NMP1‐bimolecular system, b) NMP2‐unimolecular system, and c) ATRP.magnified imageThe coupling agents employed for the various approaches used here: a) NMP1‐bimolecular system, b) NMP2‐unimolecular system, and c) ATRP.
Polystyrene‐Clay Nanocomposites Prepared with Polymerizable Imidazolium Surfactants
Authors: Bottino Francesco A. , Fabbri Elena , Fragalà Ignazio L. , Malandrino Graziella , Orestano Alice , Pilati Francesco , Pollicino Antonino 
Published: February 2004

AbstractPolystyrene/montmorillonite nanocomposites were prepared via in situ bulk polymerization. New imidazolium salts were used to modify montmorillonite obtaining an improvement in thermal stability in comparison with a montmorillonite modified with a standard alkylammonium cation. The synthesized cations facilitate the formation of partially exfoliated structures because of the presence in their structure of a polymerizable group. The polystyrene/montmorillonite nanocomposites were characterized by X‐ray diffraction, thermogravimetric analysis, scanning electron microscopy (SEM), and transmission electron microscopy (TEM).The new thermally stable, reactive surfactant imidazolium salts (C12, C16 and C18) used to organically modify montmorillonite in this study.magnified imageThe new thermally stable, reactive surfactant imidazolium salts (C12, C16 and C18) used to organically modify montmorillonite in this study.
Synthesis of Well‐Defined Telechelic Aromatic Polyamides by Chain‐Growth Polycondensation: Application to the Synthesis of Block Copolymers of Polyamide and Poly(tetrahydrofuran)
Authors: Sugi Ryuji , Yokoyama Akihiro , Yokozawa Tsutomu 
Published: February 2004

AbstractWell‐defined telechelic‐type aromatic polyamides having a secondary amino group and a phenyl ester moiety at each chain end were prepared by the chain‐growth polycondensation of phenyl 4‐(octylamino)benzoate (1) with initiator 2 (N‐tert‐butoxycarbonylated 1), followed by deprotection of the N‐protecting group of the initiator unit. This polycondensation was applied to the synthesis of well‐defined di‐ and triblock copolymers of aromatic polyamides and poly(tetrahydrofuran) (poly(THF)) by the reaction of the terminal secondary amino group of the polyamide with the living cationic propagating group of poly(THF).Block copolymers of polyamide and poly(tetrahydrofuran).magnified imageBlock copolymers of polyamide and poly(tetrahydrofuran).
Synthesis of Polymer Brushes Using Atom Transfer Radical Polymerization
Authors: Pyun Jeffrey , Kowalewski Tomasz , Matyjaszewski Krzysztof 
Published: February 2004

AbstractAtom transfer radical polymerization (ATRP) is a robust method for the preparation of well‐defined (co)polymers. This process has also enabled the preparation of a wide range of polymer brushes where (co)polymers are covalently attached to either curved or flat surfaces. In this review, the general methodology for the synthesis of polymer brushes from flat surfaces, polymers and colloids is summarized focusing on reports using ATRP. Additionally, the morphology of ultrathin films from polymer brushes is discussed using atomic force microscopy (AFM) and other techniques to confirm the formation of nanoscale structure and organization.Formation of polymer brushes by ATRP.magnified imageFormation of polymer brushes by ATRP.
Synthesis and Characterization of Novel Biodegradable Copolymers of 5‐Benzyloxy‐1,3‐dioxan‐2‐one and Glycolide
Authors: Cheng Si‐Xue , Miao Zhi‐Mei , Wang Lian‐Sheng , Zhuo Ren‐Xi 
Published: February 2004

AbstractA series of novel biodegradable random copolymers of 5‐benzyloxy‐1,3‐dioxan‐2‐one (5‐benzyloxy‐trimethylene carbonate, BTMC) and glycolide were synthesized by ring‐opening polymerization. The copolymers were characterized by nuclear magnetic resonance (NMR) spectroscopy and gel permeation chromatography (GPC). The incorporation of BTMC units into the copolymer chains results in good solubility of the polymers in common solvents. The in vitro degradation rate can be tailored by adjusting the composition of the copolymers.The in vitro degradation of the homopolymers and poly(BTMC‐co‐GA) copolymers.magnified imageThe in vitro degradation of the homopolymers and poly(BTMC‐co‐GA) copolymers.
Exercise‐induced dystonia as a preceding symptom of familial Parkinson's disease
Authors: Bruno Michiko K. , Ravina Bernard , Garraux Gaetan , Hallett Mark , Ptacek Louis , Singleton Amanda , Johnson Janel , Singleton Andrew , Hanson Melissa , Considine Elaine , Gwinn‐Hardy Katrina 
Published: February 2004

AbstractParoxysmal exercise‐induced dystonia can occur with Parkinson's disease (PD), and in rare cases, this can also be the presenting symptom. We report on 2 second cousins (no known consanguinity) who presented with paroxysmal exercise‐induced dystonia who later developed clinical features of PD. Although autosomal recessive inheritance was suggested, and the dystonic features further suggest parkin as a possible cause, ssequencing for parkin mutations was negative and this family may represent a genetic variant of PD. Further genotype–phenotype studies in this and similar families may give clues to pre‐symptomatic symptoms in PD and may reflect a particular phenotype of interest for genetics studies in the future. © 2003 Movement Disorder Society
Parkinson's disease patients with bilateral subthalamic deep brain stimulation gain weight
Authors: Macia Frédéric , Perlemoine Caroline , Coman Irène , Guehl Dominique , Burbaud Pierre , Cuny Emmanuel , Gin Henri , Rigalleau Vincent , Tison François 
Published: February 2004

AbstractWeight, body mass index (BMI) and energy expenditure/energy intake (EE/EI) was studied in 19 Parkinson's disease (PD) patients after subthalamic deep brain stimulation (STN‐DBS) versus 14 nonoperated ones. Operated patients had a significant weight gain (WG, + 9.7 ± 7 kg) and BMI increase (+ 4.7 kg/m2). The fat mass was higher after STN‐DBS. Resting EE (REE; offdrug/ON stimulation) was significantly decreased in STN‐DBS patients, while their daily energy expenditure (DEI) was not significantly different. A significant correlation was found among WG, BMI increase, and pre‐operative levodopa‐equivalent daily dose, their reduction after STN‐DBS, and the differential REE related to stimulation and the REE in the offdrug/OFF stimulation condition. In conclusion, STN‐DBS in PD induces a significant WG associated with a reduction in REE without DEI adjustment. © 2003 Movement Disorder Society
Normal dopaminergic and serotonergic metabolites in cerebrospinal fluid and blood of restless legs syndrome patients
Authors: Stiasny‐Kolster Karin , Möller J. Carsten , Zschocke Johannes , Bandmann Oliver , Cassel Werner , Oertel Wolfgang H. , Hoffmann Georg F. 
Published: February 2004

AbstractCerebrospinal fluid (CSF) and blood obtained from 22 untreated or scarcely treated patients with moderate to severe restless legs syndrome (RLS; mean age, 58.6 ± 13 years) and 11 control subjects (mean age, 56.6 ± 12.9 years) were investigated for biogenic amines between 6:00 and 8:00 PM. We did not find any significant differences in the CSF concentrations of homovanillic acid, 3‐ortho‐ methyl‐dopa, levodopa, 5‐hydroxytryptophan, 5‐hydroxyindoleacetic acid, tetrahydrobiopterin, dihydrobiopterin, 5‐methyltetrahydrofolate, and neopterin. In addition, serotonin in whole blood and plasma activity of aromatic amino acid decarboxylase were all normal. Our results suggest that dopaminergic and serotonergic release is not substantially affected in RLS. © 2003 Movement Disorder Society
Phenotypic characterization of DYT13 primary torsion dystonia
Authors: Bentivoglio Anna Rita , Ialongo Tamara , Contarino M. Fiorella , Valente Enza M. , Albanese Alberto 
Published: February 2004

AbstractWe describe the phenotype of DYT13 primary torsion dystonia (PTD) in a family first examined in 1994. A complete neurological evaluation was performed on all available family members: 8 individuals were definitely affected by dystonia. The family was re‐evaluated in March 2000: at that time, 3 more individuals had developed symptoms of dystonia. Inheritance of PTD was autosomal dominant, with affected individuals spanning three consecutive generations and male‐to‐male transmission. Age at onset ranged from 5 to 43 years. Onset occurred either in the craniocervical region or in upper limbs. Progression was mild, and the disease course was benign in most affected individuals; generalization occurred only in 2 cases. We did not find anticipation of age at onset or of disease severity through generations. Most subjects presented with jerky, myoclonic‐like dystonic movements of the neck or shoulders. DYT13‐PTD is an autosomal dominant disease, with incomplete penetrance (58%). Clinical presentation and age at onset were more variable than in DYT1‐PTD, and the neck was involved in most of those affected. Moreover, the individuals with generalised dystonia were not severely disabled and were able to lead independent lives. To date, this is the only family with DYT13‐PTD. © 2003 Movement Disorder Society
Clinical and genetic features of myoclonus–dystonia in 3 cases: A video presentation
Authors: Kock Norman , Kasten Meike , Schüle Birgitt , Hedrich Katja , Wiegers Karin , Kabakci Kemal , Hagenah Johann , Pramstaller Peter P. , Nitschke Matthias F. , Münchau Alexander , Sperner Jürgen , Klein Christine 
Published: February 2004

AbstractMany cases of myoclonus–dystonia (M‐D) are caused by mutations in the ϵ‐sarcoglycan (SGCE) gene. We describe 3 children with a similar clinical picture of autosomal dominant M‐D and an SGCE mutation in only one of them, suggesting that M‐D is genetically heterogeneous. © 2003 Movement Disorder Society
Cerebrospinal fluid analysis for Whipple's disease in patients with progressive supranuclear palsy
Authors: Pezzella Francesca Romana , Paglia Maria Grazia , Colosimo Carlo 
Published: February 2004

AbstractThe clinical picture of neurological involvement in Whipple's disease (WD) may resemble progressive supranuclear palsy (PSP). We looked for WD pathogen DNA in the cerebrospinal fluid of 9 patients with a clinical diagnosis of PSP. The analysis was negative for all samples, showing that WD is not commonly involved in the aetiopathogenesis of PSP. © 2003 Movement Disorder Society
Improvement of sleep quality in patients with advanced Parkinson's disease treated with deep brain stimulation of the subthalamic nucleus
Authors: Hjort Niels , Østergaard Karen , Dupont Erik 
Published: February 2004

AbstractMost Parkinson's patients complain about sleep problems. The subjective effect of deep brain stimulation (DBS) of the subthalamic nucleus (STN) on nocturnal disabilities and sleep quality was elucidated by the recently established Parkinson's disease sleep scale (PDSS). The DBS‐treated group obtained significant improvement of motor function assessed by the Unified Parkinson's Disease Rating Scale. The mean total PDSS improved significantly after surgery whereas no change was found for the control group. Significant improvements of individual questions were obtained for overall sleep quality and motor symptoms whereas nocturia and daytime sleepiness did not change despite significant reduction of parkinsonian medication. © 2003 Movement Disorder Society
Diagnostic considerations in juvenile parkinsonism
Authors: Paviour Dominic C. , Surtees Robert A.H. , Lees Andrew J. 
Published: February 2004

AbstractJuvenile parkinsonism (JP) describes patients in whom the clinical features of parkinsonism manifest before 21 years of age. Many reported cases that had a good response to levodopa have proved to have autosomal recessive juvenile parkinsonism (AR‐JP) due to mutations in the parkin gene. With the exception of parkin mutations and dopa‐responsive dystonia, most causes are associated with the presence of additional neurological signs, resulting from additional lesions outside of the basal ganglia. Lewy body pathology has only been reported in one case, suggesting that a juvenile form of idiopathic Parkinson's disease may be extremely rare. © 2003 Movement Disorder Society
Late‐life action tremor in a southern sea otter (enhydris lutris nereis)
Authors: Louis Elan D. , Murray Michael J. , Miller Melissa A. , Pullman Seth L. , Vonsattel Jean Paul G. 
Published: February 2004

AbstractAlthough tremor is highly prevalent in human beings, there are few reports of tremor occurring in other mammals. Such tremor can further our insight into the mechanisms and anatomical basis of human tremor disorders. We report on a southern sea otter with a slowly progressive 6.5 to 8.5 Hz action tremor of late life that shared several clinical characteristics with essential tremor. The main pathological finding was in the cerebellum, where there was extensive vacuolation of Purkinje cells. © 2003 Movement Disorder Society
Complete mitochondrial DNA sequence analysis in a family with early‐onset dystonia and optic atrophy
Authors: Brown Michael D. , Hosseini Seyed , Steiner Israel , Wallace Douglas C. , Korn‐Lubetzki Isabelle 
Published: February 2004

AbstractThe combination of optic atrophy and dystonia has been etiologically associated with mitochondrial DNA (mtDNA) mutations. We report here on the complete mtDNA sequence from the proband of a consanguineous family exhibiting “mitochondrial‐like” optic atrophy and dystonia. A candidate tRNAGly mutation was identified that was unique to the family. However, the mutation was homoplasmic in both affected and unaffected family members and we were unable to demonstrate a biochemical defect in patient mitochondria. Hence, it is unlikely that a mtDNA mutation accounts for the phenotype in this family. © 2003 Movement Disorder Society
Inter‐rater reliability of the International Cooperative Ataxia Rating Scale (ICARS)
Authors: Storey Elsdon , Tuck Kate , Hester Robert , Hughes Andrew , Churchyard Andrew 
Published: February 2004

AbstractWe assessed the inter‐rater reliability of the 100‐point International Cooperative Ataxia Rating Scale (ICARS). Three neurologists independently rated videotaped ICARS examinations of 22 subjects with genetically determined ataxias (spinocerebellar ataxia [SCA] Type 1 in 11; SCA Type 2 in 1; Friedreich's ataxia in 10) and 4 controls. Scores on live ICARS assessment had ranged from 0 to 7 for controls and 11 to 74 for ataxic subjects (clinically very mildly affected to wheelchair‐bound). Inter‐rater correlation was very high for the total score (Kendall's ω 0.994, 95% confidence interval, 0.988–0.997), and high to very high for each component subscore (0.791 for speech to 0.994 for posture/gait). All correlations were significant at P < 0.00001. The ICARS exhibits very high inter‐rater reliability even without prior observer standardisation and is sensitive to a range of ataxia severities from very mild to severe. © 2003 Movement Disorder Society
Familial cortical tremor with epilepsy and cerebellar pathological findings
Authors: van Rootselaar Anne‐Fleur , Aronica Eleonora , Jansen Steur Ernst N.H. , Rozemuller‐Kwakkel Johanna M. , de Vos Rob A.I. , Tijssen Marina A.J. 
Published: February 2004

AbstractThe clinical and neuropathological findings in a patient with familial cortical tremor with epilepsy (FCTE) are described. Clinically, the patient showed cortical myoclonus, tremor, and generalized seizures. Pathological investigation showed cerebellar degeneration and somal sprouting and loss of dendritic tree in Purkinje cells. Striking similarities were found in diseases caused by channelopathies such as spinocerebellar ataxia subtype 6. © 2003 Movement Disorder Society
Development of quality of care indicators for Parkinson's disease
Authors: Cheng Eric M. , Siderowf Andrew , Swarztrauber Kari , Eisa Mahmood , Lee Martin , Vickrey Barbara G. 
Published: February 2004

AbstractParkinson's disease (PD) is a major cause of disability. To date, there have been no large‐scale efforts to measure the quality of PD care because of a lack of quality indicators for conducting an explicit review of PD care processes. We present a set of quality indicators for PD care. Based on a structured review of the medical literature, 79 potential indicators were drafted. Through a two‐round modified Delphi process, an expert panel of seven movement disorders specialists rated each indicator on criteria of validity, feasibility, impact on outcomes, room for improvement, and overall utility. Seventy‐one quality indicators met validity and feasibility thresholds. Applying thresholds for impact on outcomes, room for improvement, and overall utility, a subset of 29 indicators was identified, spanning dopaminergic therapy, assessment of functional status, assessment and treatment of depression, coordination of care, and medication use. Multivariable analysis showed that overall utility ratings were driven by validity and impact on outcomes (P < 0.01). An expert panel can reach consensus on a set of highly rated quality indicators for PD care, which can be used to assess quality of PD care and guide the design of quality improvement projects. © 2003 Movement Disorder Society
Quantification of focal dystonia in pianists using scale analysis
Authors: Jabusch Hans‐Christian , Vauth Henning , Altenmüller Eckart 
Published: February 2004

AbstractFocal dystonia in pianists is a task‐specific movement disorder that causes loss of pianistic skills and provokes irregularities in playing. So far, no method has been available for objective quantification of the disorder. Eight professional pianists with focal dystonia and eight healthy professional pianists matched by age, gender, and handedness were examined, using a newly developed MIDI‐based Scale Analysis as well as the Arm Dystonia Disability Scale (ADDS). Key velocities and timing parameters were analyzed. In 5 pianists with dystonia, follow‐up examinations were carried out after treatment with botulinum toxin‐A. In affected hands, significantly higher mean standard deviations of timing parameters were seen compared with healthy reference hands. After treatment with botulinum toxin‐A, significant improvements in performance parameters were monitored by Scale Analysis. Mean standard deviations of inter‐onset intervals correlated with ADDS scores. We conclude that Scale Analysis is an effective and precise tool for quantification of focal dystonia in pianists and provides fine resolution. It is independent of rating methods and allows reliable follow‐up examinations during treatment. © 2003 Movement Disorder Society
Accuracy of clinical diagnosis of progressive supranuclear palsy
Authors: Osaki Y. , Ben‐Shlomo Y. , Lees A. J. , Daniel S. E. , Colosimo C. , Wenning G. , Quinn N. 
Published: February 2004

AbstractWe assessed the accuracy of clinical diagnosis of progressive supranuclear palsy (PSP, Steele‐Richardson‐Olszewski disease) and the validity of existing sets of clinical diagnostic criteria for PSP (see Appendix) using neuropathologically examined cases from the Queen Square Brain Bank for Neurological Disorders. Diagnosis of PSP was made by 40 different physicians, and 60 cases clinically diagnosed as PSP when last assessed in life were studied. In 47 cases (78%), the diagnosis of PSP was confirmed pathologically. False‐positive diagnoses included Parkinson's disease with significant additional cortical Lewy body (n = 3) or Alzheimer (n = 1) pathology, multiple system atrophy (n = 4), and corticobasal degeneration, Pick's disease, motor neurone disease, cerebrovascular disease, and a sporadic case of frontotemporal dementia and parkinsonism linked to chromosome 17 (1 case each). Most cases of PSP were diagnosed accurately by neurologists at the final assessment. Although application of National Institute of Neurological Disorders and the Society for PSP possible category marginally improved the accuracy of initial clinical diagnosis, none of the existing operational criteria could significantly improve accuracy of the final clinical diagnosis. © 2003 Movement Disorder Society