bluuebunny/crossref_metadata_2025_split huggingface dataset:
Do CTG expansions at the SCA8 locus cause ataxia?
Authors: Schöls Ludger , Bauer Ingrid , Zühlke Christine , Schulte Thorsten , Kölmel Christina , Bürk Katrin , Topka Helge , Bauer Peter , Przuntek Horst , Riess Olaf 
Published: June 2003

AbstractTo evaluate the significance of expanded CTG repeats at the SCA8 locus, we analyzed the allele distribution in 1,262 German ataxia patients. We found intermediate and expanded CTG repeats with similar frequencies in ataxia patients with and without established genetic diseases. One family linked to the SCA8 locus showed incomplete penetrance and an association of smaller CTG repeats with more severe disease. Our data question the disease‐causing character of CTG expansions for SCA8 and advise great caution in genetic testing. Ann Neurol 2003;54:110–115
Slower progression of Parkinson's disease with ropinirole versus levodopa: The REAL‐PET study
Authors: Whone Alan L. , Watts Ray L. , Stoessl A. Jon , Davis Margaret , Reske Sven , Nahmias Claude , Lang Anthony E. , Rascol Olivier , Ribeiro Maria J. , Remy Philippe , Poewe Werner H. , Hauser Robert A. , Brooks David J. ,   REAL‐PET Study Group
Published: June 2003

AbstractPreclinical studies suggest ropinirole (a D2/D3 dopamine agonist) may be neuroprotective in Parkinson's disease (PD), and a pilot clinical study using 18F‐dopa positron emission tomography (PET) suggested a slower loss of striatal dopamine storage with ropinirole compared with levodopa. This prospective, 2‐year, randomized, double‐blind, multinational study compared the rates of loss of dopamine‐terminal function in de novo patients with clinical and 18F‐dopa PET evidence of early PD, randomized 1 to 1 to receive either ropinirole or levodopa. The primary outcome measure was reduction in putamen 18F‐dopa uptake (Ki) between baseline and 2‐year PET. Of 186, 162 randomized patients were eligible for analysis. A blinded, central, region‐of‐interest analysis showed a significantly lower reduction (p = 0.022) in putamen Ki over 2 years with ropinirole (−13.4%; n = 68) compared with levodopa (−20.3%; n = 59; 95% confidence interval [CI], 0.65–13.06). Statistical parametric mapping localized lesser reductions in 18F‐dopa uptake in the putamen and substantia nigra with ropinirole. The greatest Ki decrease in each group was in the putamen (ropinirole, −14.1%; levodopa, −22.9%; 95% CI, 4.24–13.3), but the decrease was significantly lower with ropinirole compared with levodopa (p < 0.001). Ropinirole is associated with slower progression of PD than levodopa as assessed by 18F‐dopa PET. Ann Neurol 2003
Genetic interaction of CTLA‐4 with HLA‐DR15 in multiple sclerosis patients
Authors: Alizadeh Mehdi , Babron Marie‐Claude , Birebent Brigitte , Matsuda Fumihiko , Quelvennec Erwann , Liblau Roland , Cournu‐Rebeix Isabelle , Momigliano‐Richiardi Patricia , Sequeiros Jorge , Yaouanq Jacqueline , Genin Emmanuelle , Vasilescu Alexandre , Bougerie Héloise , Trojano Maria , Martins Silva Berta , Maciel Patricia , Clerget‐Darpoux Françoise , Clanet Michel , Edan Gilles , Fontaine Bertrand , Semana Gilbert 
Published: June 2003

AbstractMultiple sclerosis is a chronic inflammatory disease of the central nervous system with a genetic component. Until now, the more consistent association with the disease is found with the major histocompatibility complex, especially HLA‐DRB1*1501‐DQB1*0602 haplotype. In this report, we demonstrate the interaction of Cytotoxic T Lymphocyte‐associated antigen 4 (CTLA‐4 [CD152]) gene with DRB1*15 haplotype in multiple sclerosis genetic susceptibility. Our data were obtained from two European independent family‐based studies including 610 multiple sclerosis family trios. Ann Neurol 2003;54:119–122
Cerebrospinal fluid sulfatide is decreased in subjects with incipient dementia
Authors: Han Xianlin , Fagan Anne M. , Cheng Hua , Morris John C. , Xiong Chengjie , Holtzman David M. 
Published: June 2003

AbstractWe recently noted a profound decline in brain sulfatides (ST) in subjects who died with incipient dementia due to Alzheimer's disease. Herein, we measured ST levels in cerebrospinal fluid in cognitively normal elderly and in subjects with mild cognitive impairment due to incipient demenia of the Alzheimer type. There was a significant decrease in cerebrospinal fluid ST and in the ST to phosphatidylinositol ratio in MCI subjects. The ST to phosphatidylinositol ratio accurately differentiated very mildly impaired subjects from controls on an individual basis. The cerebrospinal fluid ST to phosphatidylinositol ratio may be a very useful biomarker for the earliest clinical stage of Alzheimer's disease. Ann Neurol 2003;54:115–119
Intraventricular hemorrhage in term neonates caused by sinovenous thrombosis
Authors: Wu Yvonne W. , Hamrick Shannon E. G. , Miller Steven P. , Haward Marlyse F. , Lai Michael C. , Callen Peter W. , Barkovich A. James , Ferriero Donna M. 
Published: June 2003

AbstractThe cause of intraventricular hemorrhage in term neonates is poorly understood. Among 29 neonates of at least 36 weeks' gestation with intraventricular hemorrhage, 9 (31%) had cerebral sinovenous thrombosis. Of the 26 neonates who underwent computed tomography or magnetic resonance studies, those with thalamic hemorrhage were more likely to have sinovenous thrombosis than those without thalamic involvement (4/5 vs 5/21, p = 0.03). Term neonates with intraventricular hemorrhage should undergo neuroimaging to evaluate the presence of sinovenous thrombosis. Ann Neurol 2003
Ciliary neurotrophic factor genotype does not influence clinical phenotype in amyotrophic lateral sclerosis
Authors: Al‐Chalabi Ammar , Scheffler Margaret D. , Smith Bradley N. , Parton Matthew J. , Cudkowicz Merit E. , Andersen Peter M. , Hayden Douglas L. , Hansen Valerie K. , Turner Martin R. , Shaw Christopher E. , Leigh P. Nigel , Brown Robert H. 
Published: June 2003

AbstractCiliary neurotrophic factor (CNTF) maintains survival of adult motor neurons. Mice lacking the CNTF gene develop mild, progressive motor neuron loss. In the normal human population, 1 to 2.3% are homozygous for a null allele, and reports suggest this mutant is associated with a younger onset of amyotrophic lateral sclerosis (ALS). We have tested this hypothesis in a study of 400 subjects with ALS and 236 controls. There was no difference in age of onset, clinical presentation, rate of progression, or disease duration for those with one or two copies of the null allele, excluding CNTF as a major disease modifier in ALS. Ann Neurol 2003;54:130–134
Hereditary motor and sensory neuropathy with agenesis of the corpus callosum
Authors: Dupré Nicolas , Howard Heidi C. , Mathieu Jean , Karpati George , Vanasse Michel , Bouchard Jean‐Pierre , Carpenter Stirling , Rouleau Guy A. 
Published: June 2003

AbstractHereditary motor and sensory neuropathy associated with agenesis of the corpus callosum (OMIM 218000) is an autosomal recessive disease of early onset characterized by a delay in developmental milestones, a severe sensory‐motor polyneuropathy with areflexia, a variable degree of agenesis of the corpus callosum, amyotrophy, hypotonia, and cognitive impairment. Although this disorder has rarely been reported worldwide, it has a high prevalence in the Saguenay‐Lac‐St‐Jean region of the province of Quebec (Canada) predominantly because of a founder effect. The gene defect responsible for this disorder recently has been identified, and it is a protein‐truncating mutation in the SLC12A6 gene, which codes for a cotransporter protein known as KCC3. Herein, we provide the first extensive review of this disorder, covering epidemiological, clinical, and molecular genetic studies. Ann Neurol 2002;54:9–18
Using aerial photography for identification of marine and coastal habitats under the EU's Habitats Directive
Authors: Ekebom Jan , Erkkilä Anne 
Published: June 2003

Abstract
Implementation of the E.U. Habitats Directive requires information on the distribution, abundance and area covered by the habitats listed in Annex I of the Directive.
In Finland, 21 of these habitats occur in marine and coastal areas. The demand for spatial information of these habitats is increasing, so rapid and relatively inexpensive mapping methods are needed.
This study examines the identification of 15 habitats using high altitude black and white aerial photographs. Our goal was to find out how well these habitats could be identified using these types of photographs. We used a test group of 34 persons who were given only brief instructions on how to identify the habitats prior to the test. Their results were compared to a set of field data from an archipelago area at the entrance of the Gulf of Finland, in August 1999 and autumn 2000.
The test group identified sandy beaches, lagoons, submerged sandbanks and cliffs with an accuracy of 82%, 71%, 66% and 65%, respectively. The main reasons for these high accuracy percentages were apparently the high contrast and/or easy delineation of the habitat from the surrounding areas.
Reefs, wooded dunes and submerged reefs were identified with an accuracy of 39%, 44% and 45%, respectively. The remaining habitats were less precisely identified, apparently due to their small size or poor contrast to the surrounding areas.
High altitude aerial photographs are shown to be a useful tool for identifying several of these habitats and can be used as a complement to field mapping methods, GIS methods and other remote sensing techniques. The use of high altitude photographs for monitoring change is discussed.
Copyright © 2002 John Wiley & Sons, Ltd.
Marine fish diversity on the Scotian Shelf, Canada
Authors: Shackell Nancy L. , Frank Kenneth T. 
Published: June 2003

Abstract
Marine life in offshore regions has not been fully censused, yet related conservation policy relies on our ability to identify areas of high biodiversity.
We assessed the census of marine finfish on the Scotian Shelf, Northwest Atlantic using data collected during annual research vessel surveys between 1970 and 2000. The species accumulation curve did not reach an asymptote reflecting that new species continued to be discovered throughout the survey period. Only 0.12% of the area of the Scotian Shelf has been sampled since 1970.
Since 1974, when over 50% of the species had been discovered, the community composition has been relatively constant. However, the dominance structure has changed dramatically as reflected in the geographic contraction of the formerly abundant, large‐bodied piscivores concomitant with the geographic expansion of their prey species.
The region is under‐sampled, and species' distribution and abundance are changing. A precise estimate of diversity is elusive. As an alternative, we searched for physical correlates of finfish diversity to identify its possible surrogates. Surrogates have potential both as a method for understanding process and as a tool for conservation management. We examined the effect of area and depth range on species richness. High species richness was associated with larger areas and greater depth range at large spatial scales.
Highly diverse areas include the Bay of Fundy, the Eastern Gully, the slopes, Western Bank and the northeastern shelf. Until now, the northeastern shelf has been under‐appreciated as a highly diverse area. Such information will be important for environmental impact assessments as well as selection of ‘sensitive’ or protected areas.
Copyright © 2003 John Wiley & Sons, Ltd.
Indicator taxa for the conservation of pond invertebrate diversity
Authors: Briers Robert A , Biggs Jeremy 
Published: June 2003

Abstract
Ponds are a valuable resource for the conservation of freshwater biodiversity, but are often extremely numerous in a given area, making assessment of the conservation value of individual sites potentially time consuming.
The use of indicator taxa, the species richness of which is representative of total site species richness, may provide one way to improve the efficiency of survey work. However, such indicators are poorly developed for freshwater systems.
A data set was used describing the occurrence of macroinvertebrate taxa in ponds in Oxfordshire, UK, to assess the extent to which variation in the species richness of selected taxa most consistently represented variation in all other taxa.
Coenagriidae (Odonata) and Limnephilidae (Trichoptera) reflected the variation in species richness of other taxa most consistently, with Coenagriidae showing the best overall performance as an indicator taxon.
For both suggested indicator taxa, selection of sites based solely on the presence of at least one species of indicator would represent over 95% of all species recorded across all sites.
Further investigation in different geographical regions is necessary to establish whether these relationships are consistent over a wider area.
Copyright © 2003 John Wiley & Sons, Ltd.
Dangerous targets? Unresolved issues and ideological clashes around marine protected areas
Authors: Agardy Tundi , Bridgewater Peter , Crosby Michael P. , Day Jon , Dayton Paul K. , Kenchington Richard , Laffoley Dan , McConney Patrick , Murray Peter A. , Parks John E. , Peau Lelei 
Published: June 2003

Abstract
While conservationists, resource managers, scientists and coastal planners have recognized the broad applicability of marine protected areas (MPAs), they are often implemented without a firm understanding of the conservation science — both ecological and socio‐economic — underlying marine protection. The rush to implement MPAs has set the stage for paradoxical differences of opinions in the marine conservation community.
The enthusiastic prescription of simplistic solutions to marine conservation problems risks polarization of interests and ultimately threatens bona fide progress in marine conservation. The blanket assignment and advocacy of empirically unsubstantiated rules of thumb in marine protection creates potentially dangerous targets for conservation science.
Clarity of definition, systematic testing of assumptions, and adaptive application of diverse MPA management approaches are needed so that the appropriate mix of various management tools can be utilized, depending upon specific goals and conditions. Scientists have a professional and ethical duty to map out those paths that are most likely to lead to improved resource management and understanding of the natural world, including the human element, whether or not they are convenient, politically correct or publicly magnetic.
The use of MPAs as a vehicle for promoting long‐term conservation and sustainable use of marine biodiversity is in need of focus, and both philosophical and applied tune ups. A new paradigm arising out of integrated, multi‐disciplinary science, management and education/outreach efforts must be adopted to help promote flexible, diverse and effective MPA management strategies. Given scientific uncertainties, MPAs should be designed so one can learn from their application and adjust their management strategies as needed, in the true spirit of adaptive management.
It is critical for the conservation community to examine why honest differences of opinion regarding MPAs have emerged, and recognize that inflexible attitudes and positions are potentially dangerous. We therefore discuss several questions — heretofore taken as implicit assumptions: (a) what are MPAs, (b) what purpose do MPAs serve, (c) are no‐take MPAs the only legitimate MPAs, (d) should a single closed area target be set for all MPAs, and (e) how should policymakers and conservation communities deal with scientific uncertainty?
Copyright © 2003 John Wiley & Sons, Ltd.
The use of littoral mesohabitats and their macroinvertebrate assemblages in the ecological assessment of lakes
Authors: White J. , Irvine K. 
Published: June 2003

Abstract
Historically, ecological assessment of lakes has depended on open‐water chemical and phytoplankton analysis, with trophic status determined by chlorophyll a and total phosphorus following the general protocol of the OECD lake classification scheme. This has been accompanied by a prevailing opinion that the littoral zone of lakes is too heterogeneous to allow meaningful inter‐lake comparisons.
Council of the European Communities Directive 2000/60/EC establishing a framework in the field of water policy (the Water Framework Directive) requires a broader approach to ecological assessment, including the need to incorporate a range of biotic variables. This paper describes the results of a monitoring programme designed to determine within‐ and among‐lake variation of macroinvertebrate communities found in defined mesohabitats of 22 lakes incorporating a range of lake types. Three sampling approaches were applied to the study.
Triplicate macroinvertebrate samples from a cobble/pebble mesohabitat of 11 lakes showed that community composition from a standard habitat was robust.
Twinned samples from contrasting habitats of pebble/cobble and macrophyte dominated mesohabitats of 21 lakes had greater similarity within than among lakes. This showed that even across contrasting habitat structure, macroinvertebrate assemblages can provide a reliable lake‐specific indicator.
Multivariate analysis of replicate samples from 15 visually distinct mesohabitats in one lake showed that within‐lake variation could, nevertheless, be identified as distinct clusters of invertebrate assemblages.
Conclusions from the work are that variations within lakes were nested in among‐lake variation across a range of lake types, and that macroinvertebrate assemblages can provide meaningful assessment of ecological differences across lakes. Monitoring can, however, produce a substantial amount of ‘noise’ from the data that reflects the complexity of macroinvertebrate community structure in littoral zones. It is recommended that incorporation of macroinvertebrates in ecological assessment is most useful when confined to well defined mesohabitats rather than attempt to incorporate a complete range of mesohabitats within a single lake.
Copyright © 2003 John Wiley & Sons, Ltd.
The concept of relevance in IR
Authors: Borlund Pia 
Published: June 2003

AbstractThis article introduces the concept of relevance as viewed and applied in the context of IR evaluation, by presenting an overview of the multidimensional and dynamic nature of the concept. The literature on relevance reveals how the relevance concept, especially in regard to the
multidimensionality of relevance, is many faceted, and does not just refer to the various relevance criteria users may apply in the process of judging relevance of retrieved information objects. From our point of view, the multidimensionality of relevance explains why some will argue that no
consensus has been reached on the relevance concept. Thus, the objective of this article is to present an overview of the many different views and ways by which the concept of relevance is used—leading to a consistent and compatible understanding of the concept. In addition, special attention
is paid to the type of situational relevance. Many researchers perceive situational relevance as the most realistic type of user relevance, and therefore situational relevance is discussed with reference to its potential dynamic nature, and as a requirement for interactive information retrieval
(IIR) evaluation.
Multidimensional Data Model and Query Language for Informetrics
Authors: Niemi Timo , Hirvonen Lasse , Järvelin Kalervo 
Published: June 2003

AbstractMultidimensional data analysis or On‐line analytical processing (OLAP) offers a single subject‐oriented source for analyzing summary data based on various dimensions. We demonstrate that the OLAP approach gives a promising starting point for advanced analysis and comparison among summary data in informetrics applications. At the moment there is no single precise, commonly accepted logical/conceptual model for multidimensional analysis. This is because the requirements of applications vary considerably. We develop a conceptual/logical multidimensional model for supporting the complex and unpredictable needs of informetrics. Summary data are considered with respect of some dimensions. By changing dimensions the user may construct other views on the same summary data. We develop a multidimensional query language whose basic idea is to support the definition of views in a way, which is natural and intuitive for lay users in the informetrics area. We show that this view‐oriented query language has a great expressive power and its degree of declarativity is greater than in contemporary operation‐oriented or SQL (Structured Query Language)‐like OLAP query languages.
An emerging view of scientific collaboration: Scientists' perspectives on collaboration and factors that impact collaboration
Authors: Hara Noriko , Solomon Paul , Kim Seung‐Lye , Sonnenwald Diane H. 
Published: June 2003

AbstractCollaboration is often a critical aspect of scientific research, which is dominated by complex problems, rapidly changing technology, dynamic growth of knowledge, and highly specialized areas of expertise. An individual scientist can seldom provide all of the expertise and resources necessary to address complex research problems. This paper describes collaboration among a group of scientists, and considers how their experiences are socially shaped. The scientists were members of a newly formed distributed, multi‐disciplinary academic research center that was organized into four multi‐disciplinary research groups. Each group had 14 to 34 members, including faculty, postdoctoral fellows and students, at four geographically dispersed universities. To investigate challenges that emerge in establishing scientific collaboration, data were collected about members' previous and current collaborative experiences, perceptions regarding collaboration, and work practices during the center's first year of operation. The data for the study includes interviews with members of the center, observations of videoconferences and meetings, and a center‐wide sociometric survey. Data analysis has led to the development of a framework that identifies forms of collaboration that emerged among scientists (e.g., complementary and integrative collaboration) and associated factors, which influenced collaboration including personal compatibility, work connections, incentives, and infrastructure. These results may inform the specification of social and organizational practices, which are needed to establish collaboration in distributed, multi‐disciplinary research centers.
Multi‐agent information classification using dynamic acquaintance lists
Authors: Mukhopadhyay Snehasis , Peng Shengquan , Raje Rajeev , Palakal Mathew , Mostafa Javed 
Published: June 2003

AbstractThere has been considerable interest in recent years in providing automated information services, such as information classification, by means of a society of collaborative agents. These agents augment each other's knowledge structures (e.g., the vocabularies) and assist each other in providing efficient information services to a human user. However, when the number of agents present in the society increases, exhaustive communication and collaboration among agents result in a large communication overhead and increased delays in response time. This paper introduces a method to achieve selective interaction with a relatively small number of potentially useful agents, based on simple agent modeling and acquaintance lists. The key idea presented here is that the acquaintance list of an agent, representing a small number of other agents to be collaborated with, is dynamically adjusted. The best acquaintances are automatically discovered using a learning algorithm, based on the past history of collaboration. Experimental results are presented to demonstrate that such dynamically learned acquaintance lists can lead to high quality of classification, while significantly reducing the delay in response time.