Cruise report hydro acoustic survey for blue whiting (Micromesistius poutassou) with R.V. Tridens, 17 March - 04 April 2008

This is the report of the Dutch part of the international North East Atlantic hydro acoustic survey for blue whiting. The survey is coordinated by ICES and has been executed annually. The purpose of the survey is to estimate the blue whiting stock of the North East Atlantic. The ICES uses this estimation is as a “tuning index” to assess the North East Atlantic blue whiting stock. The applied method was echo integration. By sailing transects over the survey area, the total acoustic cross-section can be calculated by surface area sampled. Trawling identified species composition of localized schools. The length composition of each species was determined. Blue whiting was examined on age and fecundity from which a split up stock structure was mad

Relational arenas in a regional Higher Education system: Insights from an empirical analysis

Extant indicators on research and higher education do not consider the complex relational structure in which universities are embedded and that influences their performance on one side, and the impact of policies on the other. This article investigates the overall pattern of universities' relational arenas in a Regional environment by considering their two main domains of activity, namely research and teaching. We study their structure, determinants, and existing interactions, in order to understand the possible consequences for policy making and management, and to identify synthetic indicators to represent the

Natural course of Myoclonus-Dystonia in adulthood: stable motor signs but increased psychiatry

Myoclonus‐dystonia (M‐D) is a rare hyperkinetic movement disorder characterized by upper body–predominant myoclonus and dystonia.1 A large proportion of cases are caused by autosomal‐dominant inherited mutations in the SGCE gene. In addition to the motor manifestations, psychiatric disorders are frequently reported.2 Several studies have suggested that they may form a primary component of the M‐D phenotype.3, 4 This study represents the first long‐term follow‐up study of both motor and psychiatric symptomatology in adults with M‐D (SGCE mutation), providing further insights into the natural history of M‐D and enabling more prognostic information

Which disease features run in essential tremor families?:A systematic review

Essential tremor is a common and highly heritable movement disorder. It is largely unknown, however, to what extent family members share overlapping symptoms. Such knowledge would be useful, as it may lead to the definition of familial essential tremor phenotypes, which will aid the ongoing search for genotypes. Also, this information can be used by clinicians in patient counselling. Therefore, we conducted a systematic review to provide an overview of the evidence on which essential tremor features run in families, to assess the literature's strengths and weaknesses, and to provide recommendations for future studies. PubMed was searched resulting in 460 titles: sixteen articles ultimately proved fit for inclusion. The results are represented in line with the Axis 1 classification of tremor as published in the latest Consensus Statement. In summary, we found varying levels of positive evidence for familial aggregation of age at onset, disease progression, alcohol responsiveness, parkinsonism and dystonia. Evidence on midline tremor was conflicting. The evidence on familial clustering was negative for cerebellar signs and action tremor asymmetry. Although the level of evidence is modest, it seems that some disease features are indeed familial, while other features are not. We discuss complicating factors, such as state-vs-trait dependency of characteristics, the place of familial dystonia, and the development of diagnostic criteria for essential tremor over time. In the future, comprehensive replication studies are needed, with the addition of several characteristics that have not been investigated so far, as the next step towards discovery of essential tremor phenotypes

Long-term survival and transmission of INI1-mutation via nonpenetrant males in a family with rhabdoid tumour predisposition syndrome

Rhabdoid tumour predisposition syndrome (RTPS) is a rare syndrome caused by inheritance of a mutated INI1 gene for which only two multigeneration families have been reported. To further characterise the genotype and phenotype of RTPS, we present a third family in which at least three cousins developed an atypical teratoid/rhabdoid tumour (AT/RT) at a young age. Two of these patients showed unusual long survival, and one of these developed an intracranial meningioma and a myoepithelioma of the lip in adulthood. Mutation analysis of INI1 revealed a germline G>A mutation in the donor splice site of exon 4 (c.500+1G>A) in the patients and in their unaffected fathers. This mutation prevents normal splicing and concomitantly generates a stop codon, resulting in nonsense-mediated mRNA decay. Biallelic inactivation of INI1 in the tumours, except for the meningioma, was confirmed by absence of nuclear INI1-protein staining. The myoepithelioma of one of the patients carried an identical somatic rearrangement in the NF2 gene as the AT/RT, indicating that both tumours originated from a common precursor cell. In conclusion, this study demonstrates for the first time transmission of a germline INI1-mutation in a RTPS family via nonpenetrant males, long-term survival of two members of this family with an AT/RT, and involvement of INI1 in the pathogenesis of myoepithelioma

The Leiden Ranking 2011/2012: Data collection, indicators, and interpretation

The Leiden Ranking 2011/2012 is a ranking of universities based on bibliometric indicators of publication output, citation impact, and scientific collaboration. The ranking includes 500 major universities from 41 different countries. This paper provides an extensive discussion of the Leiden Ranking 2011/2012. The ranking is compared with other global university rankings, in particular the Academic Ranking of World Universities (commonly known as the Shanghai Ranking) and the Times Higher Education World University Rankings. Also, a detailed description is offered of the data collection methodology of the Leiden Ranking 2011/2012 and of the indicators used in the ranking. Various innovations in the Leiden Ranking 2011/2012 are presented. These innovations include (1) an indicator based on counting a university's highly cited publications, (2) indicators based on fractional rather than full counting of collaborative publications, (3) the possibility of excluding non-English language publications, and (4) the use of stability intervals. Finally, some comments are made on the interpretation of the ranking, and a number of limitations of the ranking are pointed out

The spectrum of involuntary vocalizations in humans: A video atlas

In clinical practice, involuntary vocalizing behaviors are typically associated with Tourette syndrome and other tic disorders. However, they may also be encountered throughout the entire tenor of neuropsychiatry, movement disorders, and neurodevelopmental syndromes. Importantly, involuntary vocalizing behaviors may often constitute a predominant clinical sign, and, therefore, their early recognition and appropriate classification are necessary to guide diagnosis and treatment. Clinical literature and video‐documented cases on the topic are surprisingly scarce. Here, we pooled data from 5 expert centers of movement disorders, with instructive video material to cover the entire range of involuntary vocalizations in humans. Medical literature was also reviewed to document the range of possible etiologies associated with the different types of vocalizing behaviors and to explore treatment options. We propose a phenomenological classification of involuntary vocalizations within different categorical domains, including (1) tics and tic‐like vocalizations, (2) vocalizations as part of stereotypies, (3) vocalizations as part of dystonia or chorea, (4) continuous vocalizing behaviors such as groaning or grunting, (5) pathological laughter and crying, (6) vocalizations resembling physiological reflexes, and (7) other vocalizations, for example, those associated with exaggerated startle responses, as part of epilepsy and sleep‐related phenomena. We provide comprehensive lists of their associated etiologies, including neurodevelopmental, neurodegenerative, neuroimmunological, and structural causes and clinical clues. We then expand on the pathophysiology of the different vocalizing behaviors and comment on available treatment options. Finally, we present an algorithmic approach that covers the wide range of involuntary vocalizations in humans, with the ultimate goal of improving diagnostic accuracy and guiding appropriate treatment

A Hemoperfusion Column Based on Activated Carbon Granules Coated with an Ultrathin Membrane of Cellulose Acetate

A hemoperfusion system has been developed which makes use of activated carbon encapsulated with cellulose acetate. Studies have revealed that there are no stagnant flow regions in the column, there i? minimal particle release and the coating is 30 Å thick. The relationships between pore size, pore volume and surface area have been examined. Twenty-five patients in grade IV coma have been treated with the column for treatment of drug overdose or agricultural chemical poisoning; the clinical course of one meprobamate-poisoned patient is described in detail