Periodicals Collections in Academic Libraries of the Federal Republic of Germany: Development and Managerial Aspects

Academic libraries in the Federal Republic of Germany had, after World War II, to devote their main activities on collection development with emphasis on periodical collections. Circumstances recommended decentralized cooperative acquisition programmes coordinated and supported by the Deutsche Forschungsgemeinschaft. Besides these decentralized special collections, local serials collections developed in number and coverage to a fair standard: the traditional universities were enlarged, former Technische Hochschulen expanded to full universities, new universities were founded. Both local and regional/supra-regional activities led up to acceptable satisfaction rates. These were positively influenced by the creation of periodical holding lists (local, regional, national) , culminating in the Berlin Periodical Data Base. The entire development was favoured by economic prosperity, which declined gradually in the late seventies and dramatically in 1982. Academic libraries in the Federal Republic had suddenly to face the same challenge as libraries in the USA and other countries some years earlier. One of the immediate reactions of academic libraries was the cancellation of more than 25,000 current periodicals. The existence of decentralized special collections (Sondersammelgebiete) was basic to the line of decision taken by local library systems. Problems and implications of the present situation, with its impact on the publishing and the user community, are briefly discussed. Given conditions in the Federal Republic determine procedures of reducing acquisition programmes and resource sharing. These are to be seen within a changing world of information development and new ways of publishing outside the paper medium. Extreme resource sharing is seen as fundamentally fatal to a desirabie balance of the socio-economic system of research, publishing and library activities

Stability effects on results of diffusion tensor imaging analysis by reduction of the number of gradient directions due to motion artifacts: an application to presymptomatic Huntington's disease.

In diffusion tensor imaging (DTI), an improvement in the signal-to-noise ratio (SNR) of the fractional anisotropy (FA) maps can be obtained when the number of recorded gradient directions (GD) is increased. Vice versa, elimination of motion-corrupted or noisy GD leads to a more accurate characterization of the diffusion tensor. We previously suggest a slice-wise method for artifact detection in FA maps. This current study applies this approach to a cohort of 18 premanifest Huntington's disease (pHD) subjects and 23 controls. By 2-D voxelwise statistical comparison of original FA-maps and FA-maps with a reduced number of GD, the effect of eliminating GD that were affected by motion was demonstrated.We present an evaluation metric that allows to test if the computed FA-maps (with a reduced number of GD) still reflect a "true" FA-map, as defined by simulations in the control sample. Furthermore, we investigated if omitting data volumes affected by motion in the pHD cohort could lead to an increased SNR in the resulting FA-maps.A high agreement between original FA maps (with all GD) and corrected FA maps (i.e. without GD corrupted by motion) were observed even for numbers of eliminated GD up to 13. Even in one data set in which 46 GD had to be eliminated, the results showed a moderate agreement

Thalamic Atrophy in Huntington's Disease Co-varies with Cognitive Performance: A Morphometric MRI Analysis

The pattern of motor, behavioral and cognitive symptoms in Huntington's disease (HD) implicates dysfunction of basal-ganglia-thalamo-cortical circuits. This study explored if cognitive performance in HD is correlated with localized cerebral changes. Psychomotor functions were investigated by verbal fluency, Stroop color word and Digit Symbol tests in 44 HD patients and 22 controls. Three-dimensional magnetic resonance imaging (MRI) data were analyzed with regard to regional gray matter changes by use of the observer-independent whole-brain-based approach of voxel-based morphometry (VBM). Using statistical parametric mapping, the MRI data of the HD patients were analyzed in an ANCOVA including the individual results of the neuropsychological tests. Besides striatal areas, symmetrical regional atrophy of the thalamus was found to co-vary significantly with cognitive performance (P < 0.001, corrected for multiple comparisons). In particular, thalamic subnuclei projecting to prefrontal areas (dorsomedial subnucleus) and connected to the striatum (centromedian/parafascicular and ventrolateral nuclear complex) displayed volume loss, in agreement with neuropathological studies. These results suggest that thalamic degeneration contributes in an important way to the impairment of executive function in early HD. Patients who are impaired in executive tests display structural double lesions of the basal-ganglia-thalamo-cortical circuitry both at the striatal and at the thalamic leve

Meaningful and measurable health domains in Huntington’s: large-scale validation of the Huntington’s disease health-related quality of life questionnaire (HDQoL) across severity stages

Objectives: While health-related quality of life is key for patients with long-term neurodegenerative conditions, measuring this is less straightforward and complex in Huntington’s disease. This study aimed to refine and validate a fully patient-derived instrument, the Huntington’s Disease health-related Quality of Life questionnaire (HDQoL), and to elucidate health domains that are meaningful to patients’ lived experience. Methods: Five-hundred and forty-one participants, from pre-manifest to end-stage disease completed the HDQoL, together with generic quality of life measures, and in-person motor, cognitive and behavioural assessments. The psychometric properties of the HDQoL were examined using factor analysis and Rasch analysis. Results: Four HDQoL domains emerged reflecting the classical triad of HD features - they were Physical-Functional, Cognitive, and two different behavioural aspects i.e. Mood-Self domain, as well as a distinct Worries domain. These domains clarify the behavioural sequelae as experienced by patients, and all showed good to excellent internal consistency. Known groups analyses illustrated significant and graded changes in clinical assesments and corresponding HDQoL domains across severity levels. Convergent and discriminant validity was demonstrated by the expected pattern of correlations between specific HDQoL domains and corresponding domain-relevant clinical assesments as well as patient-reported measures. The data demonstrate robust support for the refined HDQoL across disease stages. Conclusions: The HDQoL with its two distinct behavioural domains of Mood-Self and of Worries, as well as a Physical-Functional and a Cognitive domain, is a relevant, reliable and valid patient-derived instrument to measure the impact of Huntington’s disease across all severity stages

Caudate Nucleus and Insular Activation During a Pain Suppression Paradigm Comparing Thermal and Electrical Stimulation

Pain modulation is an integral function of the nervous system. It is needed to adapt to chronic stimuli. To gain insights into pain suppression mechanisms, two studies concerning the suppression of the feeling of pain with different stimulation modalities (heat vs. electrical stimuli) but using the same stimulation paradigms were compared: 15 subjects each had been stimulated on both hands under the instruction to suppress the feeling of pain

The gene coding for PGC-1α modifies age at onset in Huntington's Disease

Huntington's disease (HD) is one of the most common autosomal dominant inherited, neurodegenerative disorders. It is characterized by progressive motor, emotional and cognitive dysfunction. In addition metabolic abnormalities such as wasting and altered energy expenditure are increasingly recognized as clinical hallmarks of the disease. HD is caused by an unstable CAG repeat expansion in the HD gene (HTT), localized on chromosome 4p16.3. The number of CAG repeats in the HD gene is the main predictor of disease-onset, but the remaining variation is strongly heritable. Transcriptional dysregulation, mitochondrial dysfunction and enhanced oxidative stress have been implicated in the pathogenesis. Recent studies suggest that PGC-1α, a transcriptional master regulator of mitochondrial biogenesis and metabolism, is defective in HD. A genome wide search for modifier genes of HD age-of-onset had suggested linkage at chromosomal region 4p16-4p15, near the locus of PPARGC1A, the gene coding for PGC-1α. We now present data of 2-loci PPARGC1A block 2 haplotypes, showing an effect upon age-at-onset in 447 unrelated HD patients after statistical consideration of CAG repeat lengths in both HTT alleles. Block 1 haplotypes were not associated with the age-at-onset. Homozygosity for the 'protective' block 2 haplotype was associated with a significant delay in disease onset. To our knowledge this is the first study to show clinically relevant effects of the PGC-1α system on the course of Huntington's disease in humans

Overlap between age-at-onset and disease-progression determinants in Huntington disease

OBJECTIVE: A fundamental but still unresolved issue regarding Huntington disease (HD) pathogenesis is whether the factors that determine age at onset are the same as those that govern disease progression. Because elucidation of this issue is crucial for the development as well as optimal timing of administration of novel disease-modifying therapies, we aimed to assess the extent of overlap between age-at-onset and disease-progression determinants in HD. METHODS: Using observational data from Enroll-HD, the largest cohort of patients with HD worldwide, in this study we present, validate, and apply an intuitive method based on linear mixed-effect models to quantify the variability in the rate of disease progression in HD. RESULTS: A total of 3,411 patients with HD met inclusion criteria. We found that (1) about two-thirds of the rate of functional, motor, and cognitive progression in HD is determined by the same factors that also determine age at onset, with CAG repeat–dependent mechanisms having by far the largest effect; (2) although expanded HTT CAG repeat size had a large influence on average body weight, the rate of weight loss was largely independent of factors that determine age at onset in HD; and (3) about one-third of the factors that determine the rate of functional, motor, and cognitive progression are different from those that govern age at onset and need further elucidation. CONCLUSION: Our findings imply that targeting of CAG repeat–dependent mechanisms, for example through gene-silencing approaches, is likely to affect the rate of functional, motor, and cognitive impairment, but not weight loss, in manifest HD mutation carriers

Adaptive Equalization and Capacity Analysis for Amplify-and-Forward Relays

Recent research has shown that multiple-input multiple-output (MIMO) systems provide high spectral efficiencies and error performance gains. However, the use of multiple antennas in mobile terminals may not be very practical. Certainly there is limited space and other implementation issues which make this a challenging problem. Therefore, to harness the diversity gains afforded by MIMO transmitter diversity techniques, while maintaining a minimal number of antennas on each handset, cooperative diversity techniques have been proposed. In addition, attention has also been given to combining wireless relaying systems with MIMO techniques to improve capacity, coverage, and obtain better diversity at the expense of increased node complexity. This thesis considers the design and analysis of cooperative diversity systems and MIMO amplify-and-forward relaying systems. In particular, we investigate adaptive time- and frequency-domain equalization techniques for cooperative diversity systems using space-time block codes (STBC). For MIMO relaying systems, we analyze the ergodic capacity of various systems and compare different amplify-and-forward methods in terms of system capacity performance. We propose a new block time-domain adaptive equalization structure for time reversal-space time block coding (TR-STBC) systems, which eliminates the separate decoder and also the need for explicit channel state information (CSI) estimation at the receiver. Our simulation results show that the time-domain adaptive block equalizer performs better than the frequency-domain counterpart but at the cost of increased complexity. Then, we extend this time-domain adaptive equalization scheme to distributed TR-STBC systems. We also develop a frequency-domain counterpart for the distributed systems. Our simulation results show that the adaptive algorithms work well for Protocols I and III proposed by Nabar et al. The time-domain adaptive algorithms perform better than the frequency-domain algorithms, and overall the Protocol I receivers outperform the Protocol III receivers. We also show that, if only the Protocol III receiver is used, it can be susceptible to noise amplification due to a weaker source-to-relay link compared to the relay-to-destination link. This problem can be mitigated by using the Protocol I receivers with some extra complexity but much superior diversity performance. We also present an ergodic capacity analysis of an amplify-and-forward (AF) MIMO two-hop system including the direct link and validate the analysis with simulations. We show that having the direct link improves the capacity due to diversity and quantify this improvement. We also present an ergodic capacity analysis of an AF MIMO two-hop, two relay system. Our results verify the capacity gain of relaying systems with two relays due to the extra diversity compared to a single relaying system. However, the results also show that when one of the source-to-relay links has a markedly higher SNR compared to the other, a single relay system has better capacity than a two relay system. Finally, we compare three types of relay amplification methods: a) average amplification, b) instantaneous channel amplification, and c) instantaneous power amplification. The instantaneous power amplification method has a higher mean capacity but with a higher variance. Also, it requires additional information at the destination and would create enormous overheads compared to the other methods. We also find that the instantaneous channel amplification method has almost no advantage in terms of the mean capacity but its capacity is less variable than the average amplification method. On the other hand, the average amplification method is simpler to implement as it does not require channel estimation at the relaying terminal

Symptomatic treatment options for Huntington's disease (guidelines of the German Neurological Society)

INTRODUCTION Ameliorating symptoms and signs of Huntington's disease (HD) is essential to care but can be challenging and hard to achieve. The pharmacological treatment of motor signs (e.g. chorea) may favorably or unfavorably impact other facets of the disease phenotype (such as mood and cognition). Similarly, pharmacotherapy for behavioral issues may modify the motor phenotype. Sometimes synergistic effects can be achieved. In patients undergoing pragmatic polypharmacological therapy, emerging complaints may stem from the employed medications' side effects, a possibility that needs to be considered. It is recommended to clearly and precisely delineate the targeted signs and symptoms (e.g., chorea, myoclonus, bradykinesia, Parkinsonism, or dystonia). Evidence from randomized controlled trials (RCTs) is limited. Therefore, the guidelines prepared for the German Neurological Society (DGN) for German-speaking countries intentionally extend beyond evidence from RCTs and aim to synthesize evidence from RCTs and recommendations of experienced clinicians. RECOMMENDATIONS First-line treatment for chorea is critically discussed, and a preference in prescription practice for using tiapride instead of tetrabenazine is noted. In severe chorea, combining two antidopaminergic drugs with a postsynaptic (e.g., tiapride) and presynaptic mode of action (e.g., tetrabenazine) is discussed as a potentially helpful strategy. Sedative side effects of both classes of compounds can be used to improve sleep if the highest dosage of the day is given at night. Risperidone, in some cases, may ameliorate irritability but also chorea and sleep disorders. Olanzapine can be helpful in the treatment of weight loss and chorea, and quetiapine as a mood stabilizer with an antidepressant effect. CONCLUSIONS Since most HD patients simultaneously suffer from distinct motor signs and distinct psychiatric/behavioral symptoms, treatment should be individually adapted

Corrigendum: Combining Literature Review With a Ground Truth Approach for Diagnosing Huntington's Disease Phenocopy.

[This corrects the article DOI: 10.3389/fneur.2022.817753.]