bluuebunny/crossref_metadata_2025_split huggingface dataset:
The action of the rectus femoris muscle following distal tendon transfer: does it generate knee flexion moment?
Authors: Riewald Scott A. , Delp Scott L. 
Published: July 2010

Rectus femoris transfer surgery involves detaching the rectus femoris from the patella and reattaching it posterior to the knee. While this procedure is thought to convert the rectus femoris from a knee extensor to a knee flexor, the moments generated by this muscle after transfer have never been measured. We used intramuscular electrodes to stimulate the rectus femoris in four subjects, two after transfer to the semitendinosus and two after transfer to the iliotibial band, while measuring the resultant knee moment.Electromyographic activity was monitored in the quadriceps, hamstrings, and gastrocnemius muscles to verify that the rectus femoris was the only muscle activated by the stimulus. We found that the rectus femoris generated a knee extension moment in all of the subjects tested. This finding suggests that transfer surgery does not convert the rectus femoris to a knee flexor, and that a mechanism exists which may transmit the force generated by the rectus femoris anterior to the knee joint center after distal tendon transfer.
EEG and evoked potentials in infantile neuronal ceroid‐lipofuscinosis
Authors: Vanhanen Sanna‐Leena , Sainio Kimmo , Lappi Marjatta , Santavuori Pirkko 
Published: July 2010

Sixteen children with infantile neuronal ceroid‐lipofuscinosis (INCL), age range 0.5 to 5.4 years, were studied using EEG, electroretinograms (ERG), visual evoked potentials (VEP) and somatosensory evoked potentials (SEP).Electroencephalography was the first of these examinations to reveal abnormalities, however the EEG may be normal at the preclinical stage. The first abnormality to appear was an attenuated reaction to passive eye opening and closing which was followed by disturbances in background activity and diminution in amplitude, and by disappearance of sleep spindles. The gradual disappearance of posterior rhythm reactivity and of sleep spindles suggests that thalamic dysfunction progresses with time. EEG inactivity appeared by the age of 3 years. Evoked potentials were normal in the early stages of the disease. SEP showed abnormalities at Stage 2 (1.7 years), while ERG and VEP abnormalities appeared at Stage 3 (by the age of 2.5 years). All neurophysiologies! reactions examined were abolished by the age of 4 years. Follow‐up EEG gives important hints as to the early diagnosis of INCL. Progression of the disease can be followed by evoked potentials which may also be helpful in the differential diagnostics.
Biotinidase deficiency: two cases of very early presentation
Authors: Andersen Jesper Brandt , Blichfeldt Susanne , Christensen Mogens Fjord , Haagerup Annette 
Published: July 2010

Two infants with early presentation of biotinidase deficiency (age 3 weeks and 2 weeks) are described. On admission, both children had severe neurological symptoms. In the first patient, magnetic resonance imaging (MRI) of the brain showed frontal and temporal atrophy, and in the second patient, CT of the brain showed diffuse periventricular hypodensities, particularly in the frontal region. Oral treatment with biotin (15mg and 10mg per day respectively) made all symptoms disappear within a few weeks. On follow‐up 13 and 16 months later, both children were still asymptomatic on this treatment. Their psychomotor development was normal. MRI and CT of the brain had normalized. Later, a moderate hearing loss was detected in the first patient.In biotinidase deficiency, early diagnosis and treatment with oral biotin are essential in order to prevent irreversible damage to the central nervous system and early death from metabolic acidosis. Neonatal screening for biotinidase deficiency would fulfil this goal.
Can cerebral MRI at age 1 year predict motor and intellectual outcomes in very‐low‐birth weight children?
Authors: Skranes J , Vik T , Nilsen G , Smevik O , Andersson H W , Brubakk A M 
Published: July 2010

This follow‐up study reports on cerebral MRI findings in 20 very‐low‐birthweight (VLBW) infants without disabilities at age 1 year in relation to motor, intellectual, and perceptual function at age 6 years. MRI findings, anthropometrics, and Bayley Scales of Infant Development scores at age 1 year as predictors of psychomotor status at age 6 years are also evaluated and compared. Outcome parameters were the Peabody Developmental Motor Scales and the Wechsler Preschool and Primary Scale of Intelligence. The results show that infants with myelin hyperintensities including the centrum semiovale or with occipital hyperintensities with associated ventricular dilatation at age 1 scored lower on the Peabody Gross Motor Locomotion Scale at age 6 than infants with normal myelination or with isolated occipital hyperintensities. This may indicate damage to motor fibers caused by perinatal periventricular leukomalacia. No relation was found between abnormal MRI findings at age 1 and later fine motor, intellectual, and perceptual function.Comparing different age 1‐year predictors, an abnormality score defined by MRI was used as an independent predictor of gross motor locomotion function at age 6 years. However, the Bayley Mental Development Index scores and weight at age 1 were more important predictors of later motor and intellectual outcome, respectively, than MRI findings. It is recommended that cerebral MRI should not be used routinely to examine VLBW infants without disabilities at 1 year of age.
Possible strategies to protect the preterm brain against the fetal inflammatory response
Authors: Dammann Olaf 
Published: July 2010

SUMMARYThe accruing evidence that a fetal inflammatory response is the link between antenatal infection and white matter damage in the preterm newborn infant offers room for speculation how this harmful sequence could be interrupted. Enhancement of endogenous protection, response modification, and damage limitation downstream could be helpful strategies for intervention design. Appropriate observational and experimental studies are needed before clinical interventions can be initiated.
Level of purposeful hand function as a marker of clinical severity in Rett syndrome
Authors: DOWNS JENNY , BEBBINGTON AMI , JACOBY PETER , WILLIAMS ANNE‐MARIE , GHOSH SOUMYA , KAUFMANN WALTER E , LEONARD HELEN 
Published: July 2010

Aim  We investigated relationships between hand function and genotype and aspects of phenotype in Rett syndrome.Method  Video assessment in naturalistic settings was supplemented by parent‐reported data in a cross‐sectional study of 144 females with a mean age of 14 years 10 months (SD 7y 10mo; range 2y–31y 10mo), 110 of whom had a mutation of the methyl CpG binding protein 2 (MECP2) gene. Ordinal logistic regression was used to assess relationships between hand function and MECP2 mutation, age, a modified Kerr score, Functional Independence Measure for Children (WeeFIM), ambulation level, and frequency of hand stereotypies.Results  Approximately two‐thirds of participants demonstrated purposeful hand function, ranging from simple grasping skills to picking up and manipulating small objects. In participants with a confirmed MECP2 mutation, those with the p.R168X mutation had the poorest hand function on multivariate analysis with C‐terminal deletion as the baseline (odds ratio [OR] 0.19; 95% confidence interval [CI] 0.04–0.95), whereas those with the p.R133C or p.R294X mutation had better hand function. Participants aged 19 years or older had lower hand function than those aged less than 8 years (OR 0.36; 95% CI 0.14–0.92). Factors that were associated with better hand function were lower Kerr scores for a 1‐point increase in score (OR 0.77; 95% CI 0.69–0.86), higher WeeFIM scores for a 1‐point increase in score (OR 1.08; 95% CI 1.04–1.12), and greater ambulation than those completely dependent on carers for mobility (OR 22.64; 95% CI 7.02–73.08). The results for participants with a confirmed pathogenic mutation were similar to results obtained when participants without a mutation were also included.Interpretation  Our novel assessment of hand function in Rett syndrome correlated well with known profiles of common MECP2 mutations and overall clinical severity. This promising assessment could measure clinical responses to therapy.
ADJUST: An automatic EEG artifact detector based on the joint use of spatial and temporal features
Authors: Mognon Andrea , Jovicich Jorge , Bruzzone Lorenzo , Buiatti Marco 
Published: July 2010

AbstractA successful method for removing artifacts from electroencephalogram (EEG) recordings is Independent Component Analysis (ICA), but its implementation remains largely user‐dependent. Here, we propose a completely automatic algorithm (ADJUST) that identifies artifacted independent components by combining stereotyped artifact‐specific spatial and temporal features. Features were optimized to capture blinks, eye movements, and generic discontinuities on a feature selection dataset. Validation on a totally different EEG dataset shows that (1) ADJUST's classification of independent components largely matches a manual one by experts (agreement on 95.2% of the data variance), and (2) Removal of the artifacted components detected by ADJUST leads to neat reconstruction of visual and auditory event‐related potentials from heavily artifacted data. These results demonstrate that ADJUST provides a fast, efficient, and automatic way to use ICA for artifact removal.
Peripheral responses to attended and unattended angry prosody: A dichotic listening paradigm
Authors: Aue Tatjana , Cuny Caroline , Sander David , Grandjean Didier 
Published: July 2010

AbstractWe investigated the effects of angry prosody, varying focus of attention, and laterality of presentation of angry prosody on peripheral nervous system activity. Participants paid attention to either their left or their right ear while performing a sex discrimination task on dichotically presented pseudo‐words. These pseudo‐words were characterized by either angry or neutral prosody and presented stereophonically (anger/neutral, neutral/anger, or neutral/neutral, for the left/right ear, respectively). Reaction times and physiological responses (heart period, skin conductance, finger and forehead temperature) in this study were differentially sensitive to the effects of anger versus neutral prosody, varying focus of attention, and laterality of presentation of angry prosody.
Feedback processing in hypothesis testing: A Laplacian ERP study
Authors: Carbonnell Laurence , Caverni Jean‐Paul , Vidal Franck 
Published: July 2010

AbstractThe capacity to adjust behavior and evaluate performance in a changing environment is highly related to taking feedback into account. Using feedback is, hence, crucial for different cognitive capacities. The present study focused on feedback processing in hypothetico‐deductive reasoning. Electroencephalographical (EEG) activity was recorded while participants performed a hypothesis‐testing task in which a feedback signal allowing the evaluation of performance was delivered at each trial. To ameliorate the spatial resolution of EEG signals, the surface Laplacian was estimated. Results showed three distinct patterns of activation. At central and centro‐parietal electrodes, the activity was sensitive to participants' expectancies in a binary and a gradual way, respectively. At the parieto‐occipital electrode, the activity was sensitive to the evaluation of performance itself: correct versus false. In conclusion, the processing of feedback signal depends on at least three separate processes.
The effects of EEG data transformations on the solution accuracy of principal component analysis
Authors: Arruda James E. , McGee Heather A. , Zhang Hongmei , Stanny Claudia J. 
Published: July 2010

AbstractPrincipal component analysis (PCA) is a commonly used multivariate procedure that reduces the dimensionality of a data set. When applied to quantitative electroencephalogram (qEEG) data, PCA produces components that may represent functional systems within the brain. Unfortunately, qEEG, like many other physiological measures, produce distributions that are positively skewed. In response, researchers often transform qEEG data prior to conducting a PCA, which does not require univariate or multivariate normality. Despite this, researchers continue to transform qEEG data with limited knowledge of how such transformations will affect the accuracy (precision) of their component solutions. The purpose of the present investigation was to examine the effects of several commonly used data transformation procedures on PCA solution accuracy.
Pitch and loudness information encoded in auditory imagery as revealed by event‐related potentials
Authors: Wu Jianhui , Yu Zulin , Mai Xiaoqin , Wei Jinghan , Luo Yuejia 
Published: July 2010

AbstractTwo experiments using the ERP method and a task that involved comparing an imagined‐S1 (the first stimulus) with a perceived‐S2 (the second stimulus) were conducted to investigate whether imagined auditory representations encode pitch and loudness information. It was found that the amplitude of the imagery‐related late positive complex (LPC) decreased with pitch but increased with loudness of the imagined sound, which was consistent with amplitude modulations of the auditory perception‐related N1 component, thereby providing the first neural evidence that auditory imagery encodes perceptual attributes of auditory experiences.
Electrophysiological evidence for the hierarchical organization of auditory change detection in the human brain
Authors: Grimm Sabine , Escera Carles , Slabu Lavinia , Costa‐Faidella Jordi 
Published: July 2010

AbstractAuditory change detection has been associated with mismatch negativity (MMN), an event‐related potential (ERP) occurring at 100–250 ms after the onset of an acoustic change. Yet, single‐unit recordings in animals suggest much faster novelty‐specific responses in the auditory system. To investigate change detection in a corresponding early time range in humans, we measured the Middle Latency Response (MLR) and MMN during a controlled frequency oddball paradigm. In addition to MMN, an early effect of change detection was observed at about 40 ms after change onset reflected in an enhancement of the Nb component of the MLR. Both MMN and the Nb effect were shown to be free from confounding influences such as differences in refractoriness. This finding implies that early change detection processes exist in humans upstream of MMN generation, which supports the emerging view of a hierarchical organization of change detection expanding along multiple levels of the auditory pathway.
Abnormal mechanisms of antisaccade generation in schizophrenia patients and unaffected biological relatives of schizophrenia patients
Authors: Kang Seung Suk , Dionisio Daphne P. , Sponheim Scott R. 
Published: July 2010

AbstractAlthough errant saccadic eye movements may mark genetic factors in schizophrenia, little is known about abnormal brain activity that precedes saccades in individuals with genetic liability for schizophrenia. We investigated electrophysiological activity preceding prosaccades and antisaccades in schizophrenia patients, first‐degree biological relatives of schizophrenia patients, and control subjects. Prior to antisaccades, patients had reduced potentials over lateral prefrontal cortex. Smaller potentials were associated with worse antisaccade performance. Relatives also exhibited reduced pre‐saccadic potentials over lateral frontal cortex but additionally had reduced potentials over parietal cortex. Both patients and relatives tended toward increased activity over orbital frontal cortex prior to saccades. Results are consistent with lateral prefrontal dysfunction marking genetic liability for schizophrenia and underlying deficient saccadic control.
Altered cholesterol homeostasis contributes to enhanced excitotoxicity in Huntington’s disease
Authors: Del Toro Daniel , Xifró Xavier , Pol Albert , Humbert Sandrine , Saudou Frédéric , Canals Josep M. , Alberch Jordi 
Published: July 2010

J. Neurochem. (2010) 115, 153–167.AbstractRecent findings suggest that altered cholesterol homeostasis may contribute to the pathophysiology of Huntington’s disease (HD). To understand the underlying mechanisms, here we used a combination of two‐photon microscopy, epifluorescence, and biochemical methods to visualize and quantify lipid distribution in cell cultures expressing mutant huntingtin. Such expression promotes lipid imbalance, and cholesterol accumulation in cellular and murine models and in HD‐affected human brains. Interestingly, cells expressing mutant huntingtin also showed higher content of ordered domains in their plasma membranes. These findings correlated with high levels of caveolin‐1 and glycosphingolipid GM1, two well‐defined markers of cholesterol‐enriched domains, at the cell surface. In addition, cells expressing mutant huntingtin showed increased localization of NMDA receptors with cholesterol‐enriched domains, contributing to increased NMDA receptor susceptibility to excitotoxic insults. Treatment with simvastatin or β‐cyclodextrin, two cholesterol‐lowering drugs, reduced the content of ordered domains at the cell surface, which in turn, protected cells against NMDA‐mediated excitotoxicity. Taken together, our results indicate that mutant huntingtin produces accumulation of cholesterol and alters its cellular distribution that contributes to NMDA‐mediated excitotoxicity. Administration of drugs that recover this effect, such as simvastatin could be beneficial for the treatment of HD.
Involvement of ceramide in ethanol‐induced apoptotic neurodegeneration in the neonatal mouse brain
Authors: Saito Mariko , Chakraborty Goutam , Hegde Medha , Ohsie Jason , Paik Sun‐Mee , Vadasz Csaba , Saito Mitsuo 
Published: July 2010

J. Neurochem. (2010) 115, 168–177.AbstractAcute administration of ethanol to 7‐day‐old mice is known to cause robust apoptotic neurodegeneration in the brain. Our previous studies have shown that such ethanol‐induced neurodegeneration is accompanied by increases in lipids, including ceramide, triglyceride (TG), cholesterol ester (ChE), and N‐acylphosphatidylethanolamine (NAPE) in the brain. In this study, the effects of ethanol on lipid profiles as well as caspase 3 activation were examined in the cortex, hippocampus, cerebellum, and inferior colliculus of the postnatal day 7 mouse brain. We found that the cortex, hippocampus, and inferior colliculus, which showed substantial caspase 3 activation by ethanol, manifested significant elevations in ceramide, TG, and NAPE. In contrast, the cerebellum, with the least caspase 3 activation, failed to show significant changes in ceramide and TG, and exhibits much smaller increases in NAPE than other brain regions. Ethanol‐induced increases in ChE were observed in all brain regions tested. Inhibitors of serine palmitoyltransferase effectively blocked ethanol‐induced caspase 3 activation as well as elevations in ceramide, ChE, and NAPE. Immunohistochemical studies indicated that the expression of serine palmitoyltransferase was mainly localized in neurons and was enhanced in activated caspase 3‐positive neurons generated by ethanol. These results indicate that de novo ceramide synthesis has a vital role in ethanol‐induced apoptotic neurodegeneration in the developing brain.
Semantic analysis according to Peep Koort – a substance‐oriented research methodology
Authors: Sivonen Kerstin , Kasén Anne , Eriksson Katie 
Published: July 2010

Scand J Caring Sci; 2010; 24; 12–20

Semantic analysis according to Peep Koort – a substance‐oriented research methodology The aim of this article is to describe the hermeneutic semantic analysis created by professor Peep Koort (1920–1977) and to discuss it as a methodology for research within caring science. The methodology is developed with a hermeneutic approach that differs from the traditions of semantic analysis in philosophy or linguistics. The research objects are core concepts and theoretical constructs (originally within the academic discipline of education science, later on within the academic discipline of caring science), focusing deeper understanding of essential meaning content when developing a discipline. The qualitative methodology of hermeneutic semantic analysis is described step by step as created by Koort, interpreted and developed by the authors. An etymological investigation and an analysis of synonymy between related concepts within a conceptual family guides the researcher to understand and discriminate conceptual dimensions of meaning content connected to the word studied, thus giving opportunities to summarise it in a theoretical definition, a discovery that can be tested in varying contexts. From a caring science perspective, we find the hermeneutic methodology of semantic analysis fruitful and suitable for researchers developing their understanding of core concepts and theoretical constructs connected to the development of the academic discipline.
Polycystic liver: clinical characteristics of patients with isolated polycystic liver disease compared with patients with polycystic liver and autosomal dominant polycystic kidney disease
Authors: Hoevenaren Inge A. , Wester Ruth , Schrier Robert W. , McFann Kim , Doctor R. Brian , Drenth Joost P. H. , Everson Gregory T. 
Published: July 2010

AbstractAim: The goal of this study was to compare the clinical features of patients with isolated polycystic liver disease (PCLD) with those of patients with polycystic liver and autosomal dominant polycystic kidney disease (ADPKD).Methods: Cases were identified from clinical records at the University of Colorado Hospital in Denver (USA) and at the Radboud University Hospital in Nijmegen (the Netherlands) by ICD‐10 codes. To be included in this analysis, patients had to have an initial diagnosis of PCLD within six years of presentation to our clinics. Medical records were reviewed for demographic information, medical history, physical examination, symptoms, complications, laboratory and imaging results, therapy and outcomes.Results: Out of a total of 94, 53 patients met our criteria for entering this study, 19 with PCLD and 34 with ADPKD. The mean time interval from diagnosis of PCLD to presentation in our clinics was 1.21 years for PCLD and 2.76 years for ADPKD (P=NS). PCLD was associated with female gender in both PCLD and ADPKD. Patients with PCLD had greater numbers (P=0.031), and larger sizes of liver cysts (P=0.0051), but had less associated morbidities than patients with ADPKD. Liver cyst decompressions were performed more frequently in PCLD patients (57.9 vs. 23.5%, P=0.012). However, serious hepatic complications, sufficient to require consideration of liver transplantation, were more frequent in patients with ADPKD (0/19 vs. 6/34, P<0.0001).Conclusions: Although PCLD in patients with PCLD is characterized by larger and greater number of hepatic cysts, the clinical course is relatively benign compared with ADPKD.