The aim of this study was to model the components of rhythmic function in a case (H.J.) of acquired rhythmic disturbance. H.J. is a right-handed, amateur male musician who acquired arrhythmia in the context of a global amusia after sustaining a right temporoparietal infarct. His rhythmic disturbance was analysed in relation to three independent components using an autoregressive extension of Wing and Kristofferson's model of rhythmic timing. This revealed preserved error-correction and motor implementation capacities, but a gross disturbance of H.J.'s central timing system ("cognitive clock"). It rendered him unable to generate a steady pulse, prevented adequate discrimination and reproduction of novel metrical rhythms, and partly contributed to bi-manual co-ordination difficulties in his instrumental performance. The findings are considered in relation to the essential components of the cognitive architecture of rhythmic function, and their respective cerebral lateralisation and localisation. Overall, the data suggested that the functioning of the right temporal auditory cortex is fundamental to 'keeping the beat' in music. The approach is presented as a new paradigm for future neuropsychological research examining rhythmic disturbances. 相似文献
Depending on the precise temporal relationship between their spiking activities, connections between neurons could be modified in opposite directions. Although the functional implications of this spike-timing-dependent plasticity are not clear, several theoretical studies have indicated that it could underlie important effects such as sequence learning, predictive learning and balancing excitation and inhibition. To explore fully this novel form of synaptic plasticity, it is crucial to understand how the modification builds up over the consecutive spikes of presynaptic and postsynaptic neurons. In the absence of solid data, many theorists assumed a linear summation model. However, recent experiments specifically devised to study this issue have demonstrated that the effects of the consecutive spikes on the overall modification steadily decline, indicating strong non-linearities in the corresponding learning rules. 相似文献
In primary hyperoxaluria type 1 (PH 1), deficiency or mistargeting of hepatic alanine glyoxylate aminotransferase (AGT) results in over-production of oxalate and hyperoxaluria, leading to nephrocalcinosis and development of end-stage renal disease (ESRD) in the majority of patients. Renal transplantation (Tx) alone carries a high risk of disease recurrence as the metabolic defect is not cured. Therefore, combined liver/kidney Tx is recommended for patients with ESRD. An alternative approach is to cure PH 1 by pre-emptive isolated liver Tx (PLTx) before ESRD has occurred, but this approach has been carried out only occasionally and there are no uniformly accepted recommendations concerning the timing of this procedure. We report follow-up 3-5.7 yr after performing successful PLTx in four children (at the age of 3-9 yrs) with PH 1 prior to the occurrence of ESRD (glomerular filtration rate [GFR] range 27-98 mL/min/1.73 m2). There was no mortality or long-term morbidity associated with the Tx procedure. Plasma and urinary oxalate levels normalized rapidly within 4 weeks, and renal function did not deteriorate under immunosuppression, even in one patient with advanced chronic renal failure (GFR 27 mL/min/1.73 m2) who showed a stable course for more than 5.7 yrs. Although treatment must be individualized in this severe metabolic disorder, and PLTx has to be regarded as an invasive procedure, we consider that PLTx should be offered and considered early in the course of PH 1. PLTx cures the metabolic defect in PH 1 and can help to prevent, or at least delay, the progression to ESRD and systemic oxalosis. 相似文献
Objective: To compare fetal/infant mortality risk associated with each additional week of expectant management with the infant mortality risk of immediate delivery in growth-restricted pregnancies.
Methods: A retrospective cohort study was conducted of singleton, nonanomalous pregnancies from the 2005–2008 California Birth Registry comparing pregnancies affected and unaffected by growth restriction, defined using birth weights as a proxy for fetal growth restriction (FGR). Birth weights were subdivided as greater than the 90th percentile, between the 10th percentile and 90th percentile, and less than the 10th percentile. Cases greater than the 90th percentile were excluded from analysis. Cases less than the 10th percentile were considered to have FGR and were further subcategorized into <10th percentile, <5th percentile, and <3rd percentile. We compared the risk of infant death at each gestational age week against a composite risk representing the mortality risk of one additional week of expectant management.
Results: We identified 1,641,000 births, of which 110,748 (6.7%) were less than 10th percentile. The risk of stillbirth increased with gestational age with the risk of stillbirth at each week of gestation inversely proportional to growth percentile. The risks of fetal and infant mortality with expectant management outweighed the risk of infant death for all FGR categories analyzed beginning at 38 weeks. However, the absolute risks differed by growth percentiles, with the highest risks of infant death and stillbirth in the <3rd percentile cohort. At 39 weeks, absolute risks were low, although the number needed to deliver to prevent 1 death ranged from 413 for <3rd percentile to 2667 in unaffected pregnancies.
Conclusion: At 38 weeks, the mortality risk of expectant management for one additional week exceeds the risk of delivery across all growth-restricted cohorts, despite variation in absolute risk by degree of growth restriction. 相似文献