Identifying cell class specific losses from serially generated electroretinogram components

Purpose. Processing of information through the cellular layers of the retina occurs in a serial manner. In the electroretinogram (ERG), this complicates interpretation of inner retinal changes as dysfunction may arise from "upstream" neurons or may indicate a direct loss to that neural generator. We propose an approach that addresses this issue by defining ERG gain relationships.

Methods. Regression analyses between two serial ERG parameters in a control cohort of rats are used to define gain relationships. These gains are then applied to two models of retinal disease.

Results. The PIIIamp to PIIamp gain is unity whereas the PIIamp to pSTRamp and PIIamp to nSTRamp gains are greater than unity, indicating "amplification" (P <0.05). Timing relationships show amplification between PIIIit to PIIit and compression for PIIit to pSTRit and PIIit to nSTRit, (P <0.05). Application of these gains to ?-3-deficiency indicates that all timing changes are downstream of photoreceptor changes, but a direct pSTR amplitude loss occurs (P <0.05). Application to diabetes indicates widespread inner retinal dysfunction which cannot be attributed to outer retinal changes (P <0.05).

Conclusions. This simple approach aids in the interpretation of inner retinal ERG changes by taking into account gain characteristics found between successive ERG components of normal animals.

The Relationship between Components of the Behavioural Phenotype in Prader-Willi Syndrome

Background

Repetitive questions and temper outbursts form part of the behavioural phenotype of Prader-Willi syndrome (PWS). We investigated the phenomenology of temper outbursts in PWS and their relationship with other PWS behavioural characteristics.

Method

Four individuals with PWS were observed (5-10 h), during a number of experimental and natural environment challenges, some of which were expected to trigger temper outbursts. Individual behaviours including crying, ignoring, arguing, questioning, stereotypy, frowning and posture changes were recorded and subjected to lag sequential analysis.

Results

All participants were significantly more likely to show repetitive questioning before more challenging behaviours such as crying, arguing or ignoring requests. Precursor behaviours such as frowning and stereotypical behaviour were identified in three participants.

Conclusions

Temper outbursts in PWS may be associated with other PWS behavioural phenotypic characteristics such as repetitive questions and 'stubbornness'. A progression of behaviours may lead up to the most challenging temper outburst behaviours. This may have important implications for effective coping strategies.

Auditory observation of stepping actions can cue both spatial and temporal components of gait in Parkinson's disease patients

Objectives: A common behavioural symptom of Parkinson’s disease (PD) is reduced step length (SL). Whilst sensory cueing strategies can be effective in increasing SL and reducing gait variability, current cueing strategies conveying spatial or temporal information are generally confined to the use of either visual or auditory cue modalities, respectively. We describe a novel cueing strategy using ecologically-valid ‘action-related’ sounds (footsteps on gravel) that convey both spatial and temporal parameters of a specific action within a single cue.
Methods: The current study used a real-time imitation task to examine whether PD affects the ability to re-enact changes in spatial characteristics of stepping actions, based solely on auditory information. In a second experimental session, these procedures were repeated using synthesized sounds derived from recordings of the kinetic interactions between the foot and walking surface. A third experimental session examined whether adaptations observed when participants walked to action-sounds were preserved when participants imagined either real recorded or synthesized sounds.
Results: Whilst healthy control participants were able to re-enact significant changes in SL in all cue conditions, these adaptations, in conjunction with reduced variability of SL were only observed in the PD group when walking to, or imagining the recorded sounds.
Conclusions: The findings show that while recordings of stepping sounds convey action information to allow PD patients to re-enact and imagine spatial characteristics of gait, synthesis of sounds purely from gait kinetics is insufficient to evoke similar changes in behaviour, perhaps indicating that PD patients have a higher threshold to cue sensorimotor resonant responses.

Modelling lay-up automation and production rate interaction on the cost of large stiffened panel components

This paper presents an integrated design and costing method for large stiffened panels for the purpose of investigating the influence and interaction of lay-up technology and production rate on manufacturing cost. A series of wing cover panels (≈586kg, 19·9m2) have been sized with realistic requirements considering manual and automated lay-up routes. The integrated method has enabled the quantification of component unit cost sensitivity to changes in annual production rate and employed equipment maximum deposition rate. Moreover the results demonstrate the interconnected relationship between lay-up process and panel design, and unit cost. The optimum unit cost solution when using automated lay-up is a combination of the minimum deposition rate and minimum number of lay-up machines to meet the required production rate. However, the location of the optimum unit cost, at the boundaries between the number of lay-up machines required, can make unit cost very sensitive to small changes in component design, production rate, and equipment maximum deposition rate. - See more at: http://aerosociety.com/News/Publications/Aero-Journal/Online/1941/Modelling-layup-automation-and-production-rate-interaction-on-the-cost-of-large-stiffened-panel-components#sthash.0fLuu9iG.dpuf

The effect of choice on motivation for young children on the autism spectrum during discrete trial teaching

Self-determination and decision-making are acknowledged internationally as key rights of persons with disabilities and should play an important role in the development of educational plans and procedures. Not only is the chance for individuals with developmental disabilities to select their own tasks, leisure activities or reinforcers a valuable way of enhancing rights-based education and personal dignity, but choice-making opportunities may also function as a useful clinical or educational tool if they actually improve the efficacy of programmes aimed at the acquisition of socially relevant behaviours and life skills or the reduction of challenging behaviours.

The study reported here assessed whether or not choice affected effectiveness of an educational procedure for three children on the autism spectrum. Following a preference assessment, a number of discrete teaching trials were conducted with each child and, contingent upon targeted responses, either the child or the therapist selected one of three preferred reinforcer items. Reinforcer choice did not affect intervention effectiveness for two of the children; however, performance and motivation improved for the third child. Results re-affirmed the importance of thorough preference assessments prior to intervention and showed that additional stimulus choice contingent on the target response may improve motivation and outcomes for some children.

Mechanochemical interconversion between discrete complexes and coordination networks - formal hydration/dehydration by LAG

Using a small planetary ball mill, liquid-assisted grinding (LAG) of metal salts or oxides (ZnO, CdO, CdCO3, Cu(OAc)(2)center dot H2O, Co(OAc)(2)center dot 4H(2)O, Mn(OAc)(2)center dot 4H(2)O, Ni(OAc)(2)center dot 4H(2)O, FeSO4 center dot 7H(2)O) with two equivalents of isonicotinic acid (HINA) and small amounts of water ( up to 5.6 molar equivalents) gave discrete aquo complexes trans-[M(INA)(2)(OH2)(4)] (M = Zn, Cd, Cu, Fe, Co, Ni, Mn) efficiently within 30 min. For M = Zn, Cd and Cu these complexes readily undergo reversible formal dehydration to the extended network structures [M(INA)(2)] (M = Zn, Cu) or [Cd(INA)(2)(OH2)]center dot DMF by further LAG with non-aqueous liquids such as methanol or DMF. Overall, the mechanochemical dehydrations are more effective than heating or immersion in bulk solvents. The work demonstrates a two-step mechanochemical synthesis of coordination networks via discrete aquo complexes which may be preferable to single step reactions or grinding-annealing procedures in some cases. For example, the two step method was the only way to prepare [Cd(INA)(2)(OH2)]center dot DMF mechanochemically and the porous network Cu(INA)(2) could not be obtained from the aquo complex by heating.