The updated European Consensus 2009 on the use of Botulinum toxin for children with cerebral palsy

An interdisciplinary European group of clinical experts in the field of movement disorders and experienced Botulinum toxin users has updated the consensus for the use of Botulinum toxin in the treatment of children with cerebral palsy (CP). A problem-orientated approach was used focussing on both published and practice-based evidence. In part I of the consensus the authors have tabulated the supporting evidence to produce a concise but comprehensive information base, pooling data and experience from 36 institutions in 9 European countries which involves more than 10,000 patients and over 45,000 treatment sessions during a period of more than 280 treatment years. In part II of the consensus the Gross Motor Function Measure (GMFM) and Gross Motor Function Classification System (GMFCS) based Motor Development Curves have been expanded to provide a graphical framework on how to treat the motor disorders in children with CP. This graph is named "CP(Graph) Treatment Modalities - Gross Motor Function" and is intended to facilitate communication between parents, therapists and medical doctors concerning (1) achievable motor function, (2) realistic goal-setting and (3) treatment perspectives for children with CP. The updated European consensus 2009 summarises the current understanding regarding an integrated, multidisciplinary treatment approach using Botulinum toxin for the treatment of children with CP.

Diffusion tensor imaging of two unrelated Chinese men with hereditary spastic paraplegia associated with thin corpus callosum

Hereditary spastic paraplegia (HSP) associated with thin corpus callosum is a rare autosomal recessive neurodegenerative disorder characterized by an abnormally thin corpus callosum, normal motor development, slowly progressive spastic paraparesis and cognitive deterioration. To investigate and localize abnormalities in the brains of two Chinese patients with HSP-TCC, with mutations in the spatacsin gene. Diffusion tensor imaging (DTI) was used to determine the mean diffusion (MD) and fractional anisotropy (FA) in the brains of the patients in comparison to 20 healthy subjects. Voxel-based analysis (VBA) of both the diffusion and anisotropy values were performed using statistical parametric mapping (SPM). Significant changes with MD increase and FA reduction were found in the already known lesions including the corpus callosum, cerebellum and thalamus. In addition, changes were also found in regions that appear to be normal in conventional MRI, such as the brain stem, internal capsule, cingulum and subcortical white matter including superior longitudinal fascicle and inferior longitudinal fascicle. Neither increase in FA nor reduction in MD was detected in the brain. Our study provides clear in vivo MR imaging evidence of a more widespread brain involvement of HSP-TCC. MD is more sensitive than FA in detecting lesions in thalamus and subcortical white matter, suggesting that MD may be a better marker of the disease progression.

Effekte spezifischer Interventionen im Sportunterricht auf die exekutiven Funktionen von Primarschulkindern

Einleitung Die Annahme, dass Sport nicht nur positive Effekte auf die körperliche Gesundheit, sondern auch auf die kognitive Leistung haben kann, konnte anhand experimenteller Studien mit Erwachsenen weitgehend bestätigt werden. Ob dieselben Effekte auch bei Kindern und Jugendlichen vorzufinden sind, kann mit Blick auf die mangelnde empirische Evidenz in dieser Altersgruppe kaum zufriedenstellend beantwortet werden (Chang et al., 2012). Will man zudem der Frage nach den Wirkmechanismen nachgehen, sind Unter-suchungsdesigns angezeigt, die theoriegeleitet verschiedene Sportinterventionen mit unterschiedlichen Beanspruchungsmodalitäten kombinieren. So ist unter der Annahme der cardiovascular fitness hypothesis (Etnier et al., 2006) zur gezielten Förderung der kognitiven Leistungsfähigkeit ein systematisches Ausdauertraining sinnvoll, während theoretische Ansätze, die neurophysiologische Korrelate zur Erklärung des Zusammenhangs zwischen Sport und Kognition heranziehen (Diamond, 2000) eher kognitiv sowie koordinativ anspruchsvolle Sportangebote nahelegen würden. Daher geht der vorliegende Beitrag der Frage nach, ob spezifisch konzipierte langfristige Interventionen im Sportunterricht einen spezifischen Effekt auf die kognitive Leistungsfähigkeit von Primarschulkindern haben können. Methode Im Rahmen der quasiexperimentellen Längsschnittstudie „Sport und Kognition“ (SpuK_5.0) wurden insgesamt 250 Schülerinnen und Schüler von 16 fünften Klassen untersucht. Während knapp zwei Monaten absolvierten je vier Klassen während zwei Lektionen des obligatorischen Sportunterrichts entweder ein spielsportbezogenes EF-Training oder ein Ausdauertraining resp. ein kognitives oder kein spezifisches Training (Kontrollgruppe mit regulärem Sportunterricht). Durch die Konzeption dieser vier Experi-mentalbedingungen wurde sichergestellt, dass alle vier möglichen Kombinationen aus hoher resp. niedriger kognitiver und körperlicher Beanspruchung im Design repräsentiert waren. Ergebnisse und Diskussion Im Beitrag werden erste Ergebnisse der noch laufendenden SpuK_5.0-Studie vorgestellt und vor dem Hintergrund aktueller theoretischer Annahmen zu den zugrundeliegenden Wirkmechanismen diskutiert. Literatur Chang, Y. K., Labban, J. D., Gapin, J. I., & Etnier, J. L. (2012). The effects of acute exercise on cognitive performance: A meta-analysis. Brain Research, 1453, 87-101. Diamond, A. (2000). Close interrelation of motor development and cognitive development and of the cere-bellum and prefrontal cortex. Child Development, 71, 44-56. Etnier, J. L., Nowell, P. M., Landers, D. M., & Sibley, B. A. (2006). A meta-regression to examine the rela-tionship between aerobic fitness and cognitive performance. BRAIN RESEARCH, 52, 119-130.

Risk factors for schizophrenia. Follow-up data from the Northern Finland 1966 Birth Cohort Study

This paper updates single risk factors identified by the Northern Finland 1966 Birth Cohort Study up to the end of year 2001 or age 34. Impaired performance (e.g., delayed motor or intellectual development) or adverse exposures (e.g., pregnancy and birth complications, central nervous system diseases) are associated with an increased risk for schizophrenia. However, upper social class girls and clever schoolboys also have an increased risk to develop schizophrenia, contrasted to their peers. Individuals who subsequently develop schizophrenia follow a developmental trajectory that partly and subtly differs from that of the general population; this trajectory lacks flexibility and responsiveness compared to control subjects, at least in the early stages. We propose a descriptive, lifespan, multilevel systems model on the development and course of schizophrenia.

A case of bilateral perisylvian syndrome with reading disability

Bilateral Perisylvian Syndrome (BPS) often presents with epilepsy and significant behavioral impairments that can include mental retardation, dysarthria, delayed speech development, and delayed fine motor development (Graff-Radford et al., 1986 and Kuzniecky et al., 1993). While a small subset of BPS cases have been described as having relatively isolated language delays (Leventer et al., 2010), BPS is not expected in children with dyslexia. As part of a Medical University of South Carolina, IRB approved multi-site study involving retrospective and de-identified dyslexia data, we unexpectedly identified a 14.05 year old male with evidence of BPS whose father had been diagnosed with dyslexia and dysgraphia. This child had been recruited for a neuroimaging study on dyslexia from a school specializing in educating children with dyslexia. The T1-weighted MRI scan from this child demonstrated a highly unusual perisylvian sulcal/gyral patterning that is a defining feature of BPS (Fig. 1). BPS cases exhibit bilateral dysgenesis of the Sylvian fissure and surrounding gyri, which appears to occur because of a limited or absent arcuate fasciculus (Kilinc, Ekinci, Demirkol, & Agan, 2015). This BPS case also had a relatively enlarged atrium of the lateral ventricle that is consistent with the BPS anatomical presentation and reduction of parietal white matter (Graff-Radford et al., 1986, Kilinc et al., 2015 and Toldo et al., 2011).

Identificação de sinais precoces de risco em crianças com SXF a partir de vídeos familiares

Com a presente investigação pretendeu-se validar a metodologia de análise retrospetiva de vídeos familiares e explorar potenciais sinais de risco precoce em crianças com SXF(N=6). Utilizando grelhas de observação de comportamentos, analisou-se a atenção social, a atenção partilhada, e o desenvolvimento sensório-motor, nas crianças com SXF, dos 0-30 meses. Validou-se a metodologia de análise de vídeos retrospetivos para a identificação de sinais de risco. Com base no estudo dos sinais de risco, sugere-se que as crianças com SXF têm preferência por estímulos não-sociais (e.g., fixação visual prolongada em objetos), têm dificuldades em dirigir a atenção para estímulos sociais (e.g., chamada e resposta ao nome) e défices na atenção partilhada, e demonstram uma interação prolongada e repetitiva com objetos e as expressões afetivas positivas. Verificou-se também que as crianças com SXF parecem conseguir discriminar entre estímulos sociais e não-sociais (e.g., vocalização para as pessoas) e apresentam estereotipias em todas as idades; Identifying early risk features in children with FXS through home movies Abstract: This research aims to validade the retrospective video analysis methodology of home movies and to explore potential early risk signs in children with FXS (N=6). Recurring to an observational grids of behaviors, social attention, joint attention, social attention and sensorymotor features were analysed, in children with FXS, at 0-30 months. Retrospective video analysis was validated for the identification of early risk signs. The study of early risk signs suggests that infants with FXS prefer non-social stimuli (e.g., visual fixation in objects), have difficulties directing attention toward social stimuli (e.g., name prompts) and impairments in joint attention, prolongued and repetitive interaction with objects, and have positive affective rating. Findings reveal that infants with FXS seem to be able to discriminate from social and non-social stimuli (e.g., vocalizing to people) and presente stereotypies through all ages.

Development of moving magnet type linear motor for dual piston compressor for pulse tube cryocooler

This paper describes the design, fabrication and testing of a moving magnet type linear motor of dual piston configuration for a pulse tube cryocooler for ground applications. Eight radially magnetized segmented magnets were used to form one set of a magnet ring. Four magnet rings of such type were constructed, in which one pair of rings has north-pole on its outer diameter and south-pole on inner diameter, while the other pair is it's complementary. The magnets were mounted with opposite poles together on the magnet holder with an axial moving shaft having a piston mounted on both ends of the shaft. The shaft movement was restricted to the axial direction by using C-clamp type flexures, mounted on both sides of the shaft. The force requirement for driving the compressor was calculated based on which the electrical circuit of motor is designed by proper selection of wire gauge and Ampere-turns. The flexure spring force estimation was done through simulation using ANSYS 11.0 and was verified experimentally; while the magnet spring force was determined experimentally. The motor with mounted piston was tested using a variable voltage and variable frequency power supply capable of driving 140 watts of load.

Staged development of Robot Motor Coordination

M.H. Lee and Q. Meng, 'Staged development of Robot Motor Coordination', IEEE International Conference on Systems, Man and Cybernetics, (IEEE SMC 05), Hawaii, USA, v3, 2917-2922, 2005.

MiR-375 is essential for human spinal motor neuron development and may be involved in motor neuron degeneration

The transcription factor REST is a key suppressor of neuronal genes in non-neuronal tissues. REST has been shown to suppress pro-neuronal microRNAs in neural progenitors indicating that REST-mediated neurogenic suppression may act in part via microRNAs. We used neural differentiation of Rest-null mouse ESC to identify dozens of microRNAs regulated by REST during neural development. One of the identified microRNAs, miR-375, was upregulated during human spinal motor neuron development. We found that miR-375 facilitates spinal motor neurogenesis by targeting the cyclin kinase CCND2 and the transcription factor PAX6. Additionally, miR-375 inhibits the tumor suppressor p53 and protects neurons from apoptosis in response to DNA damage. Interestingly, motor neurons derived from a spinal muscular atrophy patient displayed depressed miR-375 expression and elevated p53 protein levels. Importantly, SMA motor neurons were significantly more susceptible to DNA damage induced apoptosis suggesting that miR-375 may play a protective role in motor neurons.

FIPRONIL promotes motor and behavioral changes in honey bees (APIS Mellifera) and affects the development of colonies exposed to sublethal doses

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Sensor-based evaluation of Circadian motor behavior in people with Dementia. Development and validation of analysis strategies

[ITA]La demenza consiste nel deterioramento, spesso progressivo, dello stato cognitivo di un individuo. Chi è affetto da demenza, presenta alterazioni a livello cognitivo, comportamentale e motorio, ad esempio compiendo gesti ossessivi, ripetitivi, senza uno scopo preciso. La condizione dei pazienti affetti da demenza è valutata clinicamente tramite apposite scale e le informazioni relative al comportamento vengono raccolte intervistando chi se ne occupa, come familiari, il personale infermieristico o il medico curante. Spesso queste valutazioni si rivelano inaccurate, possono essere fortemente influenzate da considerazioni soggettive, e sono dispendiose in termini di tempo. Si ha quindi l'esigenza di disporre di metodiche oggettive per valutare il comportamento motorio dei pazienti e le sue alterazioni patologiche; i sensori inerziali indossabili potrebbero costituire una valida soluzione, per questo scopo. L'obiettivo principale della presente attività di tesi è stato definire e implementare un software per una valutazione oggettiva, basata su sensori, del pattern motorio circadiano, in pazienti affetti da demenza ricoverati in un'unità di terapia a lungo termine, che potrebbe evidenziare differenze nei sintomi della malattia che interessano il comportamento motorio, come descritto in ambito clinico. Lo scopo secondario è stato quello di verificare i cambiamenti motori pre- e post-intervento in un sottogruppo di pazienti, a seguito della somministrazione di un programma sperimentale di intervento basato su esercizi fisici. --------------- [ENG]Dementia involves deterioration, often progressive, of a person's cognitive status. Those who suffer from dementia, present alterations in cognitive and motor behavior, for example performing obsessive and repetitive gestures, without a purpose. The condition of patients suffering from dementia is clinically assessed by means of specific scales and information relating to the behavior are collected by interviewing caregivers, such as the family, nurses, or the doctor. Often it turns out that these are inaccurate assessments that may be heavily influenced by subjective evaluations and are costly in terms of time. Therefore, there is the need for objective methods to assess the patients' motor behavior and the pathological changes; wearable inertial sensors may represent a viable option, so this aim. The main objective of this thesis project was to define and implement a software for a sensor-based assessment of the circadian motor pattern in patients suffering from dementia, hospitalized in a long-term care unit, which could highlight differences in the disease symptoms affecting the motor behavior, as described in the clinical setting. The secondary objective was to verify pre- and post-intervention changes in the motor patterns of a subgroup of patients, following the administration of an experimental program of intervention based on physical exercises.