Pre-Production and Quality Assurance of the Mu2e Calorimeter Silicon Photomultipliers

The Mu2e electromagnetic calorimeter has to provide precise information on energy, time and position for $\sim$100 MeV electrons. It is composed of 1348 un-doped CsI crystals, each coupled to two large area Silicon Photomultipliers (SiPMs). A modular and custom SiPM layout consisting of a 3$\times$2 array of 6$\times$6 mm$^2$ UV-extended monolithic SiPMs has been developed to fulfill the Mu2e calorimeter requirements and a pre-production of 150 prototypes has been procured by three international firms (Hamamatsu, SensL and Advansid). A detailed quality assurance process has been carried out on this first batch of photosensors: the breakdown voltage, the gain, the quenching time, the dark current and the Photon Detection Efficiency (PDE) have been determined for each monolithic cell of each SiPMs array. One sample for each vendor has been exposed to a neutron fluency up to $\sim$8.5~$\times$~10$^{11}$ 1 MeV (Si) eq. n/cm$^{2}$ and a linear increase of the dark current up to tens of mA has been observed. Others 5 samples for each vendor have undergone an accelerated aging in order to verify a Mean Time To Failure (MTTF) higher than $\sim$10$^{6}$ hours.Comment: NDIP 2017 - New Developments In Photodetection, 3-7 July 2017, Tours (France

Efficacy and safety of felbamate in children under 4 years of age: a retrospective chart review.

Background and purpose: To review our experience of the efficacy and tolerability of felbamate in children younger than 4 years. Methods: We used a retrospective chart review to identify 53 children with seizures who were younger than 4 years. Efficacy was evaluated based on the occurrence of responsiveness, defined as seizure frequency reduction of more than 50% for a minimum period of 4 months. Tolerability was based on parent-reported side effects. Results: Twenty-two (41%) patients resulted to be responders and 31 (59%) did not. By univariate analysis, those achieving seizure remission were probably much older, to have a shorter history of epilepsy and a lower frequency of seizures before felbamate therapy. The number of antiepileptic drugs (AEDs) used before felbamate therapy was the only significant predictor of the duration of response to felbamate, with a longer responsiveness to the drug seen in those who were placed under fewer than three AEDs before felbamate compared with those who had taken more than three (median, 16 months vs. 7 months; P < 0.0084). Side effects occurred in 30% of the subjects, but these did not require discontinuation of the drug. Discussion: Felbamate is an effective medication for a wide range of epilepsy syndromes in children younger than 4 years. Although caution is necessary when the drug is used in children, felbamate might represent a possible option for the treatment of epilepsy in this age group. Introduction The incidence of epilepsy is high during the first year of life and it declines steadily during childhood and adolescence. Few of the new antiepileptic drugs (AEDs) are indicated officially for children younger than 4 years [1–3]. None of them is approved for children younger than 2 years [1,3,4]. There is a striking discrepancy between the high incidence of epilepsy in infancy and the relatively few approved AEDs available for this age group [5]. Felbamate (2-phenyl-1,3-propanediol dicarbamate) is a derivative of the anti-anxiety drug meprobamate, exerting additional anticonvulsant and neuroprotective properties [6–8]. The drug has been approved since 1993 for the treatment of several types of epilepsy. Experimental studies suggested that felbamate might inhibit voltage-dependen

Neurological phenotype of mowat-wilson syndrome

Mowat-Wilson Syndrome (MWS) (OMIM # 235730) is a rare disorder due to ZEB2 gene defects (heterozygous mutation or deletion). The ZEB2 gene is a widely expressed regulatory gene, extremely important for the proper prenatal development. MWS is characterized by a specific facial gestalt and multiple musculoskeletal, cardiac, gastrointestinal, and urogenital anomalies. The nervous system involvement is extensive and constitutes one of the main features in MWS, heavily affecting prognosis and life quality of affected individuals. This review aims to comprehensively organize and discuss the neurological and neurodevelopmental phenotype of MWS. First, we will describe the role of ZEB2 in the formation and development of the nervous system by reviewing the preclinical studies in this regard. ZEB2 regulates the neural crest cell differentiation and migration, as well as in the modulation of GABAergic transmission. This leads to different degrees of structural and functional impairment that have been explored and deepened by various authors over the years. Subsequently, the different neurological aspects of MWS (head and brain malformations, epilepsy, sleep disorders, and enteric and peripheral nervous system involvement, as well as developmental, cognitive, and behavioral features) will be faced one at a time and extensively examined from both a clinical and etiopathogenetic point of view, linking them to the ZEB2 related pathways

Efficacy and safety of levetiracetam in infants and young children with refractory epilepsy

SummaryThe aim of this multicentric, retrospective, and uncontrolled study was to evaluate the efficacy and safety of levetiracetam (LEV) in 81 children younger than 4 years with refractory epilepsy. At an average follow-up period of 9 months, LEV administration was found to be effective in 30% of patients (responders showing more than a 50% decrease in seizure frequency) of whom 10 (12%) became seizure free. This efficacy was observed for focal (46%) as well as for generalized seizures (42%). In addition, in a group of 48 patients, we compared the initial efficacy (evaluated at an average of 3 months of follow-up) and the retention at a mean of 12 months of LEV, with regard to loss of efficacy (defined as the return to the baseline seizure frequency). Twenty-two patients (46%) were initial responders. After a minimum of 12 months of follow-up, 9 of 48 patients (19%) maintained the improvement, 4 (8%) of whom remained seizure free. A loss of efficacy was observed in 13 of the initial responders (59%). Maintained LEV efficacy was noted in patients with focal epilepsy and West syndrome. LEV was well tolerated. Adverse events were seen in 18 (34%) patients. The main side effects were drowsiness and nervousness. Adverse events were either tolerable or resolved in time with dosage reduction or discontinuation of the drug.We conclude that LEV is safe and effective for a wide range of epileptic seizures and epilepsy syndromes and, therefore, represents a valid therapeutic option in infants and young children affected by epilepsy

Autism spectrum disorder and anorexia nervosa: an Italian prospective study

Background: Potential overlaps exist between psychopathological features of Anorexia Nervosa (AN) and Autism Spectrum Disorder (ASD). The impact of malnutrition on autistic traits in patients with AN should be considered. This study investigates possible associations among the psychopathology of Eating Disorders (EDs), ASD traits and BMI in a group of young patients with AN, using the EDI-3 (Eating Disorder Inventory-3) test and gold-standard measures for ASD. Methods: Prospective study involving 23 inpatients admitted to an Italian Centre for paediatric ED. ASD traits and ED psychopathology were assessed administering the ADOS-2 (Autism Diagnostic Observation Schedule-2), AQ (Autism Quotient) and EDI-3 tests. Both present and past autistic traits were investigated using different versions of AQ. Correlations were adjusted for BMI, Obsessive Compulsive Disorder (OCD) comorbidity and concurrent antipsychotic treatments. Results: An ASD diagnosis was possible in 22% of patients. Significant correlations were documented between ASD traits and ED psychopathology: AQ total-Interpersonal problems (IPC) (p&nbsp;= 0.041); AQ total-Global psychological maladjustment (GMPC) (p&nbsp;= 0.027); AQ social skills-Ineffectiveness (IC) (p&nbsp;= 0.018); AQ social skills-IPC (p&nbsp;= 0.019); AQ social skills-Affective problems (APC) (p&nbsp;= 0.025); AQ social skills-GMPC (p&nbsp;= 0.007); AQ attention switching-IPC (p&nbsp;= 0.020); ADOS-2 imagination-IC (p&nbsp;= 0.035). These correlations were independent of BMI, OCD and antipsychotic treatments. Conclusions: ASD traits presented high prevalence in a group of young inpatients with AN. These traits were significantly correlated to 4 specific EDI-3 subscales and independent of BMI. This is the first study to investigate the relationship between ASD traits as measured with gold-standard measures, EDI-3 scores, and BMI

Energy and time resolution for a LYSO matrix prototype of the Mu2e experiment

We have measured the performances of a LYSO crystal matrix prototype tested with electron and photon beams in the energy range 60$-$450 MeV. This study has been carried out to determine the achievable energy and time resolutions for the calorimeter of the Mu2e experiment.Comment: 2 pages, 3 figures, 13th Pisa Meeting on Advanced Detector