THGEM based photon detector for Cherenkov imaging applications

We are developing a single photon detector for Cherenkov imaging counters. This detector is based on the use of THGEM electron multipliers in a multilayer design. The major goals of our project are ion feedback suppression down to a few per cent, large gain, fast response, insensitivity to magnetic fields, and a large detector size. We report about the project status and perspectives. In particular, we present a systematic study of the THGEM response as a function of geometrical parameters, production techniques and the gas mixture composition. The first figures obtained from measuring the response of a CsI coated THGEM to single photons are presented

THGEM based photon detector for Cherenkov imaging applications

We are developing a single photon detector for Cherenkov imaging counters. This detector is based on the use of THGEM electron multipliers in a multilayer design. The major goals of our project are ion feedback suppression down to a few per cent, large gain, fast response, insensitivity to magnetic fields, and a large detector size. We report about the project status and perspectives. In particular, we present a systematic study of the THGEM response as a function of geometrical parameters, production techniques and the gas mixture composition. The first figures obtained from measuring the response of a CsI coated THGEM to single photons are presented

Development of THGEM-based photon detectors for Cherenkov Imaging Counters RID D-9840-2011

The development of a large size gaseous detector of single photons, able to stably operate at high gain and high rate, and to provide good time resolution and insensitivity to magnetic field would be beneficial to future Cherenkov Imaging Counters. The detector could be based on the use of a multilayer architecture of THGEM electron multipliers coupled to a solid state CsI photocathode. A systematic study of the response of THGEM-based counters versus the geometrical parameters has been performed and the main results will be presented. Small photon detector prototypes have been built and preliminary data obtained detecting single photoelectrons are presented as well. The key aspect of photoelectron extraction from the photocathode is illustrated presenting both simulation and dedicated measurement results

Development of THGEM-based photon detectors for Cherenkov Imaging Counters

The development of a large size gaseous detector of single photons, able to stably operate at high gain and high rate, and to provide good time resolution and insensitivity to magnetic field would be beneficial to future Cherenkov Imaging Counters. The detector could be based on the use of a multilayer architecture of THGEM electron multipliers coupled to a solid state CsI photocathode. A systematic study of the response of THGEM-based counters versus the geometrical parameters has been performed and the main results will be presented. Small photon detector prototypes have been built and preliminary data obtained detecting single photoelectrons are presented as well. The key aspect of photoelectron extraction from the photocathode is illustrated presenting both simulation and dedicated measurement results

Neurodevelopmental and Epilepsy Phenotypes in Individuals With Missense Variants in the Voltage Sensing and Pore Domain of KCNH5

OBJECTIVE: KCNH5 encodes the voltage-gated potassium channel EAG2/Kv10.2. We aimed to delineate the neurodevelopmental and epilepsy phenotypic spectrum associated with de novo KCNH5 variants. METHODS: We screened 893 individuals with developmental and epileptic encephalopathies (DEEs) for KCNH5 variants using targeted or exome sequencing. Additional individuals with KCNH5 variants were identified through an international collaboration. Clinical history, EEG, and imaging data were analyzed; seizure types and epilepsy syndromes were classified. We included three previously published individuals including additional phenotypic details. RESULTS: We report a cohort of 17 patients, including nine with a recurrent de novo missense variant p.Arg327His, four with a recurrent missense variant p.Arg333His, and four additional novel missense variants. All variants were located in or near the functionally critical voltage-sensing or pore domains, absent in the general population, and classified as pathogenic or likely pathogenic using American College of Medical Genetics and Genomics (ACMG) criteria. All individuals presented with epilepsy with a median seizure onset at six months. They had a wide range of seizure types, including focal and generalized seizures. Cognitive outcomes ranged from normal intellect to profound impairment. Individuals with the recurrent p.Arg333His variant had a self-limited drug-responsive focal or generalized epilepsy and normal intellect, while the recurrent p.Arg327His variant was associated with infantile-onset DEE. Two individuals with variants in the pore-domain were more severely affected, with a neonatal-onset movement disorder, early-infantile DEE, profound disability, and childhood death. CONCLUSIONS: We report the first cohort of 17 individuals with pathogenic or likely pathogenic missense variants in the voltage sensing and pore domains of Kv10.2, including 14 previously unreported individuals. We present evidence for a putative emerging genotype-phenotype correlation with a spectrum of epilepsy and cognitive outcomes. Overall, we expand the role of EAG proteins in human disease and establish KCNH5 as implicated in a spectrum of neurodevelopmental disorders (NDDs) and epilepsy.This article is freely available online. Click on the 'Additional Link' above to access the full-text via the publisher's site.

Histopathological findings in brain tissue obtained during epilepsy surgery

Item does not contain fulltextBackground: Detailed neuropathological information on the structural brain lesions underlying seizures is valuable for understanding drug-resistant focal epilepsy. Methods: We report the diagnoses made on the basis of resected brain specimens from 9523 patients who underwent epilepsy surgery for drug-resistant seizures in 36 centers from 12 European countries over 25 years. Histopathological diagnoses were determined through examination of the specimens in local hospitals (41%) or at the German Neuropathology Reference Center for Epilepsy Surgery (59%). Results: The onset of seizures occurred before 18 years of age in 75.9% of patients overall, and 72.5% of the patients underwent surgery as adults. The mean duration of epilepsy before surgical resection was 20.1 years among adults and 5.3 years among children. The temporal lobe was involved in 71.9% of operations. There were 36 histopathological diagnoses in seven major disease categories. The most common categories were hippocampal sclerosis, found in 36.4% of the patients (88.7% of cases were in adults), tumors (mainly ganglioglioma) in 23.6%, and malformations of cortical development in 19.8% (focal cortical dysplasia was the most common type, 52.7% of cases of which were in children). No histopathological diagnosis could be established for 7.7% of the patients. Conclusions: In patients with drug-resistant focal epilepsy requiring surgery, hippocampal sclerosis was the most common histopathological diagnosis among adults, and focal cortical dysplasia was the most common diagnosis among children. Tumors were the second most common lesion in both groups. (Funded by the European Union and others.)9 p