98 research outputs found

    Accuracy of the activation energy calculated from a thermoluminescence glow‐peak using a method that uses three points on the peak

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    The utility of thermoluminescence (TL) in detecting changes in defect concentration in insulators is well established [1–3]. The underlying premise leading to the emission of TL is that exposure of a material to ionizing radiation causes a redistribution of charge in defect centres within the material. When the material is heated at a controlled linear rate, the thermoluminescence is emitted as a temperature-dependent set of peaks collectively known as a glow-curve. The shape and intensity of each of the glow-peaks may be characterized by a set of parameters consisting of the activation energy E, the frequency factors, the number of electrons n0 trapped in defect centres at the start of the heating, and the order of kinetics b. The order of kinetics b is an indication of the retrapping probability i.e. the probability that a free electron from the conduction band will be retrapped rather than recombine with a hole at a recombination centre to produce thermoluminescence. Retrapping of electrons reduces the TL intensity at any particular temperature during the heating process. The physical mechanisms of TL associated with a given glow curve are unique and may be characterized by analysis of the glow-curve for the said kinetic parameters

    Parental experiences and perspectives on the value of seizure detection while caring for a child with epilepsy: a qualitative study

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    Introduction: Caring for a child with epilepsy has a significant impact on parental quality of life. Seizure unpredictability and complications, including sudden unexpected death in epilepsy (SUDEP), may cause high parental stress and increased anxiety. Nocturnal supervision with seizure detection devices may lower SUDEP risk and decrease parental burden of seizure monitoring, but little is known about their added value in family homes. Methods: We conducted semi-structured in-depth interviews with parents of children with refractory epilepsy participating in the PROMISE trial (NCT03909984) to explore the value of seizure detection in the daily care of their child. Children were aged 4-16 years, treated at a tertiary epilepsy center, had at least one nocturnal major motor seizure per week, and used a wearable seizure detection device (NightWatch) for two months at home. Data were analyzed using inductive thematic analysis. Results: Twenty three parents of nineteen children with refractory epilepsy were interviewed. All parents expressed their fear of missing a large seizure and the possible consequences of not intervening in time. Some parents felt the threat of child loss during every seizure, while others thought about it from time to time. The fear could fluctuate over time, mainly associated with fluctuations of seizure frequency. Most parents described how they developed a protective behavior, driven by this fear. The way parents handled the care of their child and experienced the burden of care influenced their perceptions on the added value of NightWatch. The experienced value of NightWatch depended on the amount of assurance it could offer to reduce their fear and the associated protective behavior as well as their resilience to handle the potential extra burden of care, due to false alarms or technical problems. Conclusion: Healthcare professionals and device companies should be aware of parental protective behavior and the high parental burden of care and develop tailored strategies to optimize seizure detection device care. (c) 2021 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY license (http:// creativecommons.org/licenses/by/4.0/).Paroxysmal Cerebral Disorder

    Validation of the Japanese Society for Surgery of the Hand version of the Carpal Tunnel Syndrome Instrument

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    AbstractBackgroundThe Carpal Tunnel Syndrome Instrument (CTSI) is a disease-specific, self-administered questionnaire that consists of a symptom severity scale (SS) and a functional status scale (FS). The CTSI was cross-culturally adapted and developed by the Impairment Evaluation Committee, Japanese Society for Surgery of the Hand (JSSH). The purpose of this study was to test the reliability, validity, and responsiveness of the Japanese version of the CTSI (CTSI-JSSH).MethodsA consecutive series of 87 patients with carpal tunnel syndrome completed the CTSI-JSSH, the JSSH version of the Disability of the Arm, Shoulder, and Hand questionnaire (DASH-JSSH), and the 36-Item Short-Form Health Survey (SF-36). Seventy-two of the patients were reassessed for test–retest reliability 1 or 2 weeks later. Reliability was investigated by the reproducibility and the internal consistency. To analyze the validity, a factor analysis (principal axis factoring) of the CTSI-JSSH and the correlation coefficients between the CTSI-JSSH and DASH-JSSH were obtained. The responsiveness was examined by calculating the standardized response mean (SRM; mean change/SD) and effect size (mean change/SD of baseline value) after carpal tunnel release in 42 patients.ResultsCronbach’s alpha coefficients for the CTSI-JSSH-SS and the CTSI-JSSH-FS were 0.84 and 0.90, respectively, and the intraclass correlation coefficients were 0.82 and 0.83, respectively. The unidimensionality of the CTSI-JSSH-SS was barely confirmed; the unidimensionality of the CTSI-JSSH-FS was confirmed. The correlation coefficients between the CTSI-JSSH-FS and the CTSI-JSSH-SS or DASH-JSSH were 0.58 and 0.80, respectively. The correlation coefficient between the CTSI-JSSH-SS and DASH-JSSH was 0.54. The correlation coefficients between the subscales of SF-36 and the CTSI-JSSH-SS or the CTSI-JSSH-FS ranged from -0.23 to -0.66 and from -0.19 to -0.63, respectively. The SRMs/effect sizes of the CTSI-JSSH-SS and the CTSI-JSSH-FS were -0.85/-0.99 and -0.70/-0.61, which indicated that they were more than moderately sensitive.ConclusionsThe CTSI-JSSH has sufficient reliability, validity, and responsiveness to assess the health status in carpal tunnel syndrome

    Parental experiences and perspectives on the value of seizure detection while caring for a child with epilepsy: A qualitative study

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    INTRODUCTION: Caring for a child with epilepsy has a significant impact on parental quality of life. Seizure unpredictability and complications, including sudden unexpected death in epilepsy (SUDEP), may cause high parental stress and increased anxiety. Nocturnal supervision with seizure detection devices may lower SUDEP risk and decrease parental burden of seizure monitoring, but little is known about their added value in family homes. METHODS: We conducted semi-structured in-depth interviews with parents of children with refractory epilepsy participating in the PROMISE trial (NCT03909984) to explore the value of seizure detection in the daily care of their child. Children were aged 4-16 years, treated at a tertiary epilepsy center, had at least one nocturnal major motor seizure per week, and used a wearable seizure detection device (NightWatch) for two months at home. Data were analyzed using inductive thematic analysis. RESULTS: Twenty three parents of nineteen children with refractory epilepsy were interviewed. All parents expressed their fear of missing a large seizure and the possible consequences of not intervening in time. Some parents felt the threat of child loss during every seizure, while others thought about it from time to time. The fear could fluctuate over time, mainly associated with fluctuations of seizure frequency. Most parents described how they developed a protective behavior, driven by this fear. The way parents handled the care of their child and experienced the burden of care influenced their perceptions on the added value of NightWatch. The experienced value of NightWatch depended on the amount of assurance it could offer to reduce their fear and the associated protective behavior as well as their resilience to handle the potential extra burden of care, due to false alarms or technical problems. CONCLUSION: Healthcare professionals and device companies should be aware of parental protective behavior and the high parental burden of care and develop tailored strategies to optimize seizure detection device care

    Functional abilities in children and adults with the CDKL5 disorder

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    Functional abilities in the CDKL5 disorder have been described as severely impaired, yet some individuals are able to run and use phrases for speech. Our study investigated gross motor, hand function, and expressive communication abilities in individuals with the CDKL5 disorder. Data for 108 females and 16 males registered with the International CDKL5 disorder database and with a pathogenic CDKL5 mutation were analyzed. Relationships between functional abilities, age, genotype, and gender were analyzed using regression models. Over half of the females could sit on the floor and nearly a quarter could walk 10 steps. Fewer males could complete these tasks although one boy was able to sit, walk, and run. Most females and few males were able to pick up a large object. Females mostly used gestures to communicate while males mostly used other forms of non-verbal communication. Compared to those with no functional CDKL5 protein, individuals with truncating variants after aa 781 were more likely to be able to stand (OR 5.7, 95%CI 1.2, 26.6) or walk independently (4.3, 95%CI 0.9, 20.5), and use more advanced communication methods such as words (OR 6.1, 95%CI 1.5–24.2). Although abilities were markedly impaired for the majority with the CDKL5 disorder, some females and a few males had better functional abilities. This variability may be related to underlying gene variants, with females with a late truncating variant having better levels of ability than those with no functional protein

    Prevalence and onset of comorbidities in the CDKL5 disorder differ from Rett syndrome

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    Background: Initially described as an early onset seizure variant of Rett syndrome, the CDKL5 disorder is now considered as an independent entity. However, little is currently known about the full spectrum of comorbidities that affect these patients and available literature is limited to small case series. This study aimed to use a large international sample to examine the prevalence in this disorder of comorbidities of epilepsy, gastrointestinal problems including feeding difficulties, sleep and respiratory problems and scoliosis and their relationships with age and genotype. Prevalence and onset were also compared with those occurring in Rett syndrome. Methods: Data for the CDKL5 disorder and Rett syndrome were sourced from the International CDKL5 Disorder Database (ICDD), InterRett and the Australian Rett syndrome Database (ARSD). Logistic regression (multivariate and univariate) was used to analyse the relationships between age group, mutation type and the prevalence of various comorbidities. Binary longitudinal data from the ARSD and the equivalent cross-sectional data from ICDD were examined using generalized linear models with generalized estimating equations. The Kaplan-Meier method was used to estimate the failure function for the two disorders and the log-rank test was used to compare the two functions. Results: The likelihood of experiencing epilepsy, GI problems, respiratory problems, and scoliosis in the CDKL5 disorder increased with age and males were more vulnerable to respiratory and sleep problems than females. We did not identify any statistically significant relationships between mutation group and prevalence of comorbidities. Epilepsy, GI problems and sleep abnormalities were more common in the CDKL5 disorder than in Rett syndrome whilst scoliosis and respiratory problems were less prevalent. Conclusion: This study captured a much clearer picture of the CDKL5 disorder than previously possible using the largest sample available to date. There were differences in the presentation of clinical features occurring in the CDKL5 disorder and in Rett syndrome, reinforcing the concept that CDKL5 is an independent disorder with its own distinctive characteristics

    Suprasellar cysts: clinical presentation, surgical indications, and optimal surgical treatment

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    <p>Abstract</p> <p>Background</p> <p>To describe the clinical presentation of suprasellar cysts (SSCs) and surgical indications, and compare the treatment methods of endoscopic ventriculocystostomy (VC) and ventriculocystocisternotomy (VCC).</p> <p>Methods</p> <p>We retrospectively reviewed the records of 73 consecutive patients with SSC who were treated between June 2002 and September 2009. Twenty-two patients were treated with VC and 51 with VCC. Outcome was assessed by clinical examination and magnetic resonance imaging.</p> <p>Results</p> <p>The patients were divided into five groups based on age at presentation: age less than 1 year (n = 6), 1-5 years (n = 36), 6-10 years (n = 15), 11-20 years (n = 11), and 21-53 years (n = 5). The main clinical presentations were macrocrania (100%), motor deficits (50%), and gaze disturbance (33.3%) in the age less than 1 year group; macrocrania (75%), motor deficits (63.9%), and gaze disturbance (27.8%) in the 1-5 years group; macrocrania (46.7%), symptoms of raised intracranial pressure (ICP) (40.0%), endocrine dysfunction (40%), and seizures (33.3%) in the 6-10 years group; symptoms of raised ICP (54.5%), endocrine dysfunction (54.5%), and reduced visual field or acuity (36.4%) in the 11-20 years group; and symptoms of raised ICP (80.0%) and reduced visual field or acuity (40.0%) in the 21-53 years group. The overall success rate of endoscopic fenestration was 90.4%. A Kaplan-Meier curve for long-term efficacy of the two treatment modalities showed better results for VCC than for VC (p = 0.008).</p> <p>Conclusions</p> <p>Different age groups with SSCs have different main clinical presentations. VCC appears to be more efficacious than VC.</p

    The effect of local corticosteroid injection on F-wave conduction velocity and sympathetic skin response in carpal tunnel syndrome

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    The aim of this study was to evaluate the efficacy of steroid injection for the treatment of the carpal tunnel syndrome (CTS), with F-wave parameters and sympathetic skin response (SSR). Seventeen hands of 10 women patients were treated with local steroid injection with 2-month follow-up. All patients underwent single injection into the carpal tunnel. Response to injection was measured nerve conduction studies (NCSs), median nerve F waves, and SSR before and after treatment. To determine the normal values, 42 hands of 21 healthy women were also studied. There was a significant improvement of sensory and motor nerve conduction values when compared to baseline values (P < 0.01). At the end of follow-up period, the median sensory distal latency and the sensory latency differences between the median and the ulnar nerve were improved 35 and 65%, respectively. The maximum, mean F-wave amplitudes and chronodispersion showed a slight improvement with respect to baseline values and controls, but statistical significance was not achieved after treatment. Although no statistically significant improvements were observed in SSR parameters, slightly decreased amplitudes and increased habituation of SSR were noted at the end of the treatment. The present study shows that the local steroid injection results in improvement in NCSs values, but the F-wave parameters were not effectual in short-term outcome of CTS treatment. These findings suggest that the sensory latency differences between the median and the ulnar wrist-to-digit 4 are better parameters in the median nerve recovery after treatment than the median sensory distal latency. Furthermore, the SSR does not seem to be a sensitive method in follow-up of CTS treatment
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