Outcomes of children and adolescents 1 year after being seen in a multidisciplinary psychogenic nonepileptic seizures clinic.

OBJECTIVE: Psychogenic nonepileptic seizures (PNES) are paroxysmal events that may involve altered subjective experience and change in motor activity with a psychological cause. The aim of this work is to describe a population of pediatric patients with PNES and identify factors predictive of 12-month outcomes. METHODS: We conducted a prospective observational study of children and adolescents referred to the multidisciplinary Nationwide Children's Hospital PNES clinic between November 2017 and July 2019. Information was collected from patients during clinic visits and semistructured follow-up phone calls. Descriptive statistics and Fisher exact test were used for analysis. RESULTS: Of the 139 consecutive patients referred to the PNES clinic, 104 were seen in clinic and 63 answered 12-month follow-up calls. Patients with comorbid epilepsy had increased rates of participation at 12-month follow-up (p = .04). Complete remission was achieved by 32% (20/63) of patients at 12 months. Combined PNES remission and improvement was 89% (56/63) at 12 months. Patients and families who were linked with counseling at 1 month were more likely to achieve remission at 12 months (p = .005). Less than half (44%, 28/63) of patients reached at 12 months had their events documented on video-electroencephalogram (EEG) at diagnosis; however, those who did were not more likely to be accepting of the diagnosis at 12 months (p = 1.0), be linked with counseling at 12 months (p = .59), or be event-free at 12 months (p = .79). SIGNIFICANCE: Remission occurred in one third of patients by 12 months; however, improvement in events was seen in 89%. Connection to counseling by 1 month was associated with increased remission rates at 12 months. Capturing events on video-EEG was not associated with increased acceptance or event freedom at 12 months. Diagnosis should be followed by strong encouragement to connect with counseling quickly to achieve a goal of increasing 12-month PNES remission rates.

Improving time for administration of second-line antiseizure medications for children with generalized convulsive status epilepticus using quality improvement methodology.

OBJECTIVE: Status epilepticus is a life-threatening neurological emergency. However, delay in median time to administration of second-line antiseizure medication exists. The aim of this quality improvement initiative was to decrease the average delay before fosphenytoin is administered for pediatric patients with generalized convulsive status epilepticus from 30 min (baseline data collected in 2013) to 15 min (50% reduction) by December 2015 and sustain this for 1 year. METHODS: Our team conducted an analysis of baseline data for patients with continuous generalized convulsive status epilepticus who received fosphenytoin after receiving first-line benzodiazepine treatment. Using quality improvement methodology, areas for improvement were identified and specific interventions developed and implemented. A timeline of 15 min to initiate fosphenytoin administration after failure of first-line treatment was considered reasonable and achievable as a project aim. RESULTS: A total of 199 patients were included in the dataset for the project. The database included patients aged 1 month and older. Ninety-eight percent of patients were between 1 month and 19 years of age. The gender distribution was even, with 54% of patients being White or Caucasian, 30% African American or Black, and 16% classified as "other." From January 2014 through December 2019, the average time before initiating fosphenytoin administration after failure of benzodiazepine therapy, for patients with generalized convulsive status epilepticus, decreased from 30 min (SD = 45.7) to 11.4 min (SD = 8.2, p = .043), thus reducing time to administration by 62%. SIGNIFICANCE: Quality improvement methodology can be successfully applied to decrease administration time between first- and second-line antiseizure medications for status epilepticus.

Short-term outcomes in pediatric and adolescent patients with psychogenic nonepileptic events seen by telemedicine during the COVID-19 pandemic.

INTRODUCTION: Psychogenic nonepileptic events (PNEE) are a type of Functional Neurological Symptom Disorder that present with events that appear epileptic but are not associated with abnormal electrical activity in the brain. In response to the global COVID-19 pandemic, our PNEE clinic switched to a telemedicine format, and we present here our experience with providing care to children and adolescents with PNEE in this format. METHODS: The multidisciplinary clinic shifted to a telemedicine platform in March 2020 with the same joint provider format. Follow-up phone calls are completed at one and three months following the visit. Data are presented with descriptive statistics. Referral volume and outcomes data are compared to historical patients, including rates of diagnosis acceptance, linkage to counseling, and change in event frequency. RESULTS: Twenty-three patients were scheduled to be seen via telemedicine or hybrid visits from March through June, twenty completed their visits. Sixteen (70%) were reached for follow-up at one month. Of those reached, twelve (75%) accepted the diagnosis, eight (50%) were linked with counseling, and fourteen (88%) with improvement in event frequency. Of the sixteen reached at three months, eleven (69%) had accepted the diagnosis, ten (63%) were linked with counseling, and all but two reported improvement in event frequency. In comparison, the previously published results showed 3-month rates of 75% of patients accepting the diagnosis, 76% linked with counseling, and 75% with improvement in event frequency. CONCLUSIONS: Video telemedicine visits are a feasible and effective way to provide care for children and adolescents with PNEE. At 3 months, patients seen by telemedicine had similar acceptance rates, decreased connection to counseling, and increased rate of improvement in event frequency. This study suggests telemedicine may have some benefits over traditional clinic visits, such as improved show rates and access to clinic; so should be considered a reasonable alternative to in-person visits.

A hospital-based study on caregiver preferences on acute seizure rescue medications in pediatric patients with epilepsy: Intranasal midazolam versus rectal diazepam.

RATIONALE: About 20 per 100,000 children have convulsive status epilepticus every year, a life-threatening condition. Benzodiazepines are the first-line treatment for prolonged and recurrent seizures. Our study was designed to gain understanding of caregiver perception of acute seizure treatments. METHODS: Our project uses a cross-sectional survey study design using the electronic medical record and a survey at a large academic tertiary children's medical center. Subjects were patients with epilepsy prescribed intranasal (IN) midazolam and/or per rectum (PR) diazepam. The survey was administered to caregivers of children with epilepsy regarding information on the comfort, efficacy, ease of use, and time of administration for patients receiving both abortive seizure medications. Exact binomial tests were employed to determine whether or not differences in caregiver preference exist. RESULTS: One hundred and sixty responses were obtained. Incomplete and duplicate surveys were excluded, leaving 153 responses. Of those responses, 59 respondents reported administering both medications. Among parents who expressed a preference for one medication over the other, more parents felt overall greater comfort with IN midazolam compared with rectal diazepam (p = 0.0004 and p = 0.001), IN midazolam was perceived as easier to use (68%, p = 0.0038 and 74%, p = 0.0004) and more effective (87%, p < 0.0001) than rectal diazepam. Intranasal midazolam was found to be superior to rectal diazepam in several other categories as well. CONCLUSIONS: These parents of children with epilepsy report increased ease of use, comfort, and efficacy with IN midazolam as compared with rectal diazepam suggesting that a readily available form of IN midazolam would be well received in the pediatric population.

Comparison of semiologies between tilt-induced psychogenic nonsyncopal collapse and psychogenic nonepileptic seizures.

We sought to characterize the clinical features of tilt-induced psychogenic nonsyncopal collapse (PNSC) from a cohort of young patients and to compare the semiologies between PNSC and EEG-confirmed psychogenic nonepileptic seizures (PNES). A PNSC diagnosis was made if a clinical event occurred during tilt-table testing that the patient regarded as fainting, but neither hypotension nor EEG changes were present. A diagnosis of PNSC was made in 17.6% of all patients referred during the 15-month study period. Cohorts with psychogenic nonsyncopal collapse (n=40) and PNES (n=40) did not differ in age (15.5±2.2 versus 14.6±2.7, p=.11) or female gender (80% versus 72.5%, p=.43). Psychogenic nonsyncopal collapse events were briefer than PNES events (median: 45 versus 201.5s, p<.001). Negative motor signs (head drop, body limpness) predominated in PNSC (85% versus 20%, p<.001), while the positive motor signs of convulsion occurred more often with PNES (90% versus 30%, p<.001). Behavioral arrest (25% versus 32.5%, p=.46) and eye closure (85% versus 72.5%, p=.21) did not differ between PNSC and PNES. Patients with PNSC were more likely to be tearful before (30% versus 7.5%, p=.02) and after (62.5% versus 7.5%, p<.001) an event. In conclusion, although overlap exists, the features of PNSC generally appear similar to neurally mediated syncope, while the features of PNES generally appear similar to epileptic seizures. Psychogenic nonsyncopal collapse and PNES likely represent similar disorders that differ primarily by clinical semiologies and referral patterns.

Fc gamma receptor IIa-H131R polymorphism and malaria susceptibility in sympatric ethnic groups, Fulani and Dogon of Mali.

It has been previously shown that there are some interethnic differences in susceptibility to malaria between two sympatric ethnic groups of Mali, the Fulani and the Dogon. The lower susceptibility to Plasmodium falciparum malaria seen in the Fulani has not been fully explained by genetic polymorphisms previously known to be associated with malaria resistance, including haemoglobin S (HbS), haemoglobin C (HbC), alpha-thalassaemia and glucose-6-phosphate dehydrogenase (G6PD) deficiency. Given the observed differences in the distribution of FcγRIIa allotypes among different ethnic groups and with malaria susceptibility that have been reported, we analysed the rs1801274-R131H polymorphism in the FcγRIIa gene in a study of Dogon and Fulani in Mali (n = 939). We confirm that the Fulani have less parasite densities, less parasite prevalence, more spleen enlargement and higher levels of total IgG antibodies (anti-CSP, anti-AMA1, anti-MSP1 and anti-MSP2) and more total IgE (P < 0.05) compared with the Dogon ethnic group. Furthermore, the Fulani exhibit higher frequencies of the blood group O (56.5%) compared with the Dogon (43.5%) (P < 0.001). With regard to the FcγRIIa polymorphism and allele frequency, the Fulani group have a higher frequency of the H allele (Fulani 0.474, Dogon 0.341, P < 0.0001), which was associated with greater total IgE production (P = 0.004). Our findings show that the FcγRIIa polymorphism might have an implication in the relative protection seen in the Fulani tribe, with confirmatory studies required in other malaria endemic settings.

Change patterns of homeless individuals with mental illness: a multiple case study.

This multiple case study illuminates the individual change trajectories of four homeless men with mental illnesses who participated in a manualized life skills intervention to improve housing retention. Readiness-to-change, life skills knowledge and trauma symptoms were measured at baseline, post-intervention and at 3-6 months follow-up. Cluster analysis identified two patterns of readiness-to-change: engaged and pre-engaged. Change is non-linear and baseline readiness is not necessary to benefit from the intervention. Examining individuals' lives in context illuminated the change process and demonstrated that varied patterns can lead to successful outcomes for housing stability and community reintegration.

Multidisciplinary Care May Help to Reduce Disparities Associated With Race in Youth With Psychogenic Nonepileptic Seizures.

BACKGROUND: Psychogenic nonepileptic seizures (PNES) are a common type of functional neurological disorder in which patients experience seizurelike episodes. Health disparities based on race and socioeconomics, documented in children with epilepsy and adults with PNES, have not been reported in children and adolescents with PNES. We hypothesize that disparities exist in this population, which impact overall care and therefore influence outcomes. METHODS: We retrospectively analyzed youth referred to our multidisciplinary clinic from 2018 to 2020. All patient charts were screened by social work before the visit to identify potential barriers to care, and a nurse conducted follow-up calls. Patients' race was identified from the electronic health record and compared with several variables. Outcomes were collected via phone follow-up. Descriptive statistics were produced, and comparisons between white patients and patients of other races were completed using Fisher exact tests and multivariable logistic regressions. RESULTS: During the study period, 237 patients were eligible for the analysis. Sixty-eight patients (29%) identified as a race other than white. Only 60%, 56%, and 40% of the cohort were reached for follow-up at one, three, and 12 months, respectively. In general, outcomes were similar between racial groups; however, we found that patients of nonwhite race were more likely to receive support from social work due to barriers identified in screening (P = 0.045). CONCLUSIONS: Health disparities based on race may exist in youth with PNES. A multidisciplinary clinic including social work may help mitigate barriers leading to more equitable care and similar outcomes for white and nonwhite youth with PNES.

Increasing Provider Utilization of a Seizure Action Plan in the Outpatient Setting.

OBJECTIVES: A seizure action plan (SAP) is a powerful tool that provides actionable information for caregivers during seizures. Guidelines have expressed the need for individualized SAPs. Our quality improvement team aimed to increase implementation of an SAP within a pediatric tertiary center, initially among epilepsy providers and expanded to all neurology providers. METHODS: Process changes were implemented using Plan-Do-Study-Act cycles and data were evaluated monthly using control charts. The team focused on tracking patients who received SAPs and identified opportunities for improvement, including reminders within the electronic medical record, and standardizing clinic processes. A secondary analysis was performed to trend emergency department (ED) use among our patient population. RESULTS: The SAP utilization rate among epilepsy providers increased from a baseline of 39% to 78% by December 2019 and reached the goal of 85% by June 2020, with a further increase to 92% by February 2022 and maintained. The SAP utilization rate among general neurology providers increased from 43% in 2018 to 85% by July 2020, and further increased to 93% by February 2022 and maintained. ED visits of established patients with epilepsy decreased from a baseline of 10.2 per 1000 to 7.5 per 1000. CONCLUSIONS: Quality improvement methodologies increased the utilization of a standardized SAP within neurology outpatient care centers. The SAP is a simplified tool that allows patients and providers to navigate a complex health care system. The utility of an SAP may potentially extend to minimizing unnecessary ED visits.

RNA methyltransferase SPOUT1/CENP-32 links mitotic spindle organization with the neurodevelopmental disorder SpADMiSS.

SPOUT1/CENP-32 encodes a putative SPOUT RNA methyltransferase previously identified as a mitotic chromosome associated protein. SPOUT1/CENP-32 depletion leads to centrosome detachment from the spindle poles and chromosome misalignment. Aided by gene matching platforms, we identified 24 individuals with neurodevelopmental delays from 18 families with bi-allelic variants in SPOUT1/CENP-32 detected by exome/genome sequencing. Zebrafish spout1/cenp-32 mutants showed reduction in larval head size with concomitant apoptosis likely associated with altered cell cycle progression. In vivo complementation assays in zebrafish indicated that SPOUT1/CENP-32 missense variants identified in humans are pathogenic. Crystal structure analysis of SPOUT1/CENP-32 revealed that most disease-associated missense variants mapped to the catalytic domain. Additionally, SPOUT1/CENP-32 recurrent missense variants had reduced methyltransferase activity in vitro and compromised centrosome tethering to the spindle poles in human cells. Thus, SPOUT1/CENP-32 pathogenic variants cause an autosomal recessive neurodevelopmental disorder: SpADMiSS ( SPOUT1 Associated Development delay Microcephaly Seizures Short stature) underpinned by mitotic spindle organization defects and consequent chromosome segregation errors.

Mental health counseling is rated as most helpful by autistic adults: Service perspectives in adulthood.

LAY ABSTRACT: The number of autistic adults is growing, but there are fewer services to support them in adulthood. Many autistic adults need some support services to lead successful adult lives. We know a lot about the services autistic adults use and some of the problems with using these services, but we do not know which services are most helpful to them and how the services they use relate to how they interact with their communities. Forty autistic adults took part in a study about service use and community participation. They completed surveys, interviews, and carried a global positioning system tracker. They answered questions about which services are most helpful in adulthood, things that make it hard to use services, and what services they needed. Most participants used two services in the past 2 years, most frequently mental health and employment services. Adults who were currently seeing a mental health counselor were more likely to be working full-time and visit more locations in the community compared to those who were not seeing a counselor. Mental health services were reported as the most helpful service they received as adults, followed by employment services. We often focus on the importance of employment services after high school, but our findings show a need for both mental health and employment services for autistic adults.

Beyond Friendship: The Spectrum of Social Participation of Autistic Adults.

Difficulties with social interactions and communication that characterize autism persist in adulthood. While social participation in adulthood is often marked by social isolation and limited close friendships, this qualitative study describes the range of social participation activities and community contacts, from acquaintances to close relationships, that contributed to connection from the perspective of 40 autistic adults. Qualitative data from interviews around social and community involvement were analyzed and revealed five main contexts where social participation occurred: vocational contexts, neighborhoods, common interest groups, support services and inclusive environments, and online networks and apps. Implications for practice to support a range of social participation include engaging in newer social networking avenues, as well as traditional paths through employment and support services.

The Spike-Wave Index of the First 100 Seconds of Sleep Can Be a Reliable Scoring Method for Electrographic Status Epilepticus in Sleep.

INTRODUCTION: Electrical status epilepticus in sleep (ESES) is an electrographic pattern in which interictal epileptiform activity is augmented by the transition to sleep, with non-rapid eye movement sleep state characterized by near-continuous lateralized or bilateral epileptiform discharges. The aim of this study was to measure the reliability of the spike-wave index (SWI) of the first 100 seconds of sleep as a tool for the diagnosis of ESES. METHODS: One hundred forty studies from 60 unique patients met the inclusion. Two neurophysiologists calculated the SWI of the first 100 seconds of spontaneous stage II non-rapid eye movement sleep. This was compared with the SWI of the first 5 minutes of non-rapid eye movement sleep and the cumulative SWI of three 5-minute bins of sleep. Agreement between the three SWI methods were analyzed using several statistical tools and methods. RESULTS: Using an SWI of 50% as a diagnostic cutoff, 57% of records had a diagnosis of ESES based on the first 100 seconds of sleep. Fifty-four percent of records had a diagnosis of ESES based on the method of using the SWI of three bins. This resulted in a diagnostic accuracy of 92%, sensitivity of 96%, and specificity of 88%. Positive predictive values of children diagnosed with ESES using the first 100 seconds of sleep, compared with 3 combined bins, was determined to be 90% and a negative predictive value was determined to be 95%. CONCLUSIONS: This analysis confirmed the diagnostic accuracy of using the SWI of the first 100 seconds of sleep and the cumulative total of three 5-minute bins.

Brief Report: Psychogenic Nonepileptic Events in Pediatric Patients with Autism or Intellectual Disability.

This is a retrospective case series of pediatric patients referred to the psychogenic nonepileptic events clinic (PNEE) who had comorbid diagnoses of autism spectrum disorder (ASD) or intellectual disability (ID). We describe 15 patients, nine with ASD and six with ID who had a telephone visit follow-up at 12 months. There were higher rates of male gender (40%) and comorbid epilepsy (53%) compared to the larger PNEE cohort. Eleven patients were available for follow-up and ten patients had improvement in events or were event-free. We report that patients with ASD or ID can develop PNEE and experience improvement from events.

Multidisciplinary Care for Pediatric Patients With Psychogenic Nonepileptic Seizures May Be Associated With Decreased Health Care Utilization.

Background and Objectives: Psychogenic nonepileptic seizures (PNES) are paroxysmal events that resemble epileptic seizures but have psychological underpinnings. Patients with PNES have high health care utilization. We hypothesize that appropriate care would lead to decreases in utilization. The aim of this study was to measure the impact of a multidisciplinary PNES clinic in reducing health care utilization (HCU) in youth with PNES referred to the clinic. Methods: We reviewed the frequency of visits to the emergency department (ED), urgent care (UC), planned and unplanned hospitalizations, neurodiagnostic studies, and total charges associated with these encounters 12 months before and 12 months after initial referral to the clinic. Manual chart review of all patients referred from November 2017 to March 2020 was performed. Results: Two hundred and twelve unique patients were included in this retrospective study. Patient sex was identified as 71% female, 28% male, and 1% other, with an average age of 14 years at diagnosis. These patients visited the ED a total of 410 times before being seen in the PNES clinic, which decreased to 187 visits after (75% reduction). All measures of HCU decreased in the 12 months after the visit, and statistically significant differences were observed in all measures of HCU except for head MRIs, leading to an estimated potential cost savings of $7,978,447. Discussion: Patients with PNES were found to have decreased health care utilization in the 12 months after referral to the Nationwide Children's Hospital multidisciplinary clinic, including significant decreases in emergency services and unnecessary diagnostic testing, in the 12 months after the referral.

Readiness-to-change cluster profiles among adults with mental illness who were homeless participating in a life skills intervention.

This longitudinal study examined differences in intervention outcomes based on readiness-to-change cluster profiles among 73 adults with a mental illness at risk for homelessness participating in a manualized life skills intervention. Intervention topics included money management, food management, safe community participation, and room- and self-care. Life skill knowledge and readiness-to-change, measured using the University of Rhode Island Change Assessment, was examined at baseline, post-intervention, and 3-6 months later. Two scoring patterns emerged for readiness-to-change at each time point: Pre-Engaged and Engaged. Participants who were Engaged at the time of assessment scored significantly better than Pre-Engaged on post-intervention life skill testing, however group identification changed over time. Baseline readiness-to-change did not predict future performance or attrition, and therefore may not provide accurate indication of client investment for future learning or participation. Further investigation is needed to determine what factors contribute to Engaged membership.

[Place of malaria among febrile illnesses in two ethnic tribes living in sympatry in Mali from 1998 to 2008]. / Place du paludisme dans les syndromes fébriles dans deux groupes ethniques vivant en sympatrie au Mali de 1998 à 2008.

In Africa, malaria is responsible for 25-40% of all outpatient visits and 20-50% of all hospitalizations. In malaria-endemic areas, individuals do not behave the same toward the outcome of clinical malaria. The aim of this study is to determine the prevalence of malaria in the locality among the different ethnic groups, evaluate the place of malaria among febrile illnesses, and assess the relationship between fever and parasite density of Plasmodium falciparum. Studies on susceptibility to malaria between the Fulani and Dogon groups in Mali were conducted in Mantéourou and the surrounding villages from 1998 to 2008. We carried out six cross-sectional studies during the malaria transmission and longitudinal surveys (July to December depending on the year) during the 10-year duration. In longitudinal studies, clinical data on malaria and other diseases frequently observed in the population were recorded. It appears from this work that malaria is the leading cause of febrile syndromes. We observed a significant reduction in malaria morbidity in the study population from 1998 to 2008. The pyrogenic threshold of parasitaemia was 1,000 parasites/mm(3) of blood in the Dogon and 5,000 parasites/mm(3) of blood in the Fulani.We have also found that high parasitical densities were not always associated with fever. Malaria morbidity was higher among the Dogon than in Fulani. The immunogenetic factors might account for this difference in susceptibility to malaria between Fulani and Dogon in the area under study. With regard to this study, it is important to take into account the ethnic origin of subjects when interpreting data of clinical and malarial vaccine trials.

Delivering the Diagnosis: A Practical Approach to a Patient With a Functional Neurological Disorder.

Functional Neurological Disorders are a common and debilitating group of diseases that have been the subject of stigma and confusion across medical history. It is well-documented that prognosis and even possible resolution of symptoms are linked to successful delivery of the diagnosis by the clinician, and correct understanding of diagnosis by the patient. In the following article, we delineate the nature of these disorders and provide an overview to assist providers successfully navigate the communication of these diagnoses to patients and families.

Tablet therapy as an assistive technology-based treatment for anxiety in pediatric oncology.

The painful interventions and invasive procedures associated with pediatric cancer treatment can result in anxiety. Anxiety can be reduced or better controlled through distraction and thought retraining. Although art therapy, non-electronic play therapy, music therapy, and traditional counseling are often used to alleviate stress and anxiety, new technology innovations are proving to be additional options to decrease stress and anxiety through distraction and attention shifting. Tablet-based interventions are emerging as an easily available and effective means of reducing stress and fear prior to operations, and have potential applications to reduce anxiety for patients before receiving chemotherapy, during time spent in hospital rooms, and while experiencing distressing physiological symptoms. This paper reviews the research on tablet therapy and discusses the application of assistive technologies in clinical oncology settings to reduce pediatric anxiety throughout the treatment process.