Journal of Child Neurology最新文献

Background: Children with cerebral palsy are considered to be a population at risk for the occurrence of sleep difficulties. However, existing literature has been limited by subjective measures of sleep and has failed to examine contributing factors. Methods: Forty-five youth with cerebral palsy participated. Both youth and caregivers completed sleep-related questionnaires, while youth completed daily actigraphy for objective sleep assessments. Results: Sleep patterns, including sleep duration, wake after sleep onset, and sleep efficiency, are generally aligned with existing sleep recommendations. However, the number of awakenings was significantly higher in youth with cerebral palsy compared to these recommendations. Most youth experienced poor sleep quality, and approximately a quarter experienced insomnia. Being a boy and having a preexisting mental health diagnosis was associated with poor sleep quality and greater insomnia symptoms. Conclusions: Most youth with cerebral palsy experience a range of sleep difficulties. This study provides new information on sleep patterns in youth with cerebral palsy, highlighting the importance of addressing sleep issues in this population to improve their well-being and ultimately limit the negative impacts on overall health and quality of life.

Background: Over recent years there has been a shift in clinical practice to support care delivery via telemedicine. This study aims to highlight the patient and provider experience of telemedicine over 2.5 years within a Canadian Pediatric Neurology clinic.

Method: A REDCap survey was sent to all patients/parents and providers with a telemedicine appointment between March 2020 and September 2022.

Results: Seven providers and 272 patients responded. Ninety-one percent of patients and 100% of providers were satisfied with telemedicine. Ninety percent of patients and 100% of providers found telemedicine more convenient. Eighty-seven percent of patients and 100% of providers were interested in future telemedicine appointments. Main challenges were with performing a physical examination and technological issues.

Conclusion: Our survey shows that the majority of patients and providers had highly positive experiences with telemedicine and were interested in continuing care via telemedicine. This study supports incorporating telemedicine into future pediatric neurology practice.

Objective: Few studies have explored the efficacy of laser interstitial thermal therapy in pediatric epilepsy surgery. This study aims to evaluate seizure-free outcomes in children and adolescents with mesial temporal lobe epilepsy who underwent laser interstitial thermal therapy.

Methods: This was a retrospective cohort study performed at a level 4 epilepsy center. All patients had comprehensive presurgical epilepsy evaluations with a consensus treatment decision made by a multidisciplinary team. Brain magnetic resonance imaging (MRI) data were used to determine lesional vs nonlesional groups. All laser interstitial thermal therapy procedures were performed using Visualase laser ablation systems by the neurosurgical team. Seizure-free outcomes were measured according to the Engel surgical outcome scale.

Results: This study included 19 patients (12 girls, 7 boys). Age of epilepsy onset ranged from 2 to 17 years (mean 9.9 years), and age at time of surgery ranged from 8 to 20 years (mean 15.1 years). Ten patients (52.5%) had signs of hippocampal sclerosis on MRI (lesional group), and 9 patients (47.5%) had a normal brain MRI (nonlesional group). Engel 1 score was achieved by 14 of 19 patients (73.5%): 9 of 10 patients (90%) in the lesional group and 5 of 9 patients (55.5%) in the nonlesional group. Younger age of seizure onset was a predictor of better postsurgical outcome, but no other outcome predictors could be established.

Conclusion: Laser interstitial thermal therapy is safe and effective for the treatment of drug-resistant mesial temporal lobe epilepsy in children, rendering more favorable seizure-free outcomes in pediatric patients with hippocampal atrophy than in those with nonlesional mesial temporal lobe epilepsy.

Purpose: Atypical teratoid/rhabdoid tumor (AT/RT) is a kind of central nervous system malignant tumor in children. In this study, we aimed to develop a practically clinical nomogram and risk grouping system to predict 1-year overall survival for patients with atypical teratoid/rhabdoid tumor.

Methods: The nomogram was constructed based on the pediatric tumor registry of Xinhua Hospital affiliated to Shanghai Jiaotong University School of Medicine. Fifty-four information-integrated patients with atypical teratoid/rhabdoid tumor were included from the database. Cox regression analyses were used to select independent prognostic factors. Based on the fitted multivariate Cox regression model, a nomogram of 1-year overall survival for atypical teratoid/rhabdoid tumor patients was generated. Moreover, the nomogram was validated by assessing its discrimination and calibration.

Results: In these patients, age at diagnosis, the extent of tumor resection, radiotherapy, and chemotherapy were included in the multivariate Cox regression model. Based on this multivariate Cox regression model, a nomogram of 1-year overall survival for atypical teratoid/rhabdoid tumor patients was generated. The nomogram had good discrimination (the concordance index was 0.781) and calibration curves showed no deviation from reference lines. Decision curve analysis demonstrated this nomogram was useful for clinical practice. The risk grouping system was built based on nomogram-derived risk scores, which could classify patients into 3 risk groups. Compared with the low-risk group, the risk of 1-year death was significantly higher in the intermediate-risk group (hazard ratio = 1.42, 95%, confidence intervals = 0.49-4.11) and high-risk group (hazard ratio = 9.78, 95% confidence intervals = 3.53-27.1).

Conclusion: A nomogram and risk grouping system were built to predict for the 1-year overall survival of atypical teratoid/rhabdoid tumor patients. The nomogram could facilitate a personalized prognostic evaluation for atypical teratoid/rhabdoid tumor patients and help medical practitioners make better treatment.

Although many reports have described the characteristics of Williams-Beuren syndrome, few specifically analyzed epilepsy in patients with Williams-Beuren syndrome. In this retrospective study, we map the prevalence, types, and prognosis of epileptic seizures in a large cohort of 589 patients with Williams-Beuren syndrome, as well as associations between deletions of the membrane-associated guanylate kinase inverted-2-gene (MAGI2 gene), which is associated with infantile spasms (IS), and epilepsy in patients with Williams-Beuren syndrome.Our findings indicate that the incidence of epilepsy in patients with Williams-Beuren syndrome is approximately 1.02% (6 in 589), and is thus not higher than the incidence in the general population (0.5%-1.0%). West syndrome emerged as the most common epileptic syndrome in patients with Williams-Beuren syndrome (4 of 6) and typically has a favorable prognosis. There was no genotype-phenotype correlation between MAGI2 deletions and West syndrome in this cohort.

Background: Given the centrality of school to the lives of the more than 450 000 US children living with epilepsy, their interface with schools related to medical and educational needs is critical. This qualitative study explores parental experiences with school systems for the care and education of their children with epilepsy. Methods: Two 90-minute focus groups were empaneled with a total of 11 caregivers of children with epilepsy. Facilitators, moderators, and recorders of each group performed semistructured interviews prompting discussion of families' experiences. Conversations were audio recorded, deidentified, transcribed, and coded. Results: Five central themes emerged: Lack of School Resources (46), Lack of School Support (36), Customized Educational Support Plans (32), Academic Impacts (24), and Positive School Support (22). Families demonstrated a lack of awareness of resources available for their children and reported using other families, advocacy groups, health teams, and others to help. Some participants felt burdened as their child's sole advocate through their educational journey, including educating staff on epilepsy care and fighting for individual educational plans or supplemental resources. Families described global adverse effects of their child's epilepsy on family function related to finances/work, relationships, and sibling depression/anxiety, as well as inconsistent staff education to support students with epilepsy and variable staffing. Conclusion: Families of children with epilepsy face barriers that inform potential areas of improvement within schools. Closer interface between the medical community and educational systems may bridge gaps to ease the burden on families of children with epilepsy.

Autoimmune diseases are a heterogeneous group resulting from dysregulation or dysfunction of the immune system due to genetic predisposition and environmental triggers. It is common for these diseases to coexist. However, except for a single case secondary to varicella zoster virus infection, the association between juvenile idiopathic arthritis and acute cerebellitis has not been reported in the literature. A 9-year-old girl with juvenile idiopathic arthritis, initially presenting with symptoms of headache, nausea and vomiting, was diagnosed with diffuse bihemispheric acute cerebellitis by magnetic resonance imaging (MRI). Various tests were performed to determine the etiology of the disease. Infectious, metabolic, and autoimmune causes were excluded. This case report illustrates an ultra-rare association between juvenile idiopathic arthritis and acute cerebellitis, with a successful outcome following treatment and monitoring. There are 17 children, including our patient, who were followed with juvenile idiopathic arthritis and were found to have central nervous system disorders in the literature. Cerebral vasculitis and optic neuritis were the most commonly diagnosed neurologic disorders. Biologic agents have been implicated in some cases of optic neuritis and aseptic meningitis. Prompt treatment with corticosteroids and discontinuation of suspected biologic agents leads to favorable outcomes. This literature review highlights that early diagnosis and intervention can significantly improve outcomes.

Background: Posttraumatic headache is common in pediatrics. Studies have examined treatment of postconcussion symptoms, but few target posttraumatic headache. Biofeedback therapy has been studied in children and teens with primary headaches, but not posttraumatic headache. Our goal was to examine the changes in posttraumatic headache associated with biofeedback therapy in pediatric patients.

Methods: This retrospective study included 74 children aged 10-18 years who received biofeedback therapy for posttraumatic headache. "Positive response" to biofeedback therapy was defined as ≥50% reduction in headache days per week, or 3-point drop in pain rating between the first and last biofeedback therapy visits. Patient characteristics were analyzed using logistic regression to identify factors associated with response.

Results: In our cohort, 42% of all patients with posttraumatic headache had a positive response to biofeedback therapy. Thirty-six percent experienced ≥50% decrease in headache frequency, 13% had a ≥3-point decrease in headache severity and 8% reported both changes. For those with daily headaches, 36% had a positive response to biofeedback therapy. School absences, selective serotonin reuptake inhibitor or prescription preventive medication use were associated with nonresponse.

Conclusions: Biofeedback therapy is a reasonable treatment to consider to manage pediatric posttraumatic headache because more than 40% of our cohort had a beneficial response and biofeedback therapy has few adverse effects.

We examined the association between social determinants of health and the likelihood of sustaining a concussion among adolescents. Participants in this cross-sectional study were 7164 high school students who completed the 2021 Adolescent Behaviors and Experiences Survey (52.7% girls; mean age = 16.0 years, SD = 1.2; age range 12-18 years). Logistic regression was used to determine which social determinants of health variables were associated with a self-reported history of concussion from playing a sport or being physically active over the past year. One in 10 adolescents (n = 716; 10.0% total; 11.8% of boys, 8.3% of girls) reported sustaining a concussion during the past year. Seven of 10 adolescents (70.3%) reported experiencing at least 1 of 8 negative social determinants of health. A multivariable logistic regression was statistically significant (P < .001), indicating that the negative social determinants of health were associated with having sustained a concussion during the past year; the model explained 12.5% (Nagelkerke R²) of the variance in concussion. Controlling for all other predictors, sports participation (odds ratio [OR] = 3.72, medium effect), housing instability (OR = 3.25, small-medium effect) and limited English language proficiency (OR = 3.05, small-medium effect) were the strongest independent predictors of sustaining concussion within the past year. Adolescents who lived in a neighborhood where there is violence (OR = 1.78, small effect), who were bullied (OR = 1.57, very small effect), and who experienced food insecurity (OR = 1.36, very small effect) were more likely to have sustained a concussion. Research is needed to understand the nature of these determinant-injury associations. Whether social determinants of health are associated with specific treatment and rehabilitation needs, and time to recover following concussion, should be examined with prospective studies.

We present the case of a 15-year-old girl with new-onset psychosis and abnormal white matter activity on neuroimaging, engaging multidisciplinary care between genetics, neurology, psychiatry, and neuropsychology. She functioned well in mainstream education despite below average intellectual functioning. Physical examination findings enabled the diagnosis, and patient improved with joint psychological and behavioral outpatient services.