Journal of Child Neurology最新文献

Background: Most patients with idiopathic generalized epilepsy have good seizure control on antiseizure medications. Although idiopathic generalized epilepsy subtypes such as juvenile absence epilepsy and juvenile myoclonic epilepsy have a high risk of relapse, childhood absence epilepsy may have seizure remission. After 2 years of seizure freedom in childhood absence epilepsy, typically antiseizure medications are discontinued, but follow-up protocols are unclear. This study aims to evaluate how often patients with idiopathic generalized epilepsy undergo electroencephalography (EEG) after antiseizure medication withdrawal, how often antiseizure medications are restarted based on EEG findings, and if this varies between physicians and advanced practice providers at our institution.

Methods: This was a retrospective chart review. Data were collected using electronic medical records of pediatric patients (<18 years) with idiopathic generalized epilepsy who were successfully weaned off antiseizure medications at Penn State Children's Hospital from 2010 to 2020.

Results: We reviewed 1409 charts and found 52 patients meeting criteria. Seventeen of 52 patients (32%) had a repeat EEG within 6 months of antiseizure medication withdrawal following seizure freedom. Of those 17 patients, 3 (17.6%) had generalized epileptiform discharges on EEG. Of these 3 patients, 2 (66%) were restarted on antiseizure medications based on the abnormal EEG. None had seizure relapse.

Conclusion: Obtaining a repeat EEG in patients after antiseizure medication withdrawal following seizure freedom is common. Patients with an abnormal EEG are often restarted on antiseizure medications, irrespective of clinical seizure relapse. Considering the high health care costs of EEGs and antiseizure medication side effects, we propose that if patients with idiopathic generalized epilepsy do well clinically following antiseizure medication withdrawal, EEGs may not be necessary.

Objective: Neonatal seizures present significant treatment challenges, often requiring adjunctive antiseizure medications. This study aimed to evaluate the efficacy of lacosamide as an adjunctive antiseizure medication in managing refractory neonatal seizures.

Methods: The study included neonates up to 44 weeks of corrected gestational age who received lacosamide treatment at a level 4 neonatal intensive care unit between January 2015 and December 2023. The collected data encompassed demographic features, birth history, perinatal/postnatal complications, seizure characteristics, and treatment details. The primary outcome was the response to lacosamide, assessed by changes in total seizure burden on electroencephalography (EEG). The cohort characteristics were compared between nonresponders and responders.

Results: The final analysis was conducted on 18 neonates with available EEG data. Of all patients, 72% showed a favorable response to lacosamide treatment, as evidenced by a reduced seizure burden on EEG. The demographic and clinical characteristics of the neonates varied, including a range of seizure etiologies. Responder and nonresponder groups had no differences in clinical characteristics.

Conclusion: This study suggests that lacosamide may be an effective adjunctive antiseizure medication in treating refractory neonatal seizures. Further prospective studies are warranted to confirm these findings and explore the long-term outcomes and safety profile of lacosamide in this vulnerable patient group.

Introduction: Chorea, a movement disorder that commonly affects children, may be caused by various diseases with metabolic, structural, pharmacologic, or autoimmune origins. Celiac disease is an autoimmune enteropathy that may rarely cause neurologic symptoms in children, primarily ataxia and peripheral neuropathy, even in the absence of gastrointestinal symptoms.

Case report: A 9-year-old male patient diagnosed with Sydenham chorea was admitted to our clinic because of valproic acid resistance. He had involuntary, brief, random, and irregular movements in his arms over the past 2 weeks. A low-dose, high-potency dopamine-2 receptor-blocking agent was added to the patient's treatment regimen. After 2 weeks, he had maculopapular rashes on their hands and arms, as well as arthritis in his left hand. Oral prednisone was prescribed, and the patient's arthritis and rashes were completely resolved. Although movement disorder symptoms persisted in the distal left upper extremity, the chorea in the right upper extremity was recovered. Extensive investigations were conducted to rule out possible metabolic, autoimmune, inflammatory, infectious, and paraneoplastic diseases, all of which yielded normal results. Brain magnetic resonance imaging (MRI) results were normal, and genetic analysis results for chorea were negative. The patient's tissue glutaminase IgG levels exceeded 200 U/mL (normal range: 0-10 U/mL) and IgA levels at 24 U/mL (normal range: 0--10 U/mL), leading to a diagnosis of celiac disease. His duodenal biopsies showed changes consistent with gluten-sensitive enteropathy. After the diagnosis of celiac disease, the patient began a gluten-free diet and remained free of chorea at the 6-month follow-up.

Discussion: Chorea is a rare neurologic celiac disease manifestation that can be reversible. The celiac disease should be considered in the diagnostic workup of chorea for all ages, particularly in the treatment-resistant population, even in the absence of gastrointestinal symptoms. This report presents the first known case of chorea caused by celiac disease in a pediatric patient.

Background: Post COVID-19, caregivers of children with cerebral palsy in South Africa face unique challenges.

Methods: A qualitative exploratory approach was used. Semistructured interviews were conducted with 14 caregivers of children with cerebral palsy in Gauteng, South Africa. Interviews were audio-recorded, transcribed verbatim, and analysed using thematic analysis.

Results: Lockdown restrictions have had lasting effects on families' routines and events, reshaping their internal and external functioning. The pandemic introduced new challenges, such as increased physical pain due to the child's weight gain, persistent emotional distress, and a lack of social and governmental support.

Conclusion: Post COVID-19, it is crucial to develop innovative support mechanisms for children with cerebral palsy and their caregivers, focusing on comprehensive health services, robust social support, and targeted interventions to address the ongoing and new challenges faced by these families.

Infections are hypothesized to trigger certain autoimmune diseases; however, there is a lack of epidemiologic data surrounding pediatric neuro-autoimmune disorders during the COVID-19 pandemic. Our retrospective study assessed the incidence of pre-defined autoimmune disorders diagnosed at the Children's Hospital of Eastern Ontario in Ottawa, Canada, between October 2017 and June 2024. Inpatient and outpatient charts were queried to identify subjects with neuro-autoimmune disorders or type 1 diabetes as a nonneurologic autoimmune comparison group. Monthly incidences were calculated and compared between 3 COVID-19 pandemic restriction periods: the prerestrictions period (October 2017-March 2020), intrarestrictions period (April 2020-June 2022), and postrestrictions period (July 2022-June 2024). Poisson regression models were fit to the incidence data. New diagnoses of neuro-autoimmune disorders and type 1 diabetes were identified in 111 and 670 subjects, respectively. Incidence of neuro-autoimmune disorders, but not type 1 diabetes, decreased during the intrarestrictions period when compared to the prerestrictions period (incidence rate ratio = 0.57, 95% confidence interval 0.33-0.95, P < .05).

Objective: To describe electroencephalographic (EEG) changes in pediatric patients with cerebral edema after cardiac arrest.

Methods: A retrospective study of patients admitted to the pediatric intensive care unit from July 2021 to January 2023. We included patients with cardiac arrest and changes in EEG background with clinical changes and/or neuroimaging consistent with cerebral edema. We excluded patients with electrographic seizures. We applied American Clinical Neurophysiology Society standardized critical care EEG terminology to classify EEG background, noting timing of the change in background classification. Clinical variables included age, sex, and neuroimaging findings and were described with descriptive statistics.

Results: Nine patients met inclusion criteria, with median age 24 months (interquartile range 21-49), and 89% were male. There were 5 common EEG stages: stage 1, burst suppression/burst attenuation; stage 2, continuous/discontinuous ± multifocal sporadic epileptiform discharges ± rhythmic or periodic patterns; stage 3, discontinuous/burst suppression/burst attenuation ± rhythmic or periodic patterns; stage 4, gradual voltage suppression; and stage 5, diffuse suppression. The ranges for each stage were as follows: stage 1, 2-10 hours; stage 2, 2.5-15.5 hours; stage 3, 0.5-6.24 hours; and stage 4, 0.5-11 hours. We could not calculate the duration of stage 5 given no uniform time to EEG discontinuation. One patient had a clinical change in stage 3. Remaining patients presented with fixed and dilated pupils with global anoxic injury.

Conclusions: EEG stages of cerebral edema have not been described after pediatric cardiac arrest. These stages may be relevant to other patient populations. Early stages may be a therapeutic target for intracranial pressure-lowering medications and/or neuroprotective strategies to minimize sequalae of cerebral edema.

Introduction: Leukodystrophies are a heterogeneous group of inherited neurologic disorders. These disorders are indeed progressive and debilitating conditions with limited treatment options and high mortality rates. There is a deficiency in available data concerning both the mortality rates and the most common causes of death in leukodystrophies. Methods: We investigated the mortality rates, mean age at death, and the most common causes of death in a retrospective cohort of 165 Iranian pediatric patients who were diagnosed with leukodystrophies. Results: Death was recorded in 64 of 165 patients (38.8%) with a mean follow-up of 4.7 ± 3.25 years. The mean age of living patients was 7.9 ± 4.8 years and the mean age at death was 5.2 ± 3.9 years. Mortality rate of the entire cohort was 18.1% (30/165), 24.2% (40/165), and 35.7% (59/165) at 3, 5, and 10 years' follow-up, respectively. The mean age at death was 2.13 ± 0.68 years, 2.67 ± 1.14 years, and 4.33 ± 2.73 years, at 3-, 5-, and 10-year follow-up from first symptom onset, correspondingly. However, there was a significant difference in the mean age at death in years in hypomyelinating leukodystrophies compared with other leukodystrophies (2.19 ± 0.19 standard error [SE], confidence interval [CI] 1.81-2.56; and 6.65 ± 0.62 SE (CI 5.42-7.87); log rank P = .0001, analysis of variance P = .0001). The most common causes of death were cardiopulmonary problems (47%), seizures (11%), sepsis (9%), and miscellaneous (33%). Conclusions: We proposed that a significant majority of childhood leukodystrophy deaths occur within the first 5 years of life, with a notable concentration during the initial 3 years. Further, the results of this study suggest the potential for targeted strategies to address the specific causes of death in children with leukodystrophies.

The aim of this retrospective descriptive study was to evaluate the clinical impact and safety profile of lacosamide in neonates with symptomatic refractory seizures.Patients diagnosed with symptomatic refractory seizures who received lacosamide were included in the study. Follow-up assessments were conducted until 24 months of age, during which data on lacosamide dosage, duration of exposure, concurrent treatments, and potential side effects were collected. A total of eight patients were enrolled, with lacosamide administered as a third- or fourth-line treatment for symptomatic refractory seizures. Following loading dose, 62.5% of the patients achieved complete remission of seizure activity without recurrence. In the remaining cases, a reduction in seizure frequency was observed. No adverse effects attributable to lacosamide were reported.In conclusion, lacosamide may be effective in achieving seizure remission in newborns with symptomatic refractory seizures, and all patients demonstrate excellent tolerance. Brief exposure to lacosamide was sufficient, and no adverse effects were observed up to 24 months of age. However, randomized controlled trials are necessary to confirm these findings.

Background: The standard treatment guidelines of neurocysticercosis have been described as per computed tomography (CT)-based studies. We aimed to prospectively study if posttreatment magnetic resonance imaging (MRI) clearance rates of neurocysticercosis were like those reported in literature using CT.

Methods: A prospective observational study in newly diagnosed children with neurocysticercosis was undertaken. Children were treated with antihelminthics and steroids and followed up after 6 months. The primary objective was to study the proportion of children with single-lesion neurocysticercosis who were in radiologic resolution at 6 months and clinical remission (seizure-free for the preceding 3 months).

Results: Eighty of 128 consecutive children screened were included (single lesion, 65; multiple lesions, 15). Seventy-two children were evaluated at 6 months. Seizure recurrence was seen in 5 (6.2%). Brain MRI showed an overall clearance of lesions in 10 (14%) children. In the children with single-lesion neurocysticercosis (65), 59 were followed up at 6 months, and lesions resolved in 9 (15.3%, 95% confidence interval of 6.1-24.4).

Conclusions: In children with single-lesion neurocysticercosis treated with antihelminthics and corticosteroids, the lesion resolution rate is only 15% at 6 months. Thus, there is a need to review old recommendations and use MRI as a standard outcome measure.

Cases of isolated spinal cord ischemia resulting in symptoms in neonates are rare, and there are even fewer reported cases in atraumatic births. We present a case of a presumed isolated cervical cord ischemic injury, discuss differentials to consider when evaluating a neonatal spinal cord injury, and highlight the difficulties of diagnosing a spinal cord infarction.