Journal of Child Neurology最新文献

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).

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.

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.

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: 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.

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.

Objective: To describe the incidence, clinical characteristics, and long-term outcomes of cerebral sinus venous thrombosis in children with acute lymphoblastic leukemia.

Methods: This was a retrospective cohort study comprising pediatric patients with newly diagnosed or first-relapse acute lymphoblastic leukemia who developed cerebral sinus venous thrombosis at Texas Children's Hospital from 2002 to 2019.

Results: Nineteen cases (1.7%) with cerebral sinus venous thrombosis were identified in all pediatric patients with acute lymphoblastic leukemia (n = 1129). Increased risk of cerebral sinus venous thrombosis was observed with age >10 years (P = .006). Twelve cases (63%) occurred during the induction, 4 (21%) during maintenance, and 3 (16%) during the consolidation phases of leukemia therapy. Seizures (10/19) and headaches (9/19) were the most common presenting symptoms. After treatment with anticoagulation therapy, we observed full resolution of thrombosis in 10 (53%) and partial resolution in 8 patients (42%). Long-term neurologic outcomes at follow-up in the 14 patients who survived included normal neurologic examinations (n = 10), epilepsy (n = 3), and focal neurologic deficits (n = 2). The death occurred in 5 individuals.

Conclusion: Cerebral sinus venous thrombosis is a notable complication of pediatric acute lymphoblastic leukemia therapy. Older age (>10 years) was a risk factor for developing cerebral sinus venous thrombosis. Despite variable patient presentations and treatment durations, favorable clinical outcomes were observed in most patients after the treatment with anticoagulation for a minimum of 3 months.

A growing number of genes have been identified in individuals with cerebral palsy (CP); however, many of these studies have poor compliance with the cerebral palsy clinical description. This systematic review aimed to assess the quality of the cerebral palsy clinical description/phenotype in cerebral palsy genetic studies published between 2010 and 2024 and report clinically relevant genes based on the quality of the cerebral palsy phenotype. An expert panel developed 6 criteria to review the reported cerebral palsy phenotype/description for each included study. Clinically relevant genes were extracted from each study and stratified into 2 tiers based on the quality. Eighteen studies were included. There was high confidence in the reported cerebral palsy description/phenotype from 8 studies. Of the initial 373 clinically relevant genes, 85 were tier II genes. Individual cerebral palsy motor disorder and phenotype data were absent for 349 of these individuals, limiting further analysis. The tier I gene list was composed of 6 genes: ATL1, COL4A1, GNAO1, KIF1A, SPAST, and TUBA1A. Bilateral spasticity was the most common motor disorder reported in individuals with variants in all 6 genes, and most individuals had accompanying conditions. Prioritizing the accurate reporting of motor and nonmotor phenotypes is crucial for future cerebral palsy genetic studies to further understand the underlying neurobiology.

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease is a neuroinflammatory disorder (MOGAD) with heterogeneous phenotype including paroxysms of optic neuritis, transverse myelitis, acute disseminated encephalomyelitis, brainstem demyelination, and encephalitis. Fluid-attenuated inversion recovery hyperintense cortical lesions in MOG-associated encephalitis with seizures, or FLAMES, is a manifestation of cerebral cortical encephalitis seen less frequently than other typical MOG antibody-associated disease presentations. Cases of FLAMES are rarer in children, and frequently initially misdiagnosed with infectious meningoencephalitis. Other meningocortical manifestations of MOG antibody-associated disease have been described and likely exist along a continuum. In this retrospective single-center case series, we describe the demographic, clinical, radiographic, laboratory, and electroencephalographic features of 5 children with clinicoradiographic features consistent with the spectrum of MOG-IgG-positive meningocortical syndromes.