The US Food and Drug Administration approval for perampanel has only recently been expanded to patients as young as 4 years, and so there have been few real-life studies of the effects of perampanel in pediatric patients. The aim of this study was to determine the long-term efficacy, factors affecting treatment response, and tolerability of perampanel as an add-on therapy in pediatric patients aged 4 years or older with epilepsy. This multicenter retrospective observational study collected data from pediatric epilepsy centers of four Korean national universities. Changes in the seizure frequency from baseline, adverse events, and retention rates were obtained at 3, 6, and 12 months. Adverse events and discontinuation profiles were obtained to assess tolerability. This study included 220 children and adolescents (117 males and 103 females) aged 4 to 20 years. The overall response rate was 43.6%, and the seizure-freedom rate was 17.7%. Factors affecting a good treatment response were the absence of intellectual disability, small number of concomitant antiepileptic drugs, and low baseline seizure frequency. Eighty-eight patients (40%) experienced adverse events, but they mostly were of mild severity and resolved after the dose reduction or discontinuation of perampanel. The retention rates at 3, 6, and 12 months were 85.0%, 71.8%, and 50.5%, respectively. Adjunctive treatment with perampanel was efficacious and tolerated in pediatric patients aged 4 years or older with epilepsy. Early perampanel treatment may help to reduce the burden of their seizures and improve their quality of life.

Background: Recurrent simple febrile seizure (SFS) refers to febrile seizure (FS) that recurs within 24 hours. Patients with recurrent SFS often undergo unnecessary neurodiagnostic tests. To address this, we compared the clinical characteristics of recurrent SFS with those of SFS and investigated the risk factors associated with recurrent SFS. Methods: We retrospectively reviewed electronic medical records of patients aged six to 60 months who had been hospitalized for FS at two training hospitals between January 2016 and December 2019. The primary outcome was a comparison of the clinical features of patients with SFS and recurrent SFS. Additionally, the risk factors associated with seizure recurrence within 24 hours were evaluated. Results: Three quarters (n = 191, 75.2%) of the 254 enrolled patients experienced a single seizure episode during the febrile illness period. The remaining 63 patients (24.8%) were diagnosed with recurrent SFS. Significant differences between SFS and recurrent SFS were observed in the history of recurrent SFS, time from fever onset to seizure, and body temperature on hospital arrival. Multiple logistic regression analysis revealed that a history of previous recurrent SFS (odds ratio [OR] 10.161) and a body temperature below 39 degrees C on arrival (OR 2.377) were significantly associated with early seizure recurrence. Conclusions: This study highlights that early FS recurrence is common and has a self-limiting clinical course similar to that of SFS. We recommend close monitoring of the patient for six to eight hours when a history of early recurrence is present or if the seizure occurs at a low body temperature. (c) 2024 The Author(s). Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Perampanel (PER) is expanding the therapeutic scope for pediatric epilepsy owing to its efficacy and favorable safety profile. However, concerns about psychiatric and behavioral adverse events (PBAEs) in combination therapy with levetiracetam (LEV) continue to contribute to hesitation in its prescription. We investigated the risk profiles for PBAEs when adding PER to pediatric epilepsy treatment and analyzed the differences according to the presence of concomitant LEV. We retrospectively reviewed the medical records of children aged 4-18 years with epilepsy who were prescribed PER as adjunctive therapy from March 2016 to February 2023. We compared the occurrence and management of PBAEs between the PER without LEV and PER with LEV groups. The risk factors for PBAEs were also analyzed. Ninety-four patients (53 boys and 41 girls) were included in this study. The median age of total patients at the time of adding PER was 14.9 years (12.3-16.4 years), and 53 patients (56.4 %) had concomitant LEV. Forty-seven PBAEs occurred in 34 patients (36.2 %), with no significant differences depending on whether concomitant LEV is present or not. The most common PBAEs were aggression (14.9 %), irritability (9.6 %), affect lability (7.4 %), and acute psychosis (6.4 %). PBAEs occurred at a lower dosage (2-6 mg/day) in 70.6 % of the patients. In addition, 73.5 % of patients with PBAEs continued PER treatment by follow-up observation or by reducing the PER dosage. No risk factors, such as the presence of concomitant LEV or lamotrigine, any comorbid conditions, higher PER dosage (8-12 mg/day), two or more concomitant anti-seizure medications, and younger age (<13 years) at PER add-on, showed significant associations. When expanding the use of anti-seizure medications in pediatric patients, real-world evidence on safety issues is crucial for pediatric epileptologists. We confirmed that combination therapy with PER and LEV did not increase the risk profile of PBAEs.

Rotavirus is an important cause of severe gastroenteritis in infants and children. Generally, rotavirus infections are self-limiting benign diseases but occasionally can cause a wide range of neurological manifestations, including benign febrile or afebrile convulsions, meningoencephalitis, cerebellitis, and lethal encephalitis or encephalopathy [1]. However, it is unclear how rotavirus can affect the central nervous system without direct invasion. Posterior reversible encephalopathy syndrome (PRES) is a well-recognized clinical disorder with typical neuroimaging findings consisting of mostly transient bilateral gray and white matter abnormalities in the posterior cerebral hemispheric regions and cerebellum [2]. The common clinical symptoms are headache, confusion, seizures, and visual disturbances such as cortical blindness. These symptoms usually recover without sequelae following appropriate treatment. Common precipitants are sudden elevations of blood pressure, renal failure, fluid restriction, and treatment with immunosuppressive drugs such as cyclosporine [3]. Recently, studies related to infection-associated PRES have been published [2,4]. We describe the case of a 6-year-old boy with rotavirus gastroenteritis who developed clinical and radiological manifestations consistent with PRES.

Purpose: We aimed to evaluate the utility of facial analysis technology for genetic diagnoses in a typical pediatric genetic clinic. Methods: A retrospective review identified children (aged <18 years) who had not previously received a definitive genetic diagnosis and underwent a comprehensive genetic evaluation. Their photographs and relevant clinical non-facial features were uploaded to the CLINIC application of the Face2Gene web interface, and the resulting analysis was accessed and correlated to the molecular diagnosis. Results: Of the 23 children included, the overall diagnostic yield in this study was 60.9% (14/23). In total, 64.3% of patients had the correct condition suggested in the top 10 differential diagnoses. The gestalt similarity was only 55.6%, but the phenotypic features added by the clinician showed a similarity of more than the medium level in all patients. Conclusion: Our data underscore the usefulness of facial analysis technology as an auxiliary point-of-care tool in pediatric genetic clinics, and we also present some considerations to increase accuracy.