RESUMO
Epileptic spasms without hypsarrhythmia occur when patients do not display hypsarrhythmia on electroencephalogram (EEG) at the onset and throughout the clinical course. We report three patients of epileptic spasms in patients with early onset, all of whom experienced other types of seizures.We detail three patients (two boys and one girl) of epileptic spasms without hypsarrhythmia, occurring between 1 and 3 months of age, with no abnormalities detected on neurometabolic analysis and brain magnetic resonance imaging. Long-term video-EEG monitoring revealed epileptic spasms with focal onset seizures in two patients, and epileptic spasms followed by generalized tonic-clonic seizures in one patient. Hypsarrhythmia was never observed in repeated EEG examinations. Two patients achieved seizure freedom and improved development through treatment with topiramate alone or in combination with valproate, without requiring hormonal therapies or vigabatrin. The remaining patient achieved seizure freedom following administration of antiseizure medications, including topiramate, after a trial of adrenocorticotropic hormone therapy.We report the cases of three patients with early onset epileptic spasms without hypsarrhythmia. All patients achieved seizure freedom after topiramate treatment. Topiramate may be considered as a relatively effective antiseizure medication for early onset epileptic spasms without hypsarrhythmia.
Assuntos
Anticonvulsivantes , Eletroencefalografia , Espasmos Infantis , Humanos , Masculino , Feminino , Lactente , Anticonvulsivantes/uso terapêutico , Anticonvulsivantes/administração & dosagem , Espasmos Infantis/tratamento farmacológico , Espasmos Infantis/fisiopatologia , Topiramato/administração & dosagem , Topiramato/uso terapêutico , Imageamento por Ressonância MagnéticaRESUMO
BACKGROUND: Self-efficacy plays an important role in enhancing the teaching capabilities of attending physicians (APs). The clinical ladder (CL) is an educational approach developed in the field of nursing education that increases difficulty in an incremental manner. However, no previous study has confirmed the effectiveness of CL in medical education. Therefore, this study aimed to examine the effect of clinical clerkship integrated with clinical ladder (CC-CL) on the self-efficacy of APs. METHODS: Sixth-year medical students participated in CC-CL for 6 months starting from April 2023, and the changes in the self-efficacy of APs were retrospectively evaluated. The students were trained by the APs concurrently, and the achievement levels of each student were shared. The primary outcome measure was the physician teaching self-efficacy questionnaire (PTSQ) score. The PTSQ scores before and after CC-CL were analyzed using the Wilcoxon matched-pair signed-rank test. RESULTS: Fifteen APs from the Department of Pediatric and Child Neurology were included in this study. No significant difference was observed in the total PTSQ scores of the APs before and after CC-CL. However, a significant increase was observed in the PTSQ score of APs who participated for at least 2 h per week over a period of more than 3 months (n = 8) after CC-CL (p = 0.022). Furthermore, APs who had received their pediatrician certification < 10 years ago (n = 8) showed a significant increase in the total PTSQ score after CC-CL (p = 0.022). CONCLUSIONS: CC-CL may play an important role in cultivating the self-efficacy of less experienced APs. Further comparative studies must be conducted in the future to validate the findings of this study.
Assuntos
Estágio Clínico , Médicos , Estudantes de Medicina , Humanos , Criança , Mobilidade Ocupacional , Estudos Retrospectivos , Autoeficácia , EnsinoRESUMO
INTRODUCTION: Infantile epileptic spasms syndrome (IESS) is characterized by epileptic spasms, regardless of hypsarrhythmia on electroencephalogram or neurodevelopmental delay. In Japan, pyridoxal 5'-phosphate (PLP) is often used as the first-line treatment for IESS because it is effective in a certain number of patients. Although several studies have reported serious adverse events following PLP treatment, no study has investigated the risk factors for such occurrences. OBJECTIVE: To investigate adverse events associated with PLP therapy for the treatment of IESS and to identify the associated risk factors. MATERIALS AND METHODS: We retrospectively evaluated adverse events in 59 patients with IESS at Tottori University Hospital between January 1995 and September 2022. We subsequently collected and analyzed their clinical data and analyzed the risk factors associated with each adverse event. The cutoff values and relative risk (RR) were analyzed for items with significant associations with adverse events. RESULTS: Twenty-seven (51.9%) participants experienced adverse events, including vomiting in 16 participants (59.3%), elevated liver enzyme levels in 15 participants (55.6%), and rhabdomyolysis in two participants (3.4%). No significant differences were observed between the non-adverse events group and the overall adverse events group, as well as between the non-adverse events group and the vomiting group, in terms of the factors examined. However, when comparing the non-adverse events group with the group with elevated liver enzyme levels, age at PLP treatment showed a negative correlation, whereas PLP dose showed a positive correlation with elevated liver enzyme levels. The cutoff dose was 40 mg/kg/day (73.3% sensitivity and 60.7% specificity), and the cutoff age was 9 months (100% sensitivity and 40.0% specificity). RRs of doses ≥40 mg/kg/day and age <9 months were 2.6 and 3.6, respectively. CONCLUSIONS: Adverse events of PLP therapy, including vomiting, elevated liver enzymes, and rhabdomyolysis, were observed in approximately half of the participants. Age under 9 months and a dose ≥40 mg/kg/day were identified as risk factors for elevation of liver enzymes on PLP treatment in infants with IESS, with rhabdomyolysis can occur in the younger or higher dose cases.
Assuntos
Fosfato de Piridoxal , Espasmos Infantis , Lactente , Humanos , Fosfato de Piridoxal/efeitos adversos , Estudos Retrospectivos , Espasmos Infantis/tratamento farmacológico , Síndrome , Fatores de Risco , EspasmoRESUMO
Background: In Japan, approximately 35% of facilities experience sedation-related complications for pediatric magnetic resonance imaging (MRI), including severe complications such as respiratory and cardiac arrests. In the medical education field, simulation improves not only the quality of real emergency response but also health care workers' self-efficacy. Individuals with high self-efficacy are better prepared at handling diverse conditions. However, there is no research examining the impact of sedation simulation for pediatric MRI on the self-efficacy of health care workers. This study aimed to retrospectively investigate the impact of sedation simulation for pediatric MRI on the self-efficacy of health care workers in sedation. Methods: The study was conducted on August 18, 2023, and enrolled pediatricians, nurses, and radiological technologists. The impact of sedation simulation for pediatric MRI on the participants was analyzed using confidence scale scores and part of the Pediatric Resuscitation and Escalation of Care Self-Efficacy Scale (PRSES), before and after sedation simulation for pediatric MRI. Results: Eighteen participants (six pediatricians, six nurses, and six radiological technologists) were included in this study. Regarding confidence scale scores, a significant improvement was observed in the overall group (P = 0.002) and among the nurses (P = 0.0036). Regarding the item 'When confronted with a clinically deteriorating child, I know how to ask for assistance' of PRSES, a significant improvement was observed in the overall group (P = 0.0035) and among the radiological technologists (P = 0.048). Conclusion: There's a potential for sedation simulation for pediatric MRI to increase the self-efficacy of health care workers in MRI sedation. Our findings suggest that this training has a valuable role in preparing health care workers to practice sedation for pediatric MRI.
RESUMO
OBJECTIVE: Coronavirus disease 2019 (COVID-19) has significantly impacted medical services worldwide. During the Omicron variant-predominant era, febrile seizure (FS) in patients with COVID-19 increased compared to that in the pre-Omicron variant era. Therefore, this study aimed to demonstrate the clinical characteristics of FS in patients with COVID-19. METHODS: We surveyed patients aged < 16 years who presented with FS to the emergency room of Tottori University Hospital. The patients were divided into two groups: FS patients with COVID-19 (FS with COVID-19 group) and FS patients without COVID-19 (FS without COVID-19 group) as per the results of the respiratory multiplex array test. Patients with positive results for both SARS-CoV-2 and other microorganisms were excluded. We obtained data on the patients' clinical backgrounds, symptoms, seizure duration, type of FS (simple or complex), diagnostic examinations, laboratory test results, and treatment. We compared the data between the FS with and without COVID-19 groups. RESULT: A total of 128 patients with FS met the inclusion criteria. Of these, 18 patients and 110 patients were included in the FS with COVID-19 group and without COVID-19 group, respectively. The late FS onset (>60 months) were significantly more common in the FS with COVID-19 group than that in the FS without COVID-19 group. Moreover, patients in the FS with COVID-19 group had significantly longer seizure durations than those in the FS without COVID-19 group. A diazepam (DZP) suppository was administered to 72% of FS patients with COVID-19 after the first seizure during a febrile episode. CONCLUSION: FS patients with COVID-19 had different distributions of age at onset and seizure duration than those without COVID-19. The use of DZP suppositories was more frequent in FS patients with COVID-19 compared to those without COVID-19.
Assuntos
COVID-19 , Convulsões Febris , Criança , Humanos , Convulsões Febris/diagnóstico , COVID-19/complicações , SARS-CoV-2 , Diazepam/uso terapêuticoRESUMO
OBJECTIVE: Acute pancreatitis (AP) is a life-threatening disease in children with severe motor and intellectual disabilities (SMID). This study aimed to determine the clinical characteristics and causes of AP in children with SMID. METHODS: We enrolled 29 children with AP admitted to our hospital. Patients were divided into children with SMID (AP with SMID group) and the remaining patients (AP without SMID group). All patients in the AP without SMID group had no pre-existing neurological disabilities. We obtained data on the clinical background of patients, severity of AP, treatments, and outcomes. We enrolled 34 children with SMID without a history of AP (SMID control group) to compare their clinical characteristics with the AP with SMID group. We statistically compared the data between the AP with SMID and AP without SMID groups and between the AP with SMID and SMID control groups. RESULT: Compared to the AP without SMID group, the AP with SMID group showed significantly more severe pancreatitis (50% vs 12%) and recurrent pancreatitis (58% vs 18%). Moreover, compared with the SMID control group, the AP with SMID group showed significantly more frequent absence of voluntary movement (83% vs 26%), requirement of respiratory devices (75% vs 35%), panhypopituitarism (33% vs 6%), thermoregulatory dysfunction (58% vs 18%), and low serum albumin levels (3.6 vs 4.1 g/dL). CONCLUSION: AP in children with SMID tended to be associated with severe and recurrent pancreatitis. Children with SMID who had a history of AP had more severe neurological impairment among children with SMID. Hypothalamic-pituitary system dysfunction caused by severe cerebral damage may contribute to the development of AP.
Assuntos
Deficiência Intelectual , Pancreatite , Humanos , Criança , Pancreatite/complicações , Deficiência Intelectual/complicações , Deficiência Intelectual/epidemiologia , Doença Aguda , Fatores de Risco , Estudos RetrospectivosRESUMO
Status epilepticus (SE) often causes neuronal death in the cerebrum and consequent long-term sequelae. Acute encephalopathy with biphasic seizures and late reduced diffusion is clinically characterized by SE associated with fever and seizure clusters that occur 3-9 days after symptom onset. MRI reveals reduced diffusion in the frontal or frontoparietal subcortical white matter, with sparing of the perirolandic region following seizure clusters. Kawasaki disease (KD) is an acute self-limited vasculitis secondary to activation of the immune system; KD is rarely complicated by acute encephalopathy. We report the case of a male infant who developed SE associated with KD and showed late reduced diffusion in the subcortical white matter beneath the bilateral motor cortices (primary motor, premotor and supplementary motor areas) and the right frontal cortex. The patient had characteristic neurological sequelae in the chronic phase, including clumsiness of fingers and forearms, impaired discrimination of tactile sensation and position sense on digits in his hands and feet, corresponding to the lesions with reduced diffusion at the acute phase.
RESUMO
BACKGROUND: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) often develops in children with febrile status epilepticus (FSE) with neurological sequelae. No study has investigated the associations between prehospital emergency care and AESD onset. METHODS: We retrospectively collected the data of children with FSE (>30 min) treated in Tottori University Hospital. We evaluated the prehospital emergency care information, investigating its association with AESD development. RESULTS: We identified 11 patients with AESD and 44 with FSE. The time from onset to the arrival of the emergency medical services (EMS) (OR: 1.12, P = 0.015) and hospital arrival (OR: 1.07, P = 0.009) was positively associated with AESD development. In contrast, oxygen saturation levels in ambulances (OR: 0.901, P = 0.013) are negatively associated with AESD development. The time from onset to arrival at the hospital was associated with the time from onset to the administration of antiseizure medications (ASMs) (correlation coefficient: 0.857, P < 0.001), which was significantly associated with AESD development (OR: 1.04, P = 0.039). The cutoff values were 17 minutes from onset to the arrival of EMS (OR: 27.2, P = 0.003), 38 minutes to hospital arrival (OR: 5.71, P = 0.020), and 50 minutes of administration of ASMs (OR: 7.11, P = 0.009). CONCLUSIONS: Prolonged time from onset to hospital arrival and hypoxia in ambulances were associated with AESD development. Shortening transport time, improving respiratory management in ambulances, and the early administration of ASMs might play a role in preventing the development of AESD.
Assuntos
Encefalopatias , Serviços Médicos de Emergência , Convulsões Febris , Estado Epiléptico , Humanos , Criança , Estudos Retrospectivos , Convulsões Febris/terapia , Estado Epiléptico/etiologia , Estado Epiléptico/terapia , Fatores de RiscoRESUMO
INTRODUCTION: Carbamazepine (CBZ) is a common antiepileptic drug that may cause overdoses with seizures as a common neurological manifestation. In previous reports, patients with CBZ overdose exhibited stimulus-induced generalized clinical or electrical seizures. To date, no previous cases of focal motor seizures have been reported. CASE REPORT: We report the case of an 11-year-old girl with spontaneous and stimulus-induced clustering of focal motor seizures following CBZ overdose. The patient had been treated with CBZ (150 mg daily) for focal epilepsy since the age of six years. At the age of 11, she forgot to take a morning dose, took ten CBZ pills (CBZ 1000 mg) as compensation, and presented with generalized seizures. The patient arrived at the hospital in a coma. She demonstrated clustering of focal-to-bilateral tonic-clonic seizures induced by pain stimulus or spontaneously, with focal epileptiform discharges observed on EEG. Her CBZ blood concentration measured 40.4 µg/mL and she was diagnosed with CBZ overdose. The patient showed improvement without any specific treatment, and was later discharged without neurological sequelae. CONCLUSION: Previous cases of CBZ overdose with stimulus-induced generalized seizures resulted in death or required intensive care. Stimulus-induced focal seizures may indicate a favorable prognosis for CBZ overdose.