We retrospectively reviewed the medical records of 189 children who were admitted to the Pediatric Neurology Department at Yonsei University College of Medicine with status epilepticus (SE) between April, 1994 and April, 2003. The children were followed up for a mean duration of 17 months. We analyzed the clinical findings and the relationships between neurologic sequelae, recurrence, age of onset, presumptive causes, types of seizure, seizure duration and the presence of fever. Mean age at SE onset was 37 months. Incidences by seizure type classification were generalized convulsive SE in 73.5%, and non-convulsive SE in 26.5%. The incidences of presumptive causes of SE were idiopathic 40.7%, epilepsy 29.1%, remote 16.4% and acute symptomatic in 13.3%. Among all the patients, febrile episodes occurred in 35.4%, especially in patients under 3 year old, and 38.4% of these were associated with febrile illness regardless of presumptive cause. Neurologic sequelae occurred in 33% and the mortality rate was 3%. Neurologic sequelae were lower in patients that presented with an idiopathic etiology and higher in generalized convulsive SE patients. The recurrence of SE was higher in patients with a remote symptomatic epileptic etiology, and generalized convulsive SE showed higher rates of recurrence. Based on this retrospective study, the neurologic outcomes and recurrence of SE were found to be strongly associated with etiology and seizure type. Age, seizure duration and the presence of febrile illness were found to have no effect on outcome.
Age Distribution ; Child ; Child, Preschool ; Female ; Humans ; Incidence ; Infant ; Male ; Prognosis ; Recurrence ; Sex Distribution ; Status Epilepticus/etiology/*mortality

Background: Exclusive enteral nutrition (EEN) induces remission in pediatric Crohn's disease (CD). The exact mechanism of EEN therapy in CD is unknown, but alteration of the intestinal microflora after EEN is thought to affect mucosal healing. To determine the link between EEN therapy and therapeutic efficacy in CD, we established a murine model of dextran sulfate sodium (DSS)-induced colitis and applied EEN therapy. Methods: Eight-week-old C57BL/6 mice were administered DSS for 4 days to induce colitis, and either normal chow (NC) or EEN was administered for the following 4 days. The mice were grouped according to the feeding pattern after DSS administration: DSS/NC and DSS/ EEN groups. The clinical course was confirmed via daily observation of the weight and stool. Fecal samples were collected and 16sRNA sequencing was used. The mice were sacrificed to confirm colonic histopathology. Results: Weight reduction and increase in disease activity were observed as the day progressed for 4 days after DSS administration. There was significant weight recovery and improvement in disease activity in the EEN group compared to that in the NC group. Verrucomicrobia and Proteobacteria abundances tended to increase and Bacteroidetes abundance decreased in the EEN group. In the EEN group, significant changes in the β-diversity of the microbiota were observed. In the analysis of microbiome species, abundances of Akkermansia muciniphila, Clostridium cocleatum, mucin-degrading bacteria, Flintibacter butyricus, and Parabacteroides goldsteinii, which are beneficial microbiota, were significantly increased in the EEN group compared to those in the NC group. More abundant mucins were confirmed in the colonic histopathology of the EEN group. These microbial and histopathological differences suggested that EEN might improve colitis symptoms in a murine colitis model by promoting mucin recycling and subsequently inducing the healing effect of the gut barrier. Conclusion EEN showed clinical efficacy in a murine model of colitis. Based on the increase in mucin-degrading bacteria and the pathological increase in mucin production after EEN administration, it can be observed that mucin plays an important role in the therapeutic effect of EEN.

Ewing sarcoma and peripheral primitive neuroectodermal tumor (ES/pPNET) is an undifferentiated malignant tumor that is most prevalent in children and young adults and often radiologically mimics a meningioma. A 38-year-old female patient visited our hospital with complaints of right-sided tinnitus, right hemiparesis, and imbalance. She underwent preoperative imaging and was subsequently diagnosed as having a meningioma on the petrous ridge. After partial resection, EWSR1-FLI1 gene fusion was confirmed, and she was diagnosed with ES/pPNET. The tumor was successfully treated using a multidisciplinary approach of adjuvant chemo- and radiotherapy. This case is noteworthy because it is an extremely rare case of an intracranial ES/pPNET, and it is worth sharing our clinical experience that the tumor was successfully treated through a multidisciplinary therapeutic approach even though complete resection was not achieved.