Transition from pediatric to adult care among patients with epilepsy: Cross-sectional surveys of experts and patients in Korea.

OBJECTIVES: Many pediatric patients with epilepsy require treatment beyond the pediatric age. These patients require transition to an adult epilepsy center. Currently, many centers worldwide run epilepsy transition programs. However, a standardized protocol does not exist in Korea. The basic data required to establish a transition program are also unavailable. We aimed to assess the status and perceptions of patients and epilepsy care providers on transition. METHODS: To assess the status of epilepsy transition, we retrospectively collected data from patients with epilepsy older than 18 years who visited our pediatric epilepsy clinic between March 1990 and July 2019. To assess the perception of transition, we surveyed patients, parents, pediatric neurologists (PN), and adult epileptologists (AE). RESULTS: In a retrospective chart review, 39 of 267 (14.6%) patients visited the adult epilepsy clinic after consulting a pediatric neurologist, and three patients returned to the pediatric center. The average patient age at transition was 23.29 ± 5.10 years. A total of 94 patients or their guardians and 100 experts participated in the survey. About half of the patients or guardians (44.7%) did not want to transition and emotional dependence was the commonest reason. Most patients (52.1%) thought that the appropriate age of transition was above 20 years. PNs had greater concerns about patients' compliance than AEs. Regarding the age of transition, AEs believed that a younger age (18 years) was more appropriate than PNs (20 years). SIGNIFICANCE: This study describes difficulties in the transition from pediatric to adult epilepsy centers without appropriate support. There were differences in perspectives among patients, parents, and adult and pediatric epilepsy care providers. This study can assist in creating a standardized protocol in Korea.

Treatment Outcomes of Vagus Nerve Stimulation in Lennox-Gastaut Syndrome / 대한소아신경학회지

PURPOSE@#Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic encephalopathies and frequently patients with this syndrome respond poorly to antiepileptic drugs. The aim of this study was to evaluate the treatment outcomes of vagus nerve stimulation (VNS) in LGS patients with drug-resistant epilepsy.@*METHODS@#We analyzed the clinical files, collected over 5 years, of children with LGS who received VNS treatment.@*RESULTS@#Seven children were included in this study (four males, three females; mean age of VNS insertion 12.4±3.5 years). All the patients had generalized tonic seizures and there were various other seizure types including myoclonic seizures, atonic seizures, and atypical absence seizures. Although two patients had normal imaging, five patients had abnormalities on imaging, including pachygyria, cortical dysplasia, kernicterus, and a chromosomal anomaly. Comparing the baseline seizure frequency to the frequency after the VNS surgery, the seizure frequency at the last follow-up showed a decrease of 57.2% (0% to 100%) on average (P=0.028) and one patient achieved seizure free status. Only two children were given additional antiepileptic drugs with the aim of managing their seizures. There was no mortality or complications related to the VNS therapy except one case requiring intensive care unit admission due to pneumonia. Comparing the results before and after VNS surgery, the VNS therapy also had a tendency to have a positive effect on quality of life (P=0.066).@*CONCLUSION@#In LGS patients with drug resistant epilepsy who are not candidates for a corpus callosotomy or resective surgery, VNS could be an effective, low-risk adjunct therapy for decreasing seizure frequency.

Nasal hemangiopericytoma causing oncogenic osteomalacia.

Oncogenic osteomalacia is a rare cause that makes abnormalities of bone metabolism. Our case arose in a 47-year-old woman presenting a nasal mass associated with osteomalacia. We excised the mass carefully. After surgery, it was diagnosed as hemangiopericytoma and her symptoms related with osteomalacia were relieved and biochemical abnormalities were restored to normal range. We report and review a rare case of nasal hemangiopericytoma that caused osteomalacia.