So-Hee Eun

Estimating the Prevalence of Treated Epilepsy Using Administrative Health Data and Its Validity: ESSENCE Study

Background and Purpose Few of the epidemiologic studies of epilepsy have utilized well-validated nationwide databases. We estimated the nationwide prevalence of treated epilepsy based on a comprehensive medical payment database along with diagnostic validation. Methods We collected data on patients prescribed of antiepileptic drugs (AEDs) from the Health Insurance Review and Assessment service, which covers the entire population of Korea. To assess the diagnostic validity, a medical records survey was conducted involving 6,774 patients prescribed AEDs from 43 institutions based on regional clusters and referral levels across the country. The prevalence of treated epilepsy was estimated by projecting the diagnostic validity on the number of patients prescribed AEDs. Results The mean positive predictive value (PPV) for epilepsy was 0.810 for those prescribed AEDs with diagnostic codes that indicate epilepsy or seizure (Diagnosis-E), while it was 0.066 for those without Diagnosis-E. The PPV tended to decrease with age in both groups, with lower values seen in females. The prevalence was 3.84 per 1,000, and it was higher among males, children, and the elderly. Conclusions The prevalence of epilepsy in Korea was comparable to that in other East Asian countries. The diagnostic validity of administrative health data varies depending on the method of case ascertainment, age, and sex. The prescriptions of AEDs even without relevant diagnostic codes should be considered as a tracer for epilepsy.

Psychological characteristics of Korean children and adolescents with type 1 diabetes mellitus

Purpose This study investigates the behavioral and emotional characteristics of Korean children and adolescents with type 1 diabetes mellitus (T1DM) as compared to healthy controls, and examines whether their psychological status is associated with glycemic control, insulin regimens, or disease duration. Methods A total of 37 Korean children and adolescents with T1DM, aged 6-17 years, and 38 sex- and age-matched healthy controls were included in this study. Psychological distress was assessed using the Korean child behavior checklist (K-CBCL) and childrens depression inventory (CDI) after the subjects and their parents were interviewed. Results The CDI and K-CBCL scores were significantly higher in T1DM subjects compared to normal controls. The T1DM subjects with poorly controlled blood glucose (glycosylated hemoglobin ≥8%) and old patients (disease duration ≥1 year) had a tendency to show higher CDI and K-CBCL scores. There were no significant differences in CDI and K-CBCL scores between the intensive and conventional insulin therapy groups. Conclusion Children and adolescents with T1DM seem to have inferior psychological adjustment to their normal counterparts, which might be associated with glycemic control and disease duration. Psychological evaluation and intervention should be considered in the management of T1DM in children and adolescents.

Early neurodevelopment in very low birth weight infants with mild intraventricular hemorrhage or those without intraventricular hemorrhage

Purpose This study aimed to assess early development in very low birth weight (VLBW) infants with mild intraventricular hemorrhage (IVH) or those without IVH and to identify the perinatal morbidities affecting early neurodevelopmental outcome. Methods Bayley Scales of Infant Development-II was used for assessing neurological development in 49 infants with a birth weight <1,500 g and with low grade IVH (≤grade II) or those without IVH at a corrected age of 12 months. Results Among the 49 infants, 19 infants (38.8%) showed normal development and 14 (28.6%) showed abnormal mental and psychomotor development. Infants with abnormal mental development (n=14) were mostly male and had a longer hospitalization, a higher prevalence of patent ductus arteriosus (PDA) and bronchopulmonary dysplasia (BPD), and were under more frequent postnatal systemic steroid treatment compared with infants with normal mental development (n=35, P<0.05). Infants with abnormal psychomotor development (n=29) had a longer hospitalization and more associated PDA compared to infants with normal psychomotor development (n=20, P<0.05). Infants with abnormal mental and psychomotor development were mostly male and had a longer hospitalization and a higher prevalence of PDA and BPD compared to infants with normal mental and psychomotor development (n=19, P<0.05). Using multiple logistic regression analysis, a longer duration of hospitalization and male gender were found to be significant risk factors. Conclusion Approximately 62% of VLBW infants with low grade IVH or those without IVH had impaired early development.

Factors Predicting Poor Response to Initial Therapy in Benign Childhood Epilepsy with Centrotemporal Spikes (BCECTS)

Background and Purpose: Benign childhood epilepsy with centrotemporal spikes (BCECTS) is the most common pediatric focal epilepsy syndrome and typically has positive clinical outcomes. However, a few patients experience recurrent seizures, and therefore, require treatment with antiepileptic drugs (AEDs). This study aimed to identify risk factors associated with poor response to initial AED therapy in BCECTS patients. Methods: We retrospectively reviewed the files of 57 patients who were diagnosed with BCECTS between January 2008 and September 2013. Patients not being treated with AEDs have been excluded. We placed the patients into two groups: (1) patients using 1 AED, and (2) patients using 2 AEDs. Clinical characteristics were then collected from the medical records. Results: Of the 57 patients, 41 (72%) were successfully treated with 1 AED, and 16 (28%) required 2 AEDs to control seizures. Multiple logistic regression analysis indicated that seizure onset prior to age 5 (odds ratio [OR]: 5.65, 95% confidence interval [CI]: 1.41–22.68) and history of febrile seizures (OR: 4.97, 95% CI: 1.06–23.36) were independent risk factors for poor response to initial therapy (p<0.05). Response to AEDs was not associated with the presence of focal slowing or generalized epileptiform discharges on EEG, abnormalities on MRI of the brain, frequency of afebrile seizures before drug therapy, or family history of febrile seizures or epilepsy. Conclusions: This study revealed that 28% of patients with BCECTS experienced poor responses to initial AED therapy. Factors predicting poor response to the initial AED included onset of seizures prior to age 5 and history of febrile seizures.

Unilateral occipital condylar dysplasia: 3‐dimensional multidetector CT and MR findings

Occipital condylar dysplasia is a rare congenital anomaly that is caused by failure to form lateral elements of the 4 occipital sclerotome from the 4 week of gestation (Tubbs et al. 2013). This flattened and underdeveloped condyle occurs as an isolated anomaly or as part of complex Atlanto-occipital abnormality (Shapiro and Robinson 1976). Although its clinical manifestations are headache, stiff neck, torticollis, ataxia, and neurological signs associated with cervicomedullary compression, this congenital anomaly is difficult to be recognized, due to late onset of symptoms that usually occurs no sooner than the second decades (Kruyff 1965; Shapiro and Robinson 1976). Therefore, imaging examination is mandatory for its diagnosis. A 6-year-old girl underwent brain magnetic resonance imaging (MRI) to evaluate a recent episode of lateral deviation of the left eyeball. Contrast-enhanced brain MR images showed displaced medulla oblongata and upper cervical cord by a bony protrusion into the foramen magnum (Fig. 1a,b). Multidetector computed tomography (CT) with three-dimensional reconstruction revealed that the left occipital condyle is asymmetrically small as compared with the right one, so the craniovertebral junction (CVJ) was tilted with the SchmidtFisher angle of 130° which is no more than 125° in normal CVJ. The bony protrusion on MR was the medial part of the left occipital condyle, which was due to the pressure by atlas, leading to displaced medulla, tilting of atlas and axis, and atlanto-axial subluxation (Fig. 1c–e). Due to the tilting atlas, the course of the left vertebral artery and the canal for the left hypoglossal nerve were aberrant and elongated, as compared with the normal contralateral ones (Fig. 1f). Considering the lateral displacement of brainstem and cervical cord, we are planning an operation to decompress the deformity in order to prevent cord compression and attain stability of CVJ. Craniovertebral junction is comprised of occiput, atlas, axis, and supporting ligaments (Smoker 1994). Anatomically, this articulation surrounds the cervicomedullary junction, including medulla oblongata, spinal cord, and lower cranial nerves (Smoker 1994; Tubbs et al. 2013). Functionally, CVJ keeps movement of the head on the neck and the stability of the vertebrae, thus protecting the spinal cord (David et al. 1998). As a part of CVJ, occipital condyles articulate with the superior faces of the atlas to form the atlanto-occipital joints that contribute to the flexion and extension of the head and neck (Tubbs et al. 2013). Thus, occipital condylar hypoplasia can lead to instability of CVJ and compression of the cervicomedullary junction as well as adjacent vascular structures, resulting in various neurological disorders such as ataxia, spastic quadriparesis and lower cranial nerve palsies (Kruyff 1965). However, symptoms usually begin insidiously and generally occur near adolescence when the axial growth is accelerated (Ryken & Menezes, 1993). In our case, the symptom that made the patient visit the hospital was lateral deviation of the left eye. In addition, she had developmental delay, dysarthria, and left leg disturbance for a while without being evaluated. However, we could not identify any relations between these symptoms and the cervicomedullary displacement due to occipital condylar anomaly. Physical examination revealed torticollis that was so subtle that her parents did not even notice it before. We concluded that the left occipital condylar dysplasia in this case was an incidental finding not associated with the seizure, developmental delay, dysarthria, and shuffled leg of the patient. Since Kruyff first introduced plain radiographs of occipital dysplasia in 1965, CT is considered as the best modality to evaluate the articulations of CVJ and to investigate occipital condylar hypoplasia (Kruyff 1965; Ilkko et al. 1998). Currently, with the widespread availability of multidetector CT, we can obtain state-of-the art 3D images of CVJ, as shown in this case. In addition, MR can reveal detailed features of brainstem, spinal cord, lower cranial nerves, and ligaments in multiple orthogonal planes. In conclusion, occipital condylar dysplasia is a rare congenital anomaly that can occur in CVJ as an isolated anomaly or as part of a syndrome. Since its symptoms are diverse with late manifestation, physicians should be aware of imaging features of congenital abnormalities in CVJ. 3D CT and MR features can demonstrate detailed articulation in CVJ. A visualization of neurovascular structures surrounding CVJ is essential for surgical planning.

Parents’ Subjective Assessment of Effects of Antiepileptic Drug Discontinuation

Background and Purpose: Many parents express worries about potential negative side effects of antiepileptic drugs (AED) on cognition, behavior, mood, and academic achievement. We aimed to evaluate parents’ subjective feelings about cognitive or behavioral changes in their children and their quality of life after antiepileptic drug (AED) discontinuation. Methods: A modified questionnaire based on the Korean-Quality of Life in Childhood Epilepsy and the Korean-Child Behavior Checklist was answered by parents whose children were seizure-free over the course of 1 month after AED discontinuation. All children were seizure-free for at least 2 years before AED withdrawal. Results: Fifty-eight eligible patients (mean age, 14.1 ± 4.5 years) were examined. Except valproate in cognition (p = 0.03), parents did not feel significant change after discontinuation of different drugs. They felt improvement of behavior in generalized epilepsy (p = 0.04) and better quality of life in children less than 6 year of age at diagnosis of epilepsy (p = 0.02). Conclusions: We propose that factors such as earlier age at diagnosis of epilepsy or type of epilepsy might influence parents’ subjective feelings about their children’s well-being after drug discontinuation, rather than the drug itself.