Cognitive and other neuropsychological profiles in children with newly diagnosed benign rolandic epilepsy / 소아과

PURPOSE: Although benign rolandic epilepsy (BRE) is a benign condition, it may be associated with a spectrum of behavioral, psychiatric, and cognitive disorders. This study aimed to assess the cognitive and other neuropsychological profiles of children with BRE. METHODS: In total, 23 children with BRE were consecutively recruited. All children underwent sleep electroencephalography (EEG) and were assessed on a battery of comprehensive neuropsychological tests including the Korean versions of the Wechsler intelligence scale for children III, frontal executive neuropsychological test, rey complex figure test, Wisconsin card sorting test, attention deficit diagnostic scale, and child behavior checklist scale. RESULTS: The study subjects included 13 boys and 10 girls aged 9.0+/-1.6 years. Our subjects showed an average monthly seizure frequency of 0.9+/-0.7, and a majority of them had focal seizures (70%). The spike index (frequency/min) was 4.1+/-5.3 (right) and 13.1+/-15.9 (left). Of the 23 subjects, 9 showed frequent spikes (>10/min) on the EEG. The subjects had normal cognitive and frontal executive functions, memory, and other neuropsychological sub-domain scores, even though 8 children (35%) showed some evidence of learning difficulties, attention deficits, and aggressive behavior. CONCLUSION: Our data have limited predictive value; however, these data demonstrate that although BRE appears to be benign at the onset, children with BRE might develop cognitive, behavioral, and other psychiatric disorders during the active phase of epilepsy, and these problems may even outlast the BRE. Therefore, we recommend scrupulous follow-up for children with BRE.

Decreased Frequency of Aspiration Pneumonia after Percutaneous Endoscopic Gastrostomy in Neurodisabled Children with Dysphagia / 대한소아신경학회지

PURPOSE: Nasogastric tube or percutaneous endoscopic gastrostomy (PEG) tube can provide a means of feeding when oral intake is not adequate. This study aimed to evaluate the benefits of PEG such as reduced respiratory complications in neurodisabled children with dysphagia. METHODS: Twenty-six neurodisabled patients with dysphagia were followed-up after PEG for at least 12 months from 1999 to 2008. Medical records including characteristics, body weight, frequency of aspiration pneumonia, and grade of gastroesophageal reflux (GER) were reviewed retrospectively between the time before and after PEG. The data collected before PEG was compared with those at 0-6 months and 6-12 months after PEG. RESULTS: The 26 (male 14) enrolled patients had a mean age on PEG of 6.4+/-4.7 (0.9-16.9) years. The body weight percentile of 16 out of 26 patients was under the 3rd percentile. Underlying diseases were cerebral palsy (n=16), acquired brain-injury (n=4), spinal muscular atrophy (n=3), neurodegenerative disease (n=2), and congenital muscular dystrophy (n=1). Body weight was not significantly different before and after PEG. The frequency of aspiration pneumonia was 2.2 times per 6 months before PEG, compared to 0.35 times (0-6 months) and 0.27 times (6-12 months) after PEG, which showed a significant difference (P=0.000). CONCLUSION: The frequency of aspiration pneumonia decreased significantly by PEG in neurodisabled children with dysphagia.

Clinical Analysis of Sleep Disorders in Korean Children / 대한소아신경학회지

PURPOSE: Sleep disorders are one of the most common neurologic conditions in children and adolescents. They have an important impact on quality of their life, such as physical, psychosocial and emotional problems. This study was aimed to investigate clinical and polysomnographic features of sleep disorders in Korean children and adolescents. METHODS: Thirty two patients with sleep disorders were involved in the study. Their medical records and polysomnographs(PSG) were retrospectively reviewed. RESULTS: The mean age was 10.1+/-4.2 years, and the male to female ratio was 2.6. Chief complaints included abnormal movement during sleep(n=12, 37.5%), snoring with sleep apnea(n=9, 28.1%), excessive daytime sleepiness(n=8, 25.0%). Among 12 patients who showed abnormal behaviors during the sleep, 7 patients were diagnosed as parasomnia and 4 patients as epilepsy, 1 as sleep apnea by polysomnographic findings. Eight out of nine patients with sleep apnea and snoring revealed increased apnea-hypopnea index(AHI>1). As a result, 4 patients had tonsillectomy & adenoidectomy(T&A) and 2 patients had uvulopalatopharyngoplasty (UPPP) with good results. Among 8 patients with excessive daytime sleepiness, 4 patients were diagnosed with narcolepsy by PSG and multiple sleep latency test (MSLT). They were treated with modafinil and scheduled nap. CONCLUSION: In this study, we found there are various kinds of sleep disorders in Korean children and adolescents and PSG is still a very important diagnostic tool. However, further studies are needed to elucidate sleep disorders in them.

Clinical Spectrum of Neonatal Strokes / 대한소아신경학회지

PURPOSE: Neonatal strokes are common and may be associated with various complications. However, few studies have been conducted on the clinical spectrum in Korea. This study aimed at investigating the clinical presentation and neurological outcome of neonatal strokes. METHODS: Twenty-seven neonates with neonatal stroke were enrolled in the neonatal intensive care unit at Kyungpook National University Hospital from January 2000 to December 2009. Their medical records and neuroradiological findings were retrospectively reviewed. RESULTS: The mean age of the subjects was 4+/-5.6 days. Sixteen patients were full term, nine were prematurite and six had low birth weights. The onset of symptoms was mostly within first week (85.2%) of life, especially in the first day of life (51.9%). The most common symptom was seizure (40.7%), which were focal clonic (38.5%) or multifocal clonic (38.5%). Nine patients showed abnormal EEG findings. Thirteen patients had subdural hemorrhage, seven showed intraventricular hemorrhage, and three revealed cerebral infarction. Among 12 patients who followed-up for one year, four had mild neurologic dysfunction and two had severe impairment. CONCLUSION: We found that the onset of symptom in neonatal strokes was mostly within the first day of life, and the most common symptom was focal seizure. We, therefore recommend that neuroimaging be done when newborns have seizures within their first week of life. However, further studies are needed to elucidate this further.

Clinical Spectrum and Prognostic Factors of Acute Necrotizing Encephalopathy in Children

This study was conducted to investigate the etiology, the clinical characteristics and prognosis of acute necrotizing encephalopathy (ANE) in Korean children. Six children (1 yr to 7 yr) patients with ANE were enrolled. They were diagnosed by clinical and radiological characteristics and their clinical data were retrospectively analyzed. In a search of clinically plausible causes, brain MRI in all patients, mitochondrial DNA studies for mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) and myoclonus epilepsy and ragged red fibers (MERRF) in four patients, and genomic typing on HLA DRB/HLA DQB genes in three patients were performed. All had precedent illnesses and the main initial symptoms included mental change (83%), seizures (50%), and focal deficits (50%). MRI revealed increased T2 signal density in the bilateral thalami and/or the brainstem in all patients. Mitochodrial DNA studies for MELAS and MERRF were negative in those children and HLA-DRB1*1401, HLA-DRB3*0202, and HLA-DQB1*0502 seemed to be significant. A high dose steroid was given to all patients, which seemed to be partly effective except for 2 patients. In conclusion, ANE is relatively rare, but can result in serious neurological complication in children. Early detection and appropriate treatment may lead to a better neurological outcome.

Long-term Effectiveness and Tolerability of Topiramate in Children with Epilepsy under the Age of 2 Years: 4-Year Follow-up

This is a long-term, open label, observational study aimed to broaden our clinical experiences in managing infants and toddlers with epilepsy. The long-term retention rate and side effects of topiramate (TPM) in them were evaluated and compared with carbamazepine (CBZ). A total of 146 children were involved in the study (TPM=41, CBZ=105). The retention rates at 24 , 36, and 48 months were 46.3%, 34.1%, 26.8% for TPM and 36.2%, 23.8%, 13.3% for CBZ, respectively. At 6 months after starting antiepileptic drugs (AED), the seizure freedom or clinical efficacy (seizure reduction rate more than 50 percent) were 73.2% for TPM and 62.9% for CBZ. The major side effects led to discontinuation included psychomotor slowing, poor oral intake from TPM and sleepiness and skin rash from CBZ. TPM was discontinued due to side effects in one case (2.4%) and lack of efficacy in five cases (12.2%), whereas CBZ was discontinued due to lack of efficacy (22.9%) and side effects (6.7%). As compared with CBZ, TPM showed the same long-term retention rate in children under the age of 2 yr, and no serious side effects. It is therefore concluded that TPM can be considered as a major AED for treating children with epilepsy under the age of 2 yr.

Clinical Effectiveness and Safety of Midazolam Infusion and Topiramate Add-on Therapy against Refractory Status Epilepticus in Children / 대한간질학회지

PURPOSE: This study was aimed to evaluate the effectiveness and safety of midazolam infusion and topiramate add-on therapy for refractory status epilepticus (RSE) in children. METHODS: Nineteen children were involved in the study and their clinical data were retrospectively analyzed. After failure of initial conventional treatment, all of the children were given midazolam (MDZ) infusion, titrated until a response is achieved or maximum dose of 20 mcg/kg/min. Eleven children were given additional topiramate (TPM) at 1-2mg/kg/day by nasogastric and titrated to the favorable response every 1-3 days. We subdivided the children into 2 groups by whether TPM added (TPM group) or not (MDZ group) and compared them each other. RESULTS: The age of subjects ranged from 2 to 175 months (54.9+/-53.3 months; male 10, female 9). Seizure duration of initial presentation ranged from 30 to 1,440 minutes (218.4+/-432.9 minutes). Seizure types were convulsive in 16 children (84%) and 3 were non-convulsive (16%). In the MDZ group (4.0+/-2.9 mcg/kg/minutes), time to seizure control ranged from 1 day to 2 days (1.1+/-0.4 days), but seizures recurred in 50 %. In the TPM group (6.3+/-1.5 mg/kg/day), time to seizure control ranged from 1 day to 5 days (1.9+/-1.5 days), but seizures recurred in 36%. 1 child (5.3%) died of sepsis and 7 children (36.8%) experienced cardio-respiratoty compromise. 7 children (63.6%) in the TPM group recovered completely to baseline neurological function. CONCLUSIONS: The results indicate that midazolam infusion is still the mainstay of treatment, but TPM add-on therapy seems to be a useful option in RSE. However, further studies are needed to establish its precise role and safety.

Assessment of parental understanding of epilepsy and effects of educational programs in an epilepsy camp / 소아과

PURPOSE: To assess parental knowledge and understanding of epilepsy including social stigma and evaluate the effects of educational programs on parents in an epilepsy camp. METHODS: We conducted an epilepsy camp on August 23-24, 2008, at Gyeongsan. Twenty families with an epileptic child each participated in the camp. We performed a survey before and after the camp to obtain data concerning parental understanding of epilepsy and the effects of the camp-based educational programs on the parents. The data were analyzed with SPSS 14.0 using frequency analysis. RESULTS: The parents were sufficiently knowledgeable about epilepsy before the camp, and their knowledge and understanding of epilepsy improved further after the camp. Both perceived stigma and enacted stigma against epilepsy were found in 30.0% of the parents. The perceived stigma decreased to 15.0% after the camp. CONCLUSION: This study suggests that social approaches such as epilepsy camps are effective in improving parental knowledge and understanding about epilepsy as well as decreasing their perceived social stigma.

Assessment of parental understanding of epilepsy and effects of educational programs in an epilepsy camp / 소아과

PURPOSE: To assess parental knowledge and understanding of epilepsy including social stigma and evaluate the effects of educational programs on parents in an epilepsy camp. METHODS: We conducted an epilepsy camp on August 23-24, 2008, at Gyeongsan. Twenty families with an epileptic child each participated in the camp. We performed a survey before and after the camp to obtain data concerning parental understanding of epilepsy and the effects of the camp-based educational programs on the parents. The data were analyzed with SPSS 14.0 using frequency analysis. RESULTS: The parents were sufficiently knowledgeable about epilepsy before the camp, and their knowledge and understanding of epilepsy improved further after the camp. Both perceived stigma and enacted stigma against epilepsy were found in 30.0% of the parents. The perceived stigma decreased to 15.0% after the camp. CONCLUSION: This study suggests that social approaches such as epilepsy camps are effective in improving parental knowledge and understanding about epilepsy as well as decreasing their perceived social stigma.

Clinical evaluation of Reversible Posterior Leukoencephalopathy Syndrome in Korean Children: Multicenter Study / 대한소아신경학회지

PURPOSE: Reversible posterior leukoencephalopathy syndrome(RPLS) is a complex condition affecting gray/white matter of parieto-occipital lobes from multiple and different etiologies. This study was aimed to analyze clinical characteristics of this condition in Korean children. METHODS: A total of 36 patients from tertiary care medical centers were involved in the study. They were diagnosed as RPLS mainly by clinical and radiological manifestations. Their medical records and radiological features of brain MRI were retrospectively analyzed. RESULTS: Thirty six patients were involved in the study(21 males and 15 females, mean age: 9.8+/-4.1 years of age). Main underlying causes included drug induced(47%), renal diseases(28%), neurogenic tumors(8%) and so on. Initial manifestations were seizures, headache, visual disturbance, mental change, and nausea/vomiting. 13 patients(36%) had two or more symptoms. The brain MRI showed typical pattern of fairly symmetric, high T2 signal intensity in both parieto-occipital regions in 23 patients(64%) and various, atypical pattern in 13 patients(36%). 26 patients had the lesions affecting predominantly white matter, but 10 patients had the lesions affecting predominantly gray matter. 34 patients(94 %) had bilateral lesions, but 2 patients(6%) had unilateral lesion. This complex syndrome was associated with acute rise of blood pressure from a variety of conditions. A patient ended up a visual deficit despite the good prognosis. CONCLUSION: RPLS in Korean children seems to be complex, but a better understanding of this complex syndrome will lead to better clinical outcome by avoiding unnecessary investigations and appropriate management.

The changes of electrocardiography and signal-averaged electrocardiography after surgical repair of Tetralogy of Fallot / 소아과

PURPOSE: The purpose of this study was to analyze the changes of the late potential of Signal- averaged electrocardiography (SAECG) and Electrocardiography (ECG) parameters during follow up of those who had taken surgical repair of Tetralogy of Fallot (TOF). METHODS: Nine patients who had taken surgical repair of TOF since 1985 checked SAECG and standard 12 leads ECG twice in 1999 and 2005 in Kyungpook national university hospital. We evaluated changes of QRS duration, QT interval and JT interval, QRS dispersion, QT dispersion and JT dispersion of standard 12 leads ECG and Filtered QRS (f-QRS), High frequency low amplitude potential (HFLA), Root mean square in terminal 40 ms (RMS) and Mean voltage in terminal 40 ms (MV) of SAECG between in 1999 and 2005. RESULTS: There were significant decrease of JT dispersion (101.11+/-50.11 vs 71.11+/-22.61 ms, P< 0.05) and significant increase of HFLA (24.67+/-13.19 vs 32.89+/-14.21 ms, P<0.05). But there were no significant changes in other parameters. CONCLUSION: In repaired TOF patients, we evaluated ECG and SAECG to detect possible late complications such as tricuspid regurgitation, right ventricular enlargement, ventricualar arrythmia and sudden death. And there were significant changes of ECG and SAECG in some parameters (JT dispersion, HFLA). But to see the relationships between the changes of these parameters and the long term prognosis, we need to check more patients and longer follow-up.

Small Intestinal Mucosa Associated Lymphoid Tissue (MALT) Lymphoma in a Child Presenting with Recurrent Intussusception: A Case Report

Mucosa associated lymphoid tissue (MALT) lymphoma is a malignant B cell lymphoma that develops from MALT. The stomach is the most common site of MALT lymphomas but be found anywhere in the body. Adult MALT lymphomas are not rare diseases, but in children, intestinal MALT lymphomas are very rare. We treated a 12-year-old boy who underwent segmental resection of the small intestine due to recurrent intussusceptions, 2 months apart, and was diagnosed with an intestinal MALT lymphoma. He received multiple chemotherapeutic agents (vincristin, cyclophosphamide, prednisone, methotrexate, doxorubicin and cytosine arabinoside) according to the CCG-5961 protocol for 3 months. Currently, he is alive and disease free.

Chromosomal analyses of 4,500 cases of the peripheral blood: An experience in a single hospital for 25 years / 소아과

PURPOSE: Chromosome analysis is important in genetic study and genetic counseling. This study was performed to evaluate the type and incidence of chromosome abnormalities in a single hospital for 25 years. METHODS: Chromosome analyses were performed on peripheral blood lymphocytes, obtained from 4,856 patients with suspected chromosomal aberrations, referred to cytogenetic laboratory in Department of Pediatrics, Kyungpook National University Hospital from May 1981 to October 2005. RESULTS: We analyzed 4,567 cases. Children were 3,014 cases (66.0%) and adult were 1,553 cases (34.0 %). The most common purpose of the chromosomal analysis was growth and developmental abnormality in children and infertility in adults. Total chromosomal aberration rate was 16.9% (770/4,567). Among those cases, the numerical abnormalities were 12.2% (558 cases), the structural abnormalities were 4.1% (187 cases), and others were 0.5% (25 cases). The relative frequencies of autosomal abnormalities were 6.4% (294 cases) in Down syndrome; 0.2% (7 cases) in Edwards syndrome; 0.1% (4 cases) in Patau syndrome; 0.2% (10 cases) in other abnormalities, of sex chromosome, 2.9% (131 cases) in Klinefelter syndrome; 2.2% (99 cases) in Turner syndrome; 0.2% (8 cases) in 47, XXX; 0.1% (3 cases) in 47, XYY. Among the structural abnormalities, translocation was 1.8% (84 cases), inversion was 0.8% (37 cases), deletion was 0.4% (17 cases), and insertion was 0.3% (13 cases), in order of frequency. CONCLUSION: In this study, the type, incidence and distribution of cytogenetic abnormalities by karyotype were reviewed. We hope that our study could be used as a basic information on the diagnosis, treatment and genetic counseling for chromosome abnormalities in Korea.

Clinical and Radiological Analysis of Reversible Posterior Leukoencephalopathy Syndrome in Children / 소아과

clinical and radiologic findings that mainly involve the white/gray matter of the parieto-occipital lobes. The purpose of this study was to determine its clinical and radiological characteristics. METHODS: A total of 15 pateints were involved in the study. Their medical records and radiological features of brain MRI were retrospectively reviewed and analyzed. RESULTS: Fifteen pateints were involved. (9 males and 5 females). The patients' ages ranged from 2-20 years (mean age:10 years). The brain MRI revealed fairly symmetric areas of increased T2 signal involving both white and gray matter of parieto-occipital regions. The condition seemed to be associated with cyclosporin A and steroid therapy or a variety of other conditions in which blood pressure rises acutely. CONCLUSION: Reversible posterior leukoencephalopathy syndrome is a complicated neurological condition, but a better understanding of this complex syndrome may obviate unnecessary investigations and lead to prompt and appropriate management of the associated problems.

Comparison of Endoscopic Removal of Disk Batteries in Children / 대한소아소화기영양학회지

PURPOSE: Any battery impacted in the esophagus must be removed urgently because of the possibility of serious complications such as perforation, fistula, and mediastinitis. The use of endoscopic procedures was compared to find the most rapid, effective, and safe method to remove disk batteries. METHODS: The cases of 24 children (9 males, mean age, 1.5 years) that had accidentally ingested disk batteries were reviewed. The patients had visited the Department of Pediatrics, Kyungpook National University Hospital from July 1997 to June 2007 for the removal of disk batteries. Endoscopic removal was attempted using a retrieval net or a magnetic extractor with a balloon (condom). The procedure times for removing the ingested battery were compared. RESULTS: Children that ingested batteries with a larger diameter (20 mm) had a greater chance to have esophageal impaction with serious injury, such as esophageal mucosal necrosis or ulcers, as compared to the ingestion of smaller diameter batteries (10 mm) (p<0.01). Endoscopic removal of disk batteries was attempted either using a retrieval net in 10 children or by using a magnetic extractor with a balloon in 6 children. Endoscopic removal using a retrieval net was more effective (mean procedure time: 1.5 min) as compared to using a magnetic extractor with a balloon (mean procedure time: 3.3 min) (p<0.05). CONCLUSION: Both procedures, either using a retrieval net or a magnetic extractor with a balloon were effective in removing ingested disk batteries. Based on our experience, an endoscopic procedure using a retrieval net was a more effective method for foreign body removal.

Comparison of Endoscopic Removal of Disk Batteries in Children / 대한소아소화기영양학회지

PURPOSE: Any battery impacted in the esophagus must be removed urgently because of the possibility of serious complications such as perforation, fistula, and mediastinitis. The use of endoscopic procedures was compared to find the most rapid, effective, and safe method to remove disk batteries. METHODS: The cases of 24 children (9 males, mean age, 1.5 years) that had accidentally ingested disk batteries were reviewed. The patients had visited the Department of Pediatrics, Kyungpook National University Hospital from July 1997 to June 2007 for the removal of disk batteries. Endoscopic removal was attempted using a retrieval net or a magnetic extractor with a balloon (condom). The procedure times for removing the ingested battery were compared. RESULTS: Children that ingested batteries with a larger diameter (20 mm) had a greater chance to have esophageal impaction with serious injury, such as esophageal mucosal necrosis or ulcers, as compared to the ingestion of smaller diameter batteries (10 mm) (p<0.01). Endoscopic removal of disk batteries was attempted either using a retrieval net in 10 children or by using a magnetic extractor with a balloon in 6 children. Endoscopic removal using a retrieval net was more effective (mean procedure time: 1.5 min) as compared to using a magnetic extractor with a balloon (mean procedure time: 3.3 min) (p<0.05). CONCLUSION: Both procedures, either using a retrieval net or a magnetic extractor with a balloon were effective in removing ingested disk batteries. Based on our experience, an endoscopic procedure using a retrieval net was a more effective method for foreign body removal.