A Case of Neonatal Meningitis Caused by Streptococcus dysgalactiae subspecies dysgalactiae and Herpes Simplex Virus

Streptococcus dysgalactiae has two main subspecies: S. dysgalactiae subsp. equisimilis (SDSE) and S. dysgalactiae subsp. dysgalactiae (SDSD). SDSE often colonizes and causes infections in humans; however, SDSD is an animal pathogen which often causes pyogenic infection in domestic animals. We present a case of meningitis with SDSD and herpes simplex virus in a 22-day-old newborn baby who had no exposure to animals.

Effects of Anticonvulsant Monotherapy on Bone Mineral Density in Children / 대한소아신경학회지

PURPOSE: The use of anticonvulsants can cause side effects such as reduction of bone mineral density, requiring attention in growing children. The aim of our study is to investigate the effects of different anticonvulsants on bone mineral density in epileptic patients treated with monotherapy. METHODS: We retrospectively reviewed medical records of 60 subjects who visited the Pediatric Epilepsy Clinic of Bucheon St. Mary's Hospital from January 2013 to December 2017. Bone mineral density was measured with dual photon absorptiometry every 6 months. RESULTS: The number of patients treated with oxcarbazepine, valproate and levetiracetam was 31, 16 and 13, respectively. Reduction of bone mineral density was seen in 8 out of 31 patients (25.8%, P=0.10) treated with oxcarbazepine, 9 out of 16 patients treated with valproate (56.3%, P=0.04) and 4 out of 13 patients treated with levetiracetam (30.8%, P=0.50). CONCLUSION: There was a significant reduction of bone mineral density in patients treated with valproate compared to the other anticonvulsants in our study. We believe attention to bone mineral density is required in children treated with anticonvulsants.

Neurocognitive Outcome in Survivors of Childhood Acute Lymphoblastic Leukemia: Experience at a Tertiary Care Hospital in Korea

This study was conducted to investigate long-term neurocognitive outcomes and to determine associated risk factors in a cohort of Korean survivors of childhood acute lymphoblastic leukemia (ALL). Forty-two survivors of ALL were compared with 42 healthy controls on measures of a neurocognitive test battery. We analysed potential risk factors (cranial irradiation, sex, age at diagnosis, elapsed time from diagnosis, and ALL risk group) on neurocognitive outcomes. ALL patients had lower, but non-significant full-scale intelligence quotient (FSIQ, 107.2 +/- 12.2 vs. 111.7 +/- 10.2), verbal intelligence quotient (VIQ, 107.7 +/- 13.6 vs. 112.2 +/- 11.4), and performance intelligence quotient (PIQ, 106.3 +/- 14.2 vs. 110.1 +/- 10.7) scores than healthy controls. However, patients treated with cranial irradiation performed significantly lower on FSIQ (102.2 +/- 8.1), VIQ (103.3 +/- 11.7), and PIQ (101.4 +/- 13.2) compared to non-irradiated patients and healthy controls. ALL patients also had poor attention, concentration, and executive functions. Among ALL survivors, cranial irradiation was a risk factor for poor FSIQ, being male was a risk factor for poor PIQ, and younger age was a risk factor for poor attention. Therefore, the delayed cognitive effects of ALL treatment and its impact on quality of life require continuing monitoring and management.

Outcomes of the Monoconversion to Valproate or Lamotrigine for Absence Seizures / 대한소아신경학회지

PURPOSE: Ethosuximide (ESX) is currently not available due to various reasons in Korea. The aim of this study is to compare the efficacy of valproate (VPA) and lamotrigine (LTG) when ESX monotherapy was replaced by VPA or LTG. METHODS: A retrospective study was done for a total of 34 patients treated with ESX in 5 different hospitals affiliated with Catholic University of Korea from January, 2010 to December, 2012. They all were initially treated with ESX, but later switched to VPA or LTG. The subjects were selected based on clinical symptoms and electroencephalography findings. RESULTS: Among 34 patients, VPA was prescribed to 17 patients (50.0%) and LTG to 17 patients (50.0%). Twenty patients (58.8%) achieved the seizure freedom after 3 months of the treatments, 13 patients (76.5%) by VPA and 7 (41.2%) by LTG respectively. Four patients (23.5%) with VPA and 10 (58.8%) with LTG were replaced by other anticonvulsants due to ineffectiveness and/or side effects of medication. When we compare the efficacy of seizure reduction between VPA and LTG after 3 month period of the treatment, the efficacy of VPA was better than that of LTG (P=0.04). CONCLUSION: The results of this study suggest that the VPA is a better alternative anticonvulsant than LTG for the patients with absence epilepsy who are unable to continue ESX.

Articulation error of children with adenoid hypertrophy / 소아과

PURPOSE: Adenoid hypertrophy is a physical alteration that may affect speech, and a speech disorder can have other negative effects on a child's life. Airway obstruction leads to constricted oral breathing and causes postural alterations of several oro-facial structures, including the mouth, tongue, and hyoid bone. The postural modifications may affect several aspects of speech production. METHODS: In this study, we compared articulation errors in 19 children with adenoid hypertrophy (subject group) to those of 33 children with functional articulation disorders independent of anatomical problems (control group). RESULTS: The mean age of the subject group was significantly higher (P=0.016). Substitution was more frequent in the subject group (P=0.003; odds ratio [OR], 1.80; 95% confidence interval [CI], 1.23-2.62), while omission was less frequent (P<0.001; OR, 0.43; 95% CI, 0.27-0.67). Articulation errors were significantly less frequent in the palatal affricative in the subject group (P=0.047; OR, 0.25; 95% CI, 0.07-0.92). The number of articulation errors in other consonants was not different between the two groups. Nasalization and aspiration were significantly more frequent in the subject group (P=0.007 and 0.014; OR, 14.77 and 0.014; 95% CI, [1.62-135.04] and NA, respectively). Otherwise, there were no differences between the two groups. CONCLUSION: We identified the characteristics of articulation errors in children with adenoid hypertrophy, but our data did not show the relationship between adenoid hypertrophy and oral motor function that has been observed in previous studies. The association between adenoid hypertrophy and oral motor function remains doubtful.

Characteristics of Children with Epilepsy and Concomitant Attention-Deficit Hyperactivity Disorder / 대한소아신경학회지

PURPOSE: To investigate the prevalence of ADHD (attention-deficit hyperactivity disorder) in children with epilepsy and to determine the difference of characteristics in patients with epilepsy and concomitant ADHD as opposed to the patients without ADHD for better management. METHODS: We retrospectively reviewed 184 patients diagnosed as epilepsy and treated with antiepileptic drugs in pediatric neurology department of Seoul St. Mary's hospital from March, 2009 to May, 2012. Their ages ranged from 6 to 18 years. The subjects were included in the study those who made a regular visit for more than a year. RESULTS: 1) Out of 184 patients, 69 patients (37.5%) had both ADHD and epilepsy. 2) In epilepsy children with ADHD, male outnumbered female by almost two fold (male 67: female 33) (P=0.022). 3) In epilepsy children with ADHD, epileptiform discharges on EEG was focused in central regions in 39% of them (P=0.014). 4) In 56% of patients without ADHD, their seizures remained under the control with single anticonvulsant, as opposed to 36% of patients with both ADHD and epilepsy (P=0.001). Therefore, the presence of ADHD in patients with epilepsy might be related to the therapeutic response to anticonvulsants, and be a useful predictive factor for the response to early treatment. CONCLUSION: Patients with epilepsy and concomitant ADHD showed a significant difference and poor response to epilepsy treatment, as opposed to patients without ADHD. Therefore, early detection and establishment of countermeasures for ADHD is necessary.

Clinical Aspects of Children with Developmental Stuttering / 대한소아신경학회지

PURPOSE: The purpose of this study was to evaluate the clinical characteristics of developmental stuttering. METHODS: We retrospectively reviewed 28 children diagnosed with developmental stuttering from January 2001 to December 2010 who had been admitted to the Uijeongbu St. Mary's Hospital. RESULTS: We observed a strong male predominance. The M:F ratio was 13:1 in this study. And the onset age converged on 2-5 years (71.4%). Seven patients (25%) had a family history of language disorders. Among them, 5 cases (17.8%) was about to developmental stuttering. As for associated disorders, 5 patients (17.8%) had another language disorder as articulation disorder or developmental language delay; respectively 4 patients (14.2%) and 1 patient (3.6%). Three patients (10.7%) had psychologic disorder as ADHD or anxiety disorder; respectively 2 patients (7.1%) and 1 patient (3.6%). In addition, 2 patients (7.1%) had ankyloglossia including 1 case accompanying with articulation disorder. Another 2 patients (7.1%) had adenoid-hypertrophy also including 1 case accompanying with articulation disorder. The proportion of moderate-to-severe and severe cases was 63.6%. And we observed remarkable improvement of stuttering in 92.9% (13/14) patients during repetitive speech or text reading. CONCLUSION: Several distinctive characteristics of developmental stuttering were observed in this study. The value of this study is that it's the clinical report on developmental stuttering by pediatrician and we expect this study will contribute to the basis of forward investigation.

Diagnostic Yield of Pediatric Colonoscopies Based on Presenting Symptoms in a Secondary Hospital / 대한소아소화기영양학회지

PURPOSE: We performed this study retrospectively to review the diagnostic yield of colonoscopies in children and adolescents with various gastrointestinal symptoms and to investigate the relationship between presenting symptoms and the colonoscopic findings in a secondary hospital. METHODS: We reviewed the medical records of patients under the age of 19-years who underwent ileocolonoscopy between January 2001 and December 2010. The total number of patients (n=238) were divided into three age groups and six symptom groups. We analyzed clinical characteristics and the colonoscopic findings, and compared the colonoscopic yield between each groups. RESULTS: The median age of the patients was 16.1 (3.1~18.9) years. The most common presenting symptoms were lower gastrointestinal (GI) bleeding (48.1%) in the or =16 years group (n=126). Positive colonoscopic findings were found in 21.4% of the bowel habit change group (n=28), 51.9% of the low GI bleeding group (n=54), 37.7% of the chronic diarrhea group (n=69), and 94.4% of the group with suspected inflammatory bowel disease (IBD) (n=18), 38.9% of the chronic abdominal pain group (n=54) and 13.3% of the anemia group (n=15). The diagnostic yield of the total examination was 42.0%. The suspected IBD group had a higher yield than the presenting symptom groups (p<0.001). CONCLUSION: Colonoscopy is a safe and useful investigation in children and adolescents with suspected colonic disease. The diagnostic yield of colonoscopy is higher in patients presenting with suspected IBD. Pediatricians practicing in primary or secondary care settings should recommend colonoscopy for patients with suspected IBD.

A case of idiopathic pulmonary hemosiderosis with seasonal recurrence / 소아과

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease affecting mostly children. This disorder is characterized by recurrent episodes of hemoptysis, bilateral diffuse pulmonary infiltrates, and iron-deficiency anemia. An acute fulminant alveolar hemorrhage can be fatal due to respiratory failure, while chronic hemorrhage leads to hemosiderin-laden macrophages and pulmonary fibrosis. Genetic, autoimmune, allergic, environmental, and metabolic mechanisms of pathogenesis have been suggested, but the etiology of IPH remains unknown. We report on a 9-year-old girl with idiopathic pulmonary hemosiderosis who showed seasonal recurrences without cause.

Performance effectiveness of pediatric index of mortality 2 (PIM2) and pediatricrisk of mortality III (PRISM III) in pediatric patients with intensive care in single institution: Retrospective study / 소아과

PURPOSE: To investigate the discriminative ability of pediatric index of mortality 2 (PIM2 ) and pediatric risk of mortality III (PRISM III) in predicting mortality in children admitted into the intensive care unit (ICU). METHODS: We retrospectively analyzed variables of PIM2 and PRISM III based on medical records with children cared for in a single hospital ICU from January 2003 to December 2007. Exclusions were children who died within 2 h of admission into ICU or hopeless discharge. We used Students t test and ANOVA for general characteristics and for correlation between survivors and non-survivors for variables of PIM2 and PRISM III. In addition, we performed multiple logistic regression analysis for Hosmer-Lemeshow goodness-of-fit, receiver operating characteristic curve (ROC) for discrimination, and calculated standardized mortality ratio (SMR) for estimation of prediction. RESULTS: We collected 193 medical records but analyzed 190 events because three children died within 2 h of ICU admission. The variables of PIM2 correlated with survival, except for the presence of post-procedure and low risk. In PRISM III, there was a significant correlation for cardiovascular/neurologic signs, arterial blood gas analysis but not for biochemical and hematologic data. Discriminatory performance by ROC showed an area under the curve 0.858 (95% confidence interval; 0.779-0.938) for PIM2, 0.798 (95% CI; 0.686-0.891) for PRISM III, respectively. Further, SMR was calculated approximately as 1 for the 2 systems, and multiple logistic regression analysis showed chi-square(13)=14.986, P=0.308 for PIM2, chi-square(13)=12.899, P=0.456 for PRISM III in Hosmer-Lemeshow goodness-of-fit. However, PIM2 was significant for PRISM III in the likelihood ratio test chi-square(4)=55.3, P<0.01). CONCLUSION: We identified two acceptable scoring systems (PRISM III, PIM2 ) for the prediction of mortality in children admitted into the ICU. PIM2 was more accurate and had a better fit than PRISM III on the model tested.

Clinical Manifestation of Primary Headache with Epigastric Pain or Tenderness in Children / 대한소아신경학회지

PURPOSE: Population-based studies have shown positive associations between migraine and irritable bowel syndrome, colitis and peptic ulcer and migraine prevalence was higher among patients with dysmotility-like dyspepsia or nausea/vomiting. The aim of this study was to investigate clinical manifestation between primary headache with epigastric pain or tenderness(EPT) and primary headache without EPT. METHODS: We retrospectively reviewed the medical records of 58 patients who were diagnosed primary headache[by ICHD-II(2004)] at Incheon St. Mary Hospital from January, 2006 to December, 2007. Their clinical characteristics such as age, sex, frequency & severity of headache and associated symptoms & signs were analysed. RESULTS: The rate of headache associated with EPT were 36% of migraine cases, 50% of tension-type headache cases, and 100% of unclassified headache cases. Headache with EPT were at a high rate in female. Headache with EPT were more severe than one without EPT. Regulation of behavior, diet and sleep pattern had improved severity of headache in 71% of migraine without EPT and 94% of tension-type headache without EPT, but in 12% of migraine with EPT and 18% of tension-type headache with EPT. Headache disappeared in 64% of migraine with EPT and 53% of tension-type headache by additional regular antiacid medication. CONCLUSION: Our study supports any specific correlation between headache and EPT, but further studies are needed.

Brain-derived Neurotrophic Factor(BDNF) SNP 6265 Polymorphisms in Febrile Seizure and GEFS+ / 대한소아신경학회지

PURPOSE: Febrile seizure(FS) is one of the most common neurological conditions during childhood, but the pathogenesis of FS remains ambiguous. Various studies have shown that brain-derived neurotrophic factor(BDNF) increased neuronal excitability. In this study, to determine whether the polymorphisms of SNP 6265 within the gene encoding BDNF are associated with susceptibility to FS, the frequencies of the polymorphisms were investigated in children with FS and control subjects. In addition, we analyzed the SNP 6265 polymorphisms in Generalized epilepsy with febrile seizures plus (GEFS+) that hasn't been studied as yet in Korea. METHODS: A total of 79 children selected throughout a collaborative study of Catholic Child Neurology Research Group were divided into three groups: (1) FS(n=30); (2) GEFS+ (n=19); (3) control subjects(n=30). Genotypes and allelic frequencies for the polymorphisms of SNP 6265 located at nucleotide 196 was analyzed and compared among the groups. RESULTS: In this study, proportions for A homozygote, A/G heterozygote and G homozygote for BDNF were as follows: in FS, 46.7%, 36.7% and 16.7%, in GEFS+, 26.3%, 47.4% and 26.3% and in control subjects, 60.0%, 16.7% and 23.3%. The allele A and G frequencies for BDNF in FS were 65.0% and 35.0%, in GEFS+ were 50% and 50%, and in control subjects were 68.3% and 31.7%. However, these differences in genotype proportions and allele frequencies among three groups were not significant. CONCLUSION: These results suggest that genomic variations of BDNF might not be the susceptibility factor for FS and GEFS+ in Korean population.

The Clinical Manifestations and the Effect on Prognosis of Seizures during Acute Lymphoblastic Leukemia Treatment in Children / 대한소아신경학회지

PURPOSE: This study was designed to assess the clinical manifestations and the effect on prognosis of seizures in children with acute lymphoblastic leukemia(ALL). METHODS: The study group consisted of 20 patients(10 males and 10 females) who experienced seizures out of the 198 pediatric ALL patients(117 males and 81 females) who were diagnosed and treated at the Department of Pediatrics, St. Mary's Hospital, the Catholic University of Korea. RESULTS: The overall incidence of seizure developing after the diagnosis of ALL was 10.1% and in 11 patients(5.6%), seizure recurred one or more times. An average of 6.5+/-3.6 months(range 0-42 months) elapsed between the beginning of treatment and the new onset of seizures. In 13 patients(65%), the first seizure occurred during the induction or re-induction chemotherapy phase. Seizure type was partial seizure in 11 patients(55%), and generalized seizure in 9 patients(45%). In 15 patients(88.2%), brain imaging study showed abnormal findings and in 18 patients(90%), EEG revealed abnormal findings. 11 patients were diagnosed with epilepsy and were treated with long-term anticonvulsants. In these patients, 4 patients(36.4%) had no seizure recurrence, but 2 patients(18.2%) showed no response to anticonvulsants. The 5-year survival rate of the patients experiencing seizures was 47%, while the rate was 78% for those did not experience seizures (P<0.001). CONCLUSION: Seizures in pediatric patients with ALL was closely related to the 5-year survival rate. Therefore, these patients require early careful observation, evaluation and intensive care. Also, further studies such as ways to diminish the side effects of antileukemic agents are necessary to reduce the risk of seizure.

An analysis of one-year experience of pediatric observation unit: The first report in Korea / 소아과

PURPOSE: While pediatric observation units (POU) have become a common practice in hospitals throughout developed countries, there has been no report about POUs in Korea so far. The aims of this study were to analyze our one-year's experience of the POU and to decide which disease entities are suitable for the POU. METHODS: All children admitted from March 2006 to February 2007 to the POU at the Department of Pediatrics in Our Lady of Mercy Hospital were included in this study. Data were collected from retrospective reviews of their medical records. RESULTS: There were a total of 1,076 POU admissions. Median age of patients was 2.4 years and median length of hospital stay 14.0 hours. The most common diagnoses were gastroenteritis (42.7%), pharyngotonsillitis (19.1%), bronchiolitis (7.8%), pneumonia (5.5%) and febrile seizure (5.2%). Overall, 7.5% of the POU patients required subsequent inpatient admissions due to hospital stays of longer than 48 hours. The disease entities that were most likely to require inpatient admission were pneumonia (17.0%), febrile seizure (12.5%) and asthma (11.5%). Diseases that allowed successful discharge from the POU were gastroenteritis (4.6%), upper respiratory tract infection (5.8%), such as otitis media and pharygnotonsillitis and seizure disorder (6.4%). Compared with the previous year when the POU was not in operation, there was a statistically significant reduction in the average length of hospital stays (from 4.69 to 3.75 days), as well as a rise in the bed turnover rate (from 78.8 to 98.2 patients/ bed). CONCLUSION: Our study shows that the POU is efficient for the management of children with certain acute illnesses. Based on this study, we suggest that the POU be used as a new modality which links between the outpatient, inpatient, and emergency departments in the field of pediatrics in Korea.

Single Nucleotide Polymorphisms of GABRG2 in Idiopathic Generalized Epilepsies(IGEs) / 대한소아신경학회지

PURPOSE: Mutations in gamma-aminobutyric acid(GABA) A receptor gamma2 subunit gene (GABRG2) were independently identified in families of generalized epilepsy with febrile seizures plus(GEFS+) and families of absence epilepsy and febrile seizures(FSs). The present study assessed the role of GABRG2 gene in idiopathic generalized epilepsies(IGEs) of Korean population. METHODS: Twenty-three IGEs and 94 healthy control subjects were selected through a collaborative study of Catholic Child Neurology Research Group. The SNP211037 of GABRG2 were screened by DHPLC. DNA fragments showing variant chromatograms were subsequently sequenced. Genotypes and allelic frequencies for GABRG2 gene polymorphism in three groups were compared. RESULTS: Genotypes and allelic frequencies of the gamma2 subunit of the GABA receptor gene(SNP211037) in both groups were not significantly different. The most common genotypes for GABRG2(SNP211037) gene in both groups were T/C heterozygote. The allele C and T frequencies for GABRG2(SNP211037) in the IGEs group were 45.7% and 54.3%, respectively and in healthy control group, 42.6% and 57.4%, respectively. The number of individuals with the GABRG2 (SNP211037)-C/C genotype in the IGEs group was greater compared with that in the healthy control group(21.7% versus 12.8%). The odds ratio for developing IGEs in individuals with the GABRG2 (SNP211037)-CC genotype was 1.65 compared with individuals with the GABRG2 (SNP211037)-T/T genotype, which was not significantly different. CONCLUSION: These data suggest that genomic variations of GABRG2 might not be one of the susceptibility factors for IGEs in the Korean population.

Clinical Features of Moyamoya Disease in Children / 대한소아신경학회지

PURPOSE: Moyamoya disease, is a chronic progressive cerebrovascular occlusive disorder that can lead to permanent neurologic deficit. The aim of this study is to analyze clinical features, radiologic findings, and prognosis of Moyamoya disease comparing with previous studies. METHODS: We retrospectively reviewed on the clinical data of 26 patients and they were analyzed in terms of age, sex, clinical features, radiologic findings, treatment and prognosis. RESULTS: The male to female ratio was 1:1.6 and the mean age was 9.3 years(male 6.1 years and female 10.9 years). There was a higher occurrence rate in 11-15 year-old group, compared to the previous studies. The most common symptoms at onset were hemiparesis and hemiplegia. Brain infarction, hemorrhage and seizure were found in five, three and two patients on brain imaging respectively. Eight patients(44%) showed reduced perfusion without structural abnormalities on brain perfusion CT. The internal carotid artery was the most common site of occlusion on conventional angiography. Indirect encephalo-duro-arterio-synangiosis was performed in 76.9% of the patients. One patient(3.85%) died and twelve patients(46.15%) showed neurologic sequelae at the time of discharge. Sequelae included hemiplegia, gaze disturbance, facial palsy, and seizures. CONCLUSION: Compared with previous studies, the age at onset was higher in our patients, with a peak incidence after the age of 10. Lower rate of severe neurologic sequelae, such as hemiparesis and hemiplegia was observed in this study. Because this study was limited by the small number of patients included, further investigations such as multi-center prospective analysis is needed.

A Case of Pulmonary Sarcoidosis in a 6-year-old Girl / 소아알레르기및호흡기학회지

Sarcoidosis is a rare multisystemic granulomatous disease. The lungs, lymph nodes, eyes, skin and liver are the most commonly involved. There are two distinct forms of sarcoidosis in children. One is early-onset(younger) childhood sarcoidosis characterized by skin rash, uveitis, and arthritis occurring before age 4 years. The other is late-onset(older) childhood sarcoidosis characterized by involvement of the lungs, the common radiographic finding is bilateral hilar lymph node enlargements, similar to the adult manifestation. We report a case of late-onset childhood sarcoidosis in a 6-year-old girl which is the first pediatric pulmonary sarcoidosis reported in Korea.

The Clinical Manifestations and the Risk Factors of Seizures after Hematopoietic Stem Cell Transplantation in Children / 대한소아신경학회지

PURPOSE:This study was designed to assess the clinical manifestations and the risk factors of seizures after hematopoietic stem cell transplantation(HSCT) in children. Also we analyzed the 3-year survival rates of those who experienced such seizures and those who did not. METHOD:The study group consisted of 28 patients(21 males and, 7 females) who experienced seizures out of the 197 patients(113 males and, 84 females) who underwent HSCT at St. Mary's Hospital HSCT Center of the Catholic University of Korea. RESULTS:The overall incidence of seizures developing after HSCT was 13.8%, with boys reporting a higher incidence than girls. No significant difference was found with regards to the onsets and the types of seizures. In terms of the possible risk factors for seizures, the age of the patient and the stage of acute graft versus host disease(aGVHD) were statistically significant. The patients who were 5 years or more showed a 4.2 times greater incidence rate of seizures(P=0.025) than those who were younger. Also, the patients with stage 2-4 aGVHD showed a 2.77 times greater incidence of seizures(P= 0.034) than those with stage 0-1 aGVHD. The 3-year survival rate of the patients experiencing seizures was 37+/-18%, while the rate increased to 67+/-8% for those who did not(P< 0.001). CONCLUSION:Among the recipients of HSCT in children, the patients aged 5 years or older and aGVHD of the stage 2 or above showed a greater likelihood of seizures occurring after HSCT. The patients who experienced seizures should undergo a close observation and an intensive care since those patients reported a lower 3-year survival rate than those who did not.

Interleukin-1beta Promoter Polymorphisms in Febrile Seizures and GEFS+ / 대한소아신경학회지

PURPOSE: Studies gave conflicting results as to the association between febrile seizures(FSs) and IL1B promoter polymorphisms. In the present study, to determine whether or not the function-related two single nucleotide base C/T biallelic polymorphisms in the promoter region at positions -31 and -511 of the IL1B gene are associated with susceptibility to FSs, the frequencies of the polymorphisms were investigated in children with FSs and GEFS+, and normal control subjects. METHODS: 72 FSs, 23 GEFS+ and 174 healthy control subjects were selected throughout a collaborative study of Catholic Child Neurology Research Group. IL1B promoter -31 C/T and -511 C/T genotyping was performed by means of PCR-restriction fragment length polymorphism. RESULTS: The distribution of IL1B -31 genotypes and the frequencies of allele in children with FSs and GEFS+, and healthy control subjects were not significantly different. The distributions of IL1B -31 genotypes(CC, CT, TT) are 22.2%, 50%, and 27.8% in children with FSs, 21.7%, 43.5% and 34.8% in children with GEFS+, and 27.6%, 49.3% and 24.1% in healthy control subjects. The distribution of IL1B -511 genotypes and the frequencies of allele in children with FSs and GEFS+, and healthy control subjects were not significantly different. The distributions of IL1B -511 genotypes(CC, CT, TT) are 23.6%, 47.2%, and 29.2% in children with FSs, 26.1%, 39.1% and 34.8% in children with GEFS+, and 27.6%, 49.3% and 24.1% in healthy control subjects. CONCLUSION: Theses data suggest that genomic variations of IL1B promoter might not be one of the susceptibility factors for FSs in the Korean population.

Gelastic Seizure with Ataxic Hemiparesis Following Aseptic Meningitis / 대한소아신경학회지

Gelastic seizure(ictal laughter) is quite rare and associated with variable etiologies. The relationship between infection and gelastic seizure is not clear. The seizure usually begins in infancy or childhood and may be accompanied with precocious puberty and cognitive decline. It is known to be poorly responded to medical or surgical treatments. A 5-year-old boy diagnosed and aseptic meningitis visited our hospital again twenty days later because of paroxysmal and frequent involuntary laughing regardless of emotional changes, left side dominant muscle weakness and ataxia with gait disturbance for 5 days. Initial brain magnetic resonance image(MRI) showed no abnormalities but follow-up MRI taken twenty days later showed high signal intensity on right thalamus. The interictal electroencephalogram(EEG) revealed multiple polyspikes over the frontal area. Other etiologies could not be documented. It was suspected that previous aseptic meningitis resulted in gelastic seizure with ataxic hemiparesis. After medical treatment, he acquired good outcomes with a seizure free state.