Rethinking the next step after unexpected results associated with minimally invasive radical hysterectomy for early cervical cancer / 부인종양

No abstract available.

Outbreak of Influenza B related to Benign Acute Childhood Myositis / 대한소아신경학회지

PURPOSE: Benign acute childhood myositis (BACM) is a rare syndrome caused by a viral infection and is characterized by gait disturbance with calf pain in children. I investigated whether BACM is related to the incidences of seasonal influenza and of specific influenza subtypes in Korea. METHODS: Patients diagnosed with BACM between March, 2010 and December, 2014 were investigated retrospectively. The occurrence of BACM was compared with the incidence of influenza for each influenza season, as obtained from the Korea Centers for Disease Control and Prevention. RESULTS: All patients had previous infection preceding calf muscle pain or gait disturbance. Laboratory findings showed elevated levels of creatine kinase (median, 1243 U/L) and leukopenia ( < 4500/µL). Patients exhibited benign courses that resolved within a few days without any complications. The rate of influenza B was correlated with BACM occurrence (P=0.05) although this relationship was not statistically significant. Neither the rate of total influenza nor the rate of influenza subtype A was associated with BACM occurrence. CONCLUSION: The outbreak of influenza B was correlated with the occurrence of BACM. We should recognize the occurrence of this disease and avoid unnecessary evaluation to diagnose BACM when an outbreak of influenza B is expected.

Considerations in Treating Neonatal and Infantile Patients with a Retrocerebellar Arachnoid Cyst: in the Perspective of Different CSF Dynamics / 대한소아신경학회지

Arachnoid cysts found under the age of 1 year are more likely to grow in size, relatively short term follow-up is required. Retrocerebellar location predicts a high risk of hydrocephalus, and the time window until irreversible neuronal damage is often narrow if the arachnoid cyst increases in size and becomes symptomatic. However, when and how to treat a neonatal or infantile patient with a retrocerebellar arachnoid cyst is still a controversial subject. We recently experienced 3 differently treated very young pediatric patients with retrocerebellar arachnoid cysts. One patient was treated two weeks after birth by a cystoperitoneal shunt. This patient showed normal development after the surgery. The other patient was treated by a ventriculoperitoneal shunt and subsequent cystoventriculostomy at the age of 4 months because of his mother's refusal on 14th day after birth. This one showed developmental delay despite of decreasing size of ventricles after the surgery. The last patient was treated with microscopic fenestration, which failed in its initial attempt. A revision operation by cyst excision succeeded and had no problem after the surgery. Therefore, we suggest that early surgical intervention for retrocerebellar arachnoid cyst can be considered. Although there are concerns of long term complications related to shunts, a cystoperitoneal shunt would be a feasible treatment if we consider the minor cerebrospinal fluid pathway which is the dominant cerebrospinal fluid dynamic at this age.

Glucose transport 1 deficiency presenting as infantile spasms with a mutation identified in exon 9 of SLC2A1 / 소아과

Glucose transport 1 (GLUT-1) deficiency is a rare syndrome caused by mutations in the glucose transporter 1 gene (SLC2A1) and is characterized by early-onset intractable epilepsy, delayed development, and movement disorder. De novo mutations and several hot spots in N34, G91, R126, R153, and R333 of exons 2, 3, 4, and 8 of SLC2A1 are associated with this condition. Seizures, one of the main clinical features of GLUT-1 deficiency, usually develop during infancy. Most patients experience brief and subtle myoclonic jerk and focal seizures that evolve into a mixture of different types of seizures, such as generalized tonic-clonic, absence, myoclonic, and complex partial seizures. Here, we describe the case of a patient with GLUT-1 deficiency who developed infantile spasms and showed delayed development at 6 months of age. She had intractable epilepsy despite receiving aggressive antiepileptic drug therapy, and underwent a metabolic workup. Cerebrospinal fluid (CSF) examination showed CSF-glucose-to-blood-glucose ratio of 0.38, with a normal lactate level. Bidirectional sequencing of SLC2A1 identified a missense mutation (c.1198C>T) at codon 400 (p.Arg400Cys) of exon 9.

Peritoneal carcinomatosis mimicking a peritoneal tuberculosis

Symptoms of a peritoneal progression from ovarian cancer are nonspecific such as abdominal pain, abdominal distention and more. Many imaging studies and serum CA-125 help diagnosis. However, it is difficult to exclude the instances of the diffuse peritoneal diseases that mimic carcinomatosis. The elevated CA-125 level usually correlates with the peritoneal carcimatosis, but it is often found in other peritoneal diseases. Therefore, the pathologic confirmation is necessary because of other mimicking diseases. In our case, CA-125 levels were elevated. Abdominal computed tomography finding was suspected a peritoneal tuberculosis but the pathologic result was the peritoneal carcimatosis, eventually.

Quantitative Analysis of Simultaneous EEG Features during PET Studies for Childhood Partial Epilepsy

PURPOSE: To demonstrate the significance of simultaneous electroencephalography (EEG) recording during 2-deoxy-2-[18F] fluoro-D-glucose (FDG)-positron emission tomography (PET) in childhood partial epilepsy. MATERIALS AND METHODS: We included 46 children with partial epilepsy who underwent simultaneous EEG during PET. We compared the epileptogenic area of several EEG features including epileptiform discharges, focal polymorphic slow waves, and electrographic seizures, with the abnormal metabolic region on PET. We also compared the epileptogenic area of simultaneous EEG and PET with findings on magnetic resonance imaging (MRI) and video/EEG, as well as the histopathological diagnosis of the resected cortical area, in eight patients who underwent surgical resection of the epileptogenic area. RESULTS: Hypometabolic regions on interictal PET were concordant with epileptogenic areas of epileptiform discharges and focal polymorphic slow waves, according to their frequency and/or severity, with odds ratios of 1.35 and 1.81, respectively (p<0.05). Hypermetabolic PET was also concordant with epileptogenic areas of ictal events longer than 20 seconds during the period of FDG uptake. Among the eight patients who underwent surgical resection, six patients, including two with non-lesional MRI, had concordant EEG and PET findings, were confirmed pathologically, and became seizure-free after surgery. CONCLUSION: Simultaneous EEG is useful in identifying epileptogenic areas due to a high concordance with abnormal PET metabolic areas. Moreover, simultaneous EEG may also prevent false lateralization of PET from postictal and mixed metabolism during ictal events, as well as abnormal hypermetabolism, during frequent interictal epileptiform discharges.

Development of Purtscher-like retinopathy after pre-eclampsia combined with acute pancreatitis

Visual disturbances are common among women with pre-eclampsia. The obstetricians should have an understanding of the various conditions associated with visual impairments. We report a case of Purtscher-like retinopathy developed after pre-eclampsia combined with acute pancreatitis. A 33-year-old primigravida with twin pregnancy was admitted to the department of obstetrics and gynecology for preterm labor and pre-eclampsia at 36+4 weeks gestation. After a cesarean section, she complained of abdominal pain and visual acuity loss. She was diagnosed with Purtscher-like retinopathy combined with acute pancreatitis after ophthalmologic examination and an abdominal computed tomography scan. Purtscher-like retinopathy, most often observed after trauma, is very rare in pre-eclampsia. In addition, while visual disturbances from other conditions are transient, it can result in persistent visual impairments. Thus, when a patient with pre-eclampsia complains of visual problems for a long period of time, obstetricians should consider an ophthalmologic evaluation and treatments during the earliest stage of the disease.

Secondary paroxysmal dyskinesia associated with 2009 H1N1 infection / 소아과

Neurological complications associated with 2009 H1N1 infection in children have been reported and recognized worldwide. The most commonly reported neurological complications are seizures and encephalopathy. Secondary movement disorders are also associated with the infection, but such cases are rarely reported. Here, we describe the case of a 14-year-old boy with paroxysmal kinesigenic dyskinesia secondary to 2009 H1N1 infection, who presented with dystonia and choreic movement triggered by sudden voluntary movement.

Complete trisomy 14 mosaicism: first live-born case in Korea / 소아과

Trisomy 14 mosaicism is a rare chromosome disorder characterized by delayed development, failure to thrive, and facial dysmorphism. Only approximately 30 trisomy 14 mosaicism cases have been reported in the literature because trisomy 14 is associated with early spontaneous abortion. We report a case of a 17-month-old girl with abnormal skin pigmentation, delayed development, facial dysmorphism, and failure to thrive with the 47,XX,+14/46,XX chromosome complement.

The Effect of Systemic Hypertension on the Pediatric Brain

Hypertension is one of the most common chronic diseases in childhood and adolescence. Untreated hypertension adversely affects many organs including heart, brain, kidney and peripheral arteries. We reviewed the complication of central nervous system caused by pediatric hypertension. Cerebral blood flows are maintained constantly in response to changes in blood pressure by cerebral autoregulation. Severe hypertension which destructs cerebral autoregulation results in acute hypertensive encephalopathy syndrome, ischemic or hemorrhagic stroke. Chronic pediatric hypertension induces learning disability and cognitive defect which are subclinical symptom prior to brain damage caused by severe hypertension. We should consider the effect of hypertension on pediatric brain because appropriate antihypertensive drugs could prevent these complications.

A Case of an Imported Dengue Hemorrhagic Fever with Spontaneous Bleeding: Case Report and Review of the Literature / 소아감염

Dengue is an acute febrile viral disease which is found in tropical and sub-tropical regions around the world. Dengue fever has steadily increased in both incidence and distribution over the past 50 years. Even though Korea is not an endemic country for dengue fever, with the increasing numbers of overseas travelers in Korea, the numbers of imported dengue cases are steadily increasing. Here, we report a case of imported dengue hemorrhagic fever in a Korean child presenting with fever and epistaxis. Dengue fever should be considered if a patient who has a recent travel history to endemic areas showed classical symptoms.

Current Status of ADHD Treatment in Children with Epilepsy: A Retrospective, Multicenter, Cross Sectional Analysis in Korea / 대한소아신경학회지

PURPOSE: The purpose of this study was to assess the current therapeutic status of attention deficit-hyperactivity disorder (ADHD) in children with epilepsy. METHODS: A cross-sectional survey of 178 patients aged 4-20 years from ten pediatric neurology clinics in eight cities in South Korea from January 2005 to July 2010 was used to assess clinical characteristics of ADHD patients with epilepsy and risk factors associated with ADHD. RESULTS: A total of 178 pediatric epileptic patients were recruited for this study. One hundred seventy-four subjects' (M:F=4:1, mean age: 12.2+/-3.3 yrs old) records were evaluated excluding four patients due to incomplete data. One hundred twenty-five of 174 patients (71.8%) had partial epilepsy and 45 had generalized epilepsy. Eighty of 112 patients showed ADHD combined type from the DSM IV. The mean prevalence rate of ADHD treatment among the epileptic patients was 1.9%. Over 45% of patients showed complete or persistent symptoms without difficulties in school life with CNS stimulants. Adverse reactions were reported in 19.8% of patients who received ADHD medication, and 18 patients discontinued ADHD medication due to severe adverse effects such as aggravated seizures (5.6%) or ADHD symptoms (3.7%). About 60% of children with ADHD and epilepsy had psychiatric comorbid disorders. CONCLUSION: The results indicate that ADHD treatment in epilepsy patients is safe and effective. However, these data also show that ADHD in pediatric epilepsy patients in Korea is under-diagnosed and under-treated.

Ictal single-photon emission computed tomography with slow dye injection for determining primary epileptic foci in infantile spasms / 소아과

PURPOSE: We investigated whether ictal single-photon emission computed tomography (SPECT) with prolonged injection of technetium-99m (99mTc) ethyl cysteinate dimer during repeated spasms can localize the epileptogenic foci in children with infantile spasms. METHODS: Fourteen children with infantile spasms (11 boys, 3 girls; mean age, 2.2+/-1.3 years) were examined. When a cluster of spasms was detected during video electroencephalography (EEG) monitoring, 99mTc ethyl cysteinate dimer was slowly and continuously injected for 2 minutes to determine the presence of ictal SPECT. For 7 children, the ictal and interictal SPECT images were visually analyzed, while for the remaining 7 children, the SPECT images were analyzed using the subtraction ictal SPECT coregistered to magnetic resonance imaging (MRI) (SISCOM) technique. Subsequently, we analyzed the association between the ictal SPECT findings and those of other diagnostic modalities such as EEG, MRI, and positron emission tomography (PET). RESULTS: Increase in cerebral blood flow on ictal SPECT involved the epileptogenic foci in 10 cases6 cases analyzed by visual assessment and 4 analyzed by the SISCOM technique. The ictal SPECT and video-EEG findings showed moderate agreement (Kappa=0.57; 95% confidence interval, 0.18-0.96). CONCLUSION: Ictal SPECT with prolonged injection of a tracer could provide supplementary information to localize the epileptogenic foci in infantile spasms.

Diagnosis of neonatal seizures / 소아과

Neonatal seizures are generally not only brief and subtle but also not easily recognized and are usually untreated. In sick neonates, seizures are frequently not manifested clinically but are detected only by electroencephalography (subclinical EEG seizures). This phenomenon of electroclinical dissociation is fairly common in neonates. On the other hand, neonates frequently show clinical behaviors such as stiffening, apnea, or autonomic manifestations that mimic seizures, which is usually associated with underlying encephalopathy and non-epileptic seizures. Therefore, it might be difficult to confirm the diagnosis of neonatal seizures. Early recognition of neonatal seizures is important to minimize poor neurodevelopmental outcomes, including cognitive, behavioral, and learning disabilities, as well as the development of postnatal epilepsy. EEG is a reliable tool in the determination of neonatal seizures. Continuous EEG monitoring is essential for the identification of seizures, evaluation of treatment efficacy, and prediction of the neurodevelopmental outcome. However, there is not yet a wide consensus on the optimal "standard" lead montage for the continuous EEG monitoring.

Diagnosis of neonatal seizures / 소아과

Neonatal seizures are generally not only brief and subtle but also not easily recognized and are usually untreated. In sick neonates, seizures are frequently not manifested clinically but are detected only by electroencephalography (subclinical EEG seizures). This phenomenon of electroclinical dissociation is fairly common in neonates. On the other hand, neonates frequently show clinical behaviors such as stiffening, apnea, or autonomic manifestations that mimic seizures, which is usually associated with underlying encephalopathy and non-epileptic seizures. Therefore, it might be difficult to confirm the diagnosis of neonatal seizures. Early recognition of neonatal seizures is important to minimize poor neurodevelopmental outcomes, including cognitive, behavioral, and learning disabilities, as well as the development of postnatal epilepsy. EEG is a reliable tool in the determination of neonatal seizures. Continuous EEG monitoring is essential for the identification of seizures, evaluation of treatment efficacy, and prediction of the neurodevelopmental outcome. However, there is not yet a wide consensus on the optimal "standard" lead montage for the continuous EEG monitoring.

Two Cases of Benign Non - Familial Neonatal Convulsion / 대한소아신경학회지

Benign idiopathic neonatal convulsion is a rare disorder which has no family history of convulsion and develops before and after the 5th day in a healthy full-term neonate. Its characteristics appear focal, or multifocal clonic seizures but rare tonic seizures lasting about several minutes. It reveals non-specific findings in neurologic examination, neuroimaging and EEG(electroencephalography) so that it should be differentiated from those diseases such as eletronic imbalance, inborn errors of metabolism, other neonatal epileptic syndromes. We report two healthy full-term female neonates presented with multifocal clonic seizures before and after the 5th day after birth. They had no family history of convulsion, fetal asphyxia, fetal and maternal problems and the neurologic examination and neuroimagings were normal. The convulsions were controlled by intravenous phenobarbital injection. They had no more convulsions ever since and showed normal development at the follow-up performed one year later. We experienced a rare disorder, benign neonatal convulsion in healthy full-term neonates. We hope this report will help its diagnosis and treatment and prevent unnecessary long- term anticonvulsant medication.

Analysis on the association between EEG and 2-deoxy-2-18F-D-glucose (FDG)-PET findings in children with epilepsy / 소아과

PURPOSE: We performed EEG and PET on children with epilepsy concomitantly in order to evaluate the effects of epileptiform and non-epileptiform discharge of EEG on glucose metabolism. METHODS: Seventy three children with epilepsy who had PET and EEG simultaneously were included in our study. The subjects were classified in two ways: (1) based on the frequency of epileptiform discharge and (2) the severity of non- epileptiform discharge. We evaluated the clinical aspects of their seizures, the severity of focal slow waves during the interictal period with the frequency of spikes or sharp waves in order to compare with the PET results. RESULTS: The subjects were divided by the frequency of epileptiform discharge, with 13 in the no/rare group, 7 in the occasional group, and 53 children in the frequent group. The concordant rates with PET in each group were 0%, 42.9%, and 67.9%, respectively, showing high correlations with the frequency of epileptiform discharge (P<0.05, r=0.491). The subjects as divided by the severity of non-epileptiform discharge were 15 in the no group, 25 in the infrequent group, 17 in the intermediate group, and 16 in the continuous group. The concurrence rates with PET for each group were 13.3%, 52.0%, 64.7%, and 68.8%, respectively, also showing a high correlation with the severity of non-epileptiform discharge (P<0.05, r= 0.365). CONCLUSION: Epileptiform discharge and non-epileptiform discharge in EEG showed a certain association with hypometabolism in PET. We recommend EEG to reduce false lateralization and to localize lesions in cases of high frequency and severity.

Cortical Dysplasia and Microdysgenesis; Correlations between Clinical Feaures, Neuroimaging and Electrophysiology after Lobectomy for Intractable Epilepsy in Children / 대한소아신경학회지

PURPOSE: The diagnosis of cortical dysplasia(CD) and microdysgenesis(MD) is valuable because they often cause childhood intractable epilepsy. This study is to analyze clinical manifestations, EEG findings, and imaging features based on the pathologic diagnosis(cortical dysplasia and microdysgenesis) in childhood intractable epilepsy with surgical treatment. METHODS: We performed retrospective studies and analyzed 27 patients with MCD diagnosed by pathologic findings after brain lobectomy for intractable epilepsy from October 2003 to September 2006 in our hospital. We compared their clinical characteristics, EEGs, neuroimaging studies including MRI, and FDG-PET. We analyzed the locations of malformations of cortical development(MCD). The surgical outcomes were reviewed. RESULTS: There were no pathognomonic seizure types or EEG findings for microdysgenesis. The clinical and EEG features of microdysgenesis were similar to those of cortical dysplasia. Only 6(37 percent) out of 16 microdysgenesis patients showed normal MRI findings and also 2(18 percent) out of 10 cortical dysplasia patients showed normal MRI findings. The most common location of MCD was frontal lobe in both of the groups, followed by temporal lobe. 10(63%) out of 16 microdysgenesis patients and 9 (90%) out of 10 cortical dysplasia patients became seizure free. The locations of MCD was not related to the prognosis of the outcomes. All the patients who had had developmental delay showed improvement in development after the surgery. CONCLUSION: There were no significant differences in gender, seizure onset age, duration of seizures, seizure types, EEG findings, and MCD locations between CD and MD. The seizure outcomes were better in CD than in MD. All the patients whose pre- and post-oprative developmental status were compared showed developmental improvement.

Clinical and Laboratorial Characteristics of Korean Children with Mitochondrial Respiratory Chain Defect / 대한소아신경학회지

PURPOSE:The study was carried out to characterized the clinical and the laboratorial features of children with mitochondrial respiratoy chain disorders in Korea. METHODS:We retrospectively analyzed the clinical and the loboratorial data of 28 children with significantly low activities in respiratory chain complexes of muscle using spectrophotometry. RESULTS:The mean age was 6.67+/-4.44 years and the ratio males to female was 1.15:1. Eighteen patients (64.3%) showed defects in Complex I, 8 (28.6%) in Complex VI, 1 (3.6%) in Complex II, and 1 in Complex I and IV. Eight cases (28.6%) were diagnosed with Leigh disease, one with MELAS, Kearns-Sayre syndrome, and Alpers disease retrospectively, but the predominant clinical presentations were a nonspecific encephalopathy (17/28, 60.7%). Epilepsy was seen in 21 (75.0%) patients, while developmental delay in 27 (96.4%) patients. Fifteen out of 28 children (53.6%), clinical symptoms mostly appeared below age of 1 year. The brain MRI showed diffuse cortical atrophy in 18 (64.3%) patients and basal ganglia signal changes in 12 (42.9%) patients. CONCLUSION:The defects in mitochondrial respiratory chain complexes should be considered in any children with an unexplained neurological condition including even epilepsy.

Results of Surgical Treatments on Intractable Epilepsy with Infantile Spasm / 대한소아신경학회지

PURPOSE: Infantile spam is a kind of epileptic syndrome causing delayed psychomotor development in children and one of the most common type of epileptic encephalopathy. Some cases of infantile spasm are known to be caused by focal abnormalities of cerebral cortex thus curable by operating them. Here we analyzed the results of surgical treatments on intractable epilepsy with infantile spasm. METHODS: We performed retrospective study on 22 patients with infantile spasm who received epileptic surgery since 1999. Surgical outcome, pathologic findings, localization of lesions, and types of surgery were reviewed. RESULTS: Sex ratio of our 22 cases was 1:1.4(M:F) and the age at surgery was quite variable. The mean duration from diagnosing epilepsy till operation was 6-10 years in 8(36%) cases, 1-2 years in 7(32%). In preoperative EEG, abnormalities implying possible focal lesion were seen in all 22 patients. 7(32%) out of 22 cases showed focal lesions in MRI while 12(92%) out of 13 cases in ictal SPECT, 8(62%) out of 13 cases in interictal SPECT, and 11(61%) out of 18 cases of PET examination showed abnormal findings. Concerning the types of surgery, single lobectomy was most commonly performed, in 13 (59%) cases in which 11 among them received frontal lobectomy. Also, multilobar resection was performed in 6(27%) cases and hemispherotomy in 2(9%) as well. 19(86%) patients were classified as Engel class I after operations. In pathological examination, cortical dysplasia was most commonly observed, in 11(50%) cases, microdysgenesis in 3(14%), gliosis in 2(9%), and tuberous sclerosis in 2(9%). CONCLUSION: In cases of children with infantile spam not controlled by medical treatment, epileptic surgery should be strongly considered when cortical pathology can be identified from various studies.