PURPOSE: Encephalomalacia is one of the major causes of symptomatic epilepsy. However, no previous study has examined the correlation between encephalomalacia and epilepsy in children. In this study, we aimed to quantify the prevalence, clinical features, and risk factors of epilepsy associated with encephalomalacia. METHODS: We performed a retrospective review of the medical records of 95 patients who were diagnosed with encephalomalacia by neuroimaging techniques at Pusan National University Children's Hospital between November 2008 and July 2013. Patients were classified into two groups: epilepsy (Group A) and non-epilepsy (Group B). We compared the demographics, underlying causes, and distribution of encephalomalacic lesions of these two groups. RESULTS: Groups A and B comprised 35 (36.8%) and 60 (63.1%) patients, respectively. Compared to Group B, Group A showed shorter mean gestational period (35.99+/-4.63 vs. 38.09+/-3.70, P=0.02), lower birth weight (2.60+/-0.94 vs. 3.02+/-0.64, P=0.02), and earlier onset of encephalomalacia (2.74+/-3.52 vs. 5.60+/-5.96, P=0.01). In comparing the underlying cause of encephalomalacia, the occurrence of epilepsy was lower after cerebrovascular disease (P<0.01), but trended towards a higher incidence after a central nervous system infection (P=0.09). Multifocal encephalomalacic lesions were significantly higher in Group A (P=0.04). CONCLUSION: The risk factors for epilepsy associated with encephalomalacia are early gestational age, low birth weight, early onset of encephalomalacia, and multifocal encephalomalacic lesions. It may be necessary for clinicians to search for these risk factors, and make a particularly close observation on these patients.
Birth Weight ; Busan ; Central Nervous System Infections ; Child* ; Demography ; Encephalomalacia* ; Epilepsy* ; Gestational Age ; Humans ; Incidence ; Infant, Low Birth Weight ; Infant, Newborn ; Medical Records ; Neuroimaging ; Prevalence ; Retrospective Studies ; Risk Factors

PURPOSE: The purpose of this study was to investigate the clinical features of neonatal seizures and to identify prognostic factors of neurodevelopmental outcome in term infants who experienced clinical seizures. METHODS: A retrospective analysis was performed on 153full term and preterm infants with seizures from January 2008 to December 2013. Binary logistic regression analysis was applied to assess risk factors associated with neurological adverse outcomes using variables that were found to be significant on univariate analysis. RESULTS: During the study period, 102 (66.7%) term and 51 (33.3%) preterminfants were enrolled. The main cause of neonatal seizures was hypoxic ischemic encephalopathy (24.5%) in term infants and intracranial hemorrhage (74.5%) in preterm infants. The most common type of seizure was focal clonic seizure. Generalized tonic seizure was more commonly observed in preterm than in term infants. 39 out of 56 term infants with at least 12 months of neurologic follow-up showed normal outcomes while only one preterm infant showed normal development.Prognostic factors related to adverse neurodevelopmental outcomes in term infants were perinatal history of fetal distress, etiology of hypoxic ischemic encephalopathy, severity of EEG(Electroencephalogram) abnormality, evidence of hypoxic ischemic encephalopathy on brain magnetic resonance imaging, and the need for multiple antiepileptic drugs for seizure control. CONCLUSION: Preterm infants showed poorer neurodevelopmental outcomes compared to term infants. The etiology of seizures, treatment response, neuroimaging and electroencephalographic findings were important in predicting the developmental outcome in term infants with seizures.
Anticonvulsants ; Brain ; Epilepsy, Partial, Motor ; Fetal Distress ; Follow-Up Studies ; Humans ; Hypoxia-Ischemia, Brain ; Infant ; Infant, Newborn ; Infant, Premature ; Intracranial Hemorrhages ; Logistic Models ; Magnetic Resonance Imaging ; Neuroimaging ; Prognosis ; Retrospective Studies ; Risk Factors ; Seizures*

PURPOSE: Lumbar puncture is an essential and commonly practiced diagnostic tool and post-lumbar puncture headache and post-lumbar puncture back pain are common neurological complications. The aim of this study is to identify the factors that might cause post-lumbar puncture back pain and to reduce those complaints by eliminating the causes. METHODS: From June 5, 2013 to July 29, 2013, we studied 155 patients, male and female between the age 1 and 18,who received diagnostic or therapeutic lumbar puncture at Pusan National University Hospital and Pusan National University Yangsan Hospital. We collected data of variable aspects that may influence post-lumbar puncture back pain, by reviewing the medical records retrospectively. The independent variables were sex, age, symptoms, diagnosis, number of trials to successful lumbar puncture, year of the resident who carried out the procedure, opening pressure of cerebrospinal fluid(CSF), spinal level of punctured site, duration of strict bed rest, and use of sedatives for the procedure. RESULTS: Among the 155 patients whounderwent lumbar puncture, 36 (23.2%) experienced back pain after the procedure. Back pain subsided within 48hours in 24 ofthe 36 patients. Only one patient suffered from post-lumbar puncture back pain for more than 5days. There is no clear causality between the occurrence rate of post-lumbar puncture back pain and the independent variables which includeage, sex, duration of bed rest, symptoms, diagnosis, number of trials, skill, opening pressure, anatomic location, and use of sedatives. CONCLUSION: Back pain was a frequent complication of lumbar puncture. Various factors we expected to influence on the incidence of post-lumbar puncture back pain had no statistical significance. Further studies should be performed to investigate the causes and risk factors of post-lumbar puncture back pain.
Back Pain* ; Bed Rest ; Busan ; Child* ; Diagnosis ; Female ; Gyeongsangnam-do ; Humans ; Hypnotics and Sedatives ; Incidence ; Male ; Medical Records ; Post-Dural Puncture Headache ; Punctures* ; Retrospective Studies ; Risk Factors ; Spinal Puncture

PURPOSE: Hand, foot, and mouth disease (HFMD) is a common childhood illness. Enterovirus 71 (EV71) epidemics have recently been associated with HFMD-based neurologic complications in the Asia-Pacific region. This study described HFMD clinical features, and investigated clinical parameters in patients presenting with acute neurologic complications associated with HFMD. METHODS: We retrospectively reviewed medical records from 235 hospitalized patients who developed HFMD with or without neurologic complications (22 and 213 cases, respectively) in Uijengbu, Korea between 2010 and 2013. Clinical manifestations, radiologic findings, cerebrospinal fluid (CSF) analyses, virological analyses, and treatment regimens were summarized. Additionally, routinely collected baseline data from 235 patients were retrospectively analyzed to identify clinical parameters associated with neurologic complications. RESULTS: Brainstem encephalitis was the most frequent neurologic complication (11 cases), followed by aseptic meningitis (seven cases). We also found acute disseminated encephalomyelitis and meningitis retention syndrome. Both have rarely been reported in EV71-associated HFMD. Virological analyses were performed for 15 cases, and 14 demonstrated EV71 infection, while one patient demonstrated Coxsackievirus B3 infection. Multivariate logistic regression analysis indicated patients were more likely to develop neurologic complications if they experienced nausea/vomiting (OR= 13.65, P<0.001) and lethargy (OR=10.68, P=0.003). Males were more likely to develop neurologic complications compared to females (OR=2.12, P=0.005). In addition, neurologic complications were associated with a higher peak heart rate (OR=1.13, P=0.001). CONCLUSION: This study revealed usual and unusual findings of HFMD-associated neurologic complications. Male gender, nausea/vomiting, lethargy, and peak heart rate parameters predicted HFMD-associated neurologic complications. However, laboratory findings did not reliably predict HFMD-associated neurologic complications.
Brain Stem ; Cerebrospinal Fluid ; Encephalitis ; Encephalomyelitis, Acute Disseminated ; Enterovirus ; Female ; Foot* ; Hand* ; Heart Rate ; Humans ; Korea ; Lethargy ; Logistic Models ; Male ; Medical Records ; Meningitis ; Meningitis, Aseptic ; Mouth Diseases* ; Retrospective Studies

PURPOSE: The objective of this study was to describe polysomnographic charateristics in normal children and adolescents in Korea. Little is known regarding sleep architecture in Korean children. METHODS: We conducted a prospective study and examined sleep architecture, respiratory parameters, arousals, and periodic limb movement index during sleep (PLMS) in healthy children and adolescents for 2 consecutive days. RESULTS: The study population consisted of 29 subjects and 15 subjects (52%) were male. Ages ranged from 5-16 years, with a mean of 11.6+/-.7 years. The average total sleep time (TST) was 438+/-35.6 min, and the mean sleep efficiency was 90.9+/-17.7%. The distribution of sleep stages as percentage of TST was as follows: stage N1, 6.6+/-2.9%; stage N2, 54.2+/-5.8%; stage N3, 21.1+/-6.7%; and stage R, 18.1+/-5.4%. The wake after sleep onset (WASO) was 2.0+/-2.9% of TST. The total arousal index (TAI) was 7.6+/-2.2. The current study findings are similar to those of previous studies in Western countries except longer stage N2 sleep and higher WASO. CONCLUSION: This is the first report of normal polysomnography values in Korean children. In order to accurately diagnose sleep disorders, normative sleep reference values are essential and our findings will provide fundamental data for pediatric sleep research.
Adolescent* ; Arousal ; Child* ; Extremities ; Humans ; Korea ; Male ; Polysomnography ; Prospective Studies ; Reference Values ; Sleep Wake Disorders ; Sleep Stages

PURPOSE: We compared clinical findings of headaches to find out important characteristics for the differential diagnosis of headaches. We tried to find out specific electroencephalogram (EEG) findings according to the types of primary headache and the relationship between clinical characteristics and EEG findings by analyzing EEGs of migraine. METHOD: We conducted a retrospective analysis by reviewing medical records of 275 patients who visited the Department of Pediatrics of Korea University Guro Hospital from November 2010 to October 2013. The headaches were classified according to the International Classification of Headache Disorders-II (ICHD-II). EEG was analyzed in relation to the type of headache. RESULTS: 245 (89.1%) were primary headache patients, and 30 (10.9%) were secondary headache patients.There were no differences in the frequencies of abnormal EEG findings according to the type of primary headache (P=0.855), and epileptiform discharge was most common abnormal EEG finding in the primary headache. Comparing the patients with epileptiform discharges to the patients with normal EEG finding in migraine, the patients showing epileptiform discharge were younger (P=0.035) and presented with occipital and atypical (i.e., not pulsating or pressing) headache (P=0.005). CONCLUSION: History and physical examination are the most important in the diagnosis of headache in children. There were no significant differences in EEG findings among the different types of primary headaches.However,we could find a relationship between clinical features of the migraine and its epileptiform discharge, so more studies correlating EEG findings to clinical features are needed in the future.
Child* ; Classification ; Diagnosis ; Diagnosis, Differential ; Electroencephalography* ; Headache* ; Humans ; Korea ; Medical Records ; Migraine Disorders ; Pediatrics ; Physical Examination ; Retrospective Studies ; Tension-Type Headache

We present a case of cerebral germinoma that occurred in the basal ganglia with progressive hemiparesis. A 10-year-old boy was referred to our hospital complaining of progressive right hemiparesis and decreased mental functions. Brain MRI showed left cerebral hemiatrophy and high signal intensity in left thalamus, basal ganglia, cerebral peduncle, and pons. However, no mass or enhancement was observed. 11C-Methionine positron emission tomography showed discernable hot uptake in the left basal ganglia and thalamus. Stereotactic biopsy, with a guide of 11C-Methionine positron emission tomography, was performed and the diagnosis of pure germ cell tumor was determined. 11C-Methionine positron emission tomography is useful for localization of a suspicious tumor in patients with cerebral hemiatrophy without overt mass or enhancing lesions in brain MRI.
Basal Ganglia* ; Biopsy ; Brain ; Child ; Diagnosis ; Electrons* ; Germinoma* ; Humans ; Magnetic Resonance Imaging ; Male ; Neoplasms, Germ Cell and Embryonal ; Paresis ; Pons ; Positron-Emission Tomography* ; Tegmentum Mesencephali ; Thalamus

Galactosialidosis is a lysosomal storage disease associated with a combined deficiency of beta-galactosidase and alpha-neuraminidase, secondary to a defect of another lysosomal protective protein. It is a neurodegenerative disorder clinically characterized by psychomotor deterioration, cerebellar ataxia, coarse facies, generalized bony deformity and organomegaly. Three phenotypic subtype are recognized:early infantile, late infantile and juvenile/adult type. We report a 13 months old boy with a late infantile galactosialidosis. He was presented with progressive mental regression and motor disturbance and observed cherry red spot, hearing loss, moderate dysostosis multiplex and vacuolated lymphocytes in peripheral blood. He showed only beta-galactosidase deficiency in the lymphocytes and was diagnosed as GM1-gangliosidosis type 1. However, further studies revealed the possible defect of alpha-neuraminidase suggesting that he was a case of galactosialidosis which was mimicking GM1-gangliosidosis type 1.
beta-Galactosidase ; Cathepsin A ; Cerebellar Ataxia ; Congenital Abnormalities ; Dysostoses ; Facies ; Gangliosidosis, GM1 ; Hearing Loss ; Humans ; Infant ; Lymphocytes ; Lysosomal Storage Diseases ; Male ; Mucolipidoses ; Neurodegenerative Diseases ; Prunus

Chiari malformations are brain stem anomalies with or without spinal canal involvement. Type III is the rarest of the Chiari malformations and is characterized by a low occipital or high cervical encephalocele in combination with multiple cerebellar and brain stem anomalies. We report a case of Chiari III malformation of a newborn, who underwent repair of occipital meningoencephalocele and endoscopic third ventriculostomy. We present the case with a review of related literature.
Brain Stem ; Encephalocele ; Humans ; Infant, Newborn ; Spinal Canal ; Ventriculostomy

Neurofibromatosis type 1(NF1) is the most common neurocutaneous syndrome which has an autosomal dominant pattern of inheritance. The NF1 gene is located on chromosome 17q11.2 and encodes for neurofibromin known as tumor suppressor protein. The disorder affects almost every organ and shows neurologic manifestations such as tumors, mental retardation and epilepsy. Epilepsy associated with NF1 occurs in about 4% and is known to have relatively good prognosis. Malformations of cortical development are less common in patients with NF1 than in other phakomatoses. To our knowledge, no report on NF1 with cortical dysplasia has been published in Korea. We report a child with NF1 associated with extensive cortical dysplasia, who presented initially infantile spasms and in the following months developed tonic seizures.
Child ; Epilepsy ; Genes, Neurofibromatosis 1 ; Humans ; Infant ; Infant, Newborn ; Intellectual Disability ; Korea ; Malformations of Cortical Development* ; Neurocutaneous Syndromes ; Neurofibromatoses* ; Neurofibromatosis 1* ; Neurofibromin 1 ; Neurologic Manifestations ; Prognosis ; Seizures ; Spasms, Infantile ; Wills