PURPOSE: Epilepsy is one of the most important morbidities in childhood, but its underlying causes can not be found even though extensive studies have been done. Advances in diagnostic modalities, including neuroimaging such as MRI reveal the apparent underlying causes of epilepsies. Therefore, we evaluated specific causes of symptomatic epilepsy and compared clinical features with idiopathic epilepsy. METHODS: Three hundred nine epileptic children visited the Pediatric Department of Pusan National University Hospital from January, 1991 to June, 1998. Of these, 79 patients were determined as symptomatic epilepsy and the analysis of various clinical features was undertaken retrospectively, according to the presence or absence of underlying causes. RESULTS: Two hundred and thirty two patients(75.1%) were idiopathic and 79 patients(24.9%) were symptomatic, the ratios of male to female and the ages at the onset of first seizure were 1.28 : 1, 1.48 1 and 3.24+/-5.65, 2.40+/-2.86 years(p>0.05, p<0.05), respectively. Causes of symptomatic epilepsy in order of frequency were birth asphyxia(29.9%), CNS malformation(27.3%), CNS infection(22.1%) and others(20.7%). The ratios of male to female and onset age of birth asphyxia, CNS malformation, CNS infection were 2.83 : 1, 1.91+/-2.64, 0.75:1, 2.35+/-2.92 and 1.43 : 1, 2.74+/-2.56, respectively. Seizure types in symptomatic group were classified as partial seizure in 22.1%, generalized seizure in 71.4% and unclassified in 6.5%, compared with those of idiopathic group classified as 46.6%, 51.7%, 1.7%, respectively. Intervals between medication start and seizure control in idiopathic and symptomatic groups were 146.5+/-148.7 days and 246.1+/-247.6 days, repectively(p<0.05). According to their etiology of birth asphyxia, CNS malformation and CNS infection were 364.5+/-315.0 days, 175.4+/-181.6 days, 199.9+/-215.3 days, respectively(p<0.05). CONCLUSION: CNS malformation, birth asphyxia, CNS infection were main causes of symptomatic epilepsy in childhood. In symptomatic epilepsy, generalized seizures was more common and occurred at a younger age. It takes more time to control seizure of symptomatic epilepsy.
Age of Onset ; Asphyxia ; Busan ; Child ; Epilepsy* ; Epilepsy, Generalized ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Neuroimaging ; Parturition ; Retrospective Studies ; Seizures

PURPOSE: There are few rigorous studies about the side effects of antiepileptic drugs(AEDs) according to age. This study is to analyze differences of the side effects of AEDs in epileptic children according to age. METHODS: Sample are 368 children who had received AEDs for at least 1 month during January 1995 to June 1999. We reviewed their medical records including age, sex, AEDs and side effects, and analyzed these data by X2-test. RESULTS: Side effects were observed in 86(23.4%) of 368 patients. There was no difference in the frequency of overall side effects according to age. But significant differences existed in each side effect(p<0.05). The most common side effect according to age were hematologic side effect(75.0%) under 2 months, gastro intestinal side effect(41.7%) between 2 months and 1 year, CNS side effect(42.1%) between 1 year and 5 years, CNS side effect(41.5%) over 5 years. Hepatotoxicity, the increase of AST/ALT due to valproate, occurred significantly more frequently in children younger than 2 years(p<0.05). Drug eruption and post-carbamazepine leukopenia had no differences according to age. Under 1 year, valproate group had significantly more side effects in CNS. But carbamazepine, phenobarbital, and vigabatrin groups had no differences. CONCLUSION: There was significant difference in the side effects of AEDs among each system according to age. It is recommended to use valproate as monotherapy in children younger than 2 years, and pay more attention to CNS side effect in children older than 1 year.
Anticonvulsants* ; Carbamazepine ; Child* ; Drug Eruptions ; Humans ; Leukopenia ; Medical Records ; Phenobarbital ; Valproic Acid ; Vigabatrin

Alternating hemiplegia of childhood(AHC) is a rare but clinically distinct syndrome characterized by onset before 18 months of age, frequent attacks of alternating paralysis, nystagmus transient ocular palsies, other autonomic dysfunction, and the development of cognitive impairment and a choreoathetotic movement disorder. The case is a 13 month-old boy who has presented repeated episodes of alternating hemiplegia from the age of 6 months at a frequency of several times per month, which was lasting for two or three days. Ths diagnosis was based on clinical features. Frequently such hemiplegic attacks were accompanied by irratability. He had mental retardation, neurodevelopmental delay, and seizure attacks. EEG, brain MRI, brain MR angiography, and Tc-99m HMPAO-brain single photon emission computed tomography(SPECT) failed to reveal any significant abnormal finding during the hemiplegic attacks. We tried calcium-entry block and flunarizine to relieve the hemiplegic attacks, but the frequency and severity of the hemiplegic attacks were not decreased by flunarizine therapy.
Angiography ; Brain ; Diagnosis ; Electroencephalography ; Flunarizine ; Hemiplegia* ; Humans ; Infant ; Intellectual Disability ; Magnetic Resonance Imaging ; Male ; Movement Disorders ; Paralysis ; Seizures

Familial hemiplegic migraine(FHM) is an autosomal dominant subtype of migraine with aura, characterized by the occurrence of hemiplegia during the aura. Two subforms of FHM families exist; pure FHM in 80% and FHM families with cerebellar symptoms in 20%. Half of the known FHM families show genetic linkage to chromosome 19p13, and in these families FHM is caused by missense mutations in a neuronal P/Q type calcium channel alpha-1 subunit gene(CACNA1A gene). Linkages to 1q31 and 1q21-23 have also been established. Other families are linked neither to chromosome 19 nor 1. Clinical variabilities are partially associated with the various types of CACNA1A gene mutations. FHM is distinguished from more frequent migraine types by a clear, dominant inheritance pattern and the relative absense of other headache types. Further investigation of FHM will help to clarify the genetics of more common migraine. We describe a male patient with FHM with a brief review of the literature.
Calcium Channels ; Chromosomes, Human, Pair 19 ; Epilepsy ; Genetic Linkage ; Genetics ; Headache ; Hemiplegia ; Humans ; Inheritance Patterns ; Male ; Migraine Disorders ; Migraine with Aura* ; Migraine without Aura ; Mutation, Missense ; Neurons

PURPOSE: This study was performed to find out the role of routine lumbar puncture in children presented with their first seizure with Fever. METHODS: This study included 220 children, over a 5 year period, from April 1999 to March 2003, who visited or were admitted at Dae Dong Hospital with their first febrile convulsion. Lumbar puncture was performed in all children. We analyzed their age, family history, type of seizure, duration of seizure, cause of fever, and the results of lumbar puncture. RESULTS: In the sex distribution, males(58.6%) outnumbered females(41.4%) and the ratio was 1.2:1. 81.7% of the patients had febrile convulsion from 6 months- to 3 years of age(P<0.05). 30.5% also had family history of febrile convulsion. The types of seizure were generalized tonic-clonic(72.7%), generalized tonic(17.7%), and generalized clonic(6.4 %). For the duration of seizure, 90.4% of the patients were estimated less than 15 minutes(P<0.05). The causes of fever were pharyngotonsilitis(40.5%), gastroenteritis(19.1 %), pneumonia or bronchitis(13.2%), meningitis(11%), otitis media, urinary tract infection, and exanthem subitum. However, 9.1% of the patients were diagnosed meningitis, with 18 of 20 patients under 3 years of age. CONCLUSION: In the first seizure with fever, lumbar puncture is a useful method for meningitis, especially under 3 years of age.
Child* ; Exanthema ; Fever* ; Humans ; Meningitis ; Otitis Media ; Pneumonia ; Seizures* ; Seizures, Febrile ; Sex Distribution ; Spinal Puncture* ; Urinary Tract Infections

PURPOSE: The purpose of this study is to estimate the side effects of antiepileptic drug(AED) in children. METHODS: Subjects were 267 children who had received AED for at least 1 month during January 1995 to July 1998. We reviewed their sex, age at start of seizure onset, age at medication, class and number of AED, developmental delay, type and cause of seizure according to the presence of side effect. We analyzed data using Student's t-test and X2-test. RESULTS: Side effects were observed in 61 of 267(22.8%) patients. There were no significant differences in their characteristics according to the presence of side effect. There were 112 episodes of complications in 61 patients. In decreasing order, CNS(38.4%) > digestive(25.9%) > hematologic(22.3%) > skin and connective tissue(7.1%) > others(6.3%). There were 12 episodes in 11 patients who should have been taken off AED due to drug eruption, drowsiness, nausea and so on. Side effects were observed in 16 of 123(6.0%) patients in monotherapy compared with 45 of 144(16.8%) patients in polytherapy, which showed significantly fewer side effects in monotherapy(P<0.05). In monotherapy, mean age at seizure onset and medication start were older(4.63+/-3.83, 5.85+/-3.86 years, respectively) than in polytherapy(2.69+/-3.06, 3.69+/-3.58 years, respectively) (P<0.05). CONCLUSION: In the data, CNS is the most common side effect(43 episodes, 38.4%) and there were significantly fewer side effects in monotherapy compared with polytherapy. Accordingly, we concluded that monotherapy is preferred over polytherapy in reducing the side effects of AED.
Child* ; Drug Eruptions ; Humans ; Nausea ; Seizures ; Skin ; Sleep Stages

Magnetoencephalography(MEG) has many advantages over the electroencephalography (EEG) as it detects the magnetic fields originating from the intracellualr currents within neurons, which are not attenuated or distorted by intervening tissues. Over the several years, it has developed to a whole-head system that enables its routine clinical application for epilepsy patients. MEG is indicated for localization of the irritative zone in lesional and nonlesional epilepsy surgery patients, with having the superiority in neocortical epilepsy, functional mapping of eloquent cortex and defining its relationship with the brain lesion or epileptogenic area, and assessment of normal and abnormal language development. Because of its high spatial and temporal resolution, MEG provides promises to be a powerful tool in the investigation of normal and abnormal brain function including sensory, motor, memory and cognitive processes in the developing brain in the future.
Brain ; Child* ; Electroencephalography ; Epilepsy ; Humans ; Language Development ; Magnetic Fields ; Magnetoencephalography* ; Memory ; Neurons

PURPOSE: Periventricular leukomalacia(PVL) is associated with various neurologic sequelae such as cerebral palsy and cortical blindness. The aim of this study was to analyse the correlation between the degree of PVL on MRI and clinical features or its severity. METHODS:Thirty-eight children with PVL on MRI among children brought to Pusan National University Hospital between January 1996 and August 1999 with development delay, cerebral palsy or epilepsy were included into the study group. We reviewed medical records of the patients including the gestational age, birth weight and neurologic sequelae. The grade for PVL was divided into 1 to 3, and it was based on abnormally increased signal intensity in periventricular white matter or a reduced amount of periventricular white matter or both, and compensatory focal ventricular enlargement. We analysed the relationship of the grade of PVL and various clinical findings. RESULTS: The age ranged from 11 months to 13 years old with a mean of 42 months. History of cesarean delivery and ventilator care were significantly frequent for the severe grade of PVL. Birth weight was significantly lower according to grade of PVL. Gestational age was lower according to the grade of PVL, but was not significant statistically. Incidence of cerebral palsy was significantly higher on the severe grade of PVL. Incidence of epilepsy and mental retardation were 34.2Yo and 23.9M, not associated with the grade of PVL. CONCLUSION: Incidence of cerebral palsy was associated with the severe grade of PVL, so we think MRI can be helpful in predicting neurodevelopmental outcome. (J Korean Pediatr Soc 2000;43 798-805)
Adolescent ; Birth Weight ; Blindness, Cortical ; Brain* ; Busan ; Cerebral Palsy ; Child ; Epilepsy ; Gestational Age ; Humans ; Incidence ; Infant, Newborn ; Intellectual Disability ; Leukomalacia, Periventricular* ; Magnetic Resonance Imaging* ; Medical Records ; Ventilators, Mechanical

Epilepsy is one of the most common and disabling neurologic disorders in childhood. The primary goal of epilepsy treatment is to choose the treatment modality that provides the best chance of improving the patient's quality of life. In addition to classic antiepileptic drugs, newly developed antiepileptic drugs ketogenic diet, epilepsy surgery, and vagal nerve stimulation have improved the ability to treat seizure disorders during the past decades. The mainstay of treatment remains pharmacotherapy. The factors that affect decision of whether to treat seizures with medications and which antiepileptic drugs to use include the likelihood of seizure recurrence, type and severity of seizures, available antiepileptic drug efficacies and toxicities, need for hematologic monitoring, ease to dosing, underlying illness, pharmacologic interaction, and school and other social issues. In this article, we will describe the principle of drug selection for seizure disorder in pediatric patients and the efficacies and adverse effects of the typical and new antiepileptics. Information about the available antiepileptic drugs will help the pediatrician to select the best drug for children with epilepsy.
Anticonvulsants ; Child ; Drug Therapy* ; Epilepsy* ; Humans ; Ketogenic Diet ; Nervous System Diseases ; Quality of Life ; Recurrence ; Seizures ; Vagus Nerve Stimulation

Granulocytic sarcoma is an uncommon extramedullary localized tumor composed of granulocytic precursor cells. The majority of cases have been reported in association with acute myeloid leukemia(AML) especially in children, but infrequently may occur in patients with myeloproliferative disorders or myelodysplastic syndromes. Most common sites of involvement were bone, soft tissue, lymph nodes and skin, but gastrointestinal tract, testis, central nervous system are also involved rarely. We present an unusual case of granulocytic sarcoma of the intracranium and the retro-orbital soft tissue occurring in a patient with concurrent myelodysplastic syndrome, diagnosed by brain magnetic resonance images and ultrasound guided needle biopsy.
Biopsy, Needle ; Brain ; Central Nervous System ; Child ; Gastrointestinal Tract ; Granulocyte Precursor Cells ; Humans ; Lymph Nodes ; Myelodysplastic Syndromes* ; Myeloproliferative Disorders ; Sarcoma, Myeloid* ; Skin ; Testis ; Ultrasonography