Journal Detail Information

1

Cite

Share

A Case of Guillain-Barr Syndrome Complicated by Persistent Hypertension.

Jung Hyun KIM ; Kyung Tae HWANG ; Kyong Og KO ; Yun Duk YOO

Journal of the Korean Child Neurology Society.1999;7(1):124-128.

Guillain-Barr syndrome is rarely complicated by hypertension, which has been ascribed to sympathetic nervous system hyperactivity. We report a 11 years old female with Guillain-Barr syndrome complicated by persistent hypertension associated with elevated renin-angiotensin. So we report this case with brief review of related literatures.
Child ; Female ; Humans ; Hypertension* ; Sympathetic Nervous System

2

Cite

Share

A Case of 9p Tetrasomy Associated with Hydrocephalus due to Hypertrophy of Choroid Plexus.

Chul Hong KIM ; Eui Jun YANG ; Sang Ook NAM ; Su Yung KIM

Journal of the Korean Child Neurology Society.1999;7(1):119-123.

9p Tetrasomy is rare chromosomal aberration that was described in approximately 30 previous patients in the world and this is the first described in Korea. Here we report a 3 month-old boy who was referred for genetic evaluation because of facial dysmorphism, such as wide fontanells, hypertelorism, bulbous nose, low set ears, cleft lip and palate. He had also psychomotor retardation and hypotonia. He was diagnosed as tetrasomy 9p syndrome by clinical feature and chromosomal study. Thereafter, increased growing of head size compared with body weight and height was observed and brain MRI shows hydrocephaly associated with remarkable hypertrophy of choroid plexus and mild Dandy Walker syndrome.
Body Weight ; Brain ; Choroid Plexus* ; Choroid* ; Chromosome Aberrations ; Cleft Lip ; Dandy-Walker Syndrome ; Ear ; Head ; Humans ; Hydrocephalus* ; Hypertelorism ; Hypertrophy* ; Infant ; Korea ; Magnetic Resonance Imaging ; Male ; Muscle Hypotonia ; Nose ; Palate ; Tetrasomy*

3

Cite

Share

A Case of Juvenile Huntington.

Mun Su LEE ; Choong Ho SHIN ; Dong Wu SON ; Kyeong Hee PARK ; Do Hyun KIM ; Kyu Young KIM ; Sung Sup PARK ; Ji Yeon KIM

Journal of the Korean Child Neurology Society.1999;7(1):113-118.

Huntington's disease(HD) is an autosomal dominantly inherited neurodegenerative disease, which is characterized by choreic movement and progressive dementia. HD is a disease caused by CAG repeat expansion of huntintin gene and definitely diagnosed or is excluded by molecular genetic analysis. Juvenile HD, of which onset is in children or young adult, is the most severely disabled type and shows several distinct clinical and genetic features in contrast to usual late-onset type. We report a 10 year-old girl who presented with involuntary movement, seizure and moderate dysarthria confirmed by molecular genetic analysis.
Child ; Chorea ; Dementia ; Dysarthria ; Dyskinesias ; Female ; Humans ; Molecular Biology ; Neurodegenerative Diseases ; Seizures ; Young Adult

4

Cite

Share

A Case of Complex Partial Status Epilepticus.

Seung Kyoo HAN ; Pyoung JEON

Journal of the Korean Child Neurology Society.1999;7(1):107-112.

Nonconvulsive status epilepticus, which constitutes about 25% of all cases of status, has been subdivided into generalized nonconvulsive(absence) status and complex partial status. We report a case of complex partial status epilepticus in a 3-year-old male patient with altered mental function lasting about 24 hours. The electroencephalographic finding was compatible with complex partial status and the outcome of treatment is excellent, so far.
Child, Preschool ; Humans ; Male ; Status Epilepticus*

5

Cite

Share

Follow up Study of Outcome in Severe Hyperbilirubinemic Newborns Treated with Exchange Transfusion and Phototherapy.

Byoung Sun AHN ; Hyun Min PARK ; Baeck Hee LEE

Journal of the Korean Child Neurology Society.1999;7(1):96-106.

PURPOSE: Cerebral palsy or hearing disability of hyperbilirubinemic complication was reduced by blood exchange transfusion(BET) and phototherapy(PT). But in spite of these treatment, abnormal Auditory Brainstem evoked Response(ABR) finding after BET or PT and neurodevelopmental defect due to chronic bilirubin encephalopathy were observed. So we have studied risk factors and outcome of chronic bilirubin encephalopathy after BET, and treatment of hyperbilirubinemia. METHODS: We have analyzed clinical characteristics, the finding and change of ABR after BET in 17 hyperbilirubinemic neonates, and in 8 hyperbilirubinemic neonates who were treated by phototherapy and 15 normal control neonates. RESULTS: 1) Mean bilirubin concentraion were 27.5+/-4.1mg/dL in BET group and 22.1+/-2.3 mg/dL in PT group. There were no difference of clinical findings between BET and PT group. 2) Change of ABR (1) Wave I loss resulted in 4 neonates, wave III loss in 3 neonates, and wave V loss in 2 neonates in BET group(P<0.05). (2) Wave I peak latency and hearing threshold in BET group were significantly increased more than normal control group(P<0.01). 3) In 10 neonates(58.8%) among 17 BET group, 4 neonates(50%) in 8 PT group were observed abnormal initial ABR finding after jaundice treatment. Age at treatment and duration of jaundice(interval between onset of jaundice and treatment) in abnormal ABR group were significant prolongation compared with normal ABR group(P<0.05). 4) Chronic bilirubin encephalopathy(CBE) was observed in 3 neonates(17.6%) among 17 BET group and showed higher of bilirubin level than normalized group after BET (31.1mg/dL vs 26.6mg/dL), other clinical findings showed no significant differences. CONCLUSION: Bilirubin level was significantly elevated in CBE more than in BET group and duration of jaundice, age at treatment were longer in abnormal ABR group than in normal ABR group. So not only bilirubin level but also duration of jaundice shoud be considered at jaundice treatment, and ABR has a potential utility in detection of acute brain toxicity of bilirubin and follow up evaluation of bilirubin encephalopathy.
Bilirubin ; Brain ; Brain Stem ; Cerebral Palsy ; Follow-Up Studies* ; Hearing ; Humans ; Hyperbilirubinemia ; Infant, Newborn* ; Jaundice ; Kernicterus ; Phototherapy* ; Risk Factors

6

Cite

Share

The Clinical Study on the Epileptiform Discharges and Imaging Study in Newborn with Seizures.

Jin Hun JEON ; In Joon SEOL ; Soo Ji MOON

Journal of the Korean Child Neurology Society.1999;7(1):89-95.

PURPOSE: Neonatal seizures are mostly symptomatic and have variable atypical clinical features with very different EEG findings from those of post-neonatal period. This study was performed to determine the common types of epileptiform discharges and their relationship with abnormalities in imaging study in neonates with seizures. METHODS: Fourty one neonates under 7 days of age who had been hospitalized at the Hanyang university hospital from June 1993 to May 1997 were studied retrospectively. Their medical records, results of EEG and brain imaging study were reviewed. RESULTS: 1) The types of neonatal seizures were subtle, generalized tonic, multifocal clonic, focal clonic, myoclonic in order of frequency. 2) The most common epileptiform discharge was excessive sharp transients recorded in 28 cases and the rest showed multifocal spikes, sharp waves, repetitive alpha, repetitive theta in order. Epileptiform discharges appeared on the both hemisphere in 24 cases, right hemisphere in 9 cases and left hemisphere in 8 cases. 3) Imaging study showed no abnormal findings in 27 cases and abnormal findings in 14 cases such as brain edema, intracranial hemorrhage, leukomalacia, subarachnoid hemorrhage, cephalhematoma, craniosynostosis. 4) Among 14 cases with radiological abnormalities, 5 cases had localized abnormalities and 3 of them had the abnormal EEG discharges on the same area concomittantly. But in total only 3 cases of 17 cases with localized epileptiform discharges had abnormalities in imaging study on the same site and so significant relationship between two studies was not observed. 5) As for the prognosis of seizures, subsided in 23 cases without any antiepileptic drugs, 13 cases had no seizure after taking medicine for few days and 5 cases needed continuous antiepileptic drug. CONCLUSION: This study showed no meaningful relationship between abnormal EEG findings and localization by imaging study in neonatal seizure but we concluded that brain imaging study was needed to determine degree of encephalopathy in neonatal seizures.
Anticonvulsants ; Brain Edema ; Craniosynostoses ; Electroencephalography ; Humans ; Infant, Newborn* ; Intracranial Hemorrhages ; Medical Records ; Neuroimaging ; Prognosis ; Retrospective Studies ; Seizures* ; Subarachnoid Hemorrhage

7

Cite

Share

The Risk of Seizure Recurrence After a First Unprovoked Seizure in Childhood : A Prospective Study.

Sang Ho YOO ; Jun Young SONG ; Sung Hwan KIM

Journal of the Korean Child Neurology Society.1999;7(1):79-88.

PURPOSE: Epilepsy is generally defined as a chronic condition characterized by recurrent unprovoked seizure. It is still controversial whether the long-term antiepileptic drugs(AED) treatment will be necessary in children with first unprovoked seizure. Cognitive and behavioral side effects of AEDs are especially important in developing children. Therefore the rationale of AED treatment in the children with first unprovoked seizure depends upon the seizure recurrence rate and presence of risk factors related with seizure recurrence. We are going to evaluate the risk of seizure recurrence and risk factors after a first unprovoked seizure in children. METHODS: One hundred fifty eight patients presented with a first unprovoked seizure from July, 1994 to June, 1999 were prospectively followed by regular epilepsy clinic visit or telephone interview for a mean of 27 months. We analyzed overall recurrence risk and risk factors of seizure recurrence(EEG finding, etiology of seizure, seizure type, history of prior febrile convulsions, neurodevelopmental status prior to first seizure, presence of a Todd's paresis, family history of seizures, a seizure occuring while asleep, and duration of seizure) by Cox's proportional hazards model and Kaplan-Meier survival analysis. RESULTS: In 158 patients with first unprovoked seizure, 61 (39%) patients experienced subsequent seizure. The cumulative risk of seizure recurrence was 28.5, 37.6, and 42.3% at 6, 12, and 18 months. The median time to recurrence was 3 months, with 72% of recurrence occurring within 6 months, 92% within 1 year, and 100% within 2 years. On univariate analysis, risk factors for seizure recurrence included an etiology of seizure and abnormal EEG. In idiopathic cases, the risk factor was an EEG abnormality. CONCLUSION: Many of the children with a first unprovoked seizure will not have recurrences. The recurrence risk of a first unprovoked seizure in children have statistically correlated with the etiology of seizure(cryptogenic and symptomatic) and abnormal EEG. Children with an idiopathic first seizure and a normal EEG have a particularly favorable prognosis.
Ambulatory Care ; Child ; Electroencephalography ; Epilepsy ; Humans ; Interviews as Topic ; Paresis ; Prognosis ; Proportional Hazards Models ; Prospective Studies* ; Recurrence* ; Risk Factors ; Seizures* ; Seizures, Febrile

8

Cite

Share

The Clinical Characteristics of Status Epilepticus in Children.

Yoon Ki KANG ; Sun Jun KIM

Journal of the Korean Child Neurology Society.1999;7(1):71-78.

PURPOSE: Status epilepticus (SE) is one of the most common neurologic emergencies in children requiring immediate aggressive intervention. Despite advances in treatment of this condition, SE is still associated with a significant morbidity and mortality. To evaluate etiology and neurologic outcome of SE is very important for improving the methods of evaluation and management of SE as well as decreasing morbidity and mortality. METHODS: We retrospectively reviewed the records of 98 children aged 1 month to 15 years who were admitted to our department of pediatrics from January, 1992 to December, 1998. We analyzed age distribution, etiology, seizure type, abnormalities of EEG and brain MRI, and neurologic outcome. We try to find any coherence between neurologic sequelae and clinical factors. RESULTS: The incidence was high in toddler age, especially younger than 3 years old (67.4%). Generalized convulsive SE was the most common type (67.3%), and 54.1% of children with SE which was the first seizure episode. Major etiology of SE in young children younger than 3 years old were febrile convulsion (54.5%) and acute symptomatic causes (22.7%). Neurologic sequelae were left in 21.4% and the mortality rate was 7.1%. The neurologic complications were highly associated with the underlying causes and the seizure durations, especially over 2 hour seizures left in 75% complications. The mortality and neurologic sequelae were higher in acute symptomatic SE than other causes. Febrile SE had usually benign course, but in four in our cases showed neurologic sequale it shows 4 neurologic sequelae cases. CONCLUSION: SE is a life-threatening neurologic condition and especially occurs in young children. The early detection of seizure etiology and aggressive management are the essential factors to decrease the mortality and morbidity rates.
Age Distribution ; Brain ; Child* ; Child, Preschool ; Electroencephalography ; Emergencies ; Humans ; Incidence ; Magnetic Resonance Imaging ; Mortality ; Pediatrics ; Retrospective Studies ; Seizures ; Seizures, Febrile ; Status Epilepticus*

9

Cite

Share

Prognostic Factors for Non-lesional Temporal Lobe Epilepsy.

Gye Ri JEON ; Sung Hwan KIM

Journal of the Korean Child Neurology Society.1999;7(1):63-70.

PURPOSE: Temporal lobe epilepsy in infants and children have been the focus of many clinical investigations and observations. Several prognostic aspects of nonlesional temporal lobe epilepsy (NLTLE) in childhood remain unclear or controversial. This study was aimed to evaluate the clinical characteristics of NLTLE influencing short-term remission and whether the short-term seizure outcome has any impact on long-term prognosis. METHODS: The study was performed between June 1994 and August 1997. There were 32 newly-referred patients who had diagnosed of NLTLE from the data registry of Pediatric Epilepsy Clinic of Ajou University Medical Center. The patients identification was based on the careful review of hospital records, EEGs, and brain MRI. We have evaluated 6 months terminal remission rate (6M-TR) at one year of continuous antiepileptic drug (AED) treatment as short-term outcome. The predictive value of clinical parameters of NLTLE was comparatively analyzed between the patient who attained 6M-TR and who did not. To identify the long-term prognosis in NLTLE, we analyzed continuous seizure free rate during the next one year and compared between the patient who attained 6M-TR and who did not. RESULTS: 1) Among total 32 NLTLE patients, 18 (56.2%) patients attained 6M-TR at one year of AED treatment. 2) We cannot find any statistically significant clinical parameters influencing the short-term outcome between the two groups : age of onset (P=0.467), duration of illness (P=0.408), seizure type (P=0.725), abnormality of EEG (P=0.473), MRI findings (hippocampal sclerosis or temporal neocortical atrophy) (P=0.685). However, a previous history of perinatal asphyxia (P=0.367) and febrile seizure (P=0.253) were not statistically significant but those clinical parameters suggest clinical significance of influencing short-term outcome of NLTLE. 3) Patients with 6M-TR have showed the next one year remission in significantly higher proportion (77.8%) than those without 6M-TR (28.6%) (P=0.001). CONCLUSION: Early short-term outcome of NLTLE is relatively good and significant proportion of patients with early 6M-TR enters the next one year remission period. So we conclude that short-term remission of NLTLE may be an important determinant in predicting long-term prognosis.
Academic Medical Centers ; Age of Onset ; Asphyxia ; Brain ; Child ; Electroencephalography ; Epilepsy ; Epilepsy, Temporal Lobe* ; Hospital Records ; Humans ; Infant ; Magnetic Resonance Imaging ; Prognosis ; Sclerosis ; Seizures ; Seizures, Febrile ; Temporal Lobe*

10

Cite

Share

Effects of Arbiturary Acute Anticonvulsnat Withdrawal in Epileptic Children.

Keun Hee LIM ; Eun Sil LEE ; Han Ku MOON

Journal of the Korean Child Neurology Society.1999;7(1):48-62.

PURPOSE: The effects of arbiturary acute anticonvulsants withdrawal in epileptic children were studied and relationship between status epilepticus and anticonvulsant withdrawal was analysed. METHODS: Medical records of 88 withdrawal episodes in 72 active epileptic children were analysed retrospectively according to the types of epilepsies, anticonvulsants. RESULTS: 1) When followed up after withdrawal, 42 cases (47.7%) had a recurrence of habitual seizure, 18 cases (20.5%) had no seizure and increased seizure frequency were found in 14 cases (16.0%). Three cases (3.4%) had no change in seizure frequency or severity and in 2 cases (2.3%) the frequency or severity of seizure were decreased after withdrawal. 2) Recurrence of habitual seizure, increased seizure frequency, more intense seizure or status epilepticus were noted in 55 of 71 withdrawal episodes in focal epilepsies (77.5%), in 9 of 17 withdrawal episodes (52.9%) in generalized epilepsies (P=0.041). These changes occurred in 36 of 55 cases (65.5%) in focal epilepsies, 3 of 9 cases (30.0%) in generalized epilepsies within 4 weeks after withdrawal (P=0.137). 3) Recurrence of habitual seizure, increased seizure frequency, development of more intense seizure or status epilepticus were found in 18 of 26 cases (69.2%) in carbamazepine monotherapy, 15 of 16 cases (93.8%) in phenobarbital monotherapy, 24 of 34 cases (70.6%) in polypharmacy (P=0.143). These changes occurred in 14 of 18 cases (77.8%) in carbamazepine monotherapy, 7 of 15 cases (46.7%) in phenobarbital monotherapy and 18 of 24 cases (75.0%) in polypharmacy within 4 weeks after withdrawal (P=0.122). 4) Four cases (4.5%) of status epilepticus occurred in 4 patients with focal epilepsies at 4 months (2 cases), 5 months, 23 months later after acute anticonvulsant withdrawal. CONCLUSION: Arbiturary acute anticonvulsants withdrawal in epileptic children were more common than expectation and councelling to avoid arbiturary anticonvulsant withdrawal must be entensified. Approximately half of the cases with anticonvulsant withdrawal showed a recurrence of habitual seizure and status epilepticus occurred in 4.5% of cases only. Other factors besides acute anticonvulsant withdrawal must be investigated to clarify the underlying mechanism of status epilepticus. A quarter of cases showed no seizure and even decrease in seizure frequency or severity after anticonvulsant withdrawal. These results suggest more considerated prescription of anticonvulsant is needed in practice.
Anticonvulsants ; Carbamazepine ; Child* ; Epilepsies, Partial ; Epilepsy ; Epilepsy, Generalized ; Humans ; Medical Records ; Phenobarbital ; Polypharmacy ; Prescriptions ; Recurrence ; Retrospective Studies ; Seizures ; Status Epilepticus

DISPLAY OPTIONS

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share