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1.
Korean Journal of Radiology ; : 1822-1833, 2021.
Article in English | WPRIM | ID: wpr-918202

ABSTRACT

This is a narrative review of various treatment modalities for advanced hepatocellular carcinoma (HCC), with a focus on recent updates in radiological treatments, as well as novel treatment concepts related to immune checkpoint inhibitors and combination therapies with locoregional treatments. Interventional radiologists have made efforts toward developing alternative and/or combination treatments for first-line systemic treatment of patients with advanced HCC. Locoregional treatments with or without systemic therapy may be considered in the selected patients. Various treatment modalities for advanced HCC are emerging, and several randomized controlled trials, including those of combination treatments with immunotherapy, are ongoing.

2.
Article in English | WPRIM | ID: wpr-899147

ABSTRACT

Background@#and Purpose: Cerebral visual impairment (CVI) is an underdiagnosed condition in children, and its assessment tools have focused on older children. We aimed to develop a parental questionnaire for cerebral visual impairment (PQCVI) for screening CVI in young children. @*Methods@#The PQCVI comprised 23 questions based on a modified version of Houliston and Dutton’s questionnaire for older children. The PQCVI with neurocognitive function tests was applied to 201 child–parent pairs with typically developing children younger than 72 months (age 32.4±20.1 months, mean±standard deviation). The children were classified into six age groups. The normative data, cutoff scores, and internal reliability were assessed and item analysis was performed. We referred to the total score for all questions as the cerebral visual function (CVF) score. @*Results@#The normative data showed that the CVF score and the scores corresponding to ventral-stream and dorsal-stream visual functions plausibly increased with age. The scores rapidly reached 90% of their maximum values up to the age of 36 months, after which they increased slowly. Cronbach’s alpha for all questions across all age groups was 0.97, showing excellent consistency. The item difficulty and item discrimination coefficients showed that the questions were generally adequate for this age stage. @*Conclusions@#The PQCVI items produced reliable responses in children younger than 72 months. The rapid increase in scores before the age of 3 years supports the importance of early identification of CVI. Following additional clinical verification, the PQCVI may be useful for CVI screening.

3.
Article in English | WPRIM | ID: wpr-891443

ABSTRACT

Background@#and Purpose: Cerebral visual impairment (CVI) is an underdiagnosed condition in children, and its assessment tools have focused on older children. We aimed to develop a parental questionnaire for cerebral visual impairment (PQCVI) for screening CVI in young children. @*Methods@#The PQCVI comprised 23 questions based on a modified version of Houliston and Dutton’s questionnaire for older children. The PQCVI with neurocognitive function tests was applied to 201 child–parent pairs with typically developing children younger than 72 months (age 32.4±20.1 months, mean±standard deviation). The children were classified into six age groups. The normative data, cutoff scores, and internal reliability were assessed and item analysis was performed. We referred to the total score for all questions as the cerebral visual function (CVF) score. @*Results@#The normative data showed that the CVF score and the scores corresponding to ventral-stream and dorsal-stream visual functions plausibly increased with age. The scores rapidly reached 90% of their maximum values up to the age of 36 months, after which they increased slowly. Cronbach’s alpha for all questions across all age groups was 0.97, showing excellent consistency. The item difficulty and item discrimination coefficients showed that the questions were generally adequate for this age stage. @*Conclusions@#The PQCVI items produced reliable responses in children younger than 72 months. The rapid increase in scores before the age of 3 years supports the importance of early identification of CVI. Following additional clinical verification, the PQCVI may be useful for CVI screening.

4.
Article in English | WPRIM | ID: wpr-728820

ABSTRACT

PURPOSE: Several studies have shown that magnesium plays an important role in modulating N-methyl-D-aspartate (NMDA)-related seizures by blocking NMDA ion channel receptors. Clinicians usually measure total serum magnesium levels instead of biologically active ionized magnesium levels. We compared the serum ionized magnesium (iMg2+) level between epileptic children with and without a history of fever-triggered seizure (FTS). METHODS: All epileptic children who visited the outpatient clinic or pediatric emergency department at Korea University Guro Hospital between January 2015 and July 2017 were included. Only epileptic children aged 1–8 years who were newly diagnosed within 2 years were included. RESULTS: There were 12 children with FTS and 16 without FTS. Median serum iMg2+ level was 0.93 (0.85–1.14, quartile) mEq/L. Serum iMg2+ level was significantly lower in epileptic children with FTS (0.86 mEq/L) compared to those without FTS (1.10 mEq/L) (P=0.005). No difference was noted in clinical variables between the two groups. Lower serum iMg2+ level significantly increased the risk of having FTS in epileptic children based on multivariable logistic regression analysis (odds ratio [OR]=0.028). CONCLUSION: Serum iMg2+ level was significantly lower in epileptic children with FTS than in those without FTS. Measurement of biologically active serum iMg2+ level could be considered in epileptic children with recurrent FTS. A large-scale prospective study is warranted.


Subject(s)
Child , Humans , Ambulatory Care Facilities , Emergency Service, Hospital , Epilepsy , Ion Channels , Korea , Logistic Models , Magnesium , N-Methylaspartate , Prospective Studies , Seizures , Seizures, Febrile
5.
Article in English | WPRIM | ID: wpr-714813

ABSTRACT

BACKGROUND: Henoch-Schӧnlein purpura (HSP) is a common vasculitis of childhood. Though HSP is usually self-limiting, severe complications can occur. The management for this condition has not been established yet. Thus, this nationwide study aimed at investigating epidemiological characteristics of children with HSP in Korea. The patterns of clinical practice with regard to the complications of the condition were also investigated. METHODS: This is a national population-based study that used the National Health Insurance Database. Children below 18 years who were diagnosed with HSP in Korea between 2006 and 2015 were enrolled. Data, such as age, sex, yearly and monthly distribution of HSP, hospitalization, re-hospitalization, comorbidities, and interventions were obtained. The use of steroids was also analyzed. RESULTS: A total of 56,841 children were enrolled. The annual incidence of HSP was 55.9 per 100,000 children. The peak age was 5 years. Spring was the most prevalent season. Sex (male) and young age (< 9 years) were risk factors of hospitalization. Younger children were more likely to be re-hospitalized and suspected with intussusception, arthritis, and nephritis. Only 4 children received laparotomy. In total, 57% were managed with steroids, and mean durations of medication were 4–5 days. Children who were hospitalized and those with comorbidities used steroids more frequently (P < 0.001). CONCLUSION: The annual incidence of HSP is 55.9 per 100,000 children which is higher in Korea than that in other countries. Younger children can have a more severe clinical course. This nationwide survey provides valuable information to understand HSP in children and to inspire further research on HSP.


Subject(s)
Child , Humans , Arthritis , Comorbidity , Epidemiology , Hospitalization , Incidence , Intussusception , Korea , Laparotomy , National Health Programs , Nephritis , Purpura , Risk Factors , Seasons , Steroids , Vasculitis
6.
Article in English | WPRIM | ID: wpr-27508

ABSTRACT

PURPOSE: To evaluate the usefulness of the Korean Developmental Screening Test (K-DST) for infants and children for developmental delay assessment. METHODS: This study was based on retrospective studies of the results of the K-DST, Preschool Receptive-Expressive Language Scale (PRES), Sequenced Language Scale for Infants (SELSI), Childhood Autism Rating Scale (CARS), Modified Checklist for Autism in Toddlers (M-CHAT), electroencephalography, magnetic resonance imaging, and extensive tests conducted in 209 of 1,403 patients, of whom 758 underwent the K-DST at the Korea University Guro Hospital between January 2015 and December 2016 and 645 were referred from local clinics between January 2015 and June 2016. RESULTS: Based on the K-DST results, the male children significantly more frequently required further or follow-up examination than the female children in most test sections, except for gross motor. The male children had notably lower mean scores than the female children. The PRES/SELSI results showed that when more further or follow-up evaluations were required in the K-DST communication section, significantly more problems in language delay or disorder emerged. When further or follow-up evaluation was required in the cognitive section in the CARS/M-CHAT, the possibility of autism increased significantly. A child tended to score low in the CARS test and show autism when further or follow-up evaluation was recommended in the K-DST. CONCLUSION: This study demonstrated the usefulness of the K-DST as a screening test early in the development of infants and children in Korea. Data of normal control groups should be examined to determine the accuracy of this investigation.


Subject(s)
Child , Female , Humans , Infant , Male , Autistic Disorder , Checklist , Electroencephalography , Follow-Up Studies , Growth and Development , Korea , Language Development Disorders , Magnetic Resonance Imaging , Mass Screening , Retrospective Studies
7.
Article in English | WPRIM | ID: wpr-119362

ABSTRACT

BACKGROUND AND PURPOSE: Lacosamide (LCM) is an antiepileptic drug that enhances the slow inactivation of sodium channels and modulates collapsin response mediator protein-2. LCM was recently demonstrated to exert a neuroprotective effect in a murine model of traumatic brain injury and status epilepticus. Assuming the same underlying excitotoxicity-related brain injury mechanism, we hypothesized that LCM would have a neuroprotective effect in hypoxic-ischemic brain injury. METHODS: We divided rats into three groups at each testing session: pre- or postfed with LCM, fed with normal saline, and sham. A hypoxic-ischemic brain injury was induced by subjecting 7-day-old rats to right carotid artery coagulation followed by 2.5 h of exposure to 8% oxygen. The animals were killed on postnatal day 12 to evaluate the severity of brain damage. Open field testing was also performed between week 2 and week 6, and the Morris water maze test was performed in week 7 after hypoxia-ischemia. RESULTS: The incidence of liquefactive cerebral infarction was lower in rats prefed with LCM at 100 mg/kg/dose, with the mortality rate being higher at higher doses (200 and 300 mg/kg/dose). The infarct areas were smaller in LCM-prefed rats in several brain regions including the hemisphere, hippocampus, cortex, and striatum. Spatial learning and memory function were better in LCM-prefed rats (p<0.05). No effect was observed in postfed rats. CONCLUSIONS: This study suggests that LCM pretreatment exerts a neuroprotective effect on hypoxia-ischemia in neonatal rats. The obtained results suggest that LCM pretreatment could be used as an effective neuroprotective method for neonates under hypoxic-ischemic conditions including heart surgery.


Subject(s)
Animals , Humans , Infant, Newborn , Rats , Brain Injuries , Brain , Carotid Arteries , Cerebral Infarction , Hippocampus , Incidence , Memory , Methods , Mortality , Neuroprotection , Neuroprotective Agents , Oxygen , Semaphorin-3A , Sodium Channels , Spatial Learning , Status Epilepticus , Thoracic Surgery , Water
8.
Article in English | WPRIM | ID: wpr-79078

ABSTRACT

Norovirus causes acute gastroenteritis, occasional afebrile seizures, and rarely encephalitis. We describe a child with cerebellitis due to norovirus that has not been reported previously. A previously healthy 2-year-old girl with a recent history of acute gastroenteritis, who presented with acute cerebellar ataxia. She exhibited marked truncal ataxia, was barely able to walk, and was prone to sitting or lying down. Multiplex PCR using stool samples revealed norovirus (genogroup II) and magnetic resonance imaging showed increased T2 signal in the hemi-cerebellum. Norovirus may be a potential pathogenic cause of acute cerebellitis in children.


Subject(s)
Child , Child, Preschool , Female , Humans , Ataxia , Cerebellar Ataxia , Deception , Encephalitis , Gastroenteritis , Magnetic Resonance Imaging , Multiplex Polymerase Chain Reaction , Norovirus , Seizures
9.
Article in English | WPRIM | ID: wpr-125199

ABSTRACT

PURPOSE: There is a paucity of evidence about prognosis after a first febrile seizure in older children. We investigated the prognosis and potential risk factors associated with subsequent unprovoked seizures in children who had experienced a first febrile seizure over 6 years of age, which we termed as late-onset febrile seizure. METHODS: We included all patients six years or older who presented to the emergency department with a febrile seizure between 2009 and 2015. Clinical data was collected by chart review and parents were contacted for information on seizure progress. We used the Cox proportional-hazards model and Kaplan-Meier analysis for evaluating the risk factors for subsequent unprovoked seizures. RESULTS: Of 247 patients, we excluded 168 children who had a history of epilepsy, unprovoked, or febrile seizure and who were followed-up for period less than six months. Overall, 79 patients were classified as having had a first late-onset febrile seizure. During follow-up of 34.9±25.7(mean±SD) months, unprovoked seizure recurred in 7 of 79 patients (9%). The cumulative probability of seizure recurrence was 4% at 6 months, 6% at 1 year and 9% at 2 years. Clinical variables predictive of subsequent unprovoked seizures were not proved. CONCLUSION: This is the first multicenter study focusing on prognosis after a late-onset febrile seizure in children six years or older. The percentage of subsequent unprovoked seizure in patients with late-onset febrile seizure was 9% at 2 years of follow-up. Prospective follow-up study with longer duration is warranted.


Subject(s)
Child , Humans , Emergency Service, Hospital , Epilepsy , Follow-Up Studies , Kaplan-Meier Estimate , Parents , Prognosis , Prospective Studies , Recurrence , Risk Factors , Seizures , Seizures, Febrile
10.
Article in English | WPRIM | ID: wpr-139254

ABSTRACT

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a disease that is characterized by acute psychiatric symptoms, seizures, and central hypoventilation. Patients with anti-NMDAR encephalitis exhibit speech alterations, insomnia, seizures, and movement disorders. We describe a previously healthy 6-year-old girl who presented with seizures, disorientation, and fever. Over the five weeks of treatment, the patient exhibited progressive neurologic symptoms, including a change in mental status. Her serum and cerebrospinal fluid contained high titers of antibodies against the NMDAR, and she was diagnosed with anti-NMDAR encephalitis. She was treated with plasmapheresis, steroid pulse therapy, intravenous immunoglobulins, and repeated doses of rituximab. After the patient was diagnosed with a concomitant ovarian teratoma, a unilateral salpingo-oophorectomy was performed. A histopathologic examination revealed that neuronal elements accounted for 60% of the resected ovarian teratoma. The patient's clinical symptoms and antibody titers improved after the surgical treatment and rituximab therapy. These observations suggested that patients with high titers of anti-NMDAR antibodies should be examined for the presence and quantity of neuronal components in concurrent ovarian teratomas.


Subject(s)
Child , Female , Humans , Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Antibodies , Cerebrospinal Fluid , Encephalitis , Epilepsy , Fever , Hypoventilation , Immunoglobulins, Intravenous , Movement Disorders , Neurologic Manifestations , Neurons , Plasmapheresis , Rituximab , Seizures , Sleep Initiation and Maintenance Disorders , Teratoma
11.
Article in English | WPRIM | ID: wpr-139258

ABSTRACT

Acute hemorrhagic leukoencephalitis (AHLE) is an acute, rapidly progressing, fulminant demyelinating disease. It is a rare disease of the central nervous system with high mortality; survivors commonly present with significant neurological deficit. We report the case of a 16-month-old girl who survived AHLE and presented with the associated neurologic deficit. The patient came into the emergency department with febrile seizure. She showed bilateral pinpoint-sized pupils and hyperactive deep tendon reflexes. Her mental status was initially drowsy and rapidly progressed to stupor. Extensive demyelination and microbleeds were found in the cerebral white matter, thalamus and left cerebellum on Magnetic resonance imaging (MRI) scans. Her mental status was improved by intravenous administration of immunoglobulin and methylprednisolone. Five months after being discharged, increased white matter connectivity was found on color-coded follow-up MR diffusion tensor imaging (DTI) as compared to previous MRI. We therefore suggest adding the DTI technique when a follow-up MRI is performed in patients with AHLE. It could be useful to visualize the status of axonal injury and to encourage patients and their parents to continue the rehabilitation program.


Subject(s)
Child , Female , Humans , Infant , Administration, Intravenous , Axons , Central Nervous System , Cerebellum , Demyelinating Diseases , Diffusion Tensor Imaging , Diffusion , Emergency Service, Hospital , Follow-Up Studies , Immunoglobulins , Leukoencephalitis, Acute Hemorrhagic , Magnetic Resonance Imaging , Methylprednisolone , Mortality , Neurologic Manifestations , Parents , Pupil , Rare Diseases , Reflex, Stretch , Rehabilitation , Seizures , Seizures, Febrile , Stupor , Survivors , Thalamus , White Matter
12.
Article in English | WPRIM | ID: wpr-139259

ABSTRACT

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a disease that is characterized by acute psychiatric symptoms, seizures, and central hypoventilation. Patients with anti-NMDAR encephalitis exhibit speech alterations, insomnia, seizures, and movement disorders. We describe a previously healthy 6-year-old girl who presented with seizures, disorientation, and fever. Over the five weeks of treatment, the patient exhibited progressive neurologic symptoms, including a change in mental status. Her serum and cerebrospinal fluid contained high titers of antibodies against the NMDAR, and she was diagnosed with anti-NMDAR encephalitis. She was treated with plasmapheresis, steroid pulse therapy, intravenous immunoglobulins, and repeated doses of rituximab. After the patient was diagnosed with a concomitant ovarian teratoma, a unilateral salpingo-oophorectomy was performed. A histopathologic examination revealed that neuronal elements accounted for 60% of the resected ovarian teratoma. The patient's clinical symptoms and antibody titers improved after the surgical treatment and rituximab therapy. These observations suggested that patients with high titers of anti-NMDAR antibodies should be examined for the presence and quantity of neuronal components in concurrent ovarian teratomas.


Subject(s)
Child , Female , Humans , Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Antibodies , Cerebrospinal Fluid , Encephalitis , Epilepsy , Fever , Hypoventilation , Immunoglobulins, Intravenous , Movement Disorders , Neurologic Manifestations , Neurons , Plasmapheresis , Rituximab , Seizures , Sleep Initiation and Maintenance Disorders , Teratoma
13.
Article in English | WPRIM | ID: wpr-139263

ABSTRACT

Acute hemorrhagic leukoencephalitis (AHLE) is an acute, rapidly progressing, fulminant demyelinating disease. It is a rare disease of the central nervous system with high mortality; survivors commonly present with significant neurological deficit. We report the case of a 16-month-old girl who survived AHLE and presented with the associated neurologic deficit. The patient came into the emergency department with febrile seizure. She showed bilateral pinpoint-sized pupils and hyperactive deep tendon reflexes. Her mental status was initially drowsy and rapidly progressed to stupor. Extensive demyelination and microbleeds were found in the cerebral white matter, thalamus and left cerebellum on Magnetic resonance imaging (MRI) scans. Her mental status was improved by intravenous administration of immunoglobulin and methylprednisolone. Five months after being discharged, increased white matter connectivity was found on color-coded follow-up MR diffusion tensor imaging (DTI) as compared to previous MRI. We therefore suggest adding the DTI technique when a follow-up MRI is performed in patients with AHLE. It could be useful to visualize the status of axonal injury and to encourage patients and their parents to continue the rehabilitation program.


Subject(s)
Child , Female , Humans , Infant , Administration, Intravenous , Axons , Central Nervous System , Cerebellum , Demyelinating Diseases , Diffusion Tensor Imaging , Diffusion , Emergency Service, Hospital , Follow-Up Studies , Immunoglobulins , Leukoencephalitis, Acute Hemorrhagic , Magnetic Resonance Imaging , Methylprednisolone , Mortality , Neurologic Manifestations , Parents , Pupil , Rare Diseases , Reflex, Stretch , Rehabilitation , Seizures , Seizures, Febrile , Stupor , Survivors , Thalamus , White Matter
14.
Article in English | WPRIM | ID: wpr-159143

ABSTRACT

PURPOSE: Among syndromes of idiopathic occipital lobe epilepsy there are Panayiotopoulos syndrome (PS) and idiopathic childhood occipital epilepsy of Gastaut (ICOE-G). However, the classification of epilepsy syndrome is difficult to diagnose at the first seizure because of varying characteristics. We analyzed the different clinical characteristics and prognostic factors of idiopathic occipital lobe epilepsy. METHODS: Forty-three patients who had at least two unprovoked seizures, no abnormalities on magnetic resonance imaging, no known etiology of seizure, and mainly occipital spikes on electroencephalography were retrospectively enrolled at the Korea University Medical Center. A good prognosis was defined as being seizure-free and taking two or less drugs for 1 year. RESULTS: Among all patients, the proportion of PS was 44.2%, ICOE-G was 7.0% and unclassified group was 48.8%. The age at seizure onset was 4.5±2.6 years (mean±SD) in the PS group, and 8.3±2.1 years in the ICOE-G group. The follow-up duration was 10.8±6.0 years. The percentage that the initial diagnosis had not been changed was 66.7% in the PS group and 100% in the ICOE-G group. Among other related symptoms only emesis (P<0.001) and visual symptoms (P=0.007) had varying characteristics between the PS and ICOE-G groups. Patients with PS had a better prognosis than those with ICOE-G or unclassified group (odds ratio [OR]=58.8). Patients with 1 more autonomic symptoms had a worse prognosis (OR=3.8). CONCLUSION: This study showed that only symptoms of emesis and visual symptoms can differentiate between PS and ICOE-G. More autonomic symptoms were associated with worse prognosis in childhood occipital lobe epilepsy.


Subject(s)
Humans , Academic Medical Centers , Classification , Diagnosis , Electroencephalography , Epilepsies, Partial , Epilepsy , Follow-Up Studies , Korea , Magnetic Resonance Imaging , Occipital Lobe , Prognosis , Retrospective Studies , Seizures , Vomiting
15.
Article in English | WPRIM | ID: wpr-72767

ABSTRACT

PURPOSE: We investigated the number of test takers of the Korean-Developmental Screening Test (K-DST) in a single children's hospital within a year, according to age, referral rate, and follow-up percentage. METHODS: For this study, 4,062 children who visited and received K-DST at Woorisoa Children's Hospital between January and December 2015 were enrolled. Seven test sets were used according to the Korean National Health Screening Program for infants and children in the following age groups: 4 to 6, 9 to 12, 18 to 24, 30 to 36, 42 to 48, 54 to 60, and 66 to 71 months. The results of the K-DST were categorized into 4 groups as follows: further evaluation (1SD). RESULTS: The test participants' population and follow-up population were concentrated before the age of 24 months (2,532, 62.3%). The children most commonly referred for further evaluation were those in the 30- to 41-month age group. A mismatch was found between the results of the K-DST and the additional questions. Most of the infants and children with suspicious developmental delays showed catch-up development in their follow-up tests (43 of 55, 78.2%). CONCLUSION: The use of K-DST should be encouraged, especially among children aged over 24 months. Multiple-choice question format for the additional questions is recommended to avoid confusion. We suggest a nationwide study to evaluate and revise the K-DST.


Subject(s)
Child , Humans , Infant , Follow-Up Studies , Korea , Mass Screening , Referral and Consultation
16.
Article in English | WPRIM | ID: wpr-88935

ABSTRACT

BACKGROUND AND PURPOSE: Childhood absence epilepsy (CAE) is one of the most common types of pediatric epilepsy. It is generally treated with ethosuximide (ESM), valproic acid (VPA), or lamotrigine (LTG), but the efficacy and adverse effects of these drugs remain controversial. This study compared initial therapy treatment outcomes, including VPA-LTG combination, and assessed clinical factors that may predict treatment response and prognosis. METHODS: Sixty-seven patients with typical CAE were retrospectively enrolled at the Korea University Medical Center. We reviewed patients' clinical characteristics, including age of seizure onset, seizure-free interval, duration of seizure-free period, freedom from treatment failure, breakthrough seizures frequency, and electroencephalogram (EEG) findings. RESULTS: The age at seizure onset was 7.9±2.7 years (mean±SD), and follow-up duration was 4.4±3.7 years. Initially, 22 children were treated with ESM (32.8%), 23 with VPA (34.3%), 14 with LTG (20.9%), and 8 with VPA-LTG combination (11.9%). After 48 months of therapy, the rate of freedom from treatment failure was significantly higher for the VPA-LTG combination therapy than in the three monotherapy groups (p=0.012). The treatment dose administrated in the VPA-LTG combination group was less than that in the VPA and LTG monotherapy groups. The shorter interval to loss of 3-Hz spike-and-wave complexes and the presence of occipital intermittent rhythmic delta activity on EEG were significant factors predicting good treatment response. CONCLUSIONS: This study showed that low-dose VPA-LTG combination therapy has a good efficacy and fewer side effects than other treatments, and it should thus be considered as a firstline therapy in absence epilepsy.


Subject(s)
Child , Humans , Academic Medical Centers , Electroencephalography , Epilepsy , Epilepsy, Absence , Ethosuximide , Follow-Up Studies , Freedom , Korea , Prognosis , Retrospective Studies , Seizures , Treatment Failure , Valproic Acid
17.
Article in English | WPRIM | ID: wpr-110192

ABSTRACT

PURPOSE: Febrile seizure, the most common type of pediatric convulsive disorder, is a benign seizure syndrome distinct from epilepsy. However, as epilepsy is also common during childhood, we aimed to identify the prognostic factors that can predict epilepsy in children with febrile seizures METHODS: The study comprised 249 children at the Korea University Ansan Hospital who presented with febrile seizures. The relationship between the subsequent occurrence of epilepsy and clinical factors including seizure and fever-related variables were analyzed by multivariate analysis. RESULTS: Twenty-five patients (10.0%) had additional afebrile seizures later and were diagnosed with epilepsy. The subsequent occurrence of epilepsy in patients with a history of febrile seizures was associated with a seizure frequency of more than 10 times during the first 2 years after seizure onset (P<0.001). Factors that were associated with subsequent occurrence of epilepsy were developmental delay (P<0.001), preterm birth (P=0.001), multiple seizures during a febrile seizure attack (P=0.005), and epileptiform discharges on electroencephalography (EEG) (P=0.008). Other factors such as the age at onset of first seizure, seizure duration, and family history of epilepsy were not associated with subsequent occurrence of epilepsy in this study. CONCLUSION: Febrile seizures are common and mostly benign. However, careful observation is needed, particularly for prediction of subsequent epileptic episodes in patients with frequent febrile seizures with known risk factors, such as developmental delay, history of preterm birth, several attacks during a febrile episode, and epileptiform discharges on EEG.


Subject(s)
Child , Humans , Electroencephalography , Epilepsy , Korea , Multivariate Analysis , Premature Birth , Risk Factors , Seizures , Seizures, Febrile
18.
Korean Journal of Pediatrics ; : S129-S132, 2016.
Article in English | WPRIM | ID: wpr-201843

ABSTRACT

Ictal tachycardia and bradycardia are common arrhythmias; however, ictal sinus pause and asystole are rare. Ictal arrhythmia is mostly reported in adults with temporal lobe epilepsy. Recently, ictal arrhythmia was recognized as a major warning sign of sudden unexpected death in epilepsy. We present an interesting case of a child with ictal sinus pause and asystole. A 27-month-old girl was hospitalized due to 5 episodes of convulsions during the past 2 days. Results of routine electroencephalography (EEG) were normal, but she experienced brief generalized tonic seizure for 3 days. During video-monitored EEG and echocardiography (ECG), she showed multiple myoclonic seizures simultaneously or independently, as well as frequent sinus pauses. After treatment with valproic acid, myoclonus and generalized tonic seizures were well controlled and only 2 sinus pauses were seen on 24-hour Holter ECG monitoring. Sinus dysfunction should be recognized on EEG, and it can sometimes be treated successfully with only antiepileptic medication.


Subject(s)
Adult , Child , Child, Preschool , Female , Humans , Arrhythmias, Cardiac , Bradycardia , Echocardiography , Electrocardiography , Electroencephalography , Epilepsy , Epilepsy, Temporal Lobe , Heart Arrest , Myoclonus , Seizures , Tachycardia , Valproic Acid
19.
Article in English | WPRIM | ID: wpr-228473

ABSTRACT

Chromosome 11q13 deletion syndrome has been previously reported as either otodental syndrome or oculo-oto-dental syndrome. The otodental syndrome is characterized by dental abnormalities and high-frequency sensorineural hearing loss, and by ocular coloboma in some cases. The underlying genetic defect causing otodental syndrome is a hemizygous microdeletion involving the FGF3 gene on chromosome 11q13.3. Recently, a new form of severe deafness, microtia (small ear) and small teeth, without the appearance of eye abnormalities, was also reported. In this report, we describe a 1-year-old girl presenting with ptosis of the left upper eyelid, right auricular deformity, high-arched palate, delayed dentition, simian line on the right hand, microcephaly, and developmental delay. In this patient, we identified a deletion in the chromosome 11q13.2-q13.3 (2.75 Mb) region by using an array-comparative genomic hybridization analysis. The deletion in chromosome 11q13 results in a syndrome characterized by variable clinical manifestations. Some of these manifestations involve craniofacial dysmorphology and require a functional workup for hearing, ophthalmic examinations, and long-term dental care.


Subject(s)
Female , Humans , Coloboma , Congenital Abnormalities , Congenital Microtia , Deafness , Dental Care , Dentition , Eye Abnormalities , Eyelids , Hand , Hearing , Hearing Loss , Hearing Loss, Sensorineural , Microcephaly , Nucleic Acid Hybridization , Palate , Tooth , Tooth Abnormalities
20.
Article in English | WPRIM | ID: wpr-83158

ABSTRACT

OBJECTIVE: Perinatal hypoxic-ischemic encephalopathy (HIE) and prolonged febrile seizures (pFS) are common neurologic problems that occur during childhood. However, there is insufficient evidence from experimental studies to conclude that pFS directly induces hippocampal injury. We studied cognitive function and histological changes in a rat model and investigated which among pFS, HIE, or a dual pathologic effect is most detrimental to the health of children. METHODS: A rat model of HIE at postnatal day (PD) 7 and a pFS model at PD10 were used. Behavioral and cognitive functions were investigated by means of weekly open field tests from postnatal week (PW) 3 to PW7, and by daily testing with the Morris water maze test at PW8. Pathological changes in the hippocampus were observed in the control, pFS, HIE, and HIE+pFS groups at PW9. RESULTS: The HIE priming group showed a seizure-prone state. The Morris water maze test revealed a decline in cognitive function in the HIE and HIE+pFS groups compared with the pFS and control groups. Additionally, the HIE and HIE+pFS groups showed significant hippocampal neuronal damage, astrogliosis, and volume loss, after maturation. The pFS alone induced minimal hippocampal neuronal damage without astrogliosis or volume loss. CONCLUSION: Our findings suggest that pFS alone causes no considerable memory or behavioral impairment, or cellular change. In contrast, HIE results in lasting memory impairment and neuronal damage, gliosis, and tissue loss. These findings may contribute to the understanding of the developing brain concerning conditions caused by HIE or pFS.


Subject(s)
Animals , Child , Humans , Rats , Brain Injuries , Brain , Epilepsy , Gliosis , Hippocampus , Hypoxia-Ischemia, Brain , Memory , Models, Animal , Neurons , Seizures, Febrile
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