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1.
Article in English | WPRIM | ID: wpr-999245

ABSTRACT

Purpose@#Patients with atopic dermatitis (AD) experience a chronic relentless course which may affect emotional and behavioral problems. Moreover, AD could affect serious psychosocial problems due to its disfiguring skin conditions, particularly in adolescents who are under various stresses. The aim of this study was to investigate behavioral problems in children and adolescents with AD and to identify associated clinical factors. @*Methods@#A total of 69 subjects with AD were enrolled in this study. The severity of AD was assessed using the SCORAD (Scoring of Atopic Dermatitis) index and divided into 2 groups: mild-to-moderate and severe groups. Behavioral problems were assessed by using the Korean Child Behavior Checklist (K-CBCL). @*Results@#The severe AD group had higher total behavior problem scores and internalizing scores than the mild-to-moderate group. Social problem and attention problem scores were slightly higher in the severe AD group than in the mild-to-moderate AD group. The DSM (diagnostic and statistical manual of mental disorders)-anxiety, obsessive compulsive problems, and sluggish cognitive tempo problems were more frequently observed in the severe AD group. Longer disease duration was an independent risk factor of internalizing problems in subjects with AD. @*Conclusion@#This study showed that subjects with severe AD had higher total behavioral problem scores, particularly internalizing scales. Their behavioral problems may have poor compliance and consequently poor outcomes as well. Clinicians should be aware that children and adolescents with severe AD have more behavioral problems and that an interdisciplinary approach with medical and psychological supports is necessary for proper management.

2.
Article in Korean | WPRIM | ID: wpr-926265

ABSTRACT

Vestibular migraine and benign paroxysmal vertigo are the most common causes of vertigo in children and adolescents. This is a review of vestibular migraine of childhood dizziness.Current Concepts: In children and adolescents, the symptoms of dizziness or vertigo are not well defined. Thus, few studies have investigated the characteristics of dizziness or vertigo in children and adolescents. Vestibular migraine is categorized as episodic syndromes associated with migraine in the International Classification of Headache Disorders, 3rd edition. Despite vertigo’s various clinical features and duration, vestibular migraine can be diagnosed only when a migraine accompanies vertigo from 5 minutes to 72 hours. Benign paroxysmal vertigo is more common in children and adolescents than vestibular migraine. Benign paroxysmal vertigo is considered one of the precursor syndrome of migraine, and the duration of vertigo is short from seconds to minutes.Discussion and Conclusion: Vestibular migraine is common in older children and adults who need a differential diagnosis. This review might be helpful to diagnose, evaluate, and treat children with vestibular migraine and to reassure their parents.

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-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.

5.
Neonatal Medicine ; : 159-166, 2020.
Article in English | WPRIM | ID: wpr-895107

ABSTRACT

Purpose@#Preterm infants are known to be at a risk of neurodevelopmental delay; however, limited data are available on the outcomes of moderate-to-late preterm (MLPT) infants (born at 32 to 36 weeks’ gestation). The Korean Developmental Screening Test (K-DST) for infants and children is a recently designed screening test for Korean infants and children. The current study aimed to evaluate the neurodevelopmental outcomes of MLPT infants and investigate the risk factors associated with neurodevelopmental delay. @*Methods@#A total of 119 MLPT infants admitted to a neonatal intensive care unit (NICU) of a tertiary hospital in Korea were enrolled. The infants were assessed during two follow-up periods (first: 16 to 24 months of corrected age; second: 24 to 41 months of corrected age). The perinatal factors in the NICU that were associated with delayed development were analyzed. @*Results@#In all sections of the K-DST, the proportion of infants with developmental delay was higher in the second period (5.6% to 9.3%) than in the first period (0.9% to 5.4%). A total of 10% to 17% of the infants presented with persistent delay throughout the two periods based on five sections of the K-DST. Male sex, oxygen therapy duration, and younger maternal age were the risk factors affecting at least one section during the second period. @*Conclusion@#MLPT infants showed greater developmental delay than the general infant population. Considering that early intervention is important for good longterm outcomes, close observation of male MLPT infants and MLPT infants who received oxygen therapy is warranted.

6.
Neonatal Medicine ; : 159-166, 2020.
Article in English | WPRIM | ID: wpr-902811

ABSTRACT

Purpose@#Preterm infants are known to be at a risk of neurodevelopmental delay; however, limited data are available on the outcomes of moderate-to-late preterm (MLPT) infants (born at 32 to 36 weeks’ gestation). The Korean Developmental Screening Test (K-DST) for infants and children is a recently designed screening test for Korean infants and children. The current study aimed to evaluate the neurodevelopmental outcomes of MLPT infants and investigate the risk factors associated with neurodevelopmental delay. @*Methods@#A total of 119 MLPT infants admitted to a neonatal intensive care unit (NICU) of a tertiary hospital in Korea were enrolled. The infants were assessed during two follow-up periods (first: 16 to 24 months of corrected age; second: 24 to 41 months of corrected age). The perinatal factors in the NICU that were associated with delayed development were analyzed. @*Results@#In all sections of the K-DST, the proportion of infants with developmental delay was higher in the second period (5.6% to 9.3%) than in the first period (0.9% to 5.4%). A total of 10% to 17% of the infants presented with persistent delay throughout the two periods based on five sections of the K-DST. Male sex, oxygen therapy duration, and younger maternal age were the risk factors affecting at least one section during the second period. @*Conclusion@#MLPT infants showed greater developmental delay than the general infant population. Considering that early intervention is important for good longterm outcomes, close observation of male MLPT infants and MLPT infants who received oxygen therapy is warranted.

7.
Article in English | WPRIM | ID: wpr-728816

ABSTRACT

PURPOSE: Febrile seizure (FS) is the most common type of seizure in children between 6 months to 5 years of age. A family history of febrile seizures can increase the risk a child will have a FS. Yet, prevalence of FS regarding external environment has not been clearly proved. This study attempts to determine the association between prevalence of FS and weather. METHODS: This study included medical records from the Korea National Health Insurance Review and Assessment Service. Data were collected from 29,240 children, born after 2004, diagnosed with FS who were admitted to one of the hospitals in Seoul, Korea, between January 2009 and December 2013. During the corresponding time period, data from the Korea Meteorological Administration on daily monitoring of four meteorological factors (sea-level pressure, amount of precipitation, humidity and temperature) were collected. The relationships of FS prevalence and each meteorological factor will be designed using Poisson generalized additive model (GAM). Also, the contributory effect of viral infections on FS prevalence and weather will be discussed. RESULTS: The amount of precipitation was divided into two groups for comparison: one with less than 5 mm and the other with equal to or more than 5 mm. As a result of Poisson GAM, higher prevalence of FS showed a correlation with smaller amount of precipitation. Smoothing function was used to classify the relationships between three variables (sea-level pressure, humidity, and temperature) and prevalence of FS. FS prevalence was correlated with lower sea-level pressure and lower humidity. FS prevalence was high in two temperature ranges (-7 to -1℃ and 18–21℃). CONCLUSION: Low sea-level pressure, small amount of precipitation, and low relative air humidity may increase FS prevalence risk.


Subject(s)
Child , Humans , Fever , Humidity , Korea , Medical Records , Meteorological Concepts , National Health Programs , Prevalence , Seizures , Seizures, Febrile , Seoul , Weather
8.
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
9.
Article in English | WPRIM | ID: wpr-114696

ABSTRACT

PURPOSE: Recent data indicate that sensitization to mold contributes to the severity and persistence of asthma. In this study, we investigated the relationships between sensitization to mold and lung function parameters in children with asthma. METHODS: We retrospectively reviewed clinical data from 551 asthmatic subjects. We selected subjects who met clinical diagnostic criteria of asthma. Their spirometry, methacholine challenge tests, and measurements of blood eosinophils, serum IgE, eosinophil cationic protein (ECP) and fractional exhaled nitric oxide (FeNO) results were included. Skin prick testing (SPT) results with 13 common aeroallergens in Korea including house dust mites, animal dander, pollen, cockroach and mold were reviewed. Subjects were divided into 3 groups according to their SPT results. Subjects who showed no positive result to any aeroallergen were designated as group 1 (non-sensitized). Group 2 represented subjects who were sensitized to aeroallergens other than mold (other allergen-sensitized) and group 3 included subjects who were sensitized to mold allergens (mold-sensitized). RESULTS: Among the 551 asthmatic subjects, 67 (12.2%) were sensitized to mold and 366 (66.4%) were sensitized to other aeroallergens. The log mean IgE levels were higher in groups 2 (5.96±1.14 IU/mL) and 3 (5.81±0.97 IU/mL) compared to group 1 (3.88±1.68 IU/mL). Blood eosinophils, ECP and FeNO concentrations were significantly higher in groups 2 and 3, but no significant difference was found between the 2 groups. The mean FEV1 value was significantly lower in group 3 (86.9±12.1%pred) than in groups 2 (92.0±14.8%pred) and 1 (93.4±15.4%pred). The log mean methacholine PC20 was significantly lower in group 3 (0.08±1.91 mg/mL) than in groups 2 (1.31±1.69 mg/mL) and 1 (2.29±1.66 mg/mL). CONCLUSIONS: We observed a differential association between mold and other aeroallergen sensitization, and severity of asthma. Sensitization to mold is associated with lower lung function and increased airway hyper-responsiveness in children with asthma. Mold sensitization could be an important factor determining asthma severity particularly airflow limitation in children.


Subject(s)
Animals , Child , Humans , Allergens , Asthma , Cockroaches , Dander , Eosinophil Cationic Protein , Eosinophils , Fungi , Immunoglobulin E , Korea , Lung , Methacholine Chloride , Nitric Oxide , Pollen , Pyroglyphidae , Respiratory Hypersensitivity , Retrospective Studies , Skin , Spirometry
10.
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
11.
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
12.
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
13.
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
14.
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
15.
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
16.
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
17.
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
18.
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
19.
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
20.
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
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