PURPOSE: We investigated articulation patterns in children with ankyloglossia who developed articulation disorders in order to determine the relationship between ankyloglossia and articulation disorders, and to clinically detect children who have articulation disorders associated with ankyloglossia. METHODS: The participants of this study were 23 children with articulation disorders that accompanied ankyloglossia and 55 controls with functional articulation disorders independent of anatomical problems, who were admitted to our hospital from January 1, 2002 to December 31, 2012. All children underwent speech-language pathologic evaluation using the Picture Consonant Articulation Test (PCAT; Young-Tae Kim, 1994). We retrospectively compared collected data between the subject and control groups using Fisher's exact test and odds ratio tests with a 95% confidential interval for categorical variables and the independent Mann-Whitney U-test for continuous variables. RESULTS: The number of patients with articulation errors in the velar nasal was lower significantly only in the subject group (P=0.038). The total numbers of articulation errors in the bilabial plosive, velar plosive and velar nasal also were lower (P=0.007, P<0.001, and P=0.034, respectively). There were no differences in the numbers of patients with articulation errors according to phonological changes between the two groups. However, the total numbers of fronting and frication were lower in the subject group (both P<0.001), but the total numbers of plosivation and tensing were higher (P=0.002 and P=0.008, respectively). CONCLUSION: This study showed that the relationship between an articulation disorder and ankyloglossia is doubtful, although some results suggest that ankyloglossia may cause articulation errors only in certain individuals. Therefore, clinicians should be careful when determining the relationship between ankyloglossia and articulation disorders and use caution when making a treatment decision.
Articulation Disorders* ; Child* ; Humans ; Odds Ratio ; Retrospective Studies ; Transcutaneous Electric Nerve Stimulation

PURPOSE: South Korean adolescents have been identified as a chronic partial sleep-deprived population in several previous studies. This study was to identify the current nocturnal sleep duration by age in South Korean adolescents and to analyze the association of emotional status factors such as subjective happiness, depression and suicidal attempts, and health-risk behaviors with the nocturnal sleep duration. METHODS: The findings in this study are based on the data obtained from the 7th Korea Youth Risk Behavior Web-Based Survey (KYRBWS-VII), a cross-sectional, annual survey of the health-risk behaviors in a representative sample of South Korean middle- and high-school students aged 13-18 years, which was conducted in 2011. Out of 75,643 students from 800 schools across the nation, 75,205 students were selected by using the complex sampling design of the survey. We analyzed the relationships between the duration of nocturnal sleep and emotional status (subjective happiness, stress level, depression, and suicidal thoughts), health-risk behaviors (smoking, drinking), and sleep satisfaction. RESULTS: There was a significant relationship between the duration of sleep and the emotional status. In general, happy students had the longest nocturnal sleep duration, regardless of their grades. Self-rated stress levels and depression in adolescents were inversely proportional to nocturnal sleep duration. Health-risk behaviors such as smoking and alcohol consumptions were more common in sleep-deprived students (P<0.001). CONCLUSION: Appropriate nocturnal sleep duration is critical for emotional health as well as prevention of suicide in the adolescent population.
Adolescent* ; Depression ; Happiness ; Humans ; Korea ; Risk-Taking ; Sleep Deprivation ; Smoke ; Smoking ; Suicidal Ideation ; Suicide

PURPOSE: This study was aimed to evaluate the clinical features of hypoxic ischemic encephalopathy(HIE) in children with and without seizures. METHODS: Fifty five children who had been diagnosed as HIE at Inha University Hospital from June 1999 to December 2011 were enrolled in this study. Subjects were divided into two groups by the presence of seizures and their medical records were retrospectively analyzed. RESULTS: Among the 55 cases, 34 patients (61.8%) had seizures, while 17 patients (32.2%) did not have them. Male to female ratio was 1:1 for the 'seizure' group and 2.5:1 for the 'no seizure' group. The onset age was 9.7 months (range: 0-158 months) for the 'seizure' group and 10 months (range : 0-108 months) for the 'no seizure' group. The most common risk factor was birth asphyxia (17.7%) for the 'seizure' group, and prematurity (23.8%) for the 'no seizure' group. The most common symptom other than seizure was respiratory arrest for both groups. On radiologic imaging studies of the brain, main causative lesion was most commonly observed in the cerebral cortex in both groups. The neurologic deficits or death were detected in 67.7% of the 'seizure' group, and 76.3% of the 'no seizure' group. There were no statistically significant differences in risk factors between the two groups. CONCLUSION: Although the characteristics between patients with and without seizures from HIE revealed no significant differences, HIE still can result in death or permanent disability in children. Therefore, permanent brain damage may be minimized by early suspicion and treatment in these patients.
Age of Onset ; Asphyxia ; Brain ; Cerebral Cortex ; Child* ; Female ; Humans ; Hypoxia-Ischemia, Brain* ; Male ; Medical Records ; Neurologic Manifestations ; Parturition ; Retrospective Studies ; Risk Factors ; Seizures*

PURPOSE: Neonatal seizures are one of the most common neurologic manifestations in neonates and could be the important clinical sign of underlying brain disorders. The aim of this study is to review the clinical characteristics and to find the prognostic factors related to the outcomes of neonatal seizures. METHODS: We reviewed medical records retrospectively in 23 patients with neonatal seizures who admitted to Dankook University Hospital from July 2007 to June 2009. RESULTS: During the study period, neonatal seizures were diagnosed in 23/1,474 (1.56%) neonates. Nineteen of them (82.6%) were term and 4 were preterm. The main cause of neonatal seizures was hypoxic ischemic encephalopathy (n=8, 35%). Other various causes included metabolic disorders (n=4, 17%, carnitine palmitoyl transferase 1 deficiency, severe hypernatremic dehydration, prolonged severe hypoglycemia, and pyridoxine dependent seizure), intracranial hemorrhages (n=2, 9%), congenital brain anomaly (callosal dysgenesis, hemimegalencephaly) (n=2, 9%), and infection (congenital syphilis, early neonatal sepsis, n=2, 9%). Among nineteen neonates (82.6%) who were treated with anticonvulsants, we could not stop the anticonvulsants in six of them (32%). They had severe HIE, prolonged severe hypoglycemia with residual encephalomalatic changes, sinovenous thrombotic hemorrhages due to antithrombin III deficiency, congenital brain anomaly, and septic shock, respectively. CONCLUSION: Approximately one third of neonatal seizures were caused by HIE, and moderate to severe HIE had more serious outcomes. Neonatal seizure could be a significant clinical sign indicating specific underlying etiologies such as stroke, metabolic disturbances or congenital brain anomalies. Therefore, intensive workup and prompt management for neonatal seizures should be considered for better outcomes.
Anticonvulsants ; Antithrombin III Deficiency ; Brain ; Brain Diseases ; Carnitine ; Dehydration ; Hemorrhage ; Humans ; Hypoglycemia ; Hypoxia-Ischemia, Brain ; Infant, Newborn ; Intracranial Hemorrhages ; Medical Records ; Neurologic Manifestations ; Prognosis ; Pyridoxine ; Retrospective Studies ; Seizures* ; Sepsis ; Shock, Septic ; Stroke ; Syphilis ; Transferases

PURPOSE: Status epilepticus(SE) is one of the most common neurologic emergencies in children, which might result in significant morbidity and mortality. The aim of this study was to evaluate the changes of etiology and prognosis, clinical outcomes in children with status epilepticus during the period between the 1990s and 2000s at a single institution. METHODS: We retrospectively reviewed and analyzed the records of two groups (group A and B) of children with SE at the Department of Pediatrics of our institution. Group A consisted of 98 children aged between 2 months and 15 years and admitted from January, 1992 to December, 1998. Group B consisted of 79 children at the same age and admitted from November, 2008 to July, 2011. We compared age distribution, etiology, duration, abnormality of EEG and Brain CT/MRI, and neurologic complications between the two groups. RESULTS: Compared with group A, the mortality rate of SE in group B improved from 7.1% to 1.3%. Neurological complication rates also improved from 21.4% (group A) to 18.3% (group B). Duration of SE decreased from 71.4+/-82.6 minutes (group A) to 42.0+/-53.8 minutes (group B). Etiologies of SE between two periods were not significantly different, but the duration of SE decreased, and mortality and neurologic outcomes improved (P<0.001). The duration of SE was significantly correlated with neurologic complications (R2=0.214, P<0.001). CONCLUSION: This study showed decreased mortality and neurologic complication rates in SE over the last decade. The Decrease of mortality and neurologic complication rates may be due to the decreased duration of SE and more immediate visit of patients to the hospital than in the past.
Age Distribution ; Brain ; Child* ; Electroencephalography ; Emergencies ; Humans ; Mortality ; Pediatrics ; Prognosis ; Retrospective Studies ; Status Epilepticus*

Many children with epilepsy remain medically intractable inspire of the best available therapies. One of the most difficult seizure types to treat is infantile spasms. Many children will respond to treatment with ACTH but there are may serious side effects and many children relapse when the medication is reduced or discontinued. Thus, new therapies are critically needed for this group of patients. Early evidence from European trials indicated that vigabatrin (Sabril) was effective in this population. A randomized, single blind, high-dose vs low dose vigabatrin study was performed in a multicenter USA trial. Between January 1996 and May 1997, 89 patients from 7 pediatric epilepsy centers were started in the study. An interim evaluation was performed on September 14, 1997. Of the 89 patients, data was not yet available for 16 so 73 patients were evaluable for safety. Efficacy was evaluated in 63 of the patients. Two were eliminated because they had violated the entry criteria and 8 did not yet report the efficacy information and had to be excluded from the interim report.
Adrenocorticotropic Hormone ; Child* ; Epilepsy* ; Humans ; Infant ; Infant, Newborn ; Recurrence ; Seizures ; Spasms, Infantile ; Vigabatrin*

Neonatal seizures are only a manifestation of a variety of organic, metabolic or functional disorders of the neonatal brain. Most are caused by acute brain insults such as perinatal hypoxic-ischemic encephalopathy, intracranial hemorrhage or CNS infections. Recent years have witnessed a remarkable reduction of simple hypocalcemia, resulting in a relative increase of hypoxic-ischemic encephalopathy. These acute symptomatic seizures are different from epilepsy, a chronic seizure disorder. Most previous studies concerning seizure manifestations of the newborn have dealt with all types of neonatal seizures together, regardless of etiology. It is true that seizures in the neonate pose unique diagnostic and therapeutic problems because of the immaturity of the neonatal brain, but it is not appropriate to study seizure types in the newborn on the basis of acute symptomatic seizures such as those occurring in hypoxic-ischemic encephalopathy in which acute brain swelling or neuronal necrosis may obscure the unique developmental state of the neonatal brain. No one would attempt to classify the seizure types in older children with acute encephalitis and epilepsy together. Such acute symptomatic neonatal seizures should be excluded from the classification of epileptic seizures and syndromes, although they may evolve into symptomatic epilepsy later as a sequel. The current International Classification of Epilepsies and Epileptic Syndromes lists five epileptic syndromes in which neonatal seizures are a major manifestation; benign familial neonatal convulsions(BFNC), benign neonatal convulsion(BNC), early infantile epileptic encephalopathy with suppression-burst(EIEE), early myoclonic enecphalopathy(EME), and neonatal seizures. BFNC and BNC are categorized in idiopathic generalized epilepsies according to the International Classification. BFNC are described as being associated with clonic or apneic seizures, although the ictal EEGs of these seizures have been well documented in only a small number of cases. Seizure types reported to date were partial or generalized. Three of four patients in whom we were able to record ictal EEGs displayed definite partial seizures. One diagnosed as haying generalized seizures did not show typical electroclinical features of generalized tonic-clonic convulsions.
Brain ; Brain Edema ; Child ; Classification ; Electroencephalography ; Encephalitis ; Epilepsy ; Epilepsy, Generalized ; Humans ; Hypocalcemia ; Hypoxia-Ischemia, Brain ; Infant, Newborn ; Intracranial Hemorrhages ; Necrosis ; Neurons ; Seizures*

A 4-year-old girl developed acute motor, sensory impairment, lower abdominal pain and urinary dysfunction after URI. In acute stage of disease, MRI showed diffuse spinal cord swelling and high signal intensity on T2WI below T2 body level. The patient did not improved and one month later, follow-up MRI showed diffuse spinal cord atrophy and syrinx formation as sequelae of ATM on T1WI below T9 level. To our knowledge, this is the first case showing the sequelae of acute transverse myelitis seen in Korea.
Abdominal Pain ; Atrophy* ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Korea ; Magnetic Resonance Imaging ; Myelitis, Transverse* ; Spinal Cord*

Canavan disease(CD) is a rare autosomal recessive leukodystrophy caused by the deficiency of aspartoacylase and the accumulation in brain of N-acetylaspartate(NAA). CD has been reported mainly Ashkenazi Jews but also occurs in other ethnic groups. Usually it presents as early as the third month of life with megalencephaly, hypotonia later progressing to hypertonia, psychomotor and mental retardation, blindness, occasionally deafness and seizure. Diagnosis is based on the clinical feature, N-acetylaspartic aciduria, radiologic and pathologic findings. Histologically, the affected white matter shows extensive vacuolation and demyelination. There is no treatment for CD and the only prevention is through genetic counselling and prenatal diagnosis. We experienced a case of Canavan disease that was presented with hypotonia and developmental delay. Diagnosis was confirmed histologically. Radiologic findings are extensive high signal throughout the white matter on T2-weighted MRI and increased NAA peak and decreased choline peak of the white matter on MR spectroscopy.
Blindness ; Brain ; Canavan Disease* ; Choline ; Deafness ; Demyelinating Diseases ; Diagnosis ; Ethnic Groups ; Humans ; Intellectual Disability ; Jews ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Muscle Hypotonia ; Prenatal Diagnosis ; Seizures

Joubert syndrome is a rare hereditary brain malformation and transmitted as an autosomal recessive tarit. This disorder is clinically characterized by episodic tachypnea and apnea, abnormal ocular movements, developmental delay and ataxia. Anatomic anomalies include cerebellar vermal agenesis with dilatation of the fourth ventricle. Symptomatic onset is in the neonatal period and prognosis is severe. We have experienced a case of Joubert syndrome in a 3months old male patient, who manifested by developmental delay, periodic tachypnea and apnea, abnormal eye movement, generalized hypotonia and hypoplasia of cerebellar vermis with the 4th ventricular dilatation on brain MRI. We presented this case with a brief review of literatures.
Apnea ; Ataxia ; Brain ; Dilatation ; Eye Movements ; Fourth Ventricle ; Humans ; Magnetic Resonance Imaging ; Male ; Muscle Hypotonia ; Prognosis ; Tachypnea