After the first description of infantile spasms (IS) in 1841, extensive clinical and laboratory investigations have been done to find the pathophysiology and the optimal treatments. The concept of the “infantile spasms” has been evolved to the “epileptic spasms”, which includes the spasms outside the infancy the pathophysiology of IS, however, is still unknown. There have been a few randomized trials that proved the efficacy of the anecdotally used drugs in IS including hormonal therapy and vigabatrin. Due to its relative low incidence (1/2000) and the variable etiologies, clinical studies have difficulties in making a clear conclusion. Thus, animal models were eagerly sought to find the pathophysiology based treatments with definite efficacy and several models are now available. In this paper, the current understandings of the epileptic spasms as well as the translational researches using the animal models of IS are reviewed. The latest evidences of therapeutics in IS are discussed shortly.
Incidence ; Infant ; Infant, Newborn ; Models, Animal ; Spasm* ; Spasms, Infantile ; Translational Medical Research ; Vigabatrin

Cyclic vomiting syndrome (CVS) is a paroxysmal, recurrent vomiting disorder of unknown pathophysiology and target organ. It has been hypothesized that CVS shares the same mechanism as migraine. We describe here a 5-year-old boy with CVS characterized by episodic vomiting attacks. These recurrent vomiting episodes began at 3 years of age, occurred every month and lasted for 5 days at a time. At the time of admission, no abnormal physical or neurological findings were observed and laboratory findings, including brain MRI and endoscopic examination, revealed nothing specific. The vomiting episodes were self-limited but recurrent and severely interrupted his daily life. When this patient was treated with topiramate, he showed a marked increase of symptom-free periods.
Brain ; Child ; Child, Preschool ; Humans ; Magnetic Resonance Imaging ; Male ; Migraine Disorders ; Vomiting*

The posterior reversible encephalopathy syndrome(PRES) is characterized clinically by a combination of acute or subacute confusion, lethargy, visual disturbance, and seizures. PRES has been described in various clinical settings, including severe hypertension, chemotherapy, eclampsia, and seizure. We report a case of a 7-year-old girl who had taken cyclosporine for steroid resistant nephrotic syndrome. Twenty one days after the cyclosporine therapy, she was admitted due to generalized tonic clonic seizure and headache. Her blood pressure was 170/90 mmHg. Magnetic resonance(MR) imaging showed necrotic/cystic lesions involving the bilateral parieto-occipital region. After discontinuation of cyclosporine, and control of blood pressure, she had no more seizure and headache. The follow-up MR examination which was performed 6 months later showed the decreased extent of the lesion.
Blood Pressure ; Child* ; Cyclosporine* ; Drug Therapy ; Eclampsia ; Female ; Follow-Up Studies ; Headache ; Humans ; Hypertension ; Lethargy ; Nephrotic Syndrome* ; Posterior Leukoencephalopathy Syndrome* ; Pregnancy ; Seizures

Neonatal seizures represent a heterogeneous group of disorders with vastly different etiologies and outcomes. Benign familial neonatal convulsions (BFNC) are a distinctive epileptic syndrome of autosomal dominant inheritance with a favorable prognosis, characterized by the occurrence of unprovoked partial or generalized clonic seizures in the neonatal period or early infancy. Recently, mutations in two potassium channel genes, KCNQ2 and KCNQ3, have been described in this disorder. In this report, we describe a family with BFNC due to a KCNQ2 mutation, the first such family to be described in the Korean population. The diagnosis of BFNC can be made based on clinical suspicion and careful history taking with special emphasis on the familial nature of the disorder. KCNQ2 mutations may be associated with BFNC in a number of different races, as has been reported in other ethnic groups.
Electroencephalography ; Epilepsy, Benign Neonatal/*diagnosis/genetics ; Female ; Humans ; Infant, Newborn ; KCNQ2 Potassium Channel/*genetics ; Magnetic Resonance Imaging ; Mutation ; Pedigree ; Republic of Korea ; Sequence Analysis, DNA

Respiratory syncytial virus (RSV) is an extremely common cause of childhood respiratory infection resulting in significant morbidity and mortality especially in young infants and premature babies. There have been a few reports about seizures or encephalopathy in children with RSV infection. We describe here refractory status epilepticus in two preterm babies with severe respiratory illness by RSV infection. The seizures were refractory to phenobarbital and diphenylhydantoin, but ceased by continuous midazolam infusion. After several days with clinical improvement of respiratory illness, their seizures were stable on phenobarbital maintenance only. Although rare, status epilepticus can be a form of neurologic manifestation of severe RSV infection in preterm baby. We must be aware of their neurological manifestations; continuous EEG monitoring is helpful for the diagnosis of the status epilepticus in infants.
Child ; Electroencephalography ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Midazolam ; Neurologic Manifestations ; Phenobarbital ; Phenytoin ; Respiratory Syncytial Viruses ; Seizures ; Status Epilepticus

PURPOSE: Recurrent bacterial meningitis represents the reappearance of two or more episodes of meningitis by a different or same organism after an intervening period of full convalescence. Predisposing factors for recurrent bacterial meningitis include developmental or traumatic anatomical defects or immunodeficiencies. The purpose of this study was to characterize recurrent bacterial meningitis in children. METHODS: We identified 81 cases of bacterial meningitis diagnosed at the Asan Medical Center Children's Hospital between January 1999 and March 2009. We conducted retrospective reviews for children (below 18 years of age) with recurrent bacterial meningitis who had been diagnosed both by latex agglutination and positive cerebrospinal fluid (CSF) cultures. RESULTS: Among 81 cases, 4 patients were identified as having recurrent bacterial meningitis, among whom 13 episodes of meningitis were identified. Two episodes occurred in 2 patients, 2 in 1 patient, and 6 in 1 patient. Three patients had inner ear anomalies with CSF leakage and 1 patient had a traumatic CSF fistula, representing predisposing factors. Of the 13 episodes, Streptococcus pneumoniae was the causative agent in 10 episodes and Hemophilus influenzae and Streptococcus. mitis were the other causative organisms. After the second case of meningitis, successful repair of CSF leakage prevented further development of meningitis in 3 patients. Despite several repair operations for CSF leakage, 1 patient died of cerebral edema during the 6th episode of meningitis. In addition, 2 patients experienced further episodes of meningitis after vaccination against pneumococcus or H. influenzae type B in our study. CONCLUSION: Identification and proper management of conditions that predispose children to recurrent bacterial meningitis are essential to prevent further, potentially lethal infections.
Agglutination ; Brain Edema ; Child ; Convalescence ; Ear, Inner ; Fistula ; Haemophilus influenzae ; Humans ; Influenza, Human ; Latex ; Meningitis ; Meningitis, Bacterial ; Retrospective Studies ; Streptococcus ; Streptococcus pneumoniae ; Vaccination

PURPOSE: This study compared the efficacy and tolerability of intravenous (i.v.) phenobarbital (PHB) and i.v. levetiracetam (LEV) in children with status epilepticus (SE) or acute repetitive seizure (ARS). METHODS: The medical records of children (age range, 1 month to 15 years) treated with i.v. PHB or LEV for SE or ARS at our single tertiary center were retrospectively reviewed. Seizure termination was defined as seizure cessation within 30 minutes of infusion completion and no recurrence within 24 hours. Information on the demographic variables, electroencephalography and magnetic resonance imaging findings, previous antiepileptic medications, and adverse events after drug infusion was obtained. RESULTS: The records of 88 patients with SE or ARS (median age, 18 months; 50 treated with PHB and 38 with LEV) were reviewed. The median initial dose of i.v. PHB was 20 mg/kg (range, 10-20 mg/kg) and that of i.v. LEV was 30 mg/kg (range, 20-30 mg/kg). Seizure termination occurred in 57.9% of patients treated with i.v. LEV (22 of 38) and 74.0% treated with i.v. PHB (37 of 50) (P=0.111). The factor associated with seizure termination was the type of event (SE vs. ARS) in each group. Adverse effects were reported in 13.2% of patients treated with i.v. LEV (5 of 38; n=4, aggressive behavior and n=1, vomiting), and 28.0% of patients treated with i.v. PHB (14 of 50). CONCLUSION: Intravenous LEV was efficacious and safe in children with ARS or SE. Further evaluation is needed to determine the most effective and best-tolerated loading dose of i.v. LEV.
Child* ; Electroencephalography ; Humans ; Magnetic Resonance Imaging ; Medical Records ; Phenobarbital* ; Recurrence ; Retrospective Studies ; Seizures* ; Status Epilepticus*

PURPOSE: This study was performed to investigate the change in the causative organisms of bacterial meningitis and the prevalence of bacterial meningitis caused by antibiotics resistant strains in a single Korean tertiary center. METHODS: We retrospectively analyzed medical records of patients who had been diagnosed with bacterial meningitis from March 1999 to March 2010 at the Asan Medical Center. The bacterial meningitis was defined as the correlated clinical symptoms and the isolation of organisms from the cerebrospinal fluid culture. RESULTS: During the 11-year study period, we found 81 cases of CSF-culture proven bacterial meningitis. Streptococcus agalactiae (group B streptococcus, GBS) were most common bacteria accounting for 23 (28.3%) cases, followed by Streptococcus pneumoniae with 22 (27.2%), and Haemophilus influenzae with 8 (9.9%). After introduction of the vaccination, only one case of H. influenzae meningitis was documented. Seventeen of 18 (94.4%) cases of GBS were sensitive to the penicillin, whereas 82.4% (14/17) of S. pneumoniae were resistant to penicillin. Among the 17 cases of S. pneumoniae, 11 cases (64.7%) were multi-drug resistant strains but all were susceptible to vancomycin. CONCLUSION: In spite of the introduction of the vaccination, S. pneumoniae was the most common cause of bacterial meningitis in children older than two months. For pneumococcal meningitis, careful selection of antibiotics and ongoing research about antibiotics susceptibility will be needed due to multi-drug resistance.
Accounting ; Anti-Bacterial Agents ; Bacteria ; Child ; Drug Resistance ; Drug Resistance, Multiple ; Haemophilus influenzae ; Humans ; Influenza, Human ; Medical Records ; Meningitis ; Meningitis, Bacterial ; Meningitis, Pneumococcal ; Penicillins ; Pneumonia ; Prevalence ; Retrospective Studies ; Streptococcus ; Streptococcus agalactiae ; Streptococcus pneumoniae ; Vaccination

PURPOSE: The aim of this study was to evaluate the data of 11 patients who had excessive drooling attributable to various diseases such as hypoxic ischemic encephalopathy, spinal muscular atrophy, and esophageal stricture treated with the injection of botulinum toxin A. METHODS: Eleven children with excessive drooling were enrolled in a retrospective clinical evaluation. Eighty to a hundred units of botulinum toxin A were injected into the patients' parotids, submandibular glands under sonographic guide. Subjective measures including Teacher Drooling Scale(TDS) by the patients' parents or caregivers were used to determine the effect of botulinum toxin A on drooling and to document the severity and frequency of children's drooling. RESULTS: The TDS and number of suctions per day demonstrated a significant reduction at 1 week, 1 month, and 3 months in most of the patients. We defined a 2 point decrease on the TDS as "success to therapy". Five of the eleven patients(45.5%) responded to botulinum toxin A injections. Of children who responded, the definite reduction of drooling was noticed at 4 weeks after the injections in three children, at 12 weeks in one, and at 1 week in the other. No adverse effects were observed during and after the injections in this study. CONCLUSION: Parotid and submandibular botulinum toxin A injection is an effective method for the reduction of excessive drooling, demonstrating a high response rate up to 12 weeks. The procedure is simple to perform, and safe when ultrasonographic guidance is used.
Botulinum Toxins ; Caregivers ; Cerebral Palsy ; Child ; Esophageal Stenosis ; Humans ; Hypoxia-Ischemia, Brain ; Muscular Atrophy, Spinal ; Parents ; Retrospective Studies ; Sialorrhea ; Sorbitol ; Submandibular Gland ; Suction ; Tyramine

PURPOSE: We aimed to compare the effectiveness of 2 developmental tests-Korean Infant and Child Development Test (KICDT) and Korean Bayley Scale of Infant Development-II (K-BSID-II)-in the assessment of children with developmental delay. METHODS: Twenty-eight children with suspected developmental delay, who visited the Department of Pediatrics in Asan Medical Center from February 2007 to June 2008 were enrolled. They were examined using both KICDT and K-BSID-II. The results of the tests were compared on the basis of 2 parameters: age group of the children and detection of organic brain lesion on magnetic resonance imaging (MRI). The correlation between the results of the 2 tests was analyzed using SPSS. Further, intraclass correlation coefficient was calculated using SAS to examine consistency between the results of the 2 tests. RESULTS: The mental developmental index of K-BSID-II showed significant correlation with every KICDT score (P<0.01). The psychomotor developmental index of K-BSID-II was also significantly correlated with every KICDT score except the fine motor score (P<0.01). The mental developmental index of K-BSID-II showed significant correlation [Editor20] with the KICDT gross motor, fine motor, and language scores (P<0.05). Further, there was significant correlation between the psychomotor developmental index of K-BSID-II and the KICDT gross motor score (P<0.05). CONCLUSION: There was significant [Editor21]correlation between the results of KICDT and K-BSID-II for infants and children with developmental delay. Although our results suggest that KICDT is useful in assessing developmental delay, further research would be needed to standardize this test.
Brain ; Child ; Child Development ; Humans ; Infant ; Magnetic Resonance Imaging ; Pediatrics ; Phenothiazines