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

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

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: The isolated microscopic hematuria is the most common abnormality detected by school urinary screening, but there is no consensus about the range of investigations and long-term outcomes of isolated hematuria in children yet. This study aims to elucidate the prognosis of hematuria and the range of diagnostic studies by follow-up results. METHODS: Students with isolated hematuria who were referred to the Department of Pediatrics, Asan Medical Center from Aug. 1990 to Feb. 2004 were analysed retrospectively. Cases that presented Through significant proteinuria(>250 mg/day), other symptoms of nephritis or renal dysfunction (creatinine clearance <85 mL/min/1.73m2) were excluded. Follow-up was done every six months with checking urinalysis, serum creatinine, protein and albumin. When albuminuria was detected, 24 hour urine protein was checked. Renal biopsy was done when urine protein was over 500 mg/day. RESULTS: A total of 331 students were enrolled in this study. There were 157 males and 174 females. The mean age at presentation was 9.9+/-2.3 years(7-15 years) and mean follow-up period was 2.2+/-1.6 years(1-10 years). Seventy five(22.7 percent) patients showed the resolution of microscopic hematuria. The mean resolution period was 2.6+/-1.7 years(1-8 years). Eight(2.4 percent) patients developed significant proteinuria and renal biopsy was done in four of them. Two cases of mild IgA nephropathy and two of minimal change were detected. None of them developed hypertension. At the end of the follow-up, renal function had remained stable in all subsets of patients. CONCLUSION: The prognosis of isolated microscopic hematuria was good. This study suggests that invasive studies including renal biopsy are not necessary and a regular follow-up of urinalysis is enough for children with isolated microscopic hematuria.
Albuminuria ; Biopsy ; Child* ; Chungcheongnam-do ; Consensus ; Creatinine ; Female ; Follow-Up Studies* ; Glomerulonephritis, IGA ; Hematuria* ; Humans ; Hypertension ; Male ; Mass Screening* ; Nephritis ; Pediatrics ; Prognosis ; Proteinuria ; Retrospective Studies ; Urinalysis

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 majority of patients with idiopathic generalized epilepsy (IGE) are controlled by an antiepileptic drug when appropriately selected. So far, valproate is regarded as the first-line treatment for IGE. Also, it is postulated that lamotrigine and topiramate may have efficacy in IGE but there are a few evidence to support the usage. We examined the remission rates of seizures on valproate, lamotrigine, topiramate and factors predicting the outcome. METHODS: We reviewed the patients who diagnosed as IGE in pediatric neurology clinics in Asan Medical Center from March, 1995 to August, 2005. The patients with childhood absence epilepsy and patients without generalized spike and slow waves in EEG were excluded. Data were collected retrospectively on demographics, seizure types, antiepileptic drug treatment details, and remission rates. RESULTS: 64 of 80 patients had achieved one year period of remission by monotherapy with following drugs: Valproate (73.7% of 38 patients), lamotrigine (72.4% of 29 patients) or topiramate (83.3% of 18 patients). Among patients who failed to achieve remission by monotherapy, the combination of the drugs showed a remission rate of 72.7% (8/11 patients). Factors such as age, sex, family history, and history of febrile convulsions did not affect the remission rates. The existence of photoparoxysmal responses or focal epileptiform discharges, and syndrome diagnoses also couldn't be the predictive factors as well. CONCLUSIONS: As in adult patients with IGE, topiramate and lamotrigine as well as valproate can be used as the most effective anti-epileptic drugs in children with IGE without significant side effect.
Adult ; Child ; Chungcheongnam-do ; Demography ; Diagnosis ; Electroencephalography ; Epilepsy, Absence ; Epilepsy, Generalized* ; Humans ; Immunoglobulin E ; Neurology ; Retrospective Studies ; Seizures ; Seizures, Febrile ; Valproic Acid*

Anti-N-methyl D-aspartate receptor (anti-NMDAR) encephalitis, recently recognized as a form of paraneoplastic encephalitis, is characterized by a prodromal phase of unspecific illness with fever that resembles a viral disease. The prodromal phase is followed by seizures, disturbed consciousness, psychiatric features, prominent abnormal movements, and autonomic imbalance. Here, we report a case of anti-NMDAR encephalitis with initial symptoms of epilepsia partialis continua in the absence of tumor. Briefly, a 3-year-old girl was admitted to the hospital due to right-sided, complex partial seizures without preceding febrile illness. The seizures evolved into epilepsia partialis continua and were accompanied by epileptiform discharges from the left frontal area. Three weeks after admission, the patient's seizures were reduced with antiepileptic drugs; however, she developed sleep disturbances, cognitive decline, noticeable oro-lingual-facial dyskinesia, and choreoathetoid movements. Anti-NMDAR encephalitis was confirmed by positive detection of NMDAR antibodies in the patient's serum and cerebrospinal fluid, and her condition slowly improved with immunoglobulin, methylprednisolone, and rituximab. At present, the patient is no longer taking multiple antiepileptic or antihypertensive drugs. Moreover, the patient showed gradual improvement of motor and cognitive function. This case serves as an example that a diagnosis of anti-NMDAR encephalitis should be considered when children with uncontrolled seizures develop dyskinesias without evidence of malignant tumor. In these cases, aggressive immunotherapies are needed to improve the outcome of anti-NMDAR encephalitis.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis* ; Antibodies ; Anticonvulsants ; Antihypertensive Agents ; Cerebrospinal Fluid ; Child* ; Child, Preschool ; Cognition ; Consciousness ; D-Aspartic Acid ; Diagnosis ; Dyskinesias ; Encephalitis ; Epilepsia Partialis Continua* ; Female ; Fever ; Humans ; Immunoglobulins ; Immunotherapy ; Korea* ; Methylprednisolone ; Rituximab ; Seizures ; Virus Diseases

Hemimegalencephaly and tuberous sclerosis complex are distinct and rare conditions which are characterized by malformations of cortical developments. Hemimegalencephaly is a cerebral malformation of unknown pathophysiology characterized by asymmetry of the hemispheres and cortical dysplasia. Tuberous sclerosis complex(TSC) is an autosomal dominant neurocutaneous disorder characterized by the formation of hamartomatous lesion in multiple organ systems. While they are currently thought to be unrelated, there are similar cases in the literature and it is conceivable that an abnormality in early cortical development could lead to both conditions in an individual. We report here a first Korean case of unusual association of hemimegalencephaly and tuberous sclerosis complex with mutation in the TSC2 gene, who presented initially frequent partial seizures and infantile spasms.
Epilepsy ; Malformations of Cortical Development ; Neurocutaneous Syndromes ; Seizures ; Tuberous Sclerosis

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*