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: 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

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

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*

Systemic lupus erythematosus (SLE) is an autoimmune disorder involving multiple organs. Neuropsychiatric symptoms are frequently associated in SLE, which is referred to as neuropsychiatric SLE (NPSLE). NPSLE contains both central and peripheral nervous systems, which includes transverse myelitis, and Guillain-Barre syndrome (GBS). We report our experience of concurrent manifestation of transverse myelitis and GBS as an initial presentation of SLE, which suggests the common immune-mediated mechanisms of diseases. We here report the case of a 14-year-old boy with SLE who first presented with features of GBS. The patient developed ascending weakness starting from low extremities, experienced difficulty voiding, and had a facial rash. An initial diagnosis of GBS was made on the basis of clinical findings and nerve conduction studies. But he did not respond to intravenous immunoglobulin therapy and following spine MRI displayed T2 weighted high signal intensities from the cervical to thoracic region of the spinal cord, and serological analysis revealed the presence of anti-dsDNA, anti-smAb, anti nuclear antibody with decreased level of complements. The diagnosis was revised to GBS and acute transverse myelitis resulting from SLE. Additional methylprednisolone pulse therapy led to rapid clinical improvement. This was followed by oral prednisolone and cyclophosphamide pulse therapy. This is the first case of concurrent manifestation of GBS and transverse myelitis as initial presentation of SLE. The cross-reactivity of autoantibodies and increased susceptibility to infection owing to immunologic changes associated with lupus may form the basis of the association. Clinicians should consider a diagnosis of SLE as an etiology of GBS or transverse myelitis.
Adolescent ; Autoantibodies ; Complement System Proteins ; Cyclophosphamide ; Exanthema ; Extremities ; Guillain-Barre Syndrome ; Humans ; Immunization, Passive ; Lupus Erythematosus, Systemic ; Methylprednisolone ; Myelitis, Transverse ; Neural Conduction ; Peripheral Nervous System ; Prednisolone ; Spinal Cord ; Spine

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*

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

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

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