This study was conducted to investigate the etiology, the clinical characteristics and prognosis of acute necrotizing encephalopathy (ANE) in Korean children. Six children (1 yr to 7 yr) patients with ANE were enrolled. They were diagnosed by clinical and radiological characteristics and their clinical data were retrospectively analyzed. In a search of clinically plausible causes, brain MRI in all patients, mitochondrial DNA studies for mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) and myoclonus epilepsy and ragged red fibers (MERRF) in four patients, and genomic typing on HLA DRB/HLA DQB genes in three patients were performed. All had precedent illnesses and the main initial symptoms included mental change (83%), seizures (50%), and focal deficits (50%). MRI revealed increased T2 signal density in the bilateral thalami and/or the brainstem in all patients. Mitochodrial DNA studies for MELAS and MERRF were negative in those children and HLA-DRB1*1401, HLA-DRB3*0202, and HLA-DQB1*0502 seemed to be significant. A high dose steroid was given to all patients, which seemed to be partly effective except for 2 patients. In conclusion, ANE is relatively rare, but can result in serious neurological complication in children. Early detection and appropriate treatment may lead to a better neurological outcome.

PurposeAlthough benign rolandic epilepsy (BRE) is a benign condition, it may be associated with a spectrum of behavioral, psychiatric, and cognitive disorders. This study aimed to assess the cognitive and other neuropsychological profiles of children with BRE.MethodsIn total, 23 children with BRE were consecutively recruited. All children underwent sleep electroencephalography (EEG) and were assessed on a battery of comprehensive neuropsychological tests including the Korean versions of the Wechsler intelligence scale for children III, frontal executive neuropsychological test, rey complex figure test, Wisconsin card sorting test, attention deficit diagnostic scale, and child behavior checklist scale.ResultsThe study subjects included 13 boys and 10 girls aged 9.0±1.6 years. Our subjects showed an average monthly seizure frequency of 0.9±0.7, and a majority of them had focal seizures (70%). The spike index (frequency/min) was 4.1±5.3 (right) and 13.1±15.9 (left). Of the 23 subjects, 9 showed frequent spikes (>10/min) on the EEG. The subjects had normal cognitive and frontal executive functions, memory, and other neuropsychological sub-domain scores, even though 8 children (35%) showed some evidence of learning difficulties, attention deficits, and aggressive behavior.ConclusionOur data have limited predictive value; however, these data demonstrate that although BRE appears to be benign at the onset, children with BRE might develop cognitive, behavioral, and other psychiatric disorders during the active phase of epilepsy, and these problems may even outlast the BRE. Therefore, we recommend scrupulous follow-up for children with BRE.

Neurologic complications of children with influenza A H1N1 2009 pandemic, diagnosed in two consecutive influenza seasons were retrospectively reviewed to seek better outcomes in future outbreaks. Patient demographics, clinical manifestations and neurologic outcomes were reviewed. A total of 1,389 children were diagnosed with influenza A H1N1 by real-time reverse transcriptase-polymerase chain reaction. Of these, 23 (1.7%) patients had neurologic involvement. Their mean age was 5.9 ± 3.6 yr (range, 6 months to 11 yr) and 16 (69.9%) were boys. None of the 23 patients had been vaccinated for influenza A H1N1 and seasonal influenzas. Twenty-two of the 23 patients presented with seizures. Clinical features included febrile convulsion (n = 19), afebrile convulsion (n = 1), aseptic meningitis (n = 1), encephalopathy (n = 1), and acute necrotizing encephalopathy (n = 1). They all were treated with Oseltamivir twice daily for 5 days immediately after nasal and throat swab testing. Twenty-one of the subjects recovered fully, but the youngest two infants experienced severe neurological sequelae. The results indicate that neurologic complications associated with influenza A H1N1 2009 pandemic were mostly mild, but rarely were serious. Prompt intervention leads to a better outcome and vaccination may prevent the disease, thus staving off serious neurological complications following influenza, especially in young infants.

Background and Purpose:The aim of this study was to evaluate the benefits and risks of oxcarbazepine (OXC) monotherapy in children with newly diagnosed, benign partial epilepsy based on clinico-electrical and neuropsychological evaluation over time.Methods:The study was open label, prospective, multicenter based. A total of 39 children with BRE were involved in the study. They were randomized into two groups (T; treatment with OXC, NT; No treatment) to compare the effectiveness of OXC treatment. All children underwent EEGs with quantification and a comprehensive battery of neuropsychological tests at the first visit and follow up visit at 6 months.Results:The subjects made a slight progress in general intelligence measures over time in both groups (95.4±10.5 to 97.6±7.5 for T, 107.6±17.3 to 111.4±18.6 for NT). Memory and frontal executive functions did not change over time in both groups in terms of the memory quotient (MQ) (106.7±27.5 to 103.4± 19.3 for T, 105.8±13.2 to 104.9±17.2 for NT) and executive intelligence quotient (EIQ) (114.7±18.3 to 108.9±12.5 for T, 100.6±25.1 to 101.2±13.9 for NT). However, when sub-domain scores were compared between the two groups, the treatment group got significantly worse over time in the verbal fluency test (11.5±3.8 to 8.0±1.4 for T, 10.3±3.9 to 11.5±2.1 for NT; p<0.05) and level 1 of Stroop test (9.3±3.0 to 7.5±1.3 for T, 11.0±3.7 to 11.2±2.6 for NT; p<0.05). The subjects might have cognitive and behavioral difficulties in association with frontal lobe dysfunctions, but these difficulties did not seem to be dependent on the number of seizures, the abundance of subclinical epileptiform discharges, or the anti-epileptic treatment.Conclusions:We think that OXC monotherapy is effective for children with BRE, but is to be given to the selected patients such as patients with prolonged or frequent seizures. However, further studies are needed to have a better understanding in this matter.

PurposeAttention deficit hyperactivity disorder (ADHD) is a common disorder in school-aged children. Patients with restless legs syndrome (RLS) often present with ADHD symptoms and vice versa. This study was the first to attempt to identify the prevalence of RLS and sleep problems in children with ADHD in Korea.MethodsPatients diagnosed with ADHD were asked to complete a sleep questionnaire. The sleep questionnaire included items to help identify the presence of four typical symptoms that are used as diagnostic criteria for RLS.ResultsA total of 56 patients, including 51 boys and 5 girls (mean age, 10.7 years old) participated. Of these, 24 complained of pain, discomfort, or an unpleasant sensation in the legs. Based on the RLS diagnostic criteria, 2 patients were diagnosed with definite RLS and 4 with probable RLS. There were no significant differences in age, medication dosage, or neuropsychological test scores between the patients with and without RLS symptoms.ConclusionApproximately 42.9% of patients with ADHD presented with RLS symptoms and 7.1% of these were diagnosed with RLS. Patients with ADHD also experienced various other sleep disorders. Thus, appropriate assessment and treatment for sleep disorders in patients with ADHD is essential.

This is a long-term, open label, observational study aimed to broaden our clinical experiences in managing infants and toddlers with epilepsy. The long-term retention rate and side effects of topiramate (TPM) in them were evaluated and compared with carbamazepine (CBZ). A total of 146 children were involved in the study (TPM=41, CBZ=105). The retention rates at 24 , 36, and 48 months were 46.3%, 34.1%, 26.8% for TPM and 36.2%, 23.8%, 13.3% for CBZ, respectively. At 6 months after starting antiepileptic drugs (AED), the seizure freedom or clinical efficacy (seizure reduction rate more than 50 percent) were 73.2% for TPM and 62.9% for CBZ. The major side effects led to discontinuation included psychomotor slowing, poor oral intake from TPM and sleepiness and skin rash from CBZ. TPM was discontinued due to side effects in one case (2.4%) and lack of efficacy in five cases (12.2%), whereas CBZ was discontinued due to lack of efficacy (22.9%) and side effects (6.7%). As compared with CBZ, TPM showed the same long-term retention rate in children under the age of 2 yr, and no serious side effects. It is therefore concluded that TPM can be considered as a major AED for treating children with epilepsy under the age of 2 yr.

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Nocturnal enuresis (NE) is one of the most common problems in childhood. NE has a multifactorial etiology and is influenced by sleep and arousal mechanisms. The aim of the present study was to prospectively evaluate sleep problems and patterns in children with NE compared with normal healthy controls. Methods: Twenty-eight children with NE and 16 healthy controls were included in the study. To evaluate sleep habits and disturbances, parents and children filled out a questionnaire that included items about sleep patterns and sleep-related behaviors prior to treatment for NE. Demographic factors and other data were compared for the two groups based on the responses to the sleep questionnaire. Results: Night awakening, sleepwalking, and periodic limb movements were more prevalent in children with NE, but symptoms of sleep-disordered breathing were not increased in this group. There were statistically significant differences in periodic limb movements and daytime sleepiness between the two groups. Conclusion: Children with NE seemed to have more sleep problems such as night awakening, sleepwalking, and periodic limb movements. In addition, a higher level of daytime sleepiness and hyperactivity in patients with NE suggested a relationship between NE and sleep disorders.