Clinical Usefulness of Simultaneous Electroencephalography and Functional Magnetic Resonance Imaging in Children With Focal Epilepsy

Background@#and Purpose The current study analyzed the interictal epileptiform discharge (IED)-related hemodynamic response and aimed to determine the clinical usefulness of simultaneous electroencephalography and functional magnetic resonance imaging (EEG-fMRI) in defining the epileptogenic zone (EZ) in children with focal epilepsy. @*Methods@#Patients with focal epilepsy showing IEDs on conventional EEG were evaluated using EEG-fMRI. Statistical analyses were performed using the times of spike as events modeled with multiple hemodynamic response functions. The area showing the most significant t-value for blood-oxygen-level-dependent (BOLD) changes was compared with the presumed EZ. Moreover, BOLD responses between -9 and +9 s around the spike times were analyzed to track the hemodynamic response patterns over time. @*Results@#Half (n=13) of 26 EEG-fMRI investigations of 19 patients were successful. Two patients showed 2 different types of spikes, resulting in 15 analyses. The maximum BOLD response was concordant with the EZ in 11 (73.3%) of the 15 analyses. In 10 (66.7%) analyses, the BOLD response localized the EZs more specifically. Focal BOLD responses in the EZs occurred before IEDs in 11 analyses and were often widespread after IEDs. Hemodynamic response patterns were consistent in the same epilepsy syndrome or when repeating the investigation in the same patients. @*Conclusions@#EEG-fMRI can provide additional information for localizing the EZ in children with focal epilepsy, and also reveal the pathogenesis of pediatric epilepsy by evaluating the patterns in the hemodynamic response across time windows of IEDs.

KCNQ2 Encephalopathy Showing a Distinct Ictal Amplitude-Integrated Electroencephalographic Pattern

KCNQ2 mutations induce a neonatal-onset epileptic encephalopathy of widely varying severity, ranging from benign familial neonatal epilepsy to severe refractory epileptic encephalopathy. Refractory seizures with KCNQ2 mutations have a positive response to sodium-channel blockers. Recently, a distinctive ictal pattern has been reported during amplitude-integrated electroencephalographic (aEEG) monitoring in infants with KCNQ2 encephalopathy. Herein, we describe a case of KCNQ2 encephalopathy with this distinctive ictal aEEG pattern, which was confirmed using conventional electroencephalography (EEG). A 3-day-old female infant presented with neonatal seizures accompanied by cyanosis and desaturation. Her seizure semiology was tonic and focal clonic. Her ictal aEEG demonstrated a sudden rise in amplitude followed by a suppressed background pattern. This pattern was also confirmed on conventional EEG. Her seizures were refractory despite the administration of multiple conventional antiepileptic drugs. Finally, c.794C>T; p. (Ala265Val) mutation was observed in the KCNQ2 gene on genetic testing, and she was diagnosed with KCNQ2 encephalopathy. Identifying this distinctive ictal pattern on aEEG monitoring facilitates the early detection of KCNQ2 encephalopathy and timely targeted treatment in patients with refractory seizures.

KCNQ2 Encephalopathy Showing a Distinct Ictal Amplitude-Integrated Electroencephalographic Pattern

KCNQ2 mutations induce a neonatal-onset epileptic encephalopathy of widely varying severity, ranging from benign familial neonatal epilepsy to severe refractory epileptic encephalopathy. Refractory seizures with KCNQ2 mutations have a positive response to sodium-channel blockers. Recently, a distinctive ictal pattern has been reported during amplitude-integrated electroencephalographic (aEEG) monitoring in infants with KCNQ2 encephalopathy. Herein, we describe a case of KCNQ2 encephalopathy with this distinctive ictal aEEG pattern, which was confirmed using conventional electroencephalography (EEG). A 3-day-old female infant presented with neonatal seizures accompanied by cyanosis and desaturation. Her seizure semiology was tonic and focal clonic. Her ictal aEEG demonstrated a sudden rise in amplitude followed by a suppressed background pattern. This pattern was also confirmed on conventional EEG. Her seizures were refractory despite the administration of multiple conventional antiepileptic drugs. Finally, c.794C>T; p. (Ala265Val) mutation was observed in the KCNQ2 gene on genetic testing, and she was diagnosed with KCNQ2 encephalopathy. Identifying this distinctive ictal pattern on aEEG monitoring facilitates the early detection of KCNQ2 encephalopathy and timely targeted treatment in patients with refractory seizures.

Real-Life Effectiveness and Tolerability of Perampanel in Pediatric Patients Aged 4 Years or Older with Epilepsy: A Korean National Multicenter Study

Efficacy and tolerability of adjunctive perampanel treatment in children under 12 years of age with refractory epilepsy / 소아과

PURPOSE: There is limited data on the use of perampanel in children under 12 years of age. We evaluated the efficacy and tolerability of adjunctive perampanel treatment in children under 12 years of age with refractory epilepsy. METHODS: This retrospective observational study was performed in Kyungpook National University Hospital from July 2016 to March 2018. A responder was defined as a patient with ≥50% reduction in monthly seizure frequency compared with the baseline. Adverse events and discontinuation data were obtained to evaluate tolerability. RESULTS: Twenty-two patients (8 males, 14 females) aged 3.1–11.4 years (mean, 8.0±2.5 years) were included in this study. After an average of 9.2 months (range, 0.5–19 months) of follow-up, 15 patients (68%) showed a reduction in seizure frequency, including 5 patients (23%) with seizure freedom. The age at epilepsy onset was significantly lower (P=0.048), and the duration of epilepsy was significantly longer (P=0.019) in responders than in nonresponders. Nine patients (41%) experienced adverse events, including somnolence (23%), respiratory depression (9%), violence (4.5%), and seizure aggravation (4.5%). The most serious adverse event was respiratory depression, which required mechanical ventilation in 2 patients (9%). Eight patients (36%) discontinued perampanel due to lack of efficacy or adverse events. Three out of 4 patients (75%) who discontinued perampanel due to adverse events had an underlying medical condition. CONCLUSION: Perampanel offers a treatment option for refractory epilepsy in children. Adjunctive treatment with perampanel requires special consideration in those with underlying medical conditions to prevent serious adverse events.

Acute Necrotizing Encephalopathy in Children: a Long Way to Go

BACKGROUND: Acute necrotizing encephalopathy (ANE) is a rare, but potentially life threatening neurological condition in children. This study aimed to investigate its clinical spectrum, diagnostic and therapeutic dilemma, and prognosis. METHODS: Twelve children with ANE were included in the study. The diagnosis was made by clinical and radiological characteristics from January 1999 to December 2017 and their clinical data were retrospectively analyzed. RESULTS: A total of 12 children aged 6 to 93 months at onset (5 male: 7 female) were evaluated. The etiology was found in 4 of them (influenza A, H1N1; coxsackie A 16; herpes simplex virus; and RANBP2 gene/mycoplasma). The most common initial presentations were seizures (67%) and altered mental status (58%). The majority of the subjects showed elevation of aspartate aminotransferase/alanine aminotransferase with normal ammonia and increased cerebrospinal fluid protein without pleocytosis. Magnetic resonance imaging revealed increased T2 signal density in bilateral thalami in all patients, but the majority of the subjects (67%) also had lesions in other areas including tegmentum and white matter. Despite the aggressive immunomodulatory treatments, the long-term outcome was variable. One child and two sisters with genetic predisposition passed away. CONCLUSION: ANE is a distinctive type of acute encephalopathy with diverse clinical spectrum. Even though the diagnostic criteria are available, they might not be watertight. In addition, treatment options are still limited. Further studies for better outcome are needed.

A novel GLA mutation in a Korean boy with an early cardiac manifestation of Fabry disease

Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by the deficiency of α-galactosidase A. Patients with classical FD present acroparesthesia, hypohidrosis, cornea verticillata, disseminated angiokeratoma, and microalbuminuria in childhood, and develop life-threatening renal, cardiac, and cerebrovascular complications typically after the fourth decade of life. To date, more than 700 mutations responsible for FD have been identified in the human GLA gene. Herein, we report a novel GLA mutation, c.1117_1141del25 (p.Gly373Profs*10), identified in an 11-year-old Korean boy with FD presenting early cardiac and neurologic manifestation and in other affected family members. The boy had acroparesthesia, hypohidrosis, cornea verticillata, and left ventricular hypertrophy. His mother and sister also had acroparesthesia. Two males on the mother's side had similar pain and died of unknown causes. The plasma α-galactosidase A activity (4.1 nmol/hr/mg protein) of the patient was markedly lower than the mean value of the controls. The plasma level of globotriaosylsphingosine was elevated in the patient and all the carriers. We concluded the novel GLA mutation c.1117_1141del25 is a pathogenic mutation for FD, probably related to the early cardiac manifestation of FD.

Effectiveness and Tolerability of Rufinamide in Korean Children with Lennox-Gastaut Syndrome

PURPOSE: Rufinamide (RFM) is known to be effective for children with Lennox-Gastaut syndrome (LGS). The aim of this study is to evaluate its efficacy and tolerability of Korean children with LGS. METHODS: This is a single center, open label, retrospective study. Patients with LGS who received rufinamide as adjunctive therapy were enrolled in this study. Their baseline clinical characteristics, the percent change in the seizure frequency per 4 weeks, and adverse events were evaluated. RESULTS: Among 32 children, 20 were males and the mean age was 11.3±6.6 years. After 1 month of rufinamide medication, the frequency of seizures was reduced by more than 50% in 31.3% of patients and 6.3% of patients had no seizures. After 6 months of rufinamide administration, patients with a 50% or less decrease in seizure frequency remained in a state of reduced seizure frequency and 3.1% of patients had no seizures. Side effects such as nausea, vomiting, anorexia, less active, somnolence, aggression, drooling were noted in 28.1% of patients. CONCLUSION: This study suggests that rufinamide can be considered as an effective and safe treatment option for intractable epileptic children such as LGS.

Clinical importance of F-waves as a prognostic factor in Guillain-Barré syndrome in children / 소아과

PURPOSE: A limited number of studies have examined the link between F-wave abnormalities and clinical presentation in pediatric Guillain-Barré syndrome (GBS). Therefore, this study examined the importance of F-wave abnormalities as a prognostic factor in pediatric GBS patients. METHODS: The records and electrodiagnostic studies (EDS) of 70 GBS patients were retrospectively evaluated, and divided into 2 groups according to the results of EDS. Group A (n=33) presented with F-wave abnormalities, and group B (n=26) exhibited normal findings. We compared laboratory reports, clinical features, response to treatment, and prognosis between the 2 groups. RESULTS: Motor weakness was the most frequently observed symptom for either group. Clinically, the incidence of fever and upper respiratory symptoms differed between the 2 groups, while the prevalence of abnormal deep tendon reflex (DTR) was significantly higher in group A than B (P<0.05). Patients diagnosed with GBS had received intravenous immunoglobulin treatment: 94% in group A and 58% in group B. Furthermore, significantly greater numbers of patients in group A showed H-reflex abnormalities and poor prognosis compared with group B (P<0.05). CONCLUSION: This study demonstrated that F-waves are a clinically important prognostic factor in GBS. F-wave abnormalities were associated with abnormal DTR and poor prognosis in patients. Limited studies have examined the link between F-wave abnormalities and clinical results; therefore, further randomized controlled studies are needed to confirm the clinical characteristics and efficacy of treatments.

Early-onset epileptic encephalopathies and the diagnostic approach to underlying causes / 소아과

Early-onset epileptic encephalopathies are one of the most severe early onset epilepsies that can lead to progressive psychomotor impairment. These syndromes result from identifiable primary causes, such as structural, neurodegenerative, metabolic, or genetic defects, and an increasing number of novel genetic causes continue to be uncovered. A typical diagnostic approach includes documentation of anamnesis, determination of seizure semiology, electroencephalography, and neuroimaging. If primary biochemical investigations exclude precipitating conditions, a trial with the administration of a vitaminic compound (pyridoxine, pyridoxal-5-phosphate, or folinic acid) can then be initiated regardless of presumptive seizure causes. Patients with unclear etiologies should be considered for a further workup, which should include an evaluation for inherited metabolic defects and genetic analyses. Targeted next-generation sequencing panels showed a high diagnostic yield in patients with epileptic encephalopathy. Mutations associated with the emergence of epileptic encephalopathies can be identified in a targeted fashion by sequencing the most likely candidate genes. Next-generation sequencing technologies offer hope to a large number of patients with cryptogenic encephalopathies and will eventually lead to new therapeutic strategies and more favorable long-term outcomes.

Pediatric Bacterial and Aseptic Meningitis in Daegu

PURPOSE: The aim of this study was to investigate the clinical characteristics and causative organisms of meningitis in the Daegu region and seek a useful tool for the early prediction of bacterial meningitis in children. METHODS: We retrospectively reviewed the medical records of 115 pediatric patients diagnosed with bacterial or aseptic meningitis at Yeungnam university hospital in Daegu from March 2012 to July 2013. We evaluated their clinical symptoms, laboratory findings, clinical courses, bacterial meningitis scores and complications. RESULTS: The subjects included 106 with aseptic meningitis and 9 with bacterial meningitis. At the time of visit, fever was the most frequent symptom, followed by headache, vomiting and neck stiffness. In cerebrospinal fluid (CSF) analysis, white blood cell (WBC) count were higher in the bacterial meningitis group (1423.8+/-1980.4 vs. 120.0+/-161.6 mg/dL). Mean CSF protein was 219.4+/-183.6 mg/dL in bacterial meningitis and 42.4+/-27.0 mg/dL in aseptic meningitis (P <0.001). Bacterial meningitis score (BMS) were higher in the group with bacterial meningitis. Abnormal radiological findings were found in 44% of the group with bacterial meningitis. CONCLUSION: Although the clinical features between the groups were similar, the CSF analysis revealed significant differences statistically. Furthermore, BMS could be helpful to predict bacterial meningitis in children. During the outbreak of aseptic meningitis, it might reduce unnecessary hospital admissions and antibiotic treatments.

The Effectiveness and Tolerability of Topiramate and Lidocaine Add-on Therapy in Children with Refractory Status Epilepticus

PURPOSE: This study was aimed to compare the effectiveness and tolerability of topiramate add-on therapy versus lidocaine add-on therapy in children with refractory status epilepticus. METHODS: We performed a retrospective analysis of children with refractory status epilepticus who were hospitalized at Kyungpook National University Hospital during the period 2005 to 2012. We divided them into two groups, Group A (midazolam, MDZ, and Topiramate, TPM:11 patients) and Group B (midazolam and lidocaine, LDC:7 patients). Furthermore, they were sub-categorized according to children's sex, age, seizure duration, and seizure type. To comepare the effectiveness and tolerability between the two groups, we evaluated seizure freedom, >50% seizure reduction, adverse events and mortality rates, respectively. RESULTS: In MDZ/TPM group, average dosage of midazolam infusion was 10.8+/-6.8 mcg/kg/min, seizure freedom was achieved in 1 out of 11 patients (9%) and >50% seizure reduction was in 2 out of 11 patients (18%). On the other hand, in MDC/LDC group, average dosage of midazolam infusion is 11.3+/-5.2 mcg/kg/min, seizure freedom was achieved in 2 out of 7 patients (29%) and >50% seizure reduction was 3 out of 7 patients (43%). Aside from the promising effects, there was no significant difference between the two groups. CONCLUSION: In children with refractory status epilepticus, topiramate or lidocaine add-on therapy seemed to be effective and fairly tolerated and showed no serious adverse effects. Therefore they can be an alternative treatment option. However, further studies are required.

Normal Polysomnographic Characteristics in Korean Children and Adolescents

PURPOSE: The objective of this study was to describe polysomnographic charateristics in normal children and adolescents in Korea. Little is known regarding sleep architecture in Korean children. METHODS: We conducted a prospective study and examined sleep architecture, respiratory parameters, arousals, and periodic limb movement index during sleep (PLMS) in healthy children and adolescents for 2 consecutive days. RESULTS: The study population consisted of 29 subjects and 15 subjects (52%) were male. Ages ranged from 5-16 years, with a mean of 11.6+/-.7 years. The average total sleep time (TST) was 438+/-35.6 min, and the mean sleep efficiency was 90.9+/-17.7%. The distribution of sleep stages as percentage of TST was as follows: stage N1, 6.6+/-2.9%; stage N2, 54.2+/-5.8%; stage N3, 21.1+/-6.7%; and stage R, 18.1+/-5.4%. The wake after sleep onset (WASO) was 2.0+/-2.9% of TST. The total arousal index (TAI) was 7.6+/-2.2. The current study findings are similar to those of previous studies in Western countries except longer stage N2 sleep and higher WASO. CONCLUSION: This is the first report of normal polysomnography values in Korean children. In order to accurately diagnose sleep disorders, normative sleep reference values are essential and our findings will provide fundamental data for pediatric sleep research.

Prevalence of restless legs syndrome and sleep problems in Korean children and adolescents with attention deficit hyperactivity disorder: a single institution study / 소아과

PURPOSE: Attention 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. METHODS: Patients 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. RESULTS: A 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. CONCLUSION: Approximately 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.

Clinical Analysis of the Correlation between Febrile Seizures and Influenza Infection

PURPOSE: Febrile seizures are common in children between the ages of 6 months and 5 years of age and are often caused by viral illnesses. Influenza infection presents with a variety of neurological conditions including seizures. This study was aimed to evaluate the correlation of influenza infection and febrile seizures. METHODS: Eighty-four children with febrile seizures were involved in the study from October 2013 to March 2014. They were divided into two groups (febrile seizures with influenza infection, febrile seizures without influenza infection). Their medical records including clinical characteristics such as seizure types, seizure frequency, seizure duration, developmental history, brain magnetic resonance imaging(MRI), cerebrospinal fluid(CSF) study and electroencephalogram(EEG) findings were reviewed. RESULTS: Twenty six out of 242 children between the ages of 6 months and 5 years diagnosed with influenza infection had febrile seizures (10.7%), which is higher than known prevalence of febrile seizures. There were no significant differences in clinical characteristics such as seizure types, seizure frequency, seizure duration, developmental history, brain MRI, CSF study and EEG findings between the two groups. However, onset age of febrile seizures with influenza infection was older than the other group without influenza infection(P<0.001). CONCLUSION: It has been considered that influenza infections are common during the cold seasons and are the main causative factor for febrile seizures. Based on the findings from this study, Influenza infection may be a significant risk factor for febrile seizures. However, further studies are needed.

Narcolepsy with Obstructive Sleep Apnea in a 4-Year-Old Korean Girl: A Case Report

A 4-yr-old girl has exhibited severe snoring, restless sleep and increasing daytime sleepiness over the last 3 months. The physical examination showed that she was not obese but had kissing tonsils. Polysomnography demonstrated increased apnea-hypopnea index (AHI) of 5.2, and multiple sleep latency tests (MSLT) showed shortened mean sleep latency and one sleep-onset REM period (SOREMP). She was diagnosed with obstructive sleep apnea (OSA) and underwent tonsillectomy and adenoidectomy. After the surgery, her sleep became much calmer, but she was still sleepy. Another sleep test showed normal AHI of 0.2, the mean sleep latency of 8 min, and two SOREMPs. Diagnosis of OSA to be effectively treated by surgery and narcolepsy without cataplexy was confirmed. Since young children exhibiting both OSA and narcolepsy can fail to be diagnosed with the latter, it's desirable to conduct MSLT when they have severe daytime sleepiness or fail to get better even with good treatment.

Cognitive and other neuropsychological profiles in children with newly diagnosed benign rolandic epilepsy / 소아과

PURPOSE: Although 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. METHODS: In 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. RESULTS: The 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. CONCLUSION: Our 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.

The Clinical Analysis of Paroxysmal Kinesigenic Dyskinesia

PURPOSE: Paroxysmal kinesigenic dyskinesia (PKD) is one of the movement disorders in which dyskinesia occurs in a part of the body by a sudden movement after a rest under a tension or a stress. This study was aimed to evaluate the clinical features of children and adolescents with PKD in Korea via analysing the patients who have treated in Department of Pediatrics, Kyungpook National University Hospital. METHODS: A total of seven children with PKD was involved in the study and their medial records were retrospectively evaluated. RESULTS: The mean age of the 7 subjects was 15.7 years (10.0-21.4 years old). The male to female ratio was 6:1. They presented with dystonia with the average duration of 10.5 seconds (3.5-17.5 seconds), which triggered by various sudden movements. No accompanying cormorbidities were noted. Their laboratory findings were unremarkable. Six of the patients, except one who refused treatment with medicine, responded well to medication and remained symptom free. The average time response to medication was 3.4 weeks (0.95-7.81 weeks). They were of treated with either oxcarbazepine (n=4, 14.9+/-5.8 mg/kg/day) or lamotrigine (n=2, 1.5+/-0.9 mg/kg/day). There was no significant difference between two groups in terms of age, response, adverse events, and so on. CONCLUSION: This study showed that clinical features of Korean children with PKD are quite similar to those of other countries. They responded well to the medication. In addition, lamotrigine can be an alternative choice for the treatment.

Neurologic Complications and Outcomes of Pandemic (H1N1) 2009 in Korean Children

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.

Current Status of ADHD Treatment in Children with Epilepsy: A Retrospective, Multicenter, Cross Sectional Analysis in Korea

PURPOSE: The purpose of this study was to assess the current therapeutic status of attention deficit-hyperactivity disorder (ADHD) in children with epilepsy. METHODS: A cross-sectional survey of 178 patients aged 4-20 years from ten pediatric neurology clinics in eight cities in South Korea from January 2005 to July 2010 was used to assess clinical characteristics of ADHD patients with epilepsy and risk factors associated with ADHD. RESULTS: A total of 178 pediatric epileptic patients were recruited for this study. One hundred seventy-four subjects' (M:F=4:1, mean age: 12.2+/-3.3 yrs old) records were evaluated excluding four patients due to incomplete data. One hundred twenty-five of 174 patients (71.8%) had partial epilepsy and 45 had generalized epilepsy. Eighty of 112 patients showed ADHD combined type from the DSM IV. The mean prevalence rate of ADHD treatment among the epileptic patients was 1.9%. Over 45% of patients showed complete or persistent symptoms without difficulties in school life with CNS stimulants. Adverse reactions were reported in 19.8% of patients who received ADHD medication, and 18 patients discontinued ADHD medication due to severe adverse effects such as aggravated seizures (5.6%) or ADHD symptoms (3.7%). About 60% of children with ADHD and epilepsy had psychiatric comorbid disorders. CONCLUSION: The results indicate that ADHD treatment in epilepsy patients is safe and effective. However, these data also show that ADHD in pediatric epilepsy patients in Korea is under-diagnosed and under-treated.