Publications (14)31.07 Total impact
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Article: Magnetic resonance imaging and spectroscopic analysis in 5 cases of Pelizaeus-Merzbacher disease: metabolic abnormalities as diagnostic tools.
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ABSTRACT: Pelizaeus-Merzbacher disease (PMD) is a rare, X-linked recessive disorder characterized by dysmyelination in the central nervous system. PMD results from deletion, mutation, or duplication of the proteolipid protein gene (PLP1) located at Xq22, leading to the failure of axon myelination by oligodendrocytes in the central nervous system. PMD may be suspected when there are clinical manifestations such as nystagmus, developmental delays, and spasticity, and genetic analysis can confirm the diagnosis. Further diagnostic manifestations of the disease include a lack of myelination on brain magnetic resonance (MR) imaging and aberrant N-acetyl aspartate (NAA) and choline concentrations that reflect axonal and myelination abnormalities on phroton MR spectroscopy. We report 5 cases of PMD (in 1 girl and 4 boys). PLP1 duplication was detected in 2 patients. Brain MR analyses and MR spectroscopy were performed for all the patients. The brain MR images showed white matter abnormalities typical of PMD, and the MR spectroscopic images showed diverse patterns of NAA, creatinine, and choline concentrations. We propose that MR spectroscopic analysis of metabolic alterations can aid the PMD diagnosis and can contribute to a better understanding of the pathogenesis of the disease.Korean Journal of Pediatrics 10/2012; 55(10):397-402. -
Article: A case of midgut malrotation presenting as multiple afebrile seizures.
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ABSTRACT: Provoked seizures are a common pediatric problem and can be caused by multiple etiologies. However, there have been few reports of benign infantile seizures associated with gastrointestinal tract abnormality. We report on a 3-month-old male infant who presented with multiple afebrile seizures, EEG abnormalities and a midgut malrotation. Surgical treatment of the malrotation resulted in seizure resolution and EEG normalization. This case emphasizes that patients presenting with multiple seizures and symptoms of gastroenteritis should be evaluated for abnormalities of the gastrointestinal tract.Brain & development 06/2011; 34(3):244-7. · 1.74 Impact Factor -
Article: Neuropsychological status of children with newly diagnosed idiopathic childhood epilepsy.
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ABSTRACT: We characterized the neuropsychological status of children with newly diagnosed idiopathic childhood epilepsy and measured differences in IQ between children with different types of epilepsy. The Korean Education Development Institute-Wechsler Intelligence Scale for Children (KEDI-WISC) was administered to 72 patients (35 males and 37 females), of mean age 8.7±2.6 years, with newly diagnosed idiopathic childhood epilepsy. Of these patients, 22 (30.6%) had generalized epilepsy, 48 (66.7%) localization-related epilepsy, and 2 (2.8%) mixed epilepsy. Children with generalized epilepsy and benign childhood epilepsy with centro-temporal spikes (BCECTS) were of similar verbal IQ and full-scale IQ, although performance IQ was significantly lower in patients with generalized epilepsy. Among children with BCECTS, those with unilateral spikes had higher full-scale and performance IQ scores than those with bilateral spikes. Follow-up studies on large numbers of patients are needed to determine the effects of epilepsy per se, and antiepileptic drugs, on intelligence.Brain & development 01/2011; 33(8):666-71. · 1.74 Impact Factor -
Article: Comparison of short- versus long-term ketogenic diet for intractable infantile spasms.
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ABSTRACT: To compare the prognoses between short-term (8 months) and conventional long-term (> 2 years) trials involving patients with refractory infantile spasms who successfully completed the ketogenic diet (KD). Of 40 patients who achieved seizure-free outcomes and showed improvement in hypsarrhythmic patterns within 6 months of the KD, with a 3:1 fat to nonfat ratio as an add-on treatment, 16 patients were randomized into the short-term trial group and the diet was tapered throughout two additional months. Twenty-four patients were randomized into a long-term trial group, and 19 patients could successfully discontinue the diet after 2 years. Primary outcome measures included seizure relapse and frequency of 35 patients for > 12 months after successful completion of the KD. Of 16 patients in the short-term trial group, two patients relapsed with clusters of spasms, and one patient had recurrence of occasional focal seizures. Of 19 patients in the long-term trial group, two patients progressed to Lennox-Gastaut syndrome and one patient experienced recurrence of occasional focal seizures with secondary generalization. An early response to the KD, evidenced by short latency before seizure freedom and disappearance of hypsarrythmia and cryptogenic etiology, may indicate a successful early discontinuation of the KD. Significant growth failure was complicated only in conventional long-term trial group. Use of the KD for only 8 months in children who become spasm-free appears to be justified, with similar outcomes, recurrence rate, and less growth disturbance than a longer-term, traditional use.Epilepsia 01/2011; 52(4):781-7. · 3.96 Impact Factor -
Article: Bilateral ballism after herpes encephalitis with thalamic lesion.
Parkinsonism & Related Disorders 11/2009; 16(4):303-4. · 3.80 Impact Factor -
Article: Factors influencing the evolution of West syndrome to Lennox-Gastaut syndrome.
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ABSTRACT: This study examines factors influencing the evolution of West syndrome to Lennox-Gastaut syndrome. The study population comprised 98 patients diagnosed with West syndrome and monitored for at least 3 years. During follow-up, West syndrome evolved to Lennox-Gastaut syndrome in 48 of the 98 patients. Etiology analysis indicated that West syndrome was cryptogenic in 36 patients (36.7%) and symptomatic in 62 (63.3%). West syndrome was managed with antiepileptic drugs in 31 patients, ketogenic diets in 33 patients, hormonal therapy with prednisolone in 45 patients and with adrenocorticotropic hormone in 15 patients, epileptic surgery in 3 patients, and either no treatment or only herbal medication in 4 patients. The risk of developing Lennox-Gastaut syndrome was significantly lower in patients who were placed on a ketogenic diet, given prednisolone or adrenocorticotropic hormone, or treated with a combination of these two therapies (bivariate logistic regression analysis, P < 0.05). There was no relationship between the development of Lennox-Gastaut syndrome and age at West syndrome onset or disease etiology. In conclusion, a ketogenic diet and hormonal therapy may play key roles in preventing encephalopathy in patients with West syndrome.Pediatric Neurology 08/2009; 41(2):111-3. · 1.52 Impact Factor -
Article: Clinical efficacy of zonisamide in Lennox-Gastaut syndrome: Korean multicentric experience.
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ABSTRACT: To evaluate the efficacy and safety of zonisamide (ZNS) as long-term adjunctive therapy in children with Lennox-Gastaut syndrome (LGS). We evaluated the seizure frequency, cognitive outcomes, and side effects of 62 LGS patients maintained on ZNS for at least 12 months in three tertiary centers. Of the 62 LGS patients maintained on ZNS, 3 (4.8%) had 100% seizure control; 14 (22.6%) had >75% to <100% reduction in seizure frequency; 15 (24.2%) had >50% to <75% reduction in seizure frequency; 6 (9.7%) had >0% to <50% reduction in seizure frequency, and 24 (38.7%) had no seizure reduction. Seizure outcomes were not related to seizure types or etiologies. Adverse events included somnolence and anorexia, but all were transient and successfully managed by careful follow-up. Our results indicate that adjunctive treatment with ZNS is safe and effective in pediatric LGS patients.Brain and Development 04/2008; 30(4):287-90. · 2.12 Impact Factor -
Article: Comparison of corpus callosotomy and vagus nerve stimulation in children with Lennox-Gastaut syndrome.
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ABSTRACT: To compare the efficacy of corpus callosotomy and vagus nerve stimulation (VNS) for long-term adjunctive therapy in children with Lennox-Gastaut syndrome (LGS). Fourteen patients underwent a total corpus callosotomy and 10 patients received VNS implantation. The patients were monitored for more than 12 months after treatment, and seizure rates and complications were retrospectively evaluated. Seizure types among the 24 patients included atonic or tonic seizures with head-drops in 17 patients, generalized tonic seizures in two patients, atypical absence seizures in one patient, generalized tonic-clonic seizures in one patient, and myoclonic seizures in three patients. Of the 14 patients who underwent a corpus callosotomy, nine (64.3%) had a greater than 50% reduction in seizure frequency and five (35.7%) had a greater than 75% reduction. Of the 10 patients who underwent VNS implantation, seven (70.0%) had a greater than 50% reduction in seizure frequency and two (20.0%) had a greater than 75% reduction. There was no significant difference between the two procedures in terms of final efficacy. Complications of corpus callosotomy included aphasia in one patient, ataxia in another, and paresis in a third. Among patients receiving VNS, one patient experienced dyspnea while sleeping and one patient suffered from drooling. These complications were transient and tolerable, and were controlled by simple adjustments of VNS treatment parameters. The efficacy and safety of corpus callosotomy and VNS were comparable in children with LGS.Brain and Development 04/2008; 30(3):195-9. · 2.12 Impact Factor -
Article: Identification of a de novo Lys304Gln mutation in the glycine receptor alpha-1 subunit gene in a Korean infant with hyperekplexia.
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ABSTRACT: Startle disease or hyperekplexia (STHE; MIM 149400) is a rare disorder that is characterized by marked muscular hypertonia in infancy and an exaggerated startle response to unexpected acoustic or tactile stimuli. Mutations in the gene encoding the alpha-1 subunit of the inhibitory glycine receptor (GLRA1) were reported as causes of STHE. Recently, we encountered a Korean male infant with generalized stiffness that was observed from the first 3 days of life. The abnormal startle response was evident from the fourth week of life, and he showed marked improvement in the startle response and muscle hypertonia after being administered phenobarbital and clonazepam. Direct sequencing analysis of the infant and his parents revealed a de novo variation (c.910A>C) in the GLRA1 gene, resulting in a novel Lys304Gln missense mutation.Movement Disorders 03/2008; 23(4):610-3. · 4.51 Impact Factor -
Article: Vagus nerve stimulation in intractable childhood epilepsy: a Korean multicenter experience.
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ABSTRACT: We evaluated the long-term outcome of vagus nerve stimulation (VNS) in 28 children with refractory epilepsy. Of these 28 children, 15 (53.6%) showed a >50% reduction in seizure frequency and 9 (32.1%) had a >75% reduction. When we compared seizure reduction rates according to seizure types (generalized vs. partial) and etiologies (symptomatic vs. cryptogenic), we found no significant differences. In addition, there was no correlation between the length of the stimulation period and treatment effect. The seizure reduction rate, however, tended to be inversely related to the seizure duration before VNS implantation and age at the time of VNS therapy. VNS also improved quality of life in this group of patients, including improved memory in 9 (32.1%), improved mood in 12 (42.9%), improved behavior in 11 (39.3%), improved alertness in 12 (42.9%), improved achievement in 6 (21.4%), and improved verbal skills in 8 (28.6%). Adverse events included hoarseness in 7 patients, dyspnea at sleep in 2 patients, and wound infection in 1 patient, but all were transient and successfully managed by careful follow-up and adjustment of parameters. These results indicate that VNS is a safe and effective alternative therapy for pediatric refractory epilepsy, without significant adverse events.Journal of Korean Medical Science 06/2007; 22(3):442-5. · 0.99 Impact Factor -
Article: Epilepsy surgery in a patient with Lennox-Gastaut syndrome and cortical dysplasia.
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ABSTRACT: Lennox-Gastaut syndrome (LGS) is classified as a generalized epilepsy, and is often intractable to antiepileptic drugs. Although corpus callosotomy may sometimes control drop attacks, curative epilepsy surgery is rarely possible in LGS. We report, here, a patient with LGS and focal cortical dysplasia, who became seizure-free after epilepsy surgery. The patient was a 24-month-old boy without perinatal insult in whom seizures began 7 days after birth and who experienced development delay. Brain magnetic resonance imaging (MRI) showed focal cortical lesion with calcification in the right frontal area. At age 13 months, his seizure type changed from tonic seizures to head drops (atonic types) and atypical absence seizures. His interictal electroencephalogram (EEG) showed generalized slow spike and wave discharges, and he was diagnosed with LGS. His seizures were intractable to medical treatment, and a ketogenic diet was not effective. He was evaluated prior to surgery by long-term video-EEG monitoring, which detected many seizures consisted of a sudden onset of falling attacks (atonic type) intermittently followed by atypical absence seizures with diffuse slow wave bursts followed by slow spike and wave discharges in ictal EEG, brain positron emission tomography (PET) and MRI. A right frontal lesionectomy with intra-operative electrocorticography (EcoG) was performed. From the time of lesionectomy to the present, the patient has been seizure free and has been developing normally. Our results suggest that focal resective surgery should be considered in patients with LGS and focal epileptic lesions.Brain and Development 05/2007; 29(3):167-70. · 2.12 Impact Factor -
Article: Use of a modified Atkins diet in intractable childhood epilepsy.
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ABSTRACT: To evaluate the efficacy, safety and tolerability of a modified Atkins diet in intractable childhood epilepsy. Fourteen children with epilepsy were treated prospectively with a modified Atkins diet. Outcome measures included seizure frequency, adverse reactions and tolerability to the diet; blood beta-hydroxybutyrate and urine ketones were also measured. Six months after diet initiation, seven (50%) remained on the diet, five (36%) had >50% seizure reduction, and three (21%) were seizure free. The diet was well tolerated by 12 (86%) patients. Most complications were transient and were successfully managed by careful follow-up and conservative strategies. A consistently strong ketosis (beta-hydroxybutyrate of >3 mmol/L) seemed to be important for maintaining the efficacy of the diet therapy. The modified Atkins diet was well tolerated and sometimes a modified Atkins diet can be substituted for the conventional ketogenic diet. Serious complications were rare, but long-term complications remain to be determined.Epilepsia 02/2007; 48(1):182-6. · 3.96 Impact Factor -
Article: Benign childhood epilepsy with centro-temporal spikes (BCECTS): early onset of seizures is associated with poorer response to initial treatment.
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ABSTRACT: Benign childhood epilepsy with centro-temporal spikes (BCECTS) is the most common idiopathic partial epilepsy in children. Treatment attitudes remain a controversial issue. We examine features that could suggest refractoriness at onset. We retrospectively reviewed the medical records of 144 children with BCECTS diagnosed at the Division of Pediatric Neurology, Asan Medical Center, from March 1, 1995, to April 30, 2002 and treated with AEDs. The patients were subdivided into two groups according to the number of antiepileptic drugs used for effective seizure control. Of the 144 patients, 75 were male and 69 were female, with a mean age at seizure-onset of 7.2 +/- 2.3 years (range, 2.1-14.3 years); 119 children were taking one antiepileptic drug (AED) (Group A), and 25 were taking more than one (Group B). There were no significant group differences in female-to-male ratio, prescribed AEDs, number of seizures before the start of treatment, interval between seizure-onset and start of treatment, presence of secondarily generalized seizures, or presence of bilateral EEG abnormalities. The groups differed however, in mean age at seizure onset (7.6 +/- 2.2 years versus 5.1 +/- 1.9 years, p < 0.05) and percentage of patients with seizure-onset before 3 years (p < 0.05). When treated with AEDs, children with BCECTS usually respond well. However, an earlier onset of seizures is associated with more frequent seizures and initial refractoriness to medical treatment.Epileptic disorders: international epilepsy journal with videotape 12/2006; 8(4):285-8. · 1.50 Impact Factor -
Article: Vigabatrin and visual field defects in pediatric epilepsy patients.
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ABSTRACT: We studied the prevalence, type and severity of vigabatrin (VGB)-attributed visual field defects (VFDs), and used these data to assess the associated risk factors in pediatric patients. Medical records were retrospectively reviewed for 67 pediatric patients who received VGB alone or in combination with other antiepileptic drugs, and who had undergone visual field examinations using a Humphrey visual field analyzer. Of the 67 patients, 15 had VGB-attributed VFDs: 13 had nasal arcuate type, 1 had nasal and temporal constricted type and 1 had nasal constricted type. In terms of severity, 7 patients had Grade I VGB-attributed VFDs, 5 had Grade II, 2 had Grade III, and 1 had Grade IV. Although there were no significant differences between the VFD and non-VFD groups with regards to all tested parameters, there were no cases of VGB-attributed VFDs in patients with total treatment durations <2 yr and cumulative doses <10 g/kg. In conclusion, the prevalence of VGB-attributed VFDs in VGB-treated pediatric epilepsy patients was 22%. The high frequency of VGB-attributed VFDs indicates that physicians should inform all patients of this risk prior to VGB treatment and perform periodic visual field examinations.Journal of Korean Medical Science 09/2006; 21(4):728-32. · 0.99 Impact Factor
Top co-authors
Top Journals
Institutions
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2012
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Ulsan University Hospital
Ulsan, Ulsan, South Korea
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2006–2011
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Inje University Paik Hospital
Goyang, Gyeonggi, South Korea -
Asan Medical Center
Seoul, Seoul, South Korea
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