• Clinical and Experimental Pediatrics
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• v.49 no.2
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• pp.187-191
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• 2006

Purpose : Malformation of cortical development(MCD) constitutes an important etiology of intractable epilepsy and is considered an indication for surgical treatments, though their efficacy is limited and variable depending on MCD's location or distribution. Ketogenic diets are widely known to be effective, but as little study has been made concerning their efficacy on epilepsy with MCD, we evaluated the efficacy of ketogenic diets on MCD patients compared with that of epileptic surgery, which is more invasive. Methods : We performed retrospective studies and analyse on 30 patients with MCD diagnosed by brain MRI and treated with ketogenic diets for intractable epilepsy since 1998, checking decreases in their seizure episodes after starting the diets. Results : Cortical dysplasia was observed in 24(80.0 percent) patients as the most common type of MCD. Also, MCD was observed in unilateral hemisphere most commonly, in 23(76.7 percent) patients; it was observed in both hemispheres in 7(23.3 percent) patients. Nine(30.0 percent) out of 30 patients became seizure-free after starting ketogenic diets, and 14(46.7 percent) patients experienced 50 percent seizure reductions as well. Age of starting the diet or the duration of epilepsy period before starting showed no statistical relationship with the efficacy of the diet. Though the younger the patient and the longer the treatment the more effective the diet seemed to be, there was no statistical correlation between them. The location of MCD showed no significance neither. Conclusion : Considering various limits and invasiveness of surgical treatment, a ketogenic diet could be a good tool in treating children with intractable epilepsy with MCD.

• Clinical and Experimental Pediatrics
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• v.50 no.9
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• pp.896-900
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• 2007

Purpose : The aim of this study was to investigate the speech problems in benign rolandic epilepsy (BRE) according to the seizure focus in EEG and semiology. Methods : Twenty three patients [right origin (13 patients) or left side (10 patients)] who met the BRE criteria by International League Against Epilepsy (ILAE) were prospectively enrolled. We excluded the patients who had abnormal MRI or showed both side spikes in EEG. Computerized Speech Lab was used to assess the speech characteristics of the patients. Results : The error pattern of laryngeal articulation in BRE was exclusively substitution of stop consonants, these errors showed more frequent in the left group (16.0% vs 25.5%). Voice onset time (VOT) of stop consonants and Total duration (TD) of word in both groups were prolonged than normal control group, especially in left group (P<0.05). The first formant of vowel /o/ and second formant of /e/ were significantly decreased in left group (P<0.05). The right group scored wider on pitch range ($192.9{\pm}54.0Hz$) and energy range in spontaneous speech ($14.2{\pm}6.4db$) than the left group ($233.3{\pm}12.5Hz$, $19.4{\pm}9.3db$, respectively, P>0.05). Duration of counting (5 to 9) in left group slower than right group ($8.6{\pm}1.7$ vs $7.9{\pm}1.8sec$). Conclusion : Our data suggested that interictal spikes and seizures in either centrotemporal sides, especially left side group, may induce speech problems. We recommend the logopedic and phoniatric evaluations of speech in BRE patients.

• The Korean Journal of Nuclear Medicine
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• v.35 no.6
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• pp.352-363
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• 2001

Purpose: To investigate ictal hyperperfusion patterns during semiologic progression of seizures, we performed SPECT subfraction in 50 patients with temporal lobe epilepsy (TLE). Materials and Methods: The patients were categorized Into five groups according to semiologic progression during ictal SPECT (group-1 having only aura; group-2 haying motionless staling with or without aura; group-3 having motionless staring and then automatism with or without aura; group-4 having motionless staring and then dystonic posturing with or without aura and automatism; group-5 having motionless staring, automatism, then head version and generalized seizures with or without aura and dystonic posturing). Results: In group-1, three patients showed ipsilateral temporal hyperperfusion and two had bilateral temporal hyperperfusion with ipsilateral predominance. In group-2, three (42.9%) patients showed bilateral temporal hyperperfusion with unilateral predominance and four (57.1%) revealed insular hyperperfusion of epileptic side. In group-3, 15 patients (88.2%) showed bilateral temporal hyperperfusion with unilateral predominance and 12 (70.6%) insular hyperperfusion. In group-4, 11 patients (84.6%) showed basal ganglia hyperperfusion on the opposite hemisphere to the side of the dystonic posturing. en group-5, there were multiple hyperperfusion areas in the frontal, temporal and basal ganglia regions. However, the injection times of radiotracer in five groups were relatively short and similar. Conclusions: The semiologic progression in TLE seizures were related to the propagation of hyperperfusion from ipsilateral temporal lobe to contralateral temporal lobe, insula, basal ganglia, and frontal lobe. Not only the radiotracer injection time but also semiologic progression after the Injection was significant in determining hyperperfusion pattern of ictal SPECT.

• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.276-279
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• 2018

Klinefelter syndrome a genetic disorder with various clinical manifestations. Neurological symptoms, such as seizures, are rarely reported with Klinefelter syndrome, and it response well to anti-epileptic drugs. A 5-month-old boy visited the Inha university hospital due to jerking movements and hiccups. The patient had been diagnosed with Klinefelter syndrome at birth and had a medical history of admission to the neonatal intensive care unit due to opisthotonus and ocular deviation at 26 days of age. The patient's serum testosterone level was decreased and his anti-$M{\ddot{u}}llerian$ hormone level was increased. The brain image examination was normal and the electoencephalography and other blood test results showed no specific findings. However, after admission, the patient recurred generalized tonic-clonic-seizures recurred intermittently even after the administration of antiepileptic drugs. This paper reports a case of non-febrile seizures in a child with Klinefelter syndrome who presented with a refractory course.

• Clinical and Experimental Pediatrics
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• v.51 no.1
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• pp.62-66
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• 2008

Purpose : In addition to epileptic seizures (ES), a variety of physiologic, organic and psychogenic disorders can manifest as paroxysmal behavioral events. Paroxysmal nonepileptic events (PNEs) are quite encountered in infants, young children, and adolescents. In a substantial proportion of cases, a careful history and examination will elucidate their nature. However, in other cases, it is necessary to differentiate PNEs from ES by video-electroencephalographic (EEG) monitoring. We report our experiences with PNEs in a group of children and adolescents who underwent video-EEG monitoring. Methods : From September, 2004 to June, 2006, one hundred thirty patients were monitored in the Pediatric Epilepsy Monitoring Units of Korea University Guro and Ansan hospitals. Their hospital charts were reviewed and video records of these events were analyzed. We observed all patients after video-EEG monitoring for more than 3 months. Results : Typical spells occurred during monitoring in 33 patients, not associated with a seizure pattern on EEG recordings. Two patients were diagnosed as frontal lobe epilepsy on basis of typical semiology and clinical characteristics, so 31 patients were documented to have PNEs finally. The mean age of patients was $7.2{\pm}5.8\;years$. The male to female ratio was 15 (48.4%) to 16 (51.6%). Among 31 patients, fifteen patients had associated disorders such as epilepsy, developmental delay, cerebral palsy, gastric ulcer, attention deficit hyperactivity disorder or depressive disorder. Somatoform disorder and factitious disorder was frequently seen in children more than 5 years old (P<0.05). Psychogenic disorder was more frequent in female (n=6) than in male (n=2) but there was no statistical significance (P>0.05). Conclusion : Our study suggests that video-EEG monitoring is an important diagnostic tool in the evaluation of paroxysmal behavioral events. With correct diagnosis of the PNEs, several unnecessary treatment could be avoided.

• Clinical and Experimental Pediatrics
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• v.52 no.1
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• pp.75-80
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• 2009

Purpose : In adults, valproate (VPA) has been reported to be associated with thrombocytopenia. However, few studies have investigated this association in children, and the factors affecting platelet counts remain controversial. This study was undertaken to investigate changes in platelet counts following VPA therapy and related factors in children with epilepsy. Methods : Our subjects comprised 75 epileptic children who had received VPA monotherapy for at least 1 year between January 2000 and December 2005. We regularly examined platelet counts at intervals of 6 to 12 months after baseline platelet counts before VPA treatment. We analyzed changes in platelet counts according to several factors such as sex, seizure type, underlying causes of epilepsy, age at the start of medication as well as at the last follow up, duration of VPA administration, VPA dose, serum VPA level, and body weight at the last follow up. Results : Overall, the platelet counts decreased significantly after VPA therapy. The average baseline platelet count was $312.5{\pm}109.1{\times}10^6/mL$. At the last follow up, the average platelet count was $261.7{\pm}72.2{\times}10^6/mL$. We observed the relationship between platelet counts and serum levels of VPA; while children with serum VPA <$75{\mu}g/mL$ showed no change in platelet counts (P=0.102), children with serum VPA >$75{\mu}g/mL$ showed a significant decrease in platelet counts. Conclusion : VPA may cause a significantly decreased platelet count in children with epilepsy. High serum VPA levels were associated with development of thrombocytopenia.

• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.280-283
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• 2018

Magnetic resonance imaging (MRI) is recommended for patients with epileptic seizures to rule out an underlying focal lesion. However, abnormalities in idiopathic generalized epilepsy, including childhood absence epilepsy, cannot usually be identified using brain imaging modalities such as MRI. Peri-ictal MRI abnormalities have been most commonly reported secondary to status epilepticus and are rarely observed in patients with focal seizures and generalized tonic-clonic seizures. Transient peri-ictal MRI abnormalities in absence epilepsy are extremely rare. A five-year-old girl presented with a three-day history of absence seizures that persisted despite continued treatment with sodium valproate. Electroencephalography showed bursts of generalized 3-Hz spike-and-wave discharges, during and after hyperventilation. Abnormal cortex thickening in the left perisylvian region was detected on T2-weighted brain MRI, and cortical dysplasia or a tumor was suspected. The patient started treatment with lamotrigine and was seizure-free after one month. The abnormal MRI lesion was completely resolved at the two-month follow-up. We report on a patient with childhood absence epilepsy and reversible brain MRI abnormalities in the perisylvian region. To our knowledge, this is the first report of transient MRI abnormalities after absence seizures. Transient peri-ictal MRI abnormalities should be considered for differential diagnosis in patients with absence seizures and a focal abnormality on brain MRI.