• Journal of Korean Neurosurgical Society
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• v.37 no.2
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• pp.124-128
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• 2005

Objective: Congenital bilateral perisylvian syndrome(CBPS) has been defined as a characteristic malformative perisylvian polymicrogyria(PMG) in patients with clinical symptoms of pseudobulbar palsy and epileptic seizures. For the present study, we investigate clinicopathologic features of CBPS associated with timing of lesion formation. Methods: Clinicopathologic features of CBPS from 6 patients with surgical resection of the cerebral lesions due to medically intractable seizures were studied. Results: Seizure onset ranged from 1 to 10years (average 6.7years) of age, and average duration of seizure was 23years. All had complex partial seizures, and two patients had additional tonic clonic seizures. Magnetic resonance (MR) images showed polymicrogyria, atropic gyri with gliosis. In the histopathologic examination, the cortical lesions revealed features of ulegyria; atrophic and sclerotic gyri, laminar loss of neurons, extensive lobular gliosis throughout the gray and white matter, neuronoglial nodule formation, and many amyloid bodies. Unlayered or four-layered PMG was not identified. Conclusion: Above data suggest that CBPS might be caused by ulegyria resulting from developmental cortical defect during early fetal stage or acquired hypoxic/ischemic injury in prenatal or postnatal life.

• Journal of the Korea Institute of Information and Communication Engineering
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• v.10 no.8
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• pp.1471-1477
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• 2006

Electrocorticogram (ECoG) was recorded in one young adult suffering from medically refractory partial seizures a few weeks before resection. ECoG of intractable focal epilepsy was analyzed usins AR model, wavelet analysis and cross-correlation analysis. The cross-correlation of the epileptic discharges was calculated between the electrodes in every unit of time, to get the phase shift. A contour map of the phase shift and the sequential two-dimensional phase shift maps were utilized to localize the epileptic foci and to study their propagation process. More than two epileptogenic foci were localized and two kinds of propagating process were shown. These investigations suggest that epileptic phenomena can be caused by at least two kinds of mechanisms in one patient.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.29-31
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• 2016

Glucose transport 1 (GLUT-1) deficiency is a rare syndrome caused by mutations in the glucose transporter 1 gene (SLC2A1) and is characterized by early-onset intractable epilepsy, delayed development, and movement disorder. De novo mutations and several hot spots in N34, G91, R126, R153, and R333 of exons 2, 3, 4, and 8 of SLC2A1 are associated with this condition. Seizures, one of the main clinical features of GLUT-1 deficiency, usually develop during infancy. Most patients experience brief and subtle myoclonic jerk and focal seizures that evolve into a mixture of different types of seizures, such as generalized tonic-clonic, absence, myoclonic, and complex partial seizures. Here, we describe the case of a patient with GLUT-1 deficiency who developed infantile spasms and showed delayed development at 6 months of age. She had intractable epilepsy despite receiving aggressive antiepileptic drug therapy, and underwent a metabolic workup. Cerebrospinal fluid (CSF) examination showed CSF-glucose-to-blood-glucose ratio of 0.38, with a normal lactate level. Bidirectional sequencing of SLC2A1 identified a missense mutation (c.1198C>T) at codon 400 (p.Arg400Cys) of exon 9.

• Journal of Yeungnam Medical Science
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• v.12 no.2
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• pp.306-318
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• 1995

To evaluate the intractability of partial epileptic patients by variables, the author studied 113 patients (uncontrolled: 45, controlled: 68) who were admitted to the Department of Neurology, College of Medicine, Yeungnam University from January, 1991 to August, 1993. The results were as follows. The items related to complex partial seizures, multiple seizure types and a histories of status epilepticus or clusters of seizures were significantly associated with drug-refractoriness (p<0.01). A high frequency of seizures before evaluation was associated with a poor outcome(p<0.01). The presences of known etiology of seizures, neurologic abnormalities and psychiatric disturbance were associated with limited treatment responses(p<0.01, p<0.05, p<0.01). An abnormal EEG findings such as background slowing, focal slowing, epileptiform discharges or secondarily bilateral synchrony were statistically significant (p<0.01). Age at onset, sex, distribution of epileptic foci, duration of seizure before evaluation, family history and abnormal neuroradiologic findings were not statistically significant. By these results, it was suggested that having at least four factors of the above variables were associated with limited treatment response.

• Journal of Korean Neurosurgical Society
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• v.29 no.12
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• pp.1650-1656
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• 2000

Objective : Little consensus exists concerning which temporal lobe structures need to be resected or how much resection should be done during hippocampal resection. The purpose of this study is to identify whether the extent of hippocampal resection influences seizure after anterior temporal lobectomy. Materials and Methods : The extent of hippocampal resection was assessed in 96 patients who underwent temporal lobectomy for medically intractable complex partial seizures originating from a unilateral seizure focus in the anteromesial temporal lobe. Patients who had structural lesion were excluded from the study. Postoperative magnetic resonance imaging in the coronal and saggital planes were used to quantify the extent of the hippocampal and lateral cortical resection. The patients were divided into two groups. Patients who underwent hippocampal resection to the level of the cerebral peduncle were included in the partial resection group, and those who had resection to the level of the colliculus were assigned to total resection group. Seizure outcomes were defined according to the Engel classification and compared between the two groups. Neuropsychologic outcomes in the selected cases were reviewed. Results : The over-all seizure-free outcome(Engel classification 1) was accomplished in 75%(72/96) of the patients (mean duration of follow-up, 36.8 months). The total hippocampectomy group had a statistically superior seizure outcome than the partial hippocampectomy group(87.3% versus 58.5% seizure-free, p-value=0.001). Also, younger patients had a more favorable outcome. Other variables such as laterality, the extent of lateral cortical resection, age at onset and gender were not significant. The pre- and postoperative memory functions were evaluated in 24 patients. A worse postoperative memory outcome was associated with partial hippocampectomy. However this was not acceptable due to a former bias. Conclusion : The result of this study conforms that aggressive hippocampectomy resulted in a better seizure outcome.

• Journal of Korean Neurosurgical Society
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• v.30 no.1
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• pp.26-32
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• 2001

Objective : The increasing use of sensitive neuroimaging techniques has demonstrated that significant percentage of patients with intractable complex partial seizures have brain masses, especially in temporal lobe. The optimal surgical solution for these patients is still open to debate. The purpose of our investigation is to evaluate the surgical outcome of patient with lesion-related temporal lobe epilepsy with respect to the types of surgery and the location of lesion. Patients and Methods : From DEC. 1993 to Dec. 1997, 35 patients with intractable epilepsy and space occupying temporal lobe lesion identified in preoperative MRI were included in this study. The types of surgery were lesionectomy, anterior temporal lobectomy with or without hippocampectomy. The location of lesion was divided as anteromedial group and lateral cortical group. The postoperative seizure outcomes according to the type of surgery and location of the lesion were compared. Results : Twenty-six of 34 patients(76.5%) were seizure-free after surgery. The Engel's class was favorable after anterior temporal lobectomy with or without hippocampectomy(p=.044) Conclusion : It is favorable to perform anterior temporal lobectomy for the treatment of intractable epilepsy with space-occipying lesion in temporal lobe. The resection of the hippocampus can be individualized.

• 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.

• Clinical and Experimental Pediatrics
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• v.51 no.10
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• pp.1085-1089
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• 2008

Purpose : This study aims to examine and compare the features of rolandic epilepsy. Methods : Of 158 patients selected retrospectively, 116 had typical (group A) and 42 had atypical (group B) rolandic epilepsy, as defined by Worrall's criteria. Results : The age at onset of the seizures in group A was $8.6{\pm}2.0y$ and $6.2{\pm}1.7y$ in group B (P>0.05). Among the 40 patients who underwent neuroimaging studies (25 patients in group A and 15 patients in group B), abnormal findings in group B included ventricular dilatation, mild cortical atrophy, and partial agenesis of corpus callosum. group A had no abnormal findings. The frequency of seizures was $2.0{\pm}1.0$ and $2.3{\pm}1.2$ per month in groups A and B respectively. Seizure control from the initial anticonvulsant treatment was achieved within 3 months in group A, and 3 to 12 months in group B. A 2-year remission rate was noted in 105 patients in group A and in 38 patients in group B. Of these, the recurrence rate after 2 y was 13 in group A and 12 in group B. Conclusion : Age of onset of seizures, gender, frequency of seizures before therapy, and 2-y remission rate were not significantly different in the 2 groups. However, neuroimaging abnormalities, the time to achieving seizure control from the initial anticonvulsant treatment, and the recurrence rate after being seizure-free for 2 y were significantly different in the 2 groups.

• Journal of The Korean Society of Clinical Toxicology
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• v.6 no.2
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• pp.142-145
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• 2008

Lamotrigine is a newer anti-epileptic drug for adjunctive treatment of refractory epilepsy, partial seizures, generalized tonic-clonic seizures, and bipolar disorder. Lamotrigine overdose causes serious central nervous and cardiovascular problems, but reports are uncommon. Few lamotrigine overdoses have been described because anti-epileptic drug use is limited and usually used with combination of other anti-epileptic drugs. In addition, most patients visit emergency departments with multi-drug overdoses, so few cases of lamotrigine poisoning alone exist. We had a female patient visit our emergency department a couple of hours after a lamotrigine overdose treated with intravenous hydration and urine alkalization by NaHCO3. She recovered successfully without any evidence of renal injury. However, she developed profound rhabdomyolysis, a previously unreported complication of this medication. We suggest that serial creatine kinase levels should be measured after lamotrigine poisoning.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.7 no.1
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• pp.92-109
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• 1996

The objectives of the present study were to provide comprehensive assessment of the impact of epilepsy on the psychological well-being of children with epilepsy and to identify the neurological factors associated with the psychopathology. The participant patients were recruited from the population of children and adolescent aged 7 to 16 attending the OPD of department of pediatric neurology in Seoul National University Hospital in Korea. We exclude mental retardation, pervasive developmental disorder and brain organic pathology. As control group, formal students were chosen and their sex, age, achievement, socioeconomic status were matched to patients. The first author interviewed the children and their family members and obtained the developmental history and family information. We used the following 10 scales for assessing psychological and behavioral problems in patients and their family member. The scales were standardized and their validity and reliability were confirmed before. Parent rating scales : Yale children's inventory, Disruptive behavior disorder scale, Parent's attitude to epilepsy questionnaire, Family environment scale, Symptom check-list-90 revision, Children behavior check-list. Children's self rating scales : Children's depression inventory, Spielberger's state-trait anxiety anxiety, Piers-Harris self-concept inventory and Self-administered Dependency questionnaire for Mother. The result showed the risk factors associated depression were early onset, complex partial seizure, lateralized temporal focal abnormality on EEG, Drug polypharmacy, high seizure frequency and sick factors associated anxiety were old age of patient, lateralized temporal focal abnormality EEG, Drug polypharmacy, high seizure frequency. Also the result of this present study indicated that risk factors associated oppositional defiant disorder, conduct disorder and attention deficit hyperactivity disorder were young age, male, early onset, lateral temporal EEG abnormality and high seizure frequency. According to these results, common risk factors associated psychological and behavioral problems were lateralized EEG temporal abnormality, high seizure frequency in neurological factors.