• Title/Summary/Keyword: Intractable epilepsy

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Effect of Vagus Nerve Stimulation in Post-Traumatic Epilepsy and Failed Epilepsy Surgery : Preliminary Report

  • Lee, Hai-Ong;Koh, Eun-Jeong;Oh, Young-Min;Park, Seung-Soo;Kwon, Ki-Hun;Choi, Ha-Young
    • Journal of Korean Neurosurgical Society
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    • v.44 no.4
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    • pp.196-198
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    • 2008
  • Objective : Vagus nerve stimulation (VNS) has been used in epilepsy patients refractory to standard medical treatments and unsuitable candidates for resective or disconnective surgery. In this study, we investigated the efficacy of VNS to patients who had refractory result to epilepsy surgery and patients with post-traumatic epilepsy. Methods : We analyzed the effect of VNS in 11 patients who had undergone previous epilepsy surgery and patients with intractable post-traumatic epilepsy associated with brain injury. All patients underwent VNS implantation between October 2005 and December 2006. Results : We evaluated seizure frequency before and after implantation of VNS and maximum follow up period was 24 months. In the first 6 months, 11 patients showed an average of 74.3% seizure reduction. After 12 months, 10 patients showed 85.2% seizure reduction. Eighteen months after implantation, 9 patients showed 92.4% seizure reduction and 7 patients showed 97.2% seizure reduction after 24 months. Six patients were seizure-free at this time. Conclusion : We conclude that the VNS is a helpful treatment modality in patients with surgically refractory epilepsy and in patients with post-traumatic epilepsy due to severe brain injury.

Comparison between Initial and Recent Surgical Outcome of 15-Year Series of Surgically Remediable Epilepsy

  • Lee, Myoung-Hee;Son, Eun-Ik
    • Journal of Korean Neurosurgical Society
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    • v.48 no.3
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    • pp.230-235
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    • 2010
  • Objective : The aim of this study is to compare the surgical outcome of the initial and recent surgical cases, during our 15-years experience, in terms of the surgical strategies and the prognostic factors for surgically remediable epilepsy. Methods : We retrospectively reviewed and compared the surgical outcomes between the initial 256 (Group I) and recent 139 (Group II) patients according to the time period of operation for a total of 518 consecutive epilepsy surgeries at our institution since 1992. The patients of the middle intermediate period, which were subjected to changed surgical strategies, were excluded. Results : The surgical outcome data from the initial and recent groups showed a much improved outcome for patients who underwent temporal lobe epilepsy (TLE) surgery over time. The number of patients with a good outcome (Engel class I-II) was much increased from 87.7% (178 TLE cases of Group I) to 94.8% (79 TLE cases of Group II) and this was statistically significant (p = 0.0324) on univariate analysis. Other remarkable changes were the decreased performance of intracranial invasive studies from 43.5% in Group I to 30.9% in Group II due to the advanced neuroimaging tools. The strip/grid ratio was reduced from 131/32 in Group I to 17/25 in Group II, because of a markedly reduced mesial TLE surgery and an increased extratemporal epilepsy surgery. Conclusion : Our results show that surgical outcome of epilepsy surgery has improved over time and it has shown to be efficient to control medically intractable epilepsy. Appropriate patient selection, comprehensive preoperative assessments and more extensive resection are associated with good postoperative outcomes.

Gamma Knife Radiosurgery in Patients with a Hypothalamic Hamartoma Associated with Intractable Gelastic Epilepsy : Report of Three Cases

  • Ko, Che-Kyu;Hong, Seok-Ho;Ko, Tae-Sung;Lee, Jung-Kyo
    • Journal of Korean Neurosurgical Society
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    • v.39 no.1
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    • pp.58-60
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    • 2006
  • Hypothalamic hamartoma[HH] is an unusual nonneoplastic developmental lesion associated with gelastic epilepsy and precocious puberty, mostly found in children. Although open surgery has been attempted when antiepileptic medication failed to control seizures, its deep location and surrounding vital structures often rendered surgery unsuccessful. We describe the outcome of gamma knife radiosurgery in three children with a HH associated with gelastic epilepsy and reviewed the literature for a possible therapeutic mechanism.

Glucose transport 1 deficiency presenting as infantile spasms with a mutation identified in exon 9 of SLC2A1

  • Lee, Hyun Hee;Hur, Yun Jung
    • 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.

A Case of Ring Chromosome 20 with Mental Retardation and Epilepsy (정신 지체와 간질을 동반한 20 환(Ring) 염색체 증후군 1례)

  • Jung, Yeon Kyung;Lee, Gyeong Hoon
    • Clinical and Experimental Pediatrics
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    • v.48 no.1
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    • pp.108-111
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    • 2005
  • Ring chromosome 20 mosaicism [r(20)] is a rare chromosomal anomaly associated with minor dysmorphism, mental retardation, autistic behavior, and intractable epilepsy. The proposed mechanism of ring formation is breakage of both short and long arms of a chromosome with subsequent end-to-end fusion. We encountered an 18-month-old boy who presented with developmental delay and mental retardation with seizure episodes, but showed normal brain magnetic resonance imaging. Chromosome study from peripheral blood showed 46,XY, r(20)(p13q13.3) karyotype. The authors report a case of ring chromosome 20 with mental retardation and epilepsy, with a review of the literature.

Magnetoencephalography Interictal Spike Clustering in Relation with Surgical Outcome of Cortical Dysplasia

  • Jeong, Woorim;Chung, Chun Kee;Kim, June Sic
    • Journal of Korean Neurosurgical Society
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    • v.52 no.5
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    • pp.466-471
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    • 2012
  • Objective : The aim of this study was to devise an objective clustering method for magnetoencephalography (MEG) interictal spike sources, and to identify the prognostic value of the new clustering method in adult epilepsy patients with cortical dysplasia (CD). Methods : We retrospectively analyzed 25 adult patients with histologically proven CD, who underwent MEG examination and surgical resection for intractable epilepsy. The mean postoperative follow-up period was 3.1 years. A hierarchical clustering method was adopted for MEG interictal spike source clustering. Clustered sources were then tested for their prognostic value toward surgical outcome. Results : Postoperative seizure outcome was Engel class I in 6 (24%), class II in 3 (12%), class III in 12 (48%), and class IV in 4 (16%) patients. With respect to MEG spike clustering, 12 of 25 (48%) patients showed 1 cluster, 2 (8%) showed 2 or more clusters within the same lobe, 10 (40%) showed 2 or more clusters in a different lobe, and 1 (4%) patient had only scattered spikes with no clustering. Patients who showed focal clustering achieved better surgical outcome than distributed cases (p=0.017). Conclusion : This is the first study that introduces an objective method to classify the distribution of MEG interictal spike sources. By using a hierarchical clustering method, we found that the presence of focal clustered spikes predicts a better postoperative outcome in epilepsy patients with CD.

Brain Somatic Mutations in Epileptic Disorders

  • Koh, Hyun Yong;Lee, Jeong Ho
    • Molecules and Cells
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    • v.41 no.10
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    • pp.881-888
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    • 2018
  • During the cortical development, cells in the brain acquire somatic mutations that can be implicated in various neurodevelopmental disorders. There is increasing evidence that brain somatic mutations lead to sporadic form of epileptic disorders with previously unknown etiology. In particular, malformation of cortical developments (MCD), ganglioglioma (GG) associated with intractable epilepsy and non-lesional focal epilepsy (NLFE) are known to be attributable to brain somatic mutations in mTOR pathway genes and others. In order to identify such somatic mutations presenting as low-level in epileptic brain tissues, the mutated cells should be enriched and sequenced with high-depth coverage. Nevertheless, there are a lot of technical limitations to accurately detect low-level of somatic mutations. Also, it is important to validate whether identified somatic mutations are truly causative for epileptic seizures or not. Furthermore, it will be necessary to understand the molecular mechanism of how brain somatic mutations disturb neuronal circuitry since epilepsy is a typical example of neural network disorder. In this review, we overview current genetic techniques and experimental tools in neuroscience that can address the existence and significance of brain somatic mutations in epileptic disorders as well as their effect on neuronal circuitry.

Emerging Surgical Strategies of Intractable Frontal Lobe Epilepsy with Cortical Dysplasia in Terms of Extent of Resection

  • Shin, Jung-Hoon;Jung, Na-Young;Kim, Sang-Pyo;Son, Eun-Ik
    • Journal of Korean Neurosurgical Society
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    • v.56 no.3
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    • pp.248-253
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    • 2014
  • Objective : Cortical dysplasia (CD) is one of the common causes of epilepsy surgery. However, surgical outcome still remains poor, especially with frontal lobe epilepsy (FLE), despite the advancement of neuroimaging techniques and expansion of surgical indications. The aim of this study was to focus on surgical strategies in terms of extent of resection to improve surgical outcome in the cases of FLE with CD. Methods : A total of 11 patients of FLE were selected among 67 patients who were proven pathologically as CD, out of a total of 726 epilepsy surgery series since 1992. This study categorized surgical groups into three according to the extent of resection : 1) focal corticectomy, 2) regional corticectomy, and 3) partial functional lobectomy, based on the preoperative evaluation, in particular, ictal scalp EEG onset and/or intracranial recordings, and the lesions in high-resolution MRI. Surgical outcome was assessed following Engel's classification system. Results : Focal corticectomy was performed in 5 patients and regional corticectomy in another set of 5 patients. Only 1 patient underwent partial functional lobectomy. Types I and II CD were detected with the same frequency (45.45% each) and postoperative outcome was fully satisfactory (91%). Conclusion : The strategy of epilepsy surgery is to focus on the different characteristics of each individual, considering the extent of real resection, which is based on the focal ictal onset consistent with neuroimaging, especially in the practical point of view of neurosurgery.

A Primer on Magnetic Resonance-Guided Laser Interstitial Thermal Therapy for Medically Refractory Epilepsy

  • Lee, Eun Jung;Kalia, Suneil K.;Hong, Seok Ho
    • Journal of Korean Neurosurgical Society
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    • v.62 no.3
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    • pp.353-360
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    • 2019
  • Epilepsy surgery that eliminates the epileptogenic focus or disconnects the epileptic network has the potential to significantly improve seizure control in patients with medically intractable epilepsy. Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has been an established option for epilepsy surgery since the US Food and Drug Administration cleared the use of MRgLITT in neurosurgery in 2007. MRgLITT is an ablative stereotactic procedure utilizing heat that is converted from laser energy, and the temperature of the tissue is monitored in real-time by MR thermography. Real-time quantitative thermal monitoring enables titration of laser energy for cellular injury, and it also estimates the extent of tissue damage. MRgLITT is applicable for lesion ablation in cases that the epileptogenic foci are localized and/or deep-seated such as in the mesial temporal lobe epilepsy and hypothalamic hamartoma. Seizure-free outcomes after MRgLITT are comparable to those of open surgery in well-selected patients such as those with mesial temporal sclerosis. Particularly in patients with hypothalamic hamartoma. In addition, MRgLITT can also be applied to ablate multiple discrete lesions of focal cortical dysplasia and tuberous sclerosis complex without the need for multiple craniotomies, as well as disconnection surgery such as corpus callosotomy. Careful planning of the target, the optimal trajectory of the laser probe, and the appropriate parameters for energy delivery are paramount to improve the seizure outcome and to reduce the complication caused by the thermal damage to the surrounding critical structures.

A Case Report of Central Nervous System Disorder Induced Intractable Hiccups Treated with Korean Medicine (중추신경계 질환자에게 이환된 난치성 딸꾹질의 한방치험1례)

  • Moon, Jiseong;Min, Seonwoo;Kim, Yeseul;Park, Jiyoon;Hong, Junghwa;Choi, Dong-jun
    • The Journal of Internal Korean Medicine
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    • v.43 no.2
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    • pp.191-200
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    • 2022
  • Intractable hiccups are those which persist for more than one month and can often occur in patients with disorders of the central nervous system, such as stroke, epilepsy, or brain tumor. An 80-year-old male patient undergoing conservative hemodialysis for diabetic nephropathy, recently developed paralysis and dysphagia due to central nervous system complications including stroke and epilepsy. He was admitted to the ◯◯ Oriental Medicine Hospital, and treated with herbal medicine (Gyulpijugyeo-tang, Jeonssiigong-san, and Ijin-tang) and electrical acupuncture for 30 days. No side effects were observed during hospitalization. The frequency and duration of hiccups were measured daily and found to reduce, and the effect persisted during his admission. The patient took no other nervous system drugs. In conclusion, Korean medicine like gyulpijugyeo-tang and acupuncture can be effective for patients with intractable hiccups who cannot use alternative drugs or treatment, because of other complications.