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Cryptogenic Gelastic Epilepsy of Fronto-Temporal Lobe Origin: A Case of Young Adulthood Onset.

Wook Joo KIM ; Sang Myung CHEON ; Sang Ho KIM

Journal of Korean Epilepsy Society.2002;6(2):154-157.

Gelastic epilepsy characterized by paroxysmal involuntary laughing episodes is a relatively rare type of seizure which may occur singly or, more frequently, with other types of convulsions. Gelastic seizures have been observed in many different conditions, mainly hypothalamic hamartomas. We report a 21-year-old woman whose uncontrollable laughter was the only neurologic disturbance since 20 years of age. Physical and neurological examination did not reveal any abnormality. Neuropsychologic test was also normal. Brain magnetic resonance imaging was normal. During video-EEG monitoring, the clinical events usually consisted of aura of undescriable sensation lasting 1-2seconds followed by ictal laughter, without loss of consciousness and postictal manifestation. Ictal EEG showed bilateral interruption of background activity lasting 2-3seconds, followed by semirhythmic theta frequency activities over right frontotemporal region. Interictal EEG showed intermittent sharp waves or spike activities at right anterior temporal area. After Oxcarbamazepine treatment, she has never experienced the laughing seizures.
Brain ; Electroencephalography ; Epilepsies, Partial* ; Epilepsy ; Female ; Hamartoma ; Humans ; Laughter ; Magnetic Resonance Imaging ; Neurologic Examination ; Neuropsychological Tests ; Seizures ; Sensation ; Unconsciousness ; Young Adult

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Gelastic Seizures in a Patient with Tuberous Sclerosis.

Seong Hee KIM ; Kwangsoo KIM ; Ji Won MIN ; Bong Goo YOO ; Kyung Moo YOO

Journal of Korean Epilepsy Society.2002;6(2):150-153.

BACKGROUND: Gelastic seizures are characterized by ictal inappropriate sudden laughter as the predominant seizure manifestation. they are very rare and may occur in patients with hypothalamic hamartomas, pituitary tumors, astrocytomas of the mamillary bodies, CNS infection, trauma, and dysraphic condition. CASE: A 21-year-old woman with tuberous sclerosis was admitted due to frequent sudden inappropriate laughter as a seizure manifestation. EEG showed paroxysmal brief generalized 4-5 Hz spike and wave complexes. Brain MRI revealed multiple tubers in the bilateral cerebral cortical areas, right caudate nucleus, and left anterior cingulate gyrus. We tried vigabatrin, but gelastic seizures were not controlled. COMMENT: We report a case of gelastic seizures associated with tuberous sclerosis, which has not previously been reported in Korea.
Astrocytoma ; Brain ; Caudate Nucleus ; Electroencephalography ; Female ; Gyrus Cinguli ; Hamartoma ; Humans ; Korea ; Laughter ; Magnetic Resonance Imaging ; Mamillary Bodies ; Pituitary Neoplasms ; Seizures* ; Tuberous Sclerosis* ; Vigabatrin ; Young Adult

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A Case of Juvenile Onset Lennox-Gastaut Syndrome Presenting as Atypical Absence.

Byoung Lip HA ; Sang Myeong CHEON ; Sang Ho KIM

Journal of Korean Epilepsy Society.2002;6(2):147-149.

Atypical absence is less understood than typical absence. Several conditions that produce atypical absence are known including Lennox-Gastaut syndrome, myoclonic astatic epilpsy and epileptic encephalopathy with continuous spike and waves in slow wave sleep. A 17-year-old girl with mental retardation had developed frequent loss of consciousness and occasional falling attack with traumatic facial injury for 2 years. The interictal EEG showed 2 Hz slow spike-and-wave complex with maximum over right frontotemporal area and the brain MRI was normal. Carbamazepin was prescribed initially but the drug seemed to worsen the seizures. Long term video-EEG monitoring showed very frequent atypical absence seizures consisting of sudden hypotonia of head and oral automatism with or without secondary generalization. Generalized 2 to 2.5 Hz slow spike-and-wave complexes with duration of 10 to 40 seconds were seen during ictal period. About 10% to 20% of the non REM sleep was occupied with generalized slow spike-and-wave complex and/or polyspikes or polyspikes-and-wave complex with duration of within 1 second. Valprorate monotherapy had failed, then lamotrigin was added. In spite of polytherapy, the seizure was intractable. We think this intractable atypical absence might be associated with juvenile onset Lennox-Gastaut syndrome.
Adolescent ; Automatism ; Brain ; Electroencephalography ; Epilepsy, Absence ; Facial Injuries ; Female ; Generalization (Psychology) ; Head ; Humans ; Intellectual Disability ; Magnetic Resonance Imaging ; Muscle Hypotonia ; Seizures ; Sleep, REM ; Unconsciousness

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A Case of Electrical Status Epilepticus during Sleep.

Hong Ki SONG

Journal of Korean Epilepsy Society.2002;6(2):143-146.

Electrical status epilepticus during sleep (ESES), an EEG defined syndrome characterized by the occurrence of almost continuous spike and/or slow waves during nonREM sleep, is considered to be rare in incidence. It broadly overlaps with benign rolandic epilepsy, pseudo-Lennox syndrome, and Landau-Kleffner syndrome. A 13-year-old boy has been followed up for 11 years because of intractable epilepsy. He is an adopted child and first seizure occurred at the age of 14 months. Seizures were described as tonic, atypical absence and partial motor, occurred daytime and nocturnal, more frequent in the latter. He was retarded in development and had failed to acquire speech. Serial EEG showed moderately developed posterior dominant rhythm during wakefulness, and, however, continuous 2.5 to 3 Hz bilaterally synchronous centro-temporal sharp waves were observed during sleep state. The EEG features were similar, regardless of spontaneous or induced sleep, and remained essentially unchanged during a 12 year follow up period. Brain MRI was not significant. During follow-up period, carbamazepine and vigabatrin worsened his seizures in frequency and intensity, resulting in frequent generalization. On the contrary, there were rare seizures with combination therapy of valproate, clonazepam and lamotrigine and seizures were nearly disappeared after topiramate add-on. This is a case of ESCS with cognitive, behavioral and language disturbances. Clinical and EEG features of related syndromes will be briefly reviewed.
Adolescent ; Brain ; Carbamazepine ; Child ; Clonazepam ; Electroencephalography ; Epilepsy ; Epilepsy, Rolandic ; Follow-Up Studies ; Generalization (Psychology) ; Humans ; Incidence ; Landau-Kleffner Syndrome ; Magnetic Resonance Imaging ; Male ; Seizures ; Status Epilepticus* ; Valproic Acid ; Vigabatrin ; Wakefulness

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Evaluation of Memory Impairment in Patients with Temporal Lobe Epilepsy Using the Wechsler Memory Scale.

Kwang Soo KIM ; Min Jeong KIM ; Bong Goo YOO ; Kyung Moo YOO

Journal of Korean Epilepsy Society.2002;6(2):137-142.

PURPOSE: Memory complaints are common in patients with epilepsy, particularly temporal lobe epilepsy. Many factors may adversely affect memory function in epilepsy, including seizures themselves, pathology causing the epilepsy and antiepileptic medication. The Wechsler Memory Scale (WMS) is used widely for test of memory. The aim of this study is to evaluate memory in patients with temporal lobe epilepsy using the WMS and to analyze memory impairment with respect to various factors. METHODS: Twenty four epileptic patients with temporal lobe epilepsy were evaluated with the WMS. Memory impairment was analyzed with respect to age at seizure onset, epilepsy duration, and antiepileptic medication. RESULTS: Memory test using the WMS showed significant differences between epileptic patients and controls for general information, mental control, associate learning, digit span and logical memory subtests. There was no correlation between memory quotient and age at seizure onset, epilepsy duration, or antiepileptic medication. CONCLUSIONS: Patients with temporal lobe epilepsy have a memory impairment though none of the factors to impair memory function in epileptic patients could be identified.
Epilepsy ; Epilepsy, Temporal Lobe* ; Humans ; Learning ; Logic ; Memory* ; Pathology ; Seizures ; Temporal Lobe*

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The Social Stigma of Epilepsy in Korea.

Sang Ahm LEE ; Hee Jung YOO ; Kyoung HEO ; Hyung Kook PARK ; Dong Jin SHIN ; Hong Ki SONG ; Won Young JUNG ; Oh Young KWON ; Sung Soo LEE ; Yo Sik KIM ; Sung Eun KIM ; Byung In LEE

Journal of Korean Epilepsy Society.2002;6(2):128-136.

BACKGROUND: To study social stigma of people with epilepsy (PWE) in Korea, focusing on which factors are associated with social stigma. METHODS: Data were collected from 400 adult epilepsy patients (51% male, mean age:32.9 years, range:19-64 years). Patients were recruited from 10 epilepsy centers in Korea. Clinical information about seizures was obtained by neurologists and the other information was collected by using self-completed questionnaires including stigma of epilepsy scale and several scales or questions. RESULTS: Thirty one percent of PWE felt stigmatized by epilepsy, 9% of them highly so. The presence of stigma of epilepsy was associated with duration of epilepsy, seizure remission or frequency, generalized tonic clonic seizures in the last 2 years, seizure-related injuries in the last year, the actual discrimination from society, all subscales of QOLIE-31, education, introverted and neurotic personality, helplessness, control, and confidence in problem solving, anxiety and depression, and self-esteem. Multivariate analysis identified discrimination from society, introverted personality, problem solving control, and emotional subscale of QOLIE-31. CONCLUSION: Our data showed the prevalence of social stigma of PWE in Korea might be not high unexpectedly and suggested that the actual discrimination from society and patients' coping style including personality might be important in feeling social stigma of epilepsy. Our results would be basic information for the further studies to clarify the causative factors to generate the stigma of epilepsy.
Adult ; Anxiety ; Depression ; Discrimination (Psychology) ; Education ; Epilepsy* ; Humans ; Korea* ; Male ; Multivariate Analysis ; Prevalence ; Problem Solving ; Surveys and Questionnaires ; Seizures ; Social Stigma* ; Weights and Measures

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Lipid Profiles in Patient with Ketogenic Diet for Intractable Childhood Epilepsy.

Jung Chae PARK ; Mee Jung PARK ; Heung Dong KIM

Journal of Korean Epilepsy Society.2002;6(2):124-127.

PURPOSE: Ketogenic diet has been frequently used in the treatment of intractable childhood epilepsy since proven strong antiepileptic effects. Because of prolonged maintenance of high fat diet, long-term side effects of hyperlipidemia and atherosclerosis has been concerned, but there are no sufficient data revealing lipid profile from ketogenic diet. This study is intended to investigate the changes of lipid profiles in patients with ketogenic diet. METHODS: Sixty-six children (0.8-14.7 yrs, mean age 4.6+/-3.6 yrs, 33 males and 33 females) with intractable epilepsy treated with the classic (4:1) ketogenic diet were studied at Inje University Sang-gye Paik Hospital, from January 1997 to July 2001. Follow-up period was more than 1year (range:1-2 yrs). Cholesterol, HDL-cholesterol and Triglyceride (TG) concentrations were measured at 1 day, 7 days, 6 months, 12 months, 18 months and 24 months, and LDL-cholesterol concentrations were measured. RESULTS: 1) Total cholesterol concentrations were significantly increased from 7 days to 24 months after ketogenic diet than that before ketogenic diet (p<0.05). 2) HDL-cholesterol concentrations were not significantly changed for 2 years. 3) LDL-cholesterol concentrations were significantly increased at 6 months (119.9 mg/dL, p<0.05), and 24 months (128.0 mg/dL, p<0.05). 4) TG concentrations were significantly increased at 7 days (164.9 mg/dL, p<0.05), 6 months (209.7 mg/dL, p<0.01), and 12 months (192.0 mg/dL, p<0.01), and were normalized at 18 months, 24 months. CONCLUSION: Most of lipid profiles were significantly changed during ketogenic diet. Long-term complication for cardio-vascular diseases should be further investigated.
Atherosclerosis ; Child ; Cholesterol ; Diet, High-Fat ; Epilepsy* ; Follow-Up Studies ; Humans ; Hyperlipidemias ; Ketogenic Diet* ; Male ; Triglycerides

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Surgical Treatment of Medically Refractory Epilepsy in Childhood.

Hoon Chul KANG ; Heung Dong KIM ; Yong Soon HWANG ; Sang Geun PARK

Journal of Korean Epilepsy Society.2002;6(2):117-123.

PURPOSE: Recent advances in neurophysiology and neuroimaging provided surgical application in intractable childhood epilepsy with improved outcome. The aim of this study is to reveal benefit and safety of epileptic surgery in childhood by reviewing our experiences. METHOD: Thirty patients who underwent epilepsy surgery from February, 1997 to June, 2002 at epilepsy center, Inje University Sang-gye Paik Hospital, were included. We reviewed clinical features and outcomes according to temporal/extratemporal epilepsies including generalized or undertermined epileptic syndromes. RESULTS: In 8 patients of temporal lobe epilepsy, surgical procedures were as follows, anterior temporal lobectomy (ATL) was in 6 patients, ATL and extended temporal resection in 1, ATL and multiple subpial transection (MST) in 1. Pathologic findings showed cortical dysplasia, hippocampal sclerosis, tumor and old infarction. Engel class I was in 6 (75%) patients and class II was in 2 (25%) patients. Six patients (75%) showed developmental progress. In 22 patients of extratemporal lobe epilepsy, various surgical methods such as partial or total lobectomy, functional lobectomy, corticectomy, functional hemispherectomy, peri-insular hemispherotomy, multiple subpial transection, lesionectomy and palliative corpus callosotomy were done according to the location of epileptic focus and epileptic syndromes. Engel class I was in 16 (72.7%) patients, class II in 4 (18.2%) and class III in 2 (9.1%) patients. Eight patients (66.6%) among 12 patients could have been followed for more than 12 months showed developmental progress. Most of neuroimaging studies were highly concordant to ictal EEG's. CONCLUSIONS: Epilepsy surgery is effective in most of localization related intractable childhood epilepsy and developmental acceleration can be expected in successfully treated patients.
Acceleration ; Anterior Temporal Lobectomy ; Epilepsy* ; Epilepsy, Temporal Lobe ; Hemispherectomy ; Humans ; Infarction ; Malformations of Cortical Development ; Neuroimaging ; Neurophysiology ; Sclerosis

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Thalamic Damage in Temporal Lobe Epilepsy with Mesial Temporal Sclerosis.

Young Dae KIM ; Kyoung HEO ; Seung Koo LEE ; Sun Ah PARK ; Soo Chul PARK ; Jin Woo CHANG ; Byung In LEE

Journal of Korean Epilepsy Society.2002;6(2):110-116.

PURPOSE: To investigate the frequency of the thalamic damage and its relationship with clinical data in patients with temporal lobe epilepsy and mesial temporal sclerosis (MTS). METHODS: We evaluated 45 patients with temporal lobe epilepsy showing unequivocal changes of unilateral MTS by the visual inspection of magnetic resonance imaging (MRI) compared to 20 controls. We presumed the inferior margin of the lateral ventricle (IMLV) posterior to the foramen of Monro reflects the volume of the thalamus, and investigated the thalamic damage by comparing bilateral IMLVs on oblique coronal T2 MR images posterior to the foramen of Monro. The relationships between clinical data and the presence of asymmetry of IMLV were evaluated. RESULTS: None of the control group had asymmetry of the hippocampus or IMLV. Asymmetry of IMLV was observed in 42% (19 of 45) of the patient group;IMLV was downward on the ipsilateral side in 40% (18), and on the contralateral side of MTS in one of the four patients having a larger lateral ventricle on the contralateral side of MTS. An asymmetric small fornix and mamillary body was found in 42% and 29% of the patients, respectively, in all ipsilateral to the side of MTS. A history of status epilepticus (SE) was more frequent in patients with downward IMLV ipsilateral to the side of MTS than in patients with symmetry of IMLV (five of 18 versus one of 26, p=0.011). CONCLUSIONS: This study suggests that the thalamic damage may be more frequently associated with MTS, when compared with previous studies, which applied more strict methods in evaluating the thalamic damage. There is a significant correlation between the presence of asymmetrical IMLV and history of SE, and our study suggests that excessive and prolonged seizure activity related to SE, can provoke thalamic injury in patients with temporal lobe epilepsy and MTS.
Cerebral Ventricles ; Epilepsy, Temporal Lobe* ; Hippocampus ; Humans ; Lateral Ventricles ; Magnetic Resonance Imaging ; Mamillary Bodies ; Sclerosis* ; Seizures ; Status Epilepticus ; Temporal Lobe* ; Thalamus

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Clinical Review of Severe Myoclonic Epilepsy in Infancy.

Hoon Chul KANG ; Oyungerel DAMBAJAMTS ; Heung Dong KIM

Journal of Korean Epilepsy Society.2002;6(2):104-109.

PURPOSE: Severe myoclonic epilepsy in infancy (SMEI) seems to be more common than realized, because it is often overlooked. In addition, the prognosis is poor despite of recent advances of antiepileptic treatment. This study is intended to provide better identification and treatment of SMEI by reviewing our experiences. METHOD: From April 1995 to September 2002, clinical and electrophysiologic features were reviewed for 18 patients with SMEI, who were diagnosed at epilepsy center, Inje University Sang-gye Paik Hospital. RESULTS: 1) Seizure onset age was 5.1+/-2.8 months (mean+/-SD) old. 2) Onset of febrile seizure was from 2 to 11 months of age (7.8+/-3.0 months) and febrile status epilepticus was also noted in 9 patients (50%). 3) Initial afebile seizure was noticed in 11 patients (61.1%). 4) Various types of seizures were manifested durign the patient's clinical courses 5) Myoclonic seizures were started from 7 to 48 months of age (26.5+/-12.1 months) and persisted in 14 (77.8%) patients. 6) Four patients (22.2%) had photosensitivity. 7) Mitochondrial cytopathy was suspected in 8 patients, and complex IV deficiency was confirmed in 1 patient. 8) Ketogenic diet was effective in 8 patients (61.5%) with seizure reduction more than 50% among 13 patients. 9) All sixteen patients who could be followed up for more than 12 months, showed normal initial development, and subsequently, all of them showed progressive delvelopmental delay with mild degree in 4 patients (22.2%), moderate degree in 7 patients (38.8%), and severe degree in 4 patients (22.2%). CONCLUSION: SMEI is one of the intractable childhood epileptic syndromes with variable clinical seizures and progressive developmental declining. SMEI should be paid more attention in epilepsy clinic for accurate diagnosis and adequate antiepileptic treatment including ketogenic diet.
Age of Onset ; Cytochrome-c Oxidase Deficiency ; Diagnosis ; Epilepsies, Myoclonic* ; Epilepsy ; Humans ; Ketogenic Diet ; Prognosis ; Seizures ; Seizures, Febrile ; Status Epilepticus

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