Valproate is a widely used antiepileptic drug with rarely occurring serious side effects. However, valproate may induce hyperammonemic encephalopathy. A 85-year-old woman was admitted with stuporous mental status. Brain MRI showed old cerebral infarction and EEG showed sharp waves in the right frontal region. Under the impression of postictal confusion, we used phenytoin and then she was improved. However, the intermittent vacant staring was seen, and valproate was administered additionally. On the 5th day after adding valproate, her mentality was deteriorated and intermittent triphasic waves appeared on EEG. Due to confused mentality and hyperactivities, we injected lorazepam and then semicomatous mentality was developed. Follow-up EEG showed nearly continuous triphasic waves and slightly elevated ammonia with normal liver function was shown. After stopping antiepileptic drugs, we used lactulose and flumazenil, and then she was fully recovered with normalized EEG. This is a rare case of valproate-induced hyperammonemic encephalopathy with triphasic waves.
Aged, 80 and over ; Ammonia ; Anticonvulsants ; Brain ; Cerebral Infarction ; Electroencephalography ; Female ; Flumazenil ; Follow-Up Studies ; Humans ; Lactulose ; Liver ; Lorazepam ; Magnetic Resonance Imaging ; Phenytoin ; Stupor ; Valproic Acid

Peripheral neuropathy has been uncommon reported as one of neurologic adverse effects associated with phenytoin. A 53 year-old man presented with clinical and electrophysiologic signs of peripheral neuropathy after 13 years of phenytoin administration. Despite a modest dose of phenytoin (300 mg/day), blood level was 40 microg/ml. After discontinuing phenytoin about eight months, the peripheral neuropathy was improved. We present a case of reversible peripheral neuropathy caused by chronic phenytoin intoxication.
Humans ; Middle Aged ; Peripheral Nervous System Diseases* ; Phenytoin*

PURPOSE: To reveal the pattern of surgical outcome according to the location of ictal onset zone in the patients who had a history of early onset (less than two years old) hemiparesis and delayed epilepsy. Many children with acute infantile hemiplegia (AIH) develop delayed epilepsy. This can lead to HHE (Hemiplegia, hemiconvulsion, and epilepsy) syndrome. Epilepsy patients associated with AIH or HHE syndrome generally have been thought to be medically intractable and difficult to treat surgically. METHODS: Patients with a history of early onset hemiparesis with epilepsy who had undergone surgical treatment from November 1995 to May 2002 at Seoul National University Comprehensive Epilepsy Center were recruited. Diagnostic criteria include convulsions, followed by a flaccid hemiplegia, and later epilepsy with partial seizures. Multidisciplinary presurgical evaluations were performed which include a complete neurological examination, brain MRI, long-term video-EEG monitoring, FDG-PET, intracarotid amobarbital test, and ictal and interictal SPECT if possible. Patients with epileptogenic zone outside the hippocampus underwent implantation of intracranial electrodes. The surgical side was localized by the clinical, neuroimaging, and electrophysiological results includeing results of invasive studies. Anterior temporal lobectomy (ATL), cortical resection, functional hemispherectomy, and callosotomy were performed according to the results of presurgical evaluation. RESULTS: Twenty-five patients were included. Mean age was 29.8 ranging from 19 to 60 years old. Fifteen were male and 10 were female. All had a previous history of febrile convulsions. The onset age of febrile convulsion and hemiplegia was one to 48 months (mean=18.0+/-13.2) and the onset age of epileptic seizures were 0.5 to 40 years (mean=9.9+/-8.2). Seventeen of them were right-handed, eight were left-handed and one was bilateral-handed. Follow-up periods after surgery were one to eleven years (mean=5.6+/-2.2). Twelve patients were diagnosed as medial TLE and underwent ATL. The other thirteen patients were diagnosed as neocortical or multifocal epilepsy. Eleven of medial TLE patients were seizure free after ATL and only one had aura. However, only four patients with neocortical epilepsy were seizure free and nine were not. Surgical outcome was significantly different between ATL only and other surgical procedures (p=0.004). CONCLUSION: In patients of early onset hemiparesis with epilepsy, various ictal onset zones can be possible. The medial TLE patients diagnosed by comprehensive presurgical evaluation, in spite of hemiatrophy on brain MRI, showed a good surgical outcome. Surgical treatment should be considered for the selected patients.
Age of Onset ; Amobarbital ; Anterior Temporal Lobectomy ; Brain ; Child ; Electrodes ; Epilepsy* ; Female ; Follow-Up Studies ; Hemiplegia ; Hemispherectomy ; Hippocampus ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neuroimaging ; Neurologic Examination ; Paresis* ; Seizures ; Seizures, Febrile ; Seoul ; Tomography, Emission-Computed, Single-Photon

PURPOSE: Recently have there been a few reports that raised the question that Benign Rolandic Epilepsy (BRE) could be a spectrum. This study was conducted to identify whether or not the clinical and electrophysiological characteristics of typical Rolandic Spikes (RS) are different from those of concomitantly additional frontal or occipital spikes with RS. METHODS: The consecutive 39 patients who showed centro-temporal spikes were divided into typical RS and RS+ groups. We defined RS+ groups as having concomitantly additional frontal or occipital spikes with RS. The independent variables included clinical data 0(age of onset, a significant antecedent event as part of their etiology of epilepsy, nocturnal seizure, abnormal development, abnormal neurologic examination, pattern of seizure, response to medication for at least 1 year and epilepsy syndrome) and electrophysiological data (dipoles, spikes accentuated during sleep). RESULTS: Eighty two percents (32/39) of patients were RS, whereas 18% (7/39) were RS+. The median age of onset was 7 years old and only 10% showed significant antecedent events. Eighty six percents (34/39) of patients had BRE as epilepsy syndrome. Neither clinical factors nor electrophysiological characteristics were different between both groups. CONCLUSION: Although RS+ showed additional spikes more than centro-temporal area, the clinical and electrophysiological characteristics of RS+ were not different from those of RS. Centro-temporal with or without additional spikes could be a homogeneous condition rather than a spectrum.
Age of Onset ; Child ; Electroencephalography ; Epilepsy* ; Epilepsy, Rolandic ; Humans ; Neurologic Examination ; Seizures

PURPOSE: To determine whether the change of end-tidal pCO2 (ETCO2) level by hyperventilation evokes seizures or epileptiform discharges in temporal lobe epilepsy. METHODS: Twenty-four temporal lobe epilepsy patients were enrolled in this study. Hyperventilation was done for 5 min during EEG recording. We gathered data on ETCO2 levels during hyperventilation. The data included baseline ETCO2 (B-ETCO2), ETCO2 after 5 min of hyperventilation (5 min-ETCO2), the mean value of the ETCO2 (M-ETCO2), and the change in ETCO2 between baseline and the level after 5 min of hyperventilation (delta ETCO2). We counted the numbers of interictal spikes and considered the cases with more than 120% increments of the number during hyperventilation as the cases with the increased number of spikes. We compared the four variables between two groups, divided according to the increment to hyperventilation. RESULTS: Seizures were not induced by hyperventilation in all the cases. Thirteen of 24 patients were in the increment group. The M-ETCO2 was 33.0+3.3 mmHg (mean+SD) in the increment group and 29.4+5.3 in the no-increment group (p=0.064). The delta ETCO2 was 13.0+5.3 and 14.0+5.3, respectively (p=0.622). The 5 min-ETCO2 was 28.2+4.8 and 24.8+5.9, respectively (p=0.077). CONCLUSION: In this study, the frequency of interictal spikes was increased well by hyperventilation. The spike increments were not associated with the ETCO2 level or the change of that, but maybe with the individual susceptibility.
Carbon Dioxide ; Electroencephalography ; Epilepsy, Temporal Lobe* ; Humans ; Hyperventilation* ; Seizures ; Temporal Lobe*

PURPOSE: The goal of the present study was to find out whether blockade of GABAergic synaptic transmission by bicuculline (BIC) in the presence of 4-aminopyridine (4-AP) would lead to expression or suppression of epileptiform activity in the immature brain and to observe the effect of commonly used anticonvulsants (valproic acid (VPA), carbamazepine (CBZP)) and 6-cyano-7-nitroquinoxalinedione (CNQX) on that epileptiform activity. METHODS: The visual cortex slices were obtained from 14-18 day-old Sprague-Dawley rats. Extracellular cellular recording was performed to observe the induction of epileptiform activity perfused by artificial CSF (ACSF) with combined application of BIC and 4-AP and the effect of VPA, CBZP and CNQX on that epileptiform activity for at least 1 hour. RESULTS: Epileptiform activity perfused by ACSF with combined application of BIC and 4-AP was insensitive to commonly used anticonvulsants (VPA, CBZP) and sensitive to CNQX. CONCLUSION: This study suggests that the epileptiform activity induced by combined application of BIC and 4-AP is present even in immature visual cortex slices. And, the blockade of GABAergic inhibition by BIC under 4-AP showed the increase of immature brain excitability as mature brain. The attenuation of that epileptiform activity by a-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) antagonist (CNQX) showed that AMPA receptor had relevance to the induction of that epileptiform activity.
4-Aminopyridine ; 6-Cyano-7-nitroquinoxaline-2,3-dione ; alpha-Amino-3-hydroxy-5-methyl-4-isoxazolepropionic Acid* ; Animals ; Anticonvulsants* ; Bicuculline* ; Brain ; Carbamazepine ; gamma-Aminobutyric Acid ; Rats* ; Rats, Sprague-Dawley ; Receptors, AMPA ; Seizures ; Synaptic Transmission ; Visual Cortex*

PURPOSE: The aim of the present study was to determine whether an association exists between UGT1A4 gene polymorphisms (P24T and L48V) and the occurrence of lamotrigine (LTG)-induced rash. METHODS: Ten patients with LTG-induced rash were examined for the P24T and L48V genetic polymorphisms. The results were compared with 42 epilepsy patients without LTG-induced rash and 143 non-exposure epilepsy patients. RESULTS: P24T polymorphism was not found; all studied subjects were homozygous for the 24P allele. Genetic heterogeneity of the L48V polymorphism existed, but the genetic proportions and allele frequencies of L48V polymorphism were not significantly different in the three groups of patients (p>0.05, by Fisher's Exact Test). CONCLUSION: The results of our study suggest that it is unlikely that the L48V polymorphism in the UGT1A4 gene is an important factor underlying LTG-induced rash development, especially in the absence of P24T polymorphism.
Alleles ; Epilepsy ; Exanthema* ; Gene Frequency ; Genetic Heterogeneity ; Humans ; Polymorphism, Genetic

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

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

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