PURPOSE: The treatment of West syndrome is difficult since current antiepileptic drugs are rarely effective. The objective of this study is to evaluate the clinical efficacy of topiramate as add-on therapy in the refractory West Syndrome. METHODS: Nineteen children with refractory infantile spasms were included in this study. Topiramate was given as an initial dose of 25 mg per day in addition to the current antiepileptic drug (s). Dosage was increased by 25 mg every week until spasms were controlled, the maximal tolerable dose was reached, or the maximal dose of 25 mg/kg/day was achieved. RESULTS: Five (26.3%) subjects became seizure free and 7 (36.8%) achieved seizure frequency reduction more than 50%. No significant side effect of topiramate was noted. CONCLUSION: Topiramate could be a promising therapy in the treatment of refractory West syndrome.
Anticonvulsants ; Child ; Humans ; Infant ; Infant, Newborn ; Seizures ; Spasm ; Spasms, Infantile*

BACKGROUND AND OBJECTIVES: Temporal lobe epilepsy is the most common uncontrolled epileptic condition and is increasingly treated with surgery. In the absence of definitive results from noninvasive procedures, patients undergo implantation of intracranial electrodes. Intracranial EEG recordings are more accurate than scalp EEG recordings because of minimal artifact and closer approximation to the area of seizure onset. Intracranial EEG patterns between the medial and the lateral temporal lobe epilepsy were thought to have a little differences. METHODS: The authors compared the morphological pattern of seizure onset area, spread pattern, termination pattern and duration of the intracranial EEG manifestation of spontaneous seizures recorded from combined depth and subdural electrodes in 25 intractable temporal lobe epilepsy patients. RESULTS: Sixteen cases were medial temporal onset, six cases were neocortical onset and three cases were multifocal onset. The morphological onset pattern of medial temporal seizures was more likely to have high frequency rhythmic discharge (>13 Hz) and tended to show repetitive spikes prior to the seizure, whereas neocortical seizures were characterized by slow (4-to 10-Hz) and fast frequencies (>35 Hz), without evidence of repetitive spikes. The mean ictal duration at seizure onset of complex partial seizure of medial onset seizure was 121 seconds and was not different from those of neocortical seizures which were 115 seconds. Neocortical seizures take more time to propagate than medial seizures. Propagation to the opposite side of neocortical onset seizure takes 45.53 seconds, whereas medial temporal onset seizure takes 27.92 seconds. In case of second generalization, neocortical seizures continued longer than medial seizures. Of the medial onset temporal lobe seizures, except the simple partial seizures, the 35.2% of seizures initially spread to ipsilateral neocortex, and the 28.2% of seizures initially spread to the contralateral medial temporal lobe and the 25% of seizures simultaneously propagated to the ipsilateral neocortex and contralateral medial temporal lobe. Among the seizures initiated from the neocortex, 79.3% of seizures initially spread to the ipsilateral medial temporal area, but never initially spread to opposite neocortex. The termination pattern of seizures was divided into three types according to their location. In case of medial temporal lobe seizures, the mean of 31% of seizures was diffusely terminated , 38% of seizures terminated at the onset region and 38% of seizures were elsewhere within onset region. On the other hand, 48.6% of neocortical temporal lobe seizure were terminated at seizure onset region. CONCLUSIONS: The pattern of ictal intracranial EEG provides distinguishable differences between the medial temporal seizure and the neocortical temporal seizure.
Artifacts ; Electrodes ; Electroencephalography* ; Epilepsies, Partial ; Epilepsy, Temporal Lobe* ; Generalization (Psychology) ; Hand ; Humans ; Neocortex ; Scalp ; Seizures ; Temporal Lobe*

PURPOSE: The detection of epileptogenic lesion plays an important role in the management of patients with partial epilepsy. Although the development of MRI improved the examination of cerebral hemispheres greatly, many patients with neocortical temporal lobe epilepsy (TLE) or extratemporal lobe epilepsy (extra-TLE) still show no lesion in conventional two-dimensional (2D) images. To increase the yield of MRI in those patients, we performed three-dimensional (3D) surface-projection rendering (SPR) of the cerebral hemispheres. METHODS: Conventional 2D MRI (T1, T2, FLAIR, thin slice SPGR) and 3D SPR were performed in 24 patients with neocortical TLE and extra-TLE, and 20 normal subjects. Sulcogyral patterns were evaluated blindly to clinical information. The locations of the epileptogenic zone, ictal onset zone (IOZ) and irritative zone (IRZ) were determined by intracranial EEG monitoring and epilepsy surgery. RESULTS: The 2D MRI identified epileptogenic lesions in five of the 10 neocortical TLE (50%) and five of the 14 extra-TLE (35.7%). 3D SPR revealed abnormal sulcogyral patterns in 9 of the 10 neocortical TLE (90%) and 9 of the 14 extra-TLE (64.3%). Cortical deformation zones with sulcogyral anomalies included the whole area of IOZ in 10 (55.5%) and IRZ in 6 (33.3%), overlapped with IOZ in 7 (38.9%) and IRZ in 11 (61.1%), were connected to IOZ in 1 (5.6%) and IRZ in 1 (5.6%). CONCLUSION: 3D SPR of volumetric MRI data can detect epileptogenic structural lesions of neocortical epilepsy that are not visible in the conventional 2D images.
Brain* ; Cerebrum ; Electroencephalography ; Epilepsies, Partial ; Epilepsy* ; Epilepsy, Temporal Lobe ; Humans ; Magnetic Resonance Imaging*

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