OBJECTIVE: To investigate whether the tonic-clonic seizure (TCS) induced by intermittent photic stimulation (IPS)was generalized tonic-clonic seizure (GTCS)or partial secondarily tonic-clonic seizure (PGTCS),and to analyze the relationship between them. METHODS: Video-electroencephalogram (VEEG)database of Peking University First Hospital from March 2010 to October 2018 were reviewed. Fifteen cases with idiopathic epilepsy who had TCS induced by IPS were included in this study, and their clinical and electroencephalogram (EEG)characteristics were retrospectively analyzed. RESULTS: In this study, 4 of the 15 cases were boys and 11 were girls. The age of seizure onset ranged from 1 to 13 years. According to the medical records: 12 cases were considered as GTCS,while the remaining 3 cases were considered as PGTCS. The age at VEEG monitoring ranged from 2.5 to 16.0 years. All backgrounds of the VEEG were normal. Interictal discharges:generalized discharges in 11 cases, of which 4 cases coexisted with posterior discharges, 2 cases coexisted with Rolandic discharges, the other 5 cases merely had generalized discharges; merely focal discharges in two cases, one in the Rolandic area and the other in the posterior area; no interictal discharge in the remaining 2 cases. IPS induced photoparoxysmal response (PPR)results: 2 cases without PPR,the remaining 13 cases with PPR of generalized discharges, and 6 of the 13 cases coexisted with posterior discharges. IPS induced photoconvulsive response (PCR)results: GTCS in one case (contradictory to medical history),PGTCS in 11 cases (consistent with medical history),and GTCS and PGTCS hardly to distinguish in the remaining 3 cases. Of the three conditions above, there were generalized myoclonic seizures induced by IPS before TCS in 7 cases. CONCLUSION The medical history was unreliable in determining whether TCS was generalized or focal. Myoclonic seizures can coexist with PGTCS, and sometimes GTCS was indistinguishable from PGTCS, indicating that the dichotomy of seizure types need to be improved. Photosensitive TCS should be regarded as a continuum between focal and generalized seizures.
Electroencephalography ; Epilepsy, Generalized ; Epilepsy, Tonic-Clonic ; Female ; Humans ; Male ; Retrospective Studies ; Seizures

Rolandic epilepsy(or benign epilepsy with centrotemporal spikes) and childhood absence epilepsy are idiopathic epilepsies and are the most common forms of age-related epilepsies, occurring in previously neurologically normal children. Benign epilepsy with centrotemporal spikes is the most common partial childhood epilepsy, beginning between 2 and 13 years of age and characterized by typical EEG focal discharges and a self- limited course with recovery in or before puberty. Childhood absence epilepsy is one of generalized epilepsies with bilateral synchronous and symmetrical spike-wave paroxysms of 3 Hz and it has a good prognosis under a correct antiepilepsy treatment. These two epilepsies share some common features : similar age at onset, overall good prognosis and marked hereditary predisposition. A coincidence of these two epilepsies in a patient is very rare. We experienced a case of a coincidence of rolandic and childhood absence epilepsy in a 6 year-old female. The presence of an absence focus in rolandic epilepy, however, makes the coincidence of these entirely distinct phenomena, even if very rare, not excluded. We present the case with a review of related literature.
Adolescent ; Child ; Electroencephalography ; Epilepsy ; Epilepsy, Absence* ; Epilepsy, Generalized ; Epilepsy, Rolandic ; Female ; Humans ; Prognosis ; Puberty

OBJECTIVE: To examine the changes of metabolites in the bilateral thalamus of patients with secondarily generalized tonic-clonic seizure (SGTCS) and to explore the mechanism of SGTCS. METHODS: Thirty patients with SGTCS (epilepsy group) and 30 matched healthy controls (control group) were examined by 1H-magnetic resonance spectroscopy (1H-MRS). The levels of N-acetyl aspartate (NAA), choline-containing compounds (Cho), creatine phosphocreatine (Cr-PCr), and myo-inositol (mI) of the bilateral thalamus were measured in both the epilepsy group and the control group. The ratios of NAA/Cr-PCr, NAA/(Cr-PCr+Cho), Cho/Cr-PCr and mI/Cr-PCr were compared and analyzed in the 2 groups. RESULTS: The ratios of NAA/Cr-PCr, and NAA/(Cr-PCr+Cho)(1.7074 ± 0.2214; 0.9333 ± 0.2173) in the left thalamus in the epilepsy group were significantly lower than those in the control group(1.8834 ±0.2093; 1.1243 ±0.2447)(P<0.05). The ratios of NAA/Cr-PCr, and NAA/(Cr- PCr+Cho) (1.7472 ±0.2439; 0.9165 ±0.2462) in the right thalamus in the epilepsy group were also significantly lower than those in the control group(1.8925 ± 0.2004; 1.0941 ± 0.2372)(P<0.05). There were no significant differences in the ratios of NAA/Cr-PCr, NAA/(Cr-PCr+Cho), Cho/Cr- PCr, and mI/Cr-PCr between the bilateral thalamis in the epilepsy group (P>0.05). CONCLUSION There is neuronal dysfunction in the bilateral thalamus in the epilepsy group. Abnormal changes of the bilateral thalamus are involved in the mechanism of SGTCS.
Adolescent ; Adult ; Aspartic Acid ; analogs & derivatives ; analysis ; Brain Chemistry ; Case-Control Studies ; Choline ; analysis ; Energy Metabolism ; physiology ; Epilepsy, Generalized ; metabolism ; physiopathology ; Epilepsy, Tonic-Clonic ; metabolism ; physiopathology ; Female ; Humans ; Magnetic Resonance Spectroscopy ; methods ; Male ; Middle Aged ; Thalamus ; metabolism ; physiopathology ; Young Adult

PURPOSE: To verify the efficacy and safety of lamotrigine (LTG) monotherapy in newly diagnosed children with epilepsy. METHODS: We prospectively enrolled 148 children who had undergone LTG monotherapy at our institution between September 2002 and June 2009. Twenty-nine patients were excluded: 19 due to incomplete data and 10 were lost to follow up. The data of the remaining 119 patients was analyzed. RESULTS: We enrolled 119 pediatric epilepsy patients (aged 2.8-19.3 years; 66 males and 53 females) in this study. Out of 119 patients, 29 (25.2%) had generalized epilepsy and 90 (74.8%) had partial epilepsy. The responses of seizure reduction were as follows: Seizure freedom (no seizure attack for at least 6 months) in 87/111 (78.4%, n=111) patients; partial response (reduced seizure frequency compared to baseline) in 13 (11.7%) patients; and persistent seizure in 11 (9.9%) patients. The seizure freedom rate was in 81.6% in patients with partial seizure (75.9% for complex partial seizure and 90.9% for benign rolandic epilepsy) and 44.8% in patients with generalized epilepsy (30.0% for absence seizure, 35.7% for juvenile myoclonic epilepsy patients, and 100.0% for idiopathic generalized epilepsy patients). Adverse reactions were reported in 17 (14.3%) patients, and 8 patients (6.7%) discontinued LTG because of rash and tic. No patient experienced severe adverse reaction such as Stevens-Johnson syndrome. CONCLUSION: LTG showed excellent therapeutic response and had few significant adverse effects. Our findings report may contribute in promoting the use of LTG monotherapy in epileptic children.
Child ; Epilepsies, Partial ; Epilepsy ; Epilepsy, Absence ; Epilepsy, Generalized ; Exanthema ; Freedom ; Humans ; Lost to Follow-Up ; Male ; Myoclonic Epilepsy, Juvenile ; Prospective Studies ; Seizures ; Stevens-Johnson Syndrome ; Tics ; Triazines

PURPOSE: Febrile seizures are characterized by a heterogenous phenotype segregating as an autosomal dominant trait with incomplete penetrance. Mutations in GABRG2 gene were identified in two families with generalized epilepsy and febrile seizures plus (GEFS+) and with absence epilepsy and febrile seizures(FSs). The present study assessed the role of GABRG2 gene in FSs and GEFS+ of the Korean population. METHODS: 66 FSs, 20 GEFS+ and 94 healthy control subjects were selected throughout a collaborative study of Catholic Child Neurology Research Group. The SNP211037 of GABRG2 was screened by DHPLC. DNA fragments showing variant chromatograms were subsequently sequenced. Genotypes and allelic frequencies for GABRG2 gene polymorphism in three groups were compared. RESULTS: The number of individuals with the GABRG2(SNP211037)-C/C genotype in patients with FSs was significantly greater compared with that in healthy control subjects and the GABRG2(SNP211037)-C allele frequency in patients with FSs was significantly higher than that in healthy control subjects. The odds ratio for developing FSs in individuals with the GABRG2(SNP211037)-CC genotype was 5.96 compard with individuals with the GABRG2(SNP211037)-T/T genotype. In contrast, the GABRG2 (SNP211037) gene in GEFS+ and control groups was not significantly different. CONCLUSION: Theses data suggest that genomic variations of GABRG2 might be one of the susceptibility factors for FSs in the Korean population.
Child ; DNA ; Epilepsy, Absence ; Epilepsy, Generalized ; Gene Frequency ; Genotype ; Humans ; Neurology ; Odds Ratio ; Penetrance ; Phenotype ; Polymorphism, Single Nucleotide* ; Seizures, Febrile*

PURPOSE: Mutations in gamma-aminobutyric acid(GABA) A receptor gamma2 subunit gene (GABRG2) were independently identified in families of generalized epilepsy with febrile seizures plus(GEFS+) and families of absence epilepsy and febrile seizures(FSs). The present study assessed the role of GABRG2 gene in idiopathic generalized epilepsies(IGEs) of Korean population. METHODS: Twenty-three IGEs and 94 healthy control subjects were selected through a collaborative study of Catholic Child Neurology Research Group. The SNP211037 of GABRG2 were screened by DHPLC. DNA fragments showing variant chromatograms were subsequently sequenced. Genotypes and allelic frequencies for GABRG2 gene polymorphism in three groups were compared. RESULTS: Genotypes and allelic frequencies of the gamma2 subunit of the GABA receptor gene(SNP211037) in both groups were not significantly different. The most common genotypes for GABRG2(SNP211037) gene in both groups were T/C heterozygote. The allele C and T frequencies for GABRG2(SNP211037) in the IGEs group were 45.7% and 54.3%, respectively and in healthy control group, 42.6% and 57.4%, respectively. The number of individuals with the GABRG2 (SNP211037)-C/C genotype in the IGEs group was greater compared with that in the healthy control group(21.7% versus 12.8%). The odds ratio for developing IGEs in individuals with the GABRG2 (SNP211037)-CC genotype was 1.65 compared with individuals with the GABRG2 (SNP211037)-T/T genotype, which was not significantly different. CONCLUSION: These data suggest that genomic variations of GABRG2 might not be one of the susceptibility factors for IGEs in the Korean population.
Alleles ; Child ; DNA ; Epilepsy, Absence ; Epilepsy, Generalized ; Genotype ; Heterozygote ; Humans ; Neurology ; Odds Ratio ; Polymorphism, Single Nucleotide* ; Receptors, GABA ; Seizures, Febrile

Jeavons syndrome is one of the underrecognized epileptic syndromes, characterized by eyelid myoclonia with or without absence seizures, eye closure-induced seizures, electroencephalography (EEG) paroxysms, and photosensitivity. This syndrome is considered to be among idiopathic generalized epilepsies, but the underlying pathophysiology is unknown. Recent studies using functional MRI and EEG have suggested an important role of both thalamus and occipital cortex in the fundamental pathophysiology underlying Jeavons syndrome. We described here a patient with typical Jeavons syndrome, in whom SPCET studies performed ictally and interictally revealed ictal hyperperfusion mainly confined to the both occipital and parietal cortices and ictal hypoperfusion in the diffuse frontal and temporal cortices. Our SPECT findings of ictal hyperperfusion in occipital and parietal cortices and ictal hypoperfusion in widespread cortices are, to certain degree, in line with previous EEG and fMRI studies, suggesting that the interactions between occipital and other cortical areas might be implicated in generalized spike-waves generation and a photosensitivity in Jeavons syndrome.
Electroencephalography ; Epilepsy, Absence ; Epilepsy, Generalized ; Eye ; Eyelids ; Humans ; Magnetic Resonance Imaging ; Seizures ; Thalamus ; Tomography, Emission-Computed, Single-Photon

Magnetic resonance imaging (MRI) is recommended for patients with epileptic seizures to rule out an underlying focal lesion. However, abnormalities in idiopathic generalized epilepsy, including childhood absence epilepsy, cannot usually be identified using brain imaging modalities such as MRI. Peri-ictal MRI abnormalities have been most commonly reported secondary to status epilepticus and are rarely observed in patients with focal seizures and generalized tonic-clonic seizures. Transient peri-ictal MRI abnormalities in absence epilepsy are extremely rare. A five-year-old girl presented with a three-day history of absence seizures that persisted despite continued treatment with sodium valproate. Electroencephalography showed bursts of generalized 3-Hz spike-and-wave discharges, during and after hyperventilation. Abnormal cortex thickening in the left perisylvian region was detected on T2-weighted brain MRI, and cortical dysplasia or a tumor was suspected. The patient started treatment with lamotrigine and was seizure-free after one month. The abnormal MRI lesion was completely resolved at the two-month follow-up. We report on a patient with childhood absence epilepsy and reversible brain MRI abnormalities in the perisylvian region. To our knowledge, this is the first report of transient MRI abnormalities after absence seizures. Transient peri-ictal MRI abnormalities should be considered for differential diagnosis in patients with absence seizures and a focal abnormality on brain MRI.
Brain ; Diagnosis, Differential ; Electroencephalography ; Epilepsy ; Epilepsy, Absence* ; Epilepsy, Generalized ; Female ; Follow-Up Studies ; Humans ; Hyperventilation ; Magnetic Resonance Imaging* ; Malformations of Cortical Development ; Neuroimaging ; Seizures ; Status Epilepticus ; Valproic Acid

No abstract available.
Carotid Artery, Internal* ; Epilepsy, Generalized*

PURPOSE: This study was designed to evaluate the safety and the reduction in seizure frequency of levetiracetam adjunctive therapy for pediatric epilepsy. METHODS: We retrospectively reviewed the medical records of 29 pediatric intractable epilepsy patients(male 19 and female 10). They all experienced more than two seizures per month, and were not controlled by initial combined two or more conventional antiepileptic drugs. They were followed up for over at least 3 months after prescribed levetiracetam from October 2005 to December 2006 at Sanggyepaik Hospital. RESULTS: 13 patients(44.8%) out of 29 showed reduction in seizure frequency of more than 50%, and 7 patients(24.1%) more than 75%. Of 14 patients who had generalized epilepsies including 9 patients with Lennox?Gastaut syndrome, 2 patients with severe myolonic epilepsy in infancy, 1 patient with late?onset infantile spasm, and 2 patients with undetermined generalized epilepsy, 4 patients(28.6%) showed seizure reduction of more than 50% and 1 patient(7.1%) of more than 75%. Of 15 patients who had partial seizures, 9 patients(60.0%) showed seizure reduction of more than 50% and 6 patients(40.0%) of more than 75%. There were significant differences between generalized seizure and partial seizure in those with more than 75% seizure reduction rate(Fisher's exact test, P=0.03). The mean maintenance dosage of drug was 34.8 mg/kg per day(5.7?71.4 mg/kg per day). The number of patients who showed adverse reactions was 9(31.0%) patients:increased seizure frequency in 4(13.8%) patients who discontinued levetiracetam, sedation in 3 patients and increased secretion and loss of appetite in one patient respectively in a descending order of of frequency. CONCLUSION: Levetiracetam is believed to be an effective, and safe anticonvulsant when used as an adjunctive therapy for various refractory childhood epilepsies.
Anticonvulsants ; Appetite ; Child ; Epilepsy ; Epilepsy, Generalized ; Female ; Humans ; Infant ; Infant, Newborn ; Medical Records ; Piracetam ; Retrospective Studies ; Seizures ; Spasms, Infantile