OBJECTIVETo study and compare the clinical and electroencephalography (EEG) features in children with benign occipital epilepsy (BOE) of Gastaut and Panayiotopoulos types.

METHODSThe clinical data of 23 children with BOE (16 Gastaut type and 7 Panayiotopoulos type) were retrospectively studied.

RESULTSThe mean age of onset in the Panayiotopoulos and Gastaut groups were 4.5 and 9.1 years, respectively. The children in the Panayiotopoulos group were characterized by ictal vomiting, frequent deviation of eyes and head, frequent nocturnal seizures, and secondary generalized seizures with longer duration but less frequency. By comparison, the children in the Gastaut group were characterized by visual symptoms as ictal events, higher seizure frequency, shorter seizure duration and more frequent diurnal seizures. The EEG showed that the majority of both groups had occipital spike-wave discharges when the eyes were opened. Eleven children in the Panayiotopoulos group and all of 7 children in the Gastaut group received antiepileptic mono-drug therapy. All of the 11 children in the Panayiotopoulos group responded to the therapy, but 2 cases in the Gastaut group did not respond and 2 cases had subtle cognitive deficits.

CONCLUSIONSThere are differences in the age of onset, clinical symptoms, seizure frequency and duration, and therapeutic responses between children with Panayiotopoulos and Gastaut type BOE.

Child ; Child, Preschool ; Electroencephalography ; Epilepsies, Partial ; drug therapy ; physiopathology ; Female ; Humans ; Male

OBJECTIVETo investigate the clinical, neurophysiologic characteristics and therapeutic considerations of epileptic negative myoclonus (ENM) in atypical benign partial epilepsy of childhood (ABPE).

METHODSVideo-EEG monitoring with outstretched arm tests were carried out in 17 patients, and 9 of them were examined with simultaneous electromyography (EMG). The ENM manifestations, electrophysiologic features and responses to antiepileptic drugs (AED) were analyzed.

RESULTSSeventeen patients were diagnosed as having benign childhood epilepsy with centrotemporal spikes (BECT) during the early course of the disease and were treated with AED. During the course of the disease, hand trembling, objects dropping, head nodding and instability during standing might be clues for ENM occurrence. ENM had been confirmed in our patients by outstretched arm tests during video-EEG recording. The ictal EEG showed that high-amplitude spikes followed by a slow wave over the contralateral motor areas. This was further confirmed by time-locked silent EMG in 9 patients. During ENM occurrence or recurrence, the habitual seizures and interictal discharges were exaggerated. Atypical absence seizures also occurred in 6 patients. The alteration of therapeutic options of AED relating to ENM appearance in some patients included the add-on therapy with carbamazepine (CBZ), oxcarbazepine, phenobarbital, or withdrawal of valproate (VPA). ENM was controlled in most cases by using VPA, clonazepam (CZP) and corticosteroid with different combination.

CONCLUSIONENM could occur during the course of ABPE. Outstretching arm tests during video-EEG monitoring in combination with EMG was essential to confirm ENM. The ENM occurrence was always associated with the frequency increasing of habitual seizures and the aggravation of interictal discharges. Some AED such as CBZ might induce ENM. VPA, benzodiazepines and corticosteroid with different combination were relatively effective in treatment of ENM.

Anticonvulsants ; therapeutic use ; Child ; Child, Preschool ; Electroencephalography ; Electromyography ; Epilepsies, Myoclonic ; drug therapy ; physiopathology ; Epilepsies, Partial ; drug therapy ; physiopathology ; Female ; Humans ; Male

Anticonvulsants ; therapeutic use ; Child ; Child, Preschool ; Electroencephalography ; Epilepsies, Partial ; diagnosis ; drug therapy ; Humans ; Infant ; Occipital Lobe ; pathology ; Prognosis

OBJECTIVES: To study the difference in intestinal flora between children with focal epilepsy and healthy children and the change in intestinal flora after treatment in children with epilepsy. METHODS: A total of 10 children with newly diagnosed focal epilepsy were recruited as the case group and were all treated with oxcarbazepine alone. Their clinical data were recorded. Fecal specimens before treatment and after 3 months of treatment were collected. Fourteen aged-matched healthy children were recruited as the control group. Total bacterial DNA was extracted from the fecal specimens for 16S rDNA sequencing and bioinformatics analysis. RESULTS: After 3 months of carbamazepine treatment, the seizure frequency was reduced by >50% in the case group. At the phylum level, the abundance of Actinobacteria in the case group before treatment was significantly higher than that in the control group (P<0.05), and it was reduced after treatment (P<0.05). At the genus level, the abundances of Escherichia/Shigella, Streptococcus, Collinsella, and Megamonas in the case group before treatment were significantly higher than those in the control group (P<0.05), and the abundances of these bacteria decreased significantly after treatment (P<0.05). CONCLUSIONS There is a significant difference in intestinal flora between children with focal epilepsy and healthy children. Oxcarbazepine can significantly improve the symptoms and intestinal flora in children with epilepsy.
Aged ; Bacteria/genetics* ; Child ; DNA, Bacterial ; Epilepsies, Partial/drug therapy* ; Gastrointestinal Microbiome ; Humans ; RNA, Ribosomal, 16S/genetics*

Aicardi syndrome is a severe congenital disorder characterized by infantile spasms, chorioretinal lacunae, and agenesis or hypogenesis of the corpus callosum. A 6 month old female had developed abnormal eye movement and seizures of the complex partial type and myoclonic type. MRI pictures of the patient revealed the presence of genu associated with agenesis of the rest of corpus callosum. A funduscopic examination revealed bilateral small, solitary, pale areas with sharp borders, some of which had minimal surrounding pigmentation (chorioretinal lacunae), especially clustered around the disc, and they were more prominent on the left side. We report here on the unusual findings of a complex partial seizure, myoclonic seizure and the atypical EEG finding in addition to the well-known characteristic clinical and imaging findings of a patient with Aicardi syndrome.
Anticonvulsants/therapeutic use ; Arachnoid Cysts/*pathology ; Corpus Callosum/*abnormalities ; Electroencephalography ; Epilepsies, Myoclonic/drug therapy/*pathology ; Epilepsies, Partial/drug therapy/*pathology ; Female ; Humans ; Infant ; Magnetic Resonance Imaging ; Retinal Diseases/*pathology ; Spasms, Infantile/drug therapy/*pathology ; Syndrome

BACKGROUNDAn aura is usually considered to be the initial clinical sign of a seizure. The types of abnormal neuron activities (i.e., localized and generalized firing) play an important role in the diagnosis of epilepsy. The goal of this study was to investigate the types of auras and its correlation with the localization and treatment of epilepsy.

METHODSThe 426 epileptic patients with auras from a single center were reviewed with reference to International League Against Epilepsy (ILAE, 1981) classification; the clinical manifestations and incidence of auras were analyzed in this retrospective study, as well as the results of electroencephalogram (EEG), brain magnetic resonance imaging (MRI) and the treatment methods.

RESULTSAmong the 426 epileptic patients, six different types of auras were defined, including autonomic auras, sensory auras, mental and affective auras, aura as vertigo, cognitive auras, and unspeakable feelings. Duration of auras ranged from 2 s to 7 min; the median duration of auras was 64.2 s. Abnormal EEG was observed in 297 (69.72%) patients. Moreover, abnormal brain MRI was observed in 125 (29.34%) patients. Nineteen (4.46%) epilepsy patients with auras underwent both surgeries and antiepileptic drugs (AEDs) while others were treated only with AEDs.

CONCLUSIONSThis study suggested that auras played an important role in the diagnosis, classification, and localization of epilepsy. Epileptic aura could help differentiate partial seizure from generalized seizure.

Adult ; Anticonvulsants ; therapeutic use ; Electroencephalography ; Epilepsies, Partial ; diagnosis ; drug therapy ; surgery ; Epilepsy ; diagnosis ; drug therapy ; surgery ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Retrospective Studies ; Treatment Outcome ; Young Adult

OBJECTIVETo investigate the clinical efficacy and safety of oxcarbazepine (OXC) suspension in children with focal epilepsy.

METHODSA total of 118 children aged 2-14 years, who were newly diagnosed with focal epilepsy between October 2009 and December 2011, were randomly divided into experimental group (n=60) and control group (n=58). The experimental group was treated with an orally suspension of OXC and the control group was orally administered with carbamazepine (CBZ) tablets. The two treatment regimens were compared in terms of clinical efficacy and safety.

RESULTSAfter 13 and 26 weeks of treatment, the experimental group had response rates of 75% and 72% respectively and seizure-free rates of 53% and 50%, and the control group had response rates of 71% and 66% and seizure-free rates of 50% and 43% respectively. There were no significant differences in the clinical efficacy between the two groups (P>0.05). After 26 weeks of treatment, the adverse event rates of the experimental and control groups were 18% and 40% respectively, with a significant difference between the two groups (P<0.05).

CONCLUSIONSOXC suspension has a comparable clinical efficacy to that of CBZ tablets in children aged 2-14 years who are newly diagnosed with focal epilepsy, but OXC suspension causes fewer adverse events and has higher safety.

Adolescent ; Anticonvulsants ; therapeutic use ; Carbamazepine ; adverse effects ; analogs & derivatives ; therapeutic use ; Child ; Child, Preschool ; Epilepsies, Partial ; drug therapy ; Female ; Humans ; Male ; Suspensions

BACKGROUNDRecently, new anti-epileptic drugs (AEDs) have been more frequently selected to treat epilepsy. In the present study, we evaluated the dynamic changes of efficacy and safety of three newer AEDs for treating partial epilepsy in China.

METHODSPatients were collected sequentially and were divided into three groups which accepted oxcarbazepine (OXC), lamotrigine (LTG) or topiramate (TPM) therapy. Each group included monotherapy and add-on therapy subgroups. We followed all patients for one year and recorded the indexes of efficacy and safety in detail.

RESULTSA total of 909 patients finished the follow-up observation. No significant difference was found in proportion of patients with > or = 50% reduction, > or = 75% reduction and 100% seizure reduction in the LTG and OXC groups between the first and the second six months. In the TPM group there was a statistical difference between the first and the second six months in proportion of patients with > or = 50% reduction (P = 0.002), > or = 75% reduction (P < 0.0001) and 100% seizure reduction (P = 0.009) in the monotherapy subgroup, and about > or = 75% reduction and 100% seizure reduction in the add-on therapy subgroup (P < 0.0001). The efficacy between the add-on and monotherapy subgroups showed a statistical difference. The safety of the three newer AEDs was good.

CONCLUSIONSThe three newer AEDs all showed good efficacy and tolerability for partial epilepsy. And the efficacy can be maintained for at least one year.

Anticonvulsants ; therapeutic use ; Carbamazepine ; analogs & derivatives ; therapeutic use ; China ; Epilepsies, Partial ; drug therapy ; Follow-Up Studies ; Fructose ; analogs & derivatives ; therapeutic use ; Humans ; Treatment Outcome ; Triazines ; therapeutic use

Objective: To analyze the efficacy and safety of the sodium channel blockers (SCB) antiseizure medication in the treatment of focal epilepsy in infants under 6 months of age. Methods: This was a case series study. Infants with focal epilepsy with onset within 6 months of age and treated with SCB attending the Department of Neurology of Beijing Children's Hospital from June 2016 to April 2022 were collected. The clinical data, auxiliary examinations, SCB application, efficacy, adverse reactions, and prognosis were analyzed retrospectively. Patients were grouped according to type of seizure and epileptic syndrome, age of onset and etiology. Chi square test and Fisher exact test were used to analyze the differences between groups statistically. Results: A total of 118 infants were enrolled, 65 males and 53 females, with an age of epilepsy onset of 56 (4, 114) days. Developmental and epileptic encephalopathy was diagnosed in 60 infants, 39 had self-limited neonatal and (or) infantile epilepsy, and 19 had non-syndromic focal epilepsy. Application of SCB: 106 used oxcarbazepine, 2 used lacosamide, 9 switched from oxcarbazepine to lacosamide or a combination of 2 SCB, and 1 used oxcarbazepine, lacosamide, and lamotrigine successively; oxcarbazepine was the first choice in 46 cases. The age at which SCB was applied was 103 (53, 144) days. The children were followed up for 6 months to 6 years. SCB was effective in 89 cases (75.4%), including 70 cases (59.3%) who achieved seizure freedom. The seizure-free rate was higher in the focal epilepsy only group than in the group with other seizure types (64.4% (65/101) vs. 4/17, χ²=9.99, P<0.05). The responder and seizure-free rates were all higher in the group with the onset age of >3-6 months than the group >1-3 months (84.4% (38/45) vs. 62.5% (20/32), 73.3% (33/45) vs. 46.9% (15/32), χ²=4.85 and 5.58, both P<0.05). With the exception of variants in the PRRT2 gene, those with variants in sodium or potassium channels had higher responder and seizure-free rates than those with variants in other genes(86.2% (25/29) vs. 45.5% (10/22), 62.1% (18/29) vs. 22.7% (5/22), χ²=9.65 and 7.82,both P<0.05). The most common adverse event was transient hyponatremia, which happened in 66 cases (55.9%). There were 9 cases of rash, which subsided in 6 cases after discontinuing oxcarbazepine and switching to lacosamide, and 7 cases of electrocardiogram abnormalities, which improved after withdrawing oxcarbazepine and changing to lacosamide in 1 case. Conclusion: SCB are effective and tolerable in the treatment of focal epilepsy in infants under 6 months of age, with better efficacy in patients with genetic variants of the sodium or potassium channel, focal seizures only, and seizure onset >3-6 months of age.
Child ; Female ; Male ; Infant, Newborn ; Humans ; Infant ; Sodium Channel Blockers/adverse effects* ; Oxcarbazepine ; Lacosamide ; Retrospective Studies ; Epilepsies, Partial/drug therapy* ; Seizures ; Sodium ; Anticonvulsants/adverse effects*

We described a female patient with insulinoma who experienced recurrent episodes of automatism, confusion and convulsion. Furthermore, her electroencephalography (EEG) findings resembled the pattern in complex partial seizures with secondary generalization. The interictal EEG showed spikes and sharp waves, as well as focal slowing over the left temporal lobe, and the ictal EEG revealed generalized spikes and sharp waves associated with diffused slowing. She was initially misdiagnosed as pharmacoresistant epilepsy. After the insulinoma was found and surgically removed, her EEG turned normal and she was seizure-free during the 4-year follow-up. This report highlights the need for careful reassessment of all seizures refractory to medication, even for the patients associated with epileptiform discharges on EEG.
Anticonvulsants ; pharmacology ; Diagnosis, Differential ; Drug Resistance ; Electroencephalography ; Epilepsies, Partial ; diagnosis ; drug therapy ; Female ; Humans ; Insulinoma ; diagnosis ; diagnostic imaging ; Middle Aged ; Pancreatic Neoplasms ; diagnosis ; diagnostic imaging ; Tomography, X-Ray Computed