Epilepsy and driving.

Objective To identify clinical predictive factors in intractable childhood epilepsy, to provide some evidence for its early diagnosis. Methods Six hundred and twenty-four children with newly diagnosed epilepsy were retrospectively identified in Xinhua Hospital Affiliated to Shanghai Jiaotong University Medical School(1993-2009). The epileptic children were divided into a drug-responsive epilepsy group(n=584) and an intractable epilepsy group(n=40). Clinical data of patients were retrospectively analyzed, including the gender, age, pathogen, inducement, number of pre-treatment seizures, seizure type, seizure type change, seizure duration, family history, previous history, physical examination, brain CT and initial electroencephalogram(EEG) findings.Single factor analysis and Logistic regression analysis were made in 2 groups. Results Strong univariate associations suggested that some factors could increase the risks of intractable epilepsy: symptomatic or cryptogenic epilepsy, mental retardation, type of infantile spasm, positive neurological examination and large absolute number of pre-treatment seizures, and changes in seizure types in the course of the disease.With multiple Logistic regression, independent predictors of intractability were symptomatic or cryptogenic etiology(OR=3.61), large absolute number of pre-treatment seizures and changes in seizure types in the course of the disease(OR=4.76). Conclusions It's necessary to be wary of intractable epilepsy and to adjust therapy accordingly when seizures were uncontrollable and accompanied by one or more conditions such as symptomatic or cryptogenic epilepsy, large absolute number of pre-treatment seizures, changes in seizure types in the course of the disease. Key words: Child; Intractable epilepsy; Predictors

The factors relevant to intractability, types of epilepsy and impairment of dexterity in patients with intractable epilepsy were studied independently in different groups of patients. The factors relevant to intractable epilepsy that were disclosed in 202 patients, who required hospitalization more than twice, were as follows: strong seizure propensity, neuropsychiatric disorders including mental deterioration of various degrees, ataxia, personality changes and psychotic episodes, intolerance to antiepileptic drugs due to acute or chronic side effects, idiosyncrasy and internal disorders, self-induced seizure, misdiagnosis and mistreatment, and breakdown of family care of patients. The types of epilepsy in 224 patients with intractable epilepsy whose seizures were not adequately controlled and recurred on a monthly basis in spite of hospitalization were classified as follows: 101 patients with localization-related epilepsies or syndromes, 106 with generalized epilepsies or syndromes, 16 with undetermined epilepsies or syndromes and one with specific syndrome. In regard to partial epilepsies, frontal lobe epilepsy and partial epilepsy with multiple foci were at least partially intractable as temporal lobe epilepsy. With respect to intractable generalized epilepsies, miscellaneous symptomatic generalized epilepsies like intractable grand mal epilepsy with progressive mental retardation in childhood were as important as Lennox-Gastaut or West syndrome though it defies classification into any established syndromes. The proposed International Classification of Epileptic Syndromes and Epilepsies was found adequate for analysis of intractable epilepsy. The disturbance of fine motor performance found in 84 patients who participated in occupational therapy was investigated by test programs comprising nine subbatteries.(ABSTRACT TRUNCATED AT 250 WORDS)

Surgery has become an accepted treatment modality for carefully selected adults with intractable focal epilepsy. More recently, increasing numbers of pediatric patients with intractable epilepsy are also being referred for surgical consideration. Key elements of surgical candidacy include medically intractable focal epilepsy, a localized epileptogenic zone, and a low risk for new postoperative neurologic deficits. The most common etiologies of the epilepsies in pediatric surgical candidates are malformation of cortical development and low grade tumor but some patients with childhood onset temporal lobe epilepsy due to hippocampal sclerosis also present for early surgery. Based on results from several recent pediatric surgical series, the chance for favorable seizure outcome after surgery is not adversely affected by young age, with seizure-free postoperative outcome reported for 60% to 65% of infants, 59% to 67% of children, and 69% of adolescents, compared to 64% reported in a large, predominantly adult series. Some subgroups of patients have higher percentages of seizure-free outcome, including those with hippocampal sclerosis or low grade tumor. In addition to seizures, developmental issues are also a major concern in children with intractable epilepsy. Few quantitative data are available, but some anecdotal experience suggests that surgical relief of catastrophic epilepsy may result in resumption of developmental progression after surgery, although the rate of development often remains abnormal. In one series, best developmental outcomes were seen in patients with earliest surgery and highest level of preoperative development. For each patient, the timing of surgery must be carefully considered, based on a full assessment of the relative risks and benefits, derived from a detailed presurgical evaluation.

The authors undertook this study to review their experience with cortical resections in the rolandic region in children with intractable epilepsy. The authors retrospectively reviewed the medical records obtained in 22 children with intractable epilepsy arising from the rolandic region. All patients underwent preoperative electroencephalography (EEG), MR imaging, prolonged video-EEG recordings, functional MR imaging, magnetoencephalography, and in some instances PET/SPECT studies. In 21 patients invasive subdural grid and depth electrode monitoring was performed. Resection of the epileptogenic zones in the rolandic region was undertaken in all cases. Seizure outcome was graded according to the Engel classification. Functional outcome was determined using validated outcome scores. There were 10 girls and 12 boys, whose mean age at seizure onset was 3.2 years. The mean age at surgery was 10 years. Seizure duration prior to surgery was a mean of 7.4 years. Nine patients had preoperative hemiparesis. Neuropsychological testing revealed impairment in some domains in 19 patients in whom evaluation was possible. Magnetic resonance imaging abnormalities were identified in 19 patients. Magnetoencephalography was performed in all patients and showed perirolandic spike clusters on the affected side in 20 patients. The mean duration of invasive monitoring was 4.2 days. The mean number of seizures during the period of invasive monitoring was 17. All patients underwent resection that involved primary motor and/or sensory cortex. The most common pathological entity encountered was cortical dysplasia, in 13 children. Immediately postoperatively, 20 patients had differing degrees of hemiparesis, from mild to severe. The hemiparesis improved in all affected patients by 3-6 months postoperatively. With a mean follow-up of 4.1 years (minimum 2 years), seizure outcome in 14 children (64%) was Engel Class I and seizure outcome in 4 (18%) was Engel Class II. In this series, seizure outcome following perirolandic resection was intimately related to the child's age at the time of surgery. By univariate logistic regression analysis, age at surgery was a statistically significant factor predicting seizure outcome (p < 0.024). Resection of rolandic cortex for intractable epilepsy is possible with expected morbidity. Accurate mapping of regions of functional cortex and epileptogenic zones may lead to improved seizure outcome in children with intractable rolandic epilepsy. It is important to counsel patients and families preoperatively to prepare them for possible worsened functional outcome involving motor, sensory and/or language pathways.

Usefulness of liquid ketogenic milk for intractable childhood epilepsy

To investigate the expression of cell division cycle 42 GTP-binding protein (Cdc42) in the brain tissue of patients with intractable temporal epilepsy and discuss its effect on epileptogenesis. Twenty samples brain tissues were collected from the brain tissue bank of intractable temporal epilepsy established by Epilepsy Preventive and Therapeutic Center of Chongqing. The expression of Cdc42 was determined by immunohistochemistry and fluoroimmunoassay. Twenty brain tissue samples were collected from autopsy on 20 sex and age-matched persons who died from accident were used as controls. The absorption values of Cdc42 in the temporal lobe of the patients was 0.038 +/- 0.016, significantly higher than that of the controls (0.020 +/- 0.005, t = 14.59, P < 0.01). The absorption values of Cdc42 in the hippocampus of the patients was 0.049 +/- 0.009, t = 12.01, P < 0.01). Fluoroimmunoassay showed that the fluorescence intensity of Cdc42 in the temporal lobe of the patients was 18.3 +/- 2.5, significantly higher than that of the controls (11.8 +/- 2.9, t = 33.77, P < 0.01), and the Fluorescence intensity of Cdc42 in the hippocampus of the patients was and control hippocampus were 23.8 +/- 3.3, significantly higher than that of the controls (10.1 +/- 2.6, t = 12.01, P < 0.01). The overexpression of Cdc42 in the cortex of temporal lobe and hippocampus may play an important role in the epileptogenic mechanisms of intractable epilepsy.

Influencing factors of seizures in glioma patients and its relationship with recurrent epilepsy

Objective To explore the clinical manifestations, features, pathogenesis, and treatment of epilepsy secondary to stroke. Methods The seizure location and features, types, and treatment of epileptic seizures were retrospectively analyzed in 46 storoke patients with epilepsy. Results The incidence of epilepsy secondary to stroke was 6.1% (46/754). 39 of 754 patients (5.2%) developed early-onset epilepsy and the remaining 7 (0.9%) occurred late-onset epilepsy. 78.2% (36/46) was cortex epilepsy and 21.8% (10/46) was posterior cortex epilepsy. In early-onset epilepsy, generalized tonic-clonic seizure occurred in 30 patients and partial seizure in 9; while in late-onset epilepsy, 2 patients presented with generalized seizure and 5 with partial seizure. Cortex epilepsy was more common than posterior cortex epilepsy (P＜0.05). The prognosis was worse in stroke patients with epilepsy than those without epilepsy.Conclusions Cortex epilepsy secondary to epilepsy is common. Early-oneset epilepsy mainly presents with generalized tonic-clonic seizure and late-onset epilepsy presents with partial seizure. Early control of seizures is beneficial for patient recovery. Key words: Stroke; Epilepsy; Clinical feature

Stigma and quality of life at long-term follow-up after surgery for epilepsy in Uganda.

Prevalence of SCN1A mutations in children with suspected Dravet syndrome and intractable childhood epilepsy

Somatic variants are a recognized cause of epilepsy-associated focal malformations of cortical development (MCD). We hypothesized that somatic variants may underlie a wider range of focal epilepsy, including nonlesional focal epilepsy (NLFE). Through genetic analysis of brain tissue, we evaluated the role of somatic variation in focal epilepsy with and without MCD. We identified somatic variants through high-depth exome and ultra-high-depth candidate gene sequencing of DNA from epilepsy surgery specimens and leukocytes from 18 individuals with NLFE and 38 with focal MCD. We observed somatic variants in 5 cases in SLC35A2, a gene associated with glycosylation defects and rare X-linked epileptic encephalopathies. Nonsynonymous variants in SLC35A2 were detected in resected brain, and absent from leukocytes, in 3 of 18 individuals (17%) with NLFE, 1 female and 2 males, with variant allele frequencies (VAFs) in brain-derived DNA of 2 to 14%. Pathologic evaluation revealed focal cortical dysplasia type Ia (FCD1a) in 2 of the 3 NLFE cases. In the MCD cohort, nonsynonymous variants in SCL35A2 were detected in the brains of 2 males with intractable epilepsy, developmental delay, and magnetic resonance imaging suggesting FCD, with VAFs of 19 to 53%; Evidence for FCD was not observed in either brain tissue specimen. We report somatic variants in SLC35A2 as an explanation for a substantial fraction of NLFE, a largely unexplained condition, as well as focal MCD, previously shown to result from somatic mutation but until now only in PI3K-AKT-mTOR pathway genes. Collectively, our findings suggest a larger role than previously recognized for glycosylation defects in the intractable epilepsies. Ann Neurol 2018.

Surgical treatment for epilepsy

ObjectiveTrace elements such as zinc and copper have physiological effects on neuronal excitability that may play a role in the etiology of intractable epilepsy. This topic has been rarely discussed in Iranian epileptic patients.This study with the analysis of serum zinc and copper levels of children and adolescents with intractable and controlled epilepsy may identifies the potential role of these two trace elements in the development of epilepsy and intractability to antiepileptic drug treatment.Materials & MethodsSeventy patients between the ages of 6 months to 15 years that referred to Children’s Medical Center with the diagnosis of epilepsy, either controlled or intractable to treatment enrolled in the study. After informed parental consent the levels of serum zinc and copper were measured with atomic absorption spectrophotometer and analyzed with SPSS version 11.Results35 patients were enrolled in each group of intractable (IE) and controlled epilepsy (CE). 71.45% of the IE and 25.72% of the CE group had zinc deficiency that was statistically significant. 48.58% of the IE and 45.72 of the CE group were copper deficient, which was not statistically significant.ConclusionOur findings showed significant low serum zinc levels of patients with intractable epilepsy in comparison with controlled epilepsy group.We recommend that serum zinc level may play a role in the etiology of epilepsy and intractable epilepsy therefore its measurement and prescription may be regarded in the treatment of intractable epilepsy.

Increased population of oligodendroglia-like cells in pediatric intractable epilepsy

Objective To analyze the incidence and clinical characteristics of epilepsy in children with Prader-Willi syndrome (PWS), and to explore the relationship with genotype. Methods Ninety-five children with PWS were chosen from Department of Pediatric Neurology in Shengjing Hospital of China Medical University during 2008 to 2018.The general information of all children included in the study were collected and analyzed at the same time.At first, 95 children with PWS were divided into two groups according to epilepsy or not(epilepsy group 23 cases and non-epilepsy group 72 cases), the relationship between incidence of epilepsy and genotype or gender was analyzed.Furthermore, 23 PWS children with epilepsy were divided into two groups according to genotype: paternal 15q11-13 region deletion genotype group (20 cases) and maternal uni-parental disomy(UPD) group (3 cases), then the relationship between clinical characteristics and genotype was analyzed. Results A retrospective analysis of 95 patients with PWS found that the proportion of deletion in 15q11-13 was significantly higher in the epilepsy group than that in the non-epilepsy group, and the difference was statistically significant (87.0% vs.56.9%, χ2=6.832, P=0.009), while there was no statistical difference in gender (χ2=0.603, P=0.437). Among the 23 PWS children with epilepsy, 18 cases (78.2%) were generalized epilepsy, and 5 cases (21.8%) were focal epilepsy.The proportion of generalized epilepsy of deletion genotype group was higher than UPD group, and there was statistical difference(85.0% vs.33.3%, P 0.05). The effective epilepsy control rate was 91.3% in 23 children with epilepsy. Conclusion PWS children have a higher incidence of epilepsy, which can manifest with generalized features.PWS patients with a deletion genotype in paternal 15q11-13 region show a trend toward developing seizures, especially generalized epilepsy.But the seizure onset age and seizure frequency have no correlation with genotype.There is a good prognosis in most PWS with epilepsy. Key words: Prader-Willi syndrome; Epilepsy; Clinical characteristics; Genotype