Ictal EEG patterns in epilepsy with centro-temporal spikes

Purpose: To describe the EEG pattern of seizures in patients with benign childhood epilepsy with centro-temporal spikes (BCECTS). Methods: The clinical and EEG data of 701 BCECTS patients with at least a 3 years follow-up were reviewed from 10 epilepsy centers. Results: Thirty-four seizures were recorded in 30 patients. Four different ictal EEG patterns (A-D) were identified. The most frequent (pattern A) was characterized by low voltage activity of fast rhythmic spikes, increasing in amplitude and decreasing in frequency, and occurred in 14 children. Pattern B (six patients) was constituted by a discharge of spikes intermixed with sharp waves increasing in frequency and amplitude. Pattern C (seven children) consisted of monomorphic theta which progressively formed a discharge increasing in amplitude and decreasing in frequency. Pattern D (5 children) was characterized by a initial focal depression of the electrical activity, followed by one of the three above described patterns. In 21 out of 28 children, the initial ictal pattern, altered from one pattern to another one. No clinical or EEG feature was predictive of a specific ictal pattern. Discussion: We failed to identify a unique ictal EEG pattern in our patients with BCECTS. The occurrence of per-ictal features, e.g., initial EEG depression or post-ictal slowing, is common and should not be interpreted with prejudice. Alteration of ictal EEG pattern from one to another is not in conflict with the diagnosis of BCECTS.     

Rett networked database: an integrated clinical and genetic network of Rett syndrome databases

Rett syndrome (RTT) is a neurodevelopmental disorder with one principal phenotype and several distinct, atypical variants (Zappella, early seizure onset and congenital variants). Mutations in MECP2 are found in most cases of classic RTT but at least two additional genes, CDKL5 and FOXG1, can underlie some (usually variant) cases. There is only limited correlation between genotype and phenotype. The Rett Networked Database (http://www.rettdatabasenetwork.org/) has been established to share clinical and genetic information. Through an "adaptor" process of data harmonization, a set of 293 clinical items and 16 genetic items was generated; 62 clinical and 7 genetic items constitute the core dataset; 23 clinical items contain longitudinal information. The database contains information on 1838 patients from 11 countries (December 2011), with or without mutations in known genes. These numbers can expand indefinitely. Data are entered by a clinician in each center who supervises accuracy. This network was constructed to make available pooled international data for the study of RTT natural history and genotype-phenotype correlation and to indicate the proportion of patients with specific clinical features and mutations. We expect that the network will serve for the recruitment of patients into clinical trials and for developing quality measures to drive up standards of medical management.     

Focal epilepsies in adult patients attending two epilepsy centers: classification of drug-resistance, assessment of risk factors, and usefulness of "new" antiepileptic drugs

Purpose: To classify the grade of antiepileptic drug (AED) resistance in a cohort of patients with focal epilepsies, to recognize the risk factors for AED resistance, and to estimate the helpfulness of “new‐generation” AEDs. Methods: We included 1,155 adults with focal epilepsies who were observed consecutively after 1990 and followed regularly at two epilepsy centers. We systematically collected the clinical, diagnostic, and therapeutic data using a custom‐written database. We classified the patients as seizure‐free or AED resistant according to the International League Against Epilepsy (ILAE) criteria, and we evaluated the risk factors associated with AED resistance using logistic regression analysis. We further grouped AED‐resistant patients in different grades (I, II, and III) according to the number of AEDs already tried as proposed by Perucca. Key Findings: AED resistance occurred in 57.8% of the 729 patients with symptomatic focal epilepsies and was positively associated with electroencephalography (EEG) abnormalities, seizure type, and the presence of mesial temporal sclerosis. Among 426 patients without detectable causes, the percentage of AED resistance was significantly lower (39.2%) and correlated with EEG abnormalities and psychiatric symptoms. Among AED‐resistant patients, the majority (64.6%) had tried three or more AEDs, which fit the more severe grade III proposed by Perucca. Among seizure‐free patients, more than one‐half (57%) needed to try two or more AEDs before reaching seizure control (14.9% needed three or more AEDs). Furthermore, among seizure‐free patients who could be previously classified as resistant to two or more AEDs, 52.2% reached seizure freedom while receiving treatment with “new generation” AEDs. Significance: The ILAE classification of AED resistance, as well the graded classification proposed by Perucca, was easily exploitable in our patients, although these classifications systems appear to have a limited value in predicting seizure outcome. Actually, a small but not negligible percentage of patients reached seizure freedom after trying several AEDs (including “new” AEDs), suggesting repeated trials may be necessary for seizure control.     

Pleiotrophic effects of natural products in ROS-induced carcinogenesis: The role of plant-derived natural products in oral cancer chemoprevention

Cancer is a multistage process where each stage involves different molecular, biochemical and cellular events all of which, however, contribute to malignant transformation. Over the last years, substantial scientific evidence has promoted the hypothesis that ROS-induced cellular damage underlies key steps during development of the malignant phenotype including evasion of apoptosis, limitless proliferation, angiogenesis, tissue invasion and metastasis, etc. On the other hand, natural products hold great promise as anti-cancer compounds in preventing against carcinogenesis both in vitro and in vivo. Throughout this article, we aim to review the evidence as to how some of these natural products exert their chemopreventive effects in human carcinogenesis. For this reason, we have placed particular emphasis on oral cancer where significant efforts have been made in alternative therapeutic strategies such as the use of plant-derived natural products. This is of paramount importance given the disease's high morbidity and mortality rates across the world and specifically in the geographic regions of India and South-East Asia where its incidence is increasing.     

Low vitamin D status in preschool children in Greece

Vitamin D status in humans depends on the amount of sun exposure and vitamin D intake. Recent reports suggest that hypovitaminosis D (as defined by serum 25-hydroxyvitamin D [25(OH)D] <10 ng/mL) is reemerging in developed countries and in the Middle East, pointing out the significance of dietary and cultural practices. In the line of prevention, we determined vitamin D status in 393 healthy preschool children randomly selected from 7 day care centers in the Municipality of Athens in October. The data for the analysis were collected from a questionnaire regarding their actual dietary practices, voluntary sun exposure, and lifestyle conditions; clinical investigation for the determination of the skin phototype; and blood sampling for the determination of serum 25(OH)D, parathyroid hormone, and osteocalcin levels. Of the 393 children, 49 were immigrants. According to our results, 6.6% of our population had serum 25(OH)D less than 10 ng/mL. Multilinear analysis showed that the amount of sun exposure and vitamin D intake were the direct determinants of vitamin D status. Immigrant children presented lower serum 25(OH)D levels associated with lower vitamin D intake and lower socioeconomic class when compared with the Greek children. No relationship was found between 25(OH)D concentration and skin phototype, whereas 93.3% of children used topical sunscreen. We suggest that abundant sunlight exposure in Athens is not sufficient to prevent hypovitaminosis D in preschool children. The extensive use of topical sunscreens and environmental factors such as air pollution would account for inadequate sunlight exposure and the need for dietary intake of vitamin D.     

Developmental,familial and educational characteristics of a sample of children with Autism Spectrum Disorders in Greece

The aim of this study is to describe the developmental, familial and educational characteristics of 91 children with a clinical diagnosis of autism spectrum disorders (ASDs), from one educational district of Athens, Greece. Records of the 91 children, aged 4–14 years old, were examined with respect to sex, age of diagnosis, type of ASDs, school placement, co-existing disorders, parental ages, type of conception, prematurity and birth order. The results indicated that the boy:girl ratio was 4.3:1 while the average age of diagnosis was 6 years which implies a delay in the early detection of ASDs. Children with typical autism constituted 80% of the sample, while the Asperger group constituted 20%. The majority of pupils were served in general education, and only a small percentage attended special schools. The preferable type of support was one-to-one support in the classroom (47%). Among the most common disorders that coexist with the ASDs was ADHD, epilepsy, dyslexia and learning difficulties. Interesting findings were obtained for the advanced parental age, birth order and the relationship of assisted conception and premature birth with the presence of autism. The present study offers some useful insights about the characteristics of ASDs in a Greek school-based sample.     

Assessment of megakaryopoiesis in children with acute lymphoblastic leukemia

The effect of the underlying disease and chemotherapy on megakaryopoiesis has not been extensively studied in children with acute lymphoblastic leukemia (ALL) during and at the end of therapy. Using a serum-free assay, we assessed the megakaryocyte (Mk) colony formation in vitro from bone marrow mononuclear cells of 25 children with ALL during chemotherapy and shortly after the cessation of it. Twelve children with solid tumors without bone marrow involvement and cord blood from 10 full-term normal vaginal deliveries were used as controls. A significant reduction in the number of Mk colonies was observed at diagnosis of ALL, and Mk colony formation remained lower than controls throughout the different phases of leukemia treatment. Our study suggests that defects in megakaryopoiesis of children with ALL in long-term remission may persist during chemotherapy and at least shortly after the end of it.