Objective: Focal cortical dysplasia (FCD) Type 1 and its three subtypes have yet not been fully characterized at the clinical, anatomopathological, and molecular level (International League Against Epilepsy [ILAE] FCD classification from 2011). We aimed to describe the clinical phenotype of patients with histopathologically confirmed FCD1A obtained from a single epilepsy center between 2002 and 2016. Methods: Medical records were retrieved from the hospital's archive. Results from electroencephalography (EEG) video recordings, neuroimaging, and histopathology were reevaluated. Magnetic resonance imaging (MRI) post-processing was retrospectively performed in nine patients. DNA methylation studies were carried out from archival surgical brain tissue in 11 patients.Results: Nineteen children with a histopathological diagnosis of FCD1A were included. The average onset of epilepsy was 0.9 years (range 0.2-10 years). All children had severe cognitive impairment and one third had mild motor deficits, yet fine finger movements were preserved in all patients. All patients had daily seizures, being drug resistant from disease onset. Interictal electroencephalography revealed bilateral multi-regional epileptiform discharges. Interictal status epilepticus was observed in 8 and countless subclinical seizures in 11 patients.Regional continuous irregular slow waves were of higher lateralizing and localizing yield than spikes. Posterior background rhythms were normal in 16 of 19 children. Neuroimaging showed unilateral multilobar hypoplasia and increased T2-FLAIR signals of the white matter in 18 of 19 patients. All children underwent tailored multilobar resections, with seizure freedom achieved in 47% (Engel class I). There was no case with frontal involvement without involvement of the posterior quadrant by MRI and histopathology. DNA methylation profiling distinguished FCD1A samples from all other epilepsy specimens and controls.
Age does not only lead to a decline in recognition of facial expressions of emotions but age also affects the quality of observational pain recognition in others. However, given that older observers' performance was only slightly reduced, clearly suggests that older caregivers are surely not at risk of becoming visual agnostic for the pain in others.
This study describes the intellectual development of 75 children and adolescents who underwent hemispherotomy. Furthermore, we aimed to reveal predicting factors on pre-and postsurgical development with a focus on the role of aetiology. Methods. We analysed presurgical and six-month postsurgical developmental and intellectual data of 75 patients (age range: 0.87-19.78 years) and divided them into two groups: a not severely impaired group in which outcome of intellectual functioning was reported based on FSIQ score, and a severely impaired group (not testable by IQ tests) in which intellectual developmental outcome was described based on developmental quotients instead. Results. In the not severely impaired group (n = 31), the preoperative level of intellectual functioning was a strong predictor of postoperative intellectual outcome; for 22/31 (71%) patients, postoperative FSIQ and its subscales were similar to preoperative levels. Improvements were observed for FSIQ in five patients, only for Verbal IQ in one patient and only for Performance IQ in one further patient; significant losses occurred in two patients, only for Performance IQ in both. In the severely impaired group, 30/40 (75%) patients showed further development after surgery, nine (23%) patients had the same results as before surgery, and one (2%) patient showed regression. Longer duration of presurgical epilepsy was related to a marginally lower presurgical developmental level, and good seizure outcome was a predictor Some of the results were presented on the Annual Meeting of the Gesellschaft für Epilep
Influences of essential fatty acids (EFA) and triglycerides as reasons for the progression of chronic renal failure are still in debate. We studied the outcome of four diets containing different concentrations of triglycerides and EFA in 5/6 nephrectomized rats and in NZB/W mice up to 45 weeks. The results showed no significant differences in the outcome of survival rate, proteinuria, urea, and creatinine levels as well as histological findings in the different groups of both animal models. We conclude that diets containing EFA up to C18:2 or being free of EFA or that triglyceride-enriched diets have no influence on the natural course of the renal disease in these both experimental models.
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