The proposed scale seems to be a reliable and valid instrument for the assessment of pediatric and adult patients with pantothenate kinase-associated neurodegeneration. Additional validation studies with a larger sample size will be required to confirm the present results and to complete the scale validation testing. © 2017 International Parkinson and Movement Disorder Society.
Genetic generalised epilepsy or epilepsy of unknown cause can remit before adolescence. In many children, the disease does not interfere with their academic achievement. Although there are neuropsychological studies characterising the cognitive profile, there are no studies in this population focused on spatial orientation abilities. In this study, we compared children with genetic generalised epilepsy or epilepsy of unknown cause with a control group using a virtual spatial learning task. Children with epilepsy showed worse performance on the spatial orientation task, although their visuo‐spatial memory, attention, and working memory were normal. These results confirm that genetic generalised epilepsy or epilepsy of unknown cause is associated with more cognitive deficits. Virtual reality technologies can complement clinical assessment.
Summary:Purpose: To study the risk of recurrence after a first unprovoked seizure in childhood.Methods: All consecutive patients aged less than 14 years with one or more unprovoked seizures who were attended between January 1, 1987, and June 1, 1996, were included in a prospective study. Clinical features of patients attended after a first seizure and those attended after two or more seizures were compared. Recurrence risk in both groups was estimated by Kaplan-Meier curves. Univariate and multivariate analyses of the potential predictors of recurrence risk were performed for the group of patients attended after a first seizure using the Cox proportional hazards model.Results: Included in the study were 217 children. KaplanMeier estimate of recurrence risk was 64% at 5 years, when only patients being attended after a first epileptic seizure were included, compared with 74% when all patients were included. Significant differences in several clinical features were found between patients attended after a first seizure and those attended after two or more seizures. Univariate and multivariate analyses showed that in the overall cohort of patients attended after a first seizure, a symptomatic etiology increased the risk of recurrence, whereas a patient age of 3 to 10 years decreased this risk. In particular, the recurrence risk was 96% at 2 years for symptomatic seizures, compared with 46% for idiopathic/ cryptogenic seizures. In the group of patients with idiopathic/ cryptogenic seizures, an abnormal electroencephalogram and the occurrence of seizures during sleep increased the recurrence risk, whereas a patient aged 3 to 10 years reduced it. In the group of patients with symptomatic etiology, univariate analysis revealed that there was a lower recurrence risk for patients aged 3 to 10 years. This last finding was not maintained, however, in multivariate analysis.Conclusions: The recurrence risk depends on the inclusion criteria for enrolling patients. Several factors enable us to predict the recurrence risk after a first unprovoked seizure; the most important of these factors is the etiology of the seizures.
Background: Prematurity and its consequences are serious problems that can result in numerous neurosensory disabilities and cerebral cognitive dysfunctions. The Perinatal Risk Index (PERI) might provide a predictive measure of these problems. Aim:This study compared the cognitive development of prematurely born children at 4 and 5 years of age with age-matched peers born at term. The secondary objective was to determine whether a correlation exists between perinatal risk and performance on neuropsychological tests among premature children.Methods: A total of 54 children between four and five years of age were evaluated; 27 were born very premature (premature group; PG), and 27 were born at term (term group; TG). Executive function, attention, memory, language, visual perception, and spatial structuring were evaluated. Subtests from the Kaufman Assessment Battery for Children, the Rey Complex Figure Test, the McCarthy Scales of Children's Abilities, the Peabody Picture Vocabulary Test, Test A, Trails A and B, the spatial structuring questionnaire from the Child Neuropsychological Maturity Questionnaire, and the Wechsler Intelligence Scale for Children were used. A PERI score was also obtained for the PG. Results:The PG showed significantly lower scores than the TG in all the studied cognitive domains. Visualperceptive scores were significantly and negatively correlated with the PERI scores of the PG. Conclusions:The PG showed neurocognitive deficits compared with the TG. The PERI can be used to predict the development of visual-perceptive abilities in children between four and five years of age.
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