Beginning readers' use of orthographic analogies in word reading

This research re-investigated the claim that beginning readers exploit information from the orthographic rime of clue words to help them to decode unfamiliar words. In Experiment 1, first-grade children were equally able to use orthographic information from the beginning, middle, and end of clue words to identify unfamiliar target words. Moreover, the improvement in reading end- (or orthographic rime-) same target words following clue word presentation reflected phonological priming. In second-grade children, with correction for retesting effects, improvement following clue word presentation for end-same and beginning-same target words was equivalent, although end-same target words improved more than middle-same target words. In Experiment 2, both first- and second-grade children were able to use orthographic information from the beginning, middle, and end of clue words to identify unfamiliar words. Clue word presentation enhanced the reading of beginning-same and end-same target words more than middle-same target words. Improvement was the same for beginning-same and end-same target words. Target word improvement following clue word presentation was greater than that for phonologically primed words only in children reading target words sharing the beginning sequence of the clue word. (C) 1998 Academic Press.

Clinical and molecular genetics of myoclonic-astatic epilepsy and severe myoclonic epilepsy in infancy (Dravet syndrome)

The majority of severe epileptic encephalopathies of early childhood are symptomatic where a clear etiology is apparent. There is a small subgroup, however, where no etiology is found on imaging and metabolic studies, and genetic factors are important. Myoclonic-astatic epilepsy (MAE) and severe myoclonic epilepsy in infancy (SMEI), also known as Dravet syndrome, are epileptic encephalopathies where multiple seizure types begin in the first few years of life associated with developmental slowing. Clinical and molecular genetic studies of the families of probands with MAE and SMEI suggest a genetic basis. MAE was originally identified as part of the genetic epilepsy syndrome generalized epilepsy with febrile seizures plus (GEFS(+)). Recent clinical genetic studies suggest that SMEI forms the most severe end of the spectrum of the GEFS(+). GEF(+) has now been associated with molecular defects in three sodium channel subunit genes and a GABA subunit gene. Molecular defects of these genes have been identified in patients with MAE and SMEI. Interestingly, the molecular defects in MAE have been found in the setting of large GEFS(+) pedigrees, whereas, more severe truncation mutations arising de novo have been identified in patients with SMEI. It is likely that future molecular studies will shed light on the interaction of a number of genes, possibly related to the same or different ion channels, which result in a severe phenotype such as MAE and SMEI. (C) 2001 Elsevier Science B.V. All rights reserved.

Prevalence and comorbidity of behavioral problems of children aged three to six: Results of the Braunschweiger Kindergartenstudie

Prevalence and comorbidity of behavioral problems of children aged three to six: Results of the Braunschweiger Kindergartenstudie Objectives: To analyze the frequency of behavioral and emotional problems and comorbidity of kindergarten children in Braunschweig as rated by their parents. Method: The analysis is part of the Braunschweiger Kindergartenstudie. In a sample of N = 809 children aged three to six the parents rated their children using a modified version of the Child Behavior Checklist/CBCL 4-18. Results: The prevalence rates range from 0.5% to 5.0%. The most frequent behavioral problems in kindergarten children were aggressive behavior and attention problems, followed by social problems. The study also provides bidirectional comorbidity rates. Conclusion: Finally the prevalence rates and the implications of the findings for prevention of behavioral problems in children are discussed.