Epileptic disorders with onset in the first year of life: neurological and cognitive outcome.

We examined prospectively a series of 150 children with epilepsy beginning in the first year of life. We classified the types of epilepsy into five categories: West syndrome, other epileptic encephalopathies, generalized, partial and undetermined epilepsies. Of 150 patients, 15 died; 135 were followed for at least 4 years. In order to define possible factors influencing prognosis we evaluated neurological and cognitive outcome and made percentage comparisons between groups, for aetiology, age of onset, family history of epilepsy, and psychomotor development before onset. Epileptic encephalopathies as well as the symptomatic forms of West syndrome showed a very poor neurological and cognitive outcome. As previously recognized, only cryptogenic forms of West syndrome had a benign prognosis. For the generalized epilepsies, analysis of different factors, namely late age at onset, cryptogenicity and absence of primary cognitive impairment, indicated a good prognosis. In contrast, partial epilepsies usually had a poor outcome, irrespective of the aetiology, whether cryptogenic or symptomatic.

Motor and perceptual-motor competence in children with Down syndrome: variation in performance with age.

The aim of this study was to determine whether perceptual-motor competence in school-age children with Down syndrome was generally delayed or varied as a function of type of action. Twenty-two children with Down syndrome (13 males, 9 females), aged between 4.5 and 14 years were assessed on two standardized tests, the Movement Assessment Battery for Children (Movement ABC), a test of motor competence assessing gross and fine motor coordination, and on the Developmental Test of Visual-Motor Integration, a test focusing on shape copying. In order to obtain a profile of each child's performance on the different items contained in the Movement ABC, the test was used in its extended form. This involves testing any child who failed an item appropriate for his/her level, at progressively lower levels until a base-line measure was obtained. All the children obtained scores below the 5th percentile for their age on both tests. However, superimposed on this delay, we found distinct variation as a function of task. Whereas some aspects of gross motor function showed age development with delayed but regular acquisitions, all the aspects of fine motor skills assessed were more severely impaired and showed little development with age. Accuracy and timing of tasks requiring bimanual coordination were most impaired in our sample while balance and ball skills showed more variability. These results suggest that intervention in the motor domain should be varied according to each child's particular profile of performance.