Cognitive procedural learning among children and adolescents with or without spastic cerebral palsy: the differential effect of age.

INTRODUCTION: Children learn to engage their surroundings skillfully, adopting implicit knowledge of complex regularities and associations. Probabilistic classification learning (PCL) is a type of cognitive procedural learning in which different cues are probabilistically associated with specific outcomes. Little is known about the effects of developmental disorders on cognitive skill acquisition. METHODS: Twenty-four children and adolescents with cerebral palsy (CP) were compared to 24 typically developing (TD) youth in their ability to learn probabilistic associations. Performance was examined in relation to general cognitive abilities, level of motor impairment and age. RESULTS: Improvement in PCL was observed for all participants, with no relation to IQ. An age effect was found only among TD children. CONCLUSIONS: Learning curves of children with CP on a cognitive procedural learning task differ from those of TD peers and do not appear to be age sensitive.

Epilepsy in children with infantile thiamine deficiency.

OBJECTIVE: To report the follow-up findings of 7 children with severe epilepsy as a result of thiamine deficiency in infancy caused by a defective soy-based formula. METHODS: The medical records of 7 children aged 5-6 years with thiamine deficiency in infancy who developed epilepsy were reviewed and their clinical data, EEG tracings, and neuroimaging results were recorded. The clinical course and present outcome of these children, now 5 years after exposure to thiamine deficiency, are described. RESULTS: All infants displayed seizures upon presentation, either tonic, myoclonic, or focal. Six infants had an EEG recording at this stage and all showed slow background. Five of them had no epileptic activity and only 1 displayed focal activity. Following a seizure-free period of 1-9 months, the seizures recurred, and all 7 children displayed either myoclonic or complex partial seizures. Multifocal or generalized spike wave complexes were recorded on the EEGs of all 7 patients, and the tracings of 3 children evolved into hypsarrhythmia. The seizures were refractory to most antiepileptic drugs, and 4 children remain with uncontrolled seizures. All children have mental retardation and motor disabilities as well as symptoms of brainstem dysfunction. CONCLUSIONS: Our findings indicate that severe infantile thiamine deficiency may result in epilepsy.

Spinal cord ultrasonography in children with myelomeningocele.

Secondary tethering of the spinal cord in people with myelomeningocele (MMC) is almost universal but there is a relatively low incidence of the secondary tethered cord syndrome (S-TCS). In view of this, we wish to explore the notion that cord pulsation, as demonstrated by ultrasound of the spinal cord, can become an independent measure for increased tension of the spinal cord in patients with MMC. One-hundred and six patients with MMC underwent ultrasonographic examination of their back, a thorough physical examination, and a careful review of their clinical history. Of these patients, 53 had MRI of their back. Cord pulsation was graded and the statistical relation between pulsation and various clinical and MRI findings was analysed. Age and arm span were not correlated with grades of cord pulsation. The absence of cord pulsation correlated with the presence of symptoms but this relation may be explained by MRI findings of certain structural cord malformations and not by tethering per se. Assuming that cord pulsation is a reliable marker for the tension within the spinal cord, the results of our study suggest that overstretching of the cord is not the main cause for the development of the S-TCS.

Profound developmental dyscalculia: evidence for a cardinal/ordinal skills acquisition device.

This article presents an analysis of a form of dysfunctional mathematical development. A child of normal intelligence was unable to acquire basic cardinal numerical skills, despite relatively intact ordinal number use. These findings provide evidence for an innate "cardinal/ordinal skills acquisition device" (COSAD). It is argued that if this COSAD is lacking, ordinal number use may be compensated for by linguistic logic and visual skills. Cardinal number skills, however, remain limited as these demand an innate internal representation of quantity, which cannot be compensated for. The findings are discussed in terms of different approaches to number development.

Possible heterogeneity in spondyloenchondrodysplasia: quadriparesis, basal ganglia calcifications, and chondrocyte inclusions.

We report on 6 patients with short stature and progressive enchondromatous-like changes of the vertebral bodies and the metaphyses of the long bones. Parental consanguinity was observed in 5 of 6 cases, supporting autosomal recessive inheritance. In spite of the similarity in radiographic changes and body proportions, genetic heterogeneity is suggested by the presence of CNS calcifications in 3 patients. Two of the latter had progressive quadriparesis. We tentatively classified these patients into 2 provisional types. An iliac crest biopsy in one of the patients with "type I" disease did not demonstrate enchondromatosis. Light and transmission electron microscopic studies demonstrated large cisterns and small inclusion bodies containing a flocculent material within the rough endoplasmic reticulum of the chondrocytes. Based on the histological and radiographic findings, we propose to classify these conditions among the spondylometaphyseal skeletal dysplasias.