Surveillance for rare disorders by the BPSU. The British Paediatric Surveillance Unit.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to C Verity.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The assumption is often made that brain damage during the perinatal period is likely to result in neurological abnormalities such as epilepsy and cerebral palsy. However, there has been accumulating evidence that cerebral palsy is rarely, if ever, a result of intrapartum events, but few studies of other neurological abnormalities have been undertaken. We analysed data on 16,163 children from the 1970 British national cohort study and followed to age 10, focusing on the 378 who developed febrile convulsions (FCs) and 63 children with idiopathic afebrile seizures (IAS). Children with IAS were significantly more likely not to have been breast fed (P < 0.001), and this was independent of features such as birthweight and maternal disorder. A similar finding was apparent for FCs (P < 0.05). Although children with low birthweight were at increased risk of both conditions, there was no association with maternal smoking in pregnancy. No associations were found between indications of fetal distress during labour and later febrile convulsions or afebrile seizures. There was no evidence that intervention during labour would have improved these outcomes. However, associations were found with abnormalities earlier in pregnancy, suggesting a prenatal rather than an intrapartum aetiology.
PURPOSE: The aim of this study was to investigate and describe the range of white matter abnormalities in children with tuberous sclerosis complex by means of MR imaging. MATERIAL AND METHODS: A retrospective cross-sectional study was performed on the basis of MR imaging findings in 20 cases of tuberous sclerosis complex in children aged 17 years or younger. RESULTS: White matter abnormalities were present in 19/20 (95%) cases of tuberous sclerosis complex. These were most frequently (19/20 cases) found in relation to cortical tubers in the supratentorial compartment. White matter abnormalities related to tubers were found in the cerebellum in 3/20 (15%) cases. White matter abnormalities described as radial migration lines were found in relation to 5 tubers in 3 (15%) children. In 4/20 (20%) cases, white matter abnormalities were found that were not related to cortical tubers. These areas had the appearance of white matter cysts in 3 cases and infarction in the fourth. In the latter case there was a definable event in the clinical history, supporting the diagnosis of stroke. CONCLUSION: A range of white matter abnormalities were found by MR imaging in tuberous sclerosis complex, the commonest being gliosis and hypomyelination related to cortical tubers. Radial migration lines were seen infrequently in relation to cortical tubers and these are thought to represent heterotopic glia and neurons along the expected path of cortical migration.
The use of valproate has traditionally been held to be associated with a greater incidence of weight gain than that of other anticonvulsants. This paper presents an analysis of body weight data gathered during a randomized trial comparing valproate with carbamazepine in 260 children aged 4-15 years with newly-diagnosed epilepsy. There were more reports of weight gain as an adverse event in the valproate group than in the carbamazepine group (22 reports in 14 patients vs. nine reports in five patients). However, amongst the 211 patients (103 on valproate and 108 on carbamazepine) in whom objective weight measurements were taken during treatment, there were no differences between the treatments in percentage weight gain from baseline or incidence of excessive weight velocity. Eight patients reporting weight gain on valproate were switched to carbamazepine because of poor seizure control and/or adverse events including weight gain, but three of the four patients for whom body weight measurements were available continued to gain weight on carbamazepine. It is concluded that weight gain may be erroneously attributed to valproate treatment.
Four patients with infantile hemiplegia who had undergone hemispherectomy were examined. both left (LH) and right (RH) hemidecorticates obtained borderline defective scores on IQ tests. Patients with isolated right hemispheres were found not to be aphasic or apraxic. Visuospatial and constructional capacities were compromised in all patients. Individuals with an isolated right hemisphere smiled and gestured more than did those with an isolated right hemisphere. EEG and CT scan data showed that the remaining hemisphere was not normal in these patients. Therefore, inferences regarding the functional plasticity of the remaining hemisphere must be made with caution.