Seizures in patients with cerebral palsy.
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Biomedical subjects
Publications and source records attributed to G Hosking.
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This study analyses the prevalence and nature of epilepsy in all children between the ages of 5 and 16 years of age in the City of Sheffield with mild, moderate or severe intellectual impairments. An overall figure of 18% was obtained with a range of 7% for those with mild to moderate intellectual impairments to 67% for those with severe intellectual impairments and a physical disability. The severity of the epilepsy similarly varied with the most severe being in those with severe intellectual impairments and physical disabilities.
Two hundred and twenty Down's sufferers of all ages in the Sheffield district had their cervical spine X-rayed as part of a screening programme to ascertain atlanto-axial instability. Fifteen patients (6.8%) had an abnormal predental distance with four (1.8%) showing atlanto-axial subluxation. Seventy per cent of abnormal findings were in the under-25 age group. Only one child had clinical manifestations. Patients under the age of 25 years are most likely to participate in sports and are thought to be the age group most at risk. Degenerative changes in the cervical spine were found at an early age in patients with Down's syndrome, and there is both an increased incidence and severity of degenerative change at higher levels with increasing age.
Cerebral systemic lupus erythematosus is uncommon in childhood. Three further cases are presented to draw attention to the unusual and varied clinical manifestations of this condition.
A retrospective study of children attending a government hospital in Bangalore was performed to assess the causes of delay in providing appropriate treatment. Delay had occurred in 59% of children with significant illness, and in over half the cases the primary cause of delay was inappropriate treatment or delayed referral by a doctor trained in Western-style medicine. It is concluded that there are a large number of ill children in Bangalore whose parents are seeking the help of such doctors but where management is at fault.
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A biochemical study has been undertaken on the mothers of children with a major neurodevelopmental disability, without known etiology (study group), and an appropriate control group. Two hundred seventy-four mothers were studied, 137 in each group. Social and environmental details were recorded in each of the two major groups. Biochemical studies included the assay of plasma and urinary amino acids and appropriate screening tests for carbohydrate and organic acid abnormalities. To introduce an element of standardization and also to enhance the opportunities for identifying possible biochemical heterozygote states, a 50-g protein mean was given to all subjects approximately three-quarters of an hour before blood and urine samples were taken. Difference of means tests on 22 amino acids revealed 9 significant t values at the 0.05 level and all means were significantly lower in the study groups. Because of the differences noted a discriminant analysis was carried out which demonstrated an extremely high proportion of correctly classified subjects (98%) when biochemical parameters were used to attempt to classify subjects into the study or control group.
We describe three children with type V glycogen storage disease, who were reluctant to climb hills. We suggest that this condition, usually described as being of adult onset, can often be diagnosed in childhood.
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Parents from 53 families of boys with Duchenne muscular dystrophy were interviewed to explore their reactions to the diagnosis and the effects of the disease on family life. The findings are discussed in terms of the problems reported by the parents, their experiences at the time of diagnosis, their views on neonatal screening, the effects on the marital relationship, on siblings and on the affected boys themselves, and the parents' need for better information about the disorder.
An eight year old boy with multiple simple congenital ring constrictions is described with peripheral nerve palsies caused by entrapment in the constrictions. It is suggested that ring constrictions should be considered in the differential diagnosis of entrapment neuropathy.
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A 10-month-old boy presented with dermatitis and alopecia and became severely hypotonic. Screening for urinary organic acids revealed a large quantity of 3-hydroxyisovaleric acid and raised levels of beta-methylcrotonylglycine and 3-hydroxypropionate. Activities of propionyl CoA carboxylase, beta-methylcrotonyl CoA carboxylase, and pyruvate carboxylase in cultured fibroblasts were normal. Treatment with oral biotin resulted in a dramatic clinical improvement, which might therefore suggest a defect in biotin absorption or transport.