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Biomedical subjects

S J Wallace

Publications and source records attributed to S J Wallace.

At least 19 recordsLinked to original sources

Alexander's disease in infancy and childhood: a report of two cases.

Two cases of Alexander's disease are described. One case of infantile onset died at the age of 6 months and the second case was of the juvenile type with onset at 2 years and death at 10 years. A clinical diagnosis of this disease is difficult since signs can vary according to the age of the patient. The severity of the pathological changes can also depend upon the age of onset of this disease, but they are restricted to the central nervous system. The Rosenthal fibre is the characteristic feature of Alexander's disease and we have examined for the first time its ultrastructure and immunocytochemical characteristics at the electron microscopical level and demonstrated coexpression of anti-glial fibrillary acidic protein and anti-ubiquitin antisera.

Astrocytes

Herpes simplex virus encephalitis: problems in diagnosis.

Six children aged 13 days to nine years with herpes simplex encephalitis (HSE) are presented. Institution of appropriate antiviral treatment was later than six days in three cases; original diagnosis in these cases were post-traumatic epilepsy, bacterial meningitis and febrile convulsion. Initially pyrexia was absent in two cases and cranial CT was normal in two cases. Encephalitic changes were observed on the EEGs of five children. Diagnosis was confirmed by paired serological titres, brain biopsy, vesicle culture and CSF titres. The outcome for all six children was poor. HSE should always be considered in children presenting with focal seizures, even when apyrexial and with normal CT findings. In such situations, saving CSF for antibody titres or antigen identification should be routine practice. Treatment with acyclovir is justified before precise virological diagnosis has been established.

Adolescent

Kawasaki disease.

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Brain Diseases

Management of epilepsy in childhood.

Epilepsy is the commonest neurological disorder of children. Between 0.5 and 1% of the childhood population is affected either temporarily or chronically. In the individual it is important to be certain that the diagnosis is correct and thereafter to classify the seizure type(s) and, if possible, the epileptic syndrome. This will facilitate optimal therapeutic intervention and rational management of any associated problems.

Anticonvulsants

A survey of adolescents with epilepsy.

Thirty-four adolescents with epilepsy, controls matched for age and sex (A) and controls matched for age, sex and general ability (B), were studied. The adolescents with epilepsy were more likely to arrive at school by car or taxi and to have more difficult behaviour in class. Competitive sports were less popular with them and significantly fewer anticipated ever driving a car. Illness and parental marital problems were not a feature of their families. Their comprehension of reading material was significantly poorer than that of control group A. Within the group, the lowest over-all reading scores were found in children with myoclonic seizures, partial seizures with secondary generalisation, or generalised tonic-clonic seizures; and in those whose EEG findings included two-per-second spike and wave, photosensitivity, generalised slow waves, or generalised spike and wave of non-specific frequency. Right focal slow waves, sharp waves and spikes on EEG were associated with problems of comprehension, even when the over-all reading score was acceptable.

Adaptation, Psychological

EEG monitoring of therapy for neonatal seizures.

Eleven neonates with seizures had continuous EEGs recorded before and up to several hours after administration of anticonvulsant therapy. Six of seven babies given phenobarbitone responded clinically, but in four of these the EEG showed that seizure discharges either persisted or recurred within two hours. Chloral hydrate had no immediate effect in one case and only a doubtful influence on EEG seizure activity in another. Three babies were given benzodiazepines: after an apparently rapidly effective bolus dose of diazepam, recurrence of seizure discharges was almost immediate. Infusions of diazepam or clonazepam eventually were associated with persistent control of clinical and EEG seizures. In the treatment of neonatal seizures, conventional anticonvulsants are rarely completely effective: EEG monitoring is essential for optimal therapeutic intervention.

Anticonvulsants

Rapid anti-epileptic drug assay.

The usefulness of rapid anti-epileptic drug assay in a paediatric neurology clinic was assessed. Compared with a method in which results were not available for seven to 10 days, there was an increased likelihood that levels would be within the target range, particularly for carbamazepine. Increased parental discussion, and therefore involvement with dosage, was possible. Management of children receiving phenytoin was more efficient and non-compliance could be dealt with immediately. When anti-epileptic drug levels are measured, rapid assay, with results available while the patient is still in the clinic, should be a standard facility.

Anticonvulsants

Risk of seizures.

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Adolescent

Effects of puberty on seizure frequency.

Seizure frequency was documented before, during and in some cases after puberty for 12 patients with continuing generalised tonic-clonic seizures (GTCS) and for 14 with complex partial seizures (CPS) who were receiving anti-epileptic drugs. For the patients with GTCS there was a significant increase in seizure frequency during the pubertal growth-spurt, with a subsequent decrease after growth ceased. There appeared to be no relationship between puberty and the frequency of CPS. Both groups were more likely to have suboptimal plasma drug-levels during puberty, suggesting that medication was not the cause of the increased GTCS frequency. Further examination of hormonal levels in relation to frequency of GTCS during puberty could provide a better understanding of the influence of hormones.

Adolescent

Early diagnosis and secondary prevention of Duchenne muscular dystrophy.

A total of 33 young boys (mean age 3.4 years) with Duchenne muscular dystrophy and 21 normal controls (mean age 3.5 years) were assessed using the Griffiths's mental development scales and the Reynell language scales. The boys with Duchenne muscular dystrophy were significantly developmentally delayed when compared with the control group. The developmental delay was most pronounced in locomotor function and language. There was no significant difference in social class distribution. Early diagnosis of Duchenne muscular dystrophy is of vital importance if secondary cases within families are to be prevented. While diagnosis is still unacceptably late in most cases, it can be improved if all boys with this pattern of developmental delay are screened for Duchenne muscular dystrophy by measurement of creatine kinase activity.

Child

Juvenile myoclonic epilepsy.

The clinical and electroencephalographic features of 10 adolescents with juvenile myoclonic epilepsy are presented. The mean age on onset was 12.3 years. Myoclonic jerks, predominantly on awakening, occurred in all 10 and were associated with infrequent generalised tonic-clonic seizures in nine. Five had first degree relatives with seizures. The neurodevelopmental status was normal in eight and social integration good in seven. Waking interictal electroencephalograms showed normal background activity in nine, polyspike and wave in six, and single spike and wave in eight. Four were photosensitive. Failure to respond to other antiepileptic drugs was usual, but valproate monotherapy resulted in good or complete seizure control. Juvenile myoclonic epilepsy is a well defined clinical entity that responds well to valproate and is usually associated with a good outlook.

Adolescent