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Biomedical subjects

C D Ferrie

Publications and source records attributed to C D Ferrie.

36 records · Page 2Linked to original sources
PubMed1999

Vagal nerve stimulation in epileptic encephalopathies.

OBJECTIVE: To study the effect of vagal nerve stimulation (VNS) in children with epileptic encephalopathies. METHODS AND MATERIALS: All children receiving VNS during a 2-year period at our center were studied prospectively for changes in seizure frequency, electroencephalogram (EEG), adaptive behavior, quality of life, and where appropriate, verbal/nonverbal performance. Assessments were made before and for at least 1 year after implant. RESULTS: Sixteen children were studied. One device was removed because of infection. Of the remaining 15 children, 4 had a >50% reduction and 2 had a >50% increase in seizure frequency at 1 year after implant. Median reduction in seizure frequency was 17%. There was no trend toward improvement of the EEG or adaptive behavior. Quality of life was unchanged in most areas, except in perceived treatment side effects and general behavior that were improved. In 6 children undergoing further assessment, there was a significant improvement in verbal performance; this did not correlate with reduction in seizure frequency. CONCLUSION: VNS did not significantly improve seizure frequency, severity, adaptive behavior, or the EEG during the first year of treatment for the group as a whole, although 4 children (27%) had a worthwhile reduction in seizure frequency. There were significant improvements in perceived treatment side effects and general behavior.

Adolescent↗
PubMed1998

Neuroimaging and spectroscopy in children with epileptic encephalopathies.

AIMS: To investigate the nature of the unifocal cortical abnormalities on FDG positron emission tomography (PET) in children with an epileptic encephalopathy but no focal abnormality on electroencephalogram or standard magnetic resonance imaging (MRI). METHODS: Repeat FDG PET, surface rendered high resolution MRI, and single voxel magnetic resonance proton spectroscopy of the areas of abnormal metabolism compared to the contralateral side in 11 children aged 2 to 12 years. Imaging was repeated after a median of 13 months. RESULTS: Visual analysis of repeat FDG PET revealed similar abnormalities in 10 of 11 children. Semiquantitative analysis revealed similar sited abnormalities in eight children. One child with ictal hypermetabolism initially had an inconsistent second scan. Magnetic resonance spectra in three children showed the N-acetyl-aspartate/choline ratio was lower in the hypometabolic focus than in the reciprocal area on the opposite side, in two children it was higher, and in one child it was equal. Surface rendered MRI was normal in seven of eight children, and showed temporal lobe asymmetry in one. CONCLUSION: In children with established epileptic encephalopathies most hypometabolic areas on FDG PET are stable over time. While focal neuronal loss is likely in these areas in some children, microdysplasias or other focal cortical dysplasias are probable in others.

Brain↗
PubMed1997

Adaptive and maladaptive behaviour in children with epileptic encephalopathies: correlation with cerebral glucose metabolism.

In the childhood epileptic encephalopathies mental impairment is common and severe. Traditional cognitive assessment is difficult because of the low level of performance, autistic features, and the unpredictable effect of seizures. An alternative is to measure adaptive and maladaptive behaviour using instruments administered to the caregivers. Adults with different types of dementia have characteristic patterns of cortical glucose hypometabolism. Thirty-two children were studied using visual and semiquantitative analysis of 18fluorodeoxyglucose positron emission tomographic (PET) scans. The Vineland Scales and the Conners' Questionnaires were used to assess adaptive and maladaptive behaviour. The mean adaptive behaviour composite score was 37.3+/-15.6; all but one subject had a low adaptive level. A profile of relative strength in socialisation and weakness in daily living skills emerged. Up to two-thirds of children had abnormal behaviour patterns, particularly attention-deficit disorders and hyperactivity. Adaptive and maladaptive behaviour was not related to the presence or absence of focal cortical PET abnormalities. However, adaptive behaviour scores showed an inverse correlation with the degree of metabolic abnormality in the frontal lobes.

Adaptation, Psychological↗
PubMed1997

Early-onset benign occipital seizure susceptibility syndrome.

PURPOSE: Childhood epilepsy with occipital paroxysms (CEOP) is characterised by ictal visual hallucinations and occipital epileptiform activity on interictal EEG. A variant has been described with nonvisual symptoms including tonic head and eye deviation, vomiting, and episodes of partial status epilepticus. We fully documented the electroclinical features of such patients to determine whether classification separate from CEOP is justified. METHODS: This was a multicentre study with participating investigators submitting details of patients with idiopathic occipital seizures characterised by ictal head or eye deviation and vomiting. RESULTS: One hundred thirteen patients were recruited. Seizures began in early childhood (mean, 4.6 years) and occurred infrequently (mean total seizures, 3); 30% of patients had only a single seizure. Two thirds of seizures were nocturnal. Ictal eye deviation occurred in 79%, vomiting in 70%, and head deviation in 35%. Seizures were predominantly complex partial in type. Partial status epilepticus occurred in 44% of patients. Seventy-four percent of patients had occipital interictal EEG epileptiform activity, predominantly right sided, with fixation-off sensitivity. Extraoccipital EEG abnormalities occurred in 35% of patients. Prognosis was excellent: the mean duration of active seizures was 1 year. CONCLUSIONS: Although the two groups shared identical EEG features, the distinct clinical symptoms probably justify separate classification. Early-onset benign occipital seizure syndrome (EBOSS) is suggested as an appropriate name for the variant group.

Adolescent↗
PubMed1997

Cortical and subcortical glucose metabolism in childhood epileptic encephalopathies.

OBJECTIVES: Nearly one third of children with cryptogenic epileptic encephalopathies have been reported to have focal cortical defects on 18fluorodeoxyglucose (FDG) PET. As diffuse cortical dysfunction and involvement of subcortical structures, particularly the thalami, is postulated to underlie the propensity to seizures in these conditions, the aim was to determine the frequency of bilateral and diffuse cortical metabolic defects and of subcortical metabolic abnormalities in the same patients. METHODS: The interictal uptake of FDG was studied in 32 children with epileptic encephalopathies. Using a semiquantitative technique, the ratio of uptake in cortical regions and subcortical structures to that in the cerebellum was compared with that of age matched historical controls. Uptake more than 2 SD above ("hypermetabolic") or below ("hypometabolic") that of age matched controls was considered abnormal. RESULTS: Diffusely abnormal cortical up-take (nearly always hypometabolic) occurred in almost two thirds of patients; in all but two of the remaining patients at least one cortical region showed significantly decreased uptake bilaterally. When analysed as age cohorts, the mean cortical:cerebellar FDG uptake was significantly lower than that of controls in all cortical regions (P<0.005). Ninety per cent of patients had evidence of relative thalamic hypometabolism and in each age group there was a significant reduction in relative thalamic FDG uptake compared with that of controls (P<0.005). In nine out of 11 patients with unilateral cortical hypometabolic defects thalamic FDG up-take was lower ipsilateral to the cortical abnormality. CONCLUSIONS: Diffuse cortical dysfunction is common in the epileptic encephalopathies and may reflect the underlying cause of the condition or arise as a consequence of uncontrolled seizures. Altered thalamic glucose metabolism is further evidence of subcortical involvement in these conditions.

Cerebellum↗
PubMed1997

Visual and semiquantitative analysis of cortical FDG-PET scans in childhood epileptic encephalopathies.

UNLABELLED: The optimal method for analyzing PET scans in children being considered for epilepsy surgery is unresolved: Fully quantified methods are invasive, and the required controls are generally unavailable. We sought to compare visual inspection with semiquantitative analysis for the detection of cortical metabolic defects. METHODS: Thirty-two children with cryptogenic epileptic encephalopathies were studied prospectively with 18F-fluorodeoxyglucose (FDG) PET. Visual inspection was performed on separate occasions by independent observers. Four-millimeter circular regions of interest were used to sample radiotracer uptake in selected cortical regions. Asymmetry between homologous regions were calculated to detect focal abnormalities. Bilateral and diffuse abnormalities were assessed by comparing the ratio of cortical-to-cerebellar uptake in patients with historical age-matched controls. The sensitivity and specificity of visual inspection was compared with that of semiquantitative analysis for the detection of focal, bilateral and diffuse cortical metabolic abnormalities. RESULTS: Visual inspection revealed full inter-rater agreement for the presence of major focal abnormalities. The sensitivity and specificity for visual inspection compared to semiquantitative analysis were 77% and 92%, respectively, with semiquantitative analysis often revealing abnormalities to be more extensive than had been suspected visually. Compared with semiquantitative analysis, visual inspection had a low sensitivity but high specificity for the detection of bilateral and diffuse hypometabolism. CONCLUSION: Semiquantitative analysis gives clinically useful information additional to that obtained from visual inspection.

Brain↗
PubMed1996

Psychotic and severe behavioural reactions with vigabatrin: a review.

Behavioural disturbances and psychotic reactions are commoner in patients with epilepsy than in the general population and may be precipitated by the majority of antiepileptic drugs, including the newer ones. These reactions may be more frequent in patients with complex partial seizures, reflecting underlying temporal lobe pathology. A review of the literature on vigabatrin found an incidence of severe abnormal behaviour in controlled trials in adults of 3.4%. In children open studies gave an incidence of around 6%. This may be related to dosage and speed of introduction. Such reactions may be related to changes in seizure control, either unaccustomed good control (force normalisation) or breakdown in control, implying non-specific causative mechanisms. Alternatively, any relationship to control may be fortuitous and specific, unknown pharmacological mechanisms may be involved. Appropriate risk reduction measures include slow introduction, limiting the dose to that required for seizure control, slow withdrawal and increased vigilance in those on polytherapy or with psychiatric histories. Such advice is pertinent to all antiepileptic medications. Additionally, vigabatrin is probably contraindicated in idiopathic generalised epilepsies. Behavioural reactions are uncommon with vigabatrin, and have not been shown to be greater with it than with other antiepileptic agents. Therefore, it maybe inappropriate to withhold the drug from those who may benefit from it.

Adult↗
PubMed1996

Focal abnormalities detected by 18FDG PET in epileptic encephalopathies.

A prospective study of 32 children with epileptic encephalopathies 12 years or younger revealed a high incidence of focal cortical metabolic defects on 18-fluorodeoxyglucose positron emission tomography (PET) not suspected from clinical, EEG, or magnetic resonance imaging findings. PET scans were normal in all five children with typical de novo Lennox-Gastaut syndrome but showed cortical metabolic abnormalities in three out of four with atypical de novo Lennox-Gastaut syndrome, five out of six with Lennox-Gastaut syndrome following infantile spasms, six out of eight with severe myoclonic epilepsy in infancy, one out of two with epilepsy with myoclonic-astatic seizures, and four out of six with an unclassified epileptic encephalopathy. This suggests that some children with epileptic encephalopathies previously thought to have primary generalised seizures or seizures due to multifocal pathology may have unifocal cortical origin for their seizures. Such an origin may be amenable to surgery.

Child↗
PubMed1995

Lamotrigine as an add-on drug in typical absence seizures.

INTRODUCTION: Lamotrigine is licensed in many countries for use in patients with partial seizures. Evidence suggests that it may also be effective in generalised epilepsies. MATERIAL & METHODS: We analysed retrospectively our patients with idiopathic generalised epilepsy with refractory absences. RESULTS: Fifteen patients with idiopathic generalised epilepsies were identified who had been treated with lamotrigine for 3 months or more. All patients were also treated with sodium valproate. Fourteen patients had active absences. Nine (64%) had a total or virtual cessation of absences and in a further patient they became milder and less frequent. One patient reported an increase in seizures. The effective dose of lamotrigine was 1.6-3.0 mg/kg/day in children and 25-50 mg/day in adults. Patients who responded did so after the first or second dose. Lamotrigine was well tolerated. CONCLUSION: Low-dose lamotrigine added to sodium valproate appears to be effective in typical absence seizures. A therapeutic interaction of the two drugs seems likely.

Adolescent↗
PubMed1995

Qualitative and quantitative abnormalities of breath counting during brief generalized 3 Hz spike and slow wave 'subclinical' discharges.

We aimed to validate the technique of breath counting during overbreathing in revealing ictal impairment of cognition during brief generalized 3 Hz spike and slow wave discharges. A retrospective study of 66 patients with video-electroencephalographic documentation of typical absence seizures revealed 8 patients in whom there was no clinical suspicion of absences but who had brief (less than 4 seconds) generalized 3 Hz spike-wave discharges. The only clinical manifestations of the absences were abnormalities in breath counting during overbreathing. These consisted of slowing of speech, delay in counting, repetition of numbers and counting out of sequence combined with delay. Abnormalities were more likely to occur during longer discharges. Detection of these abnormalities led to important changes in the classification of the patients' epilepsies and in the recommendations for treatment. The technique of breath counting during overbreathing is a simple practical and reliable method for detecting even mild cognitive impairment during 3 Hz spike-wave discharges.

Adult↗
PubMed1995

Posterior agyria-pachygyria with polymicrogyria: evidence for an inherited neuronal migration disorder.

We describe two brothers with mental retardation and refractory epilepsy. MRI revealed symmetrical agyria-pachygyria of the temporo-occipito-parietal regions, areas of deeply infolded polymicrogyric parietal cortex, and dilated occipital horns (colpocephaly). The stereotyped clinical, EEG, and MRI findings suggest that this may be a distinct inherited condition and imply that agyria-pachygyria with polymicrogyria is not always sporadic.

Brain↗
PubMed1994

Video game induced seizures.

Fifteen patients who experienced epileptic seizures while playing video games are described together with a review of 20 cases in the English literature. Nine of the 15 cases and all but two of the reported cases experienced their first seizure while playing video games. Two thirds of patients had idiopathic generalised epilepsy and mainly reported generalised tonic clonic seizures, but some had typical absence seizures and myoclonic jerks while playing video games. In this series, 30% with idiopathic generalised epilepsy had juvenile myoclonic epilepsy. Overall, 70% of patients with idiopathic generalised epilepsy were photosensitive to intermittent photic stimulation and the mechanism of seizure provocation was probably similar to that of television induced seizures, although sensitivity to specific patterns was sometimes important. Two children had self induced video game seizures. Non-photic factors such as excitement, fatigue, sleep deprivation, cognitive processing, and diurnal variation in susceptibility seemed to be important seizure precipitants, particularly in non-photo-sensitive patients. Twenty nine per cent of patients had partial (mainly occipital) video game associated seizures. Occipital spikes were common in the EEG of these patients. Photosensitivity to intermittent photic stimulation may have been important in two patients but in the others, who all played arcade video games, other mechanisms need to be considered. Video game associated seizures are a feature of several epileptic syndromes and differ in precipitants and appropriate management.

Adolescent↗
PubMed1987

Seasonal variation of glycosylated haemoglobin.

Retrospective analysis of glycosylated haemoglobin concentrations in a diabetic outpatient clinic over three and a half years showed a small seasonal variation in the mean value with a peak in mid February and a nadir in mid August.

Adolescent↗