The Castang Foundation, Bath Unit for Research in Paediatrics, National Institute of Health Research, the Royal United Hospitals Bath NHS Foundation Trust, BRONNER-BENDER Stiftung/Gernsbach, University Children's Hospital Zurich.
Aim-To determine the outcome and identify predictive factors in children with psychogenic non-epileptic seizures (PNES). Method-The biographies of 35 children with PNES, attending a tertiary paediatric neurology centre, were reviewed. Results-Thirty five children attending the department between 1987 and 1997 were evaluated at a mean follow up of 4.6 years. The age range was 6-18 years. Twenty four were girls and 11 were boys. Eleven patients had a diagnosis of epilepsy with PNES, the remainder having PNES alone. Cause fell into four categories: a history of violence, abuse, or neglect; a high level of anxiety; dysfunctional family relationships; and attention seeking or avoidance behaviour. Management in all but five cases involved assessment and follow up by a child psychologist or child psychiatrist. The outcome was encouraging, with 66% of patients becoming PNES free. A further 23% have > 50% reduction in the frequency of PNES. Only two have had no reduction. Outcome was best in the group without epilepsy. Conclusion-These results suggest that the prognosis of PNES is better in children than in adults, perhaps because causes are more likely to be external to the child, more easily identified, and more amenable to prompt intervention. The importance of good assessment, good communication, and a treatment plan that includes both symptom management and addressing the precipitating and perpetuating factors is emphasised. (Arch Dis Child 2000;82:474-478)
We have considered multiple subpial transection (MST) as a treatment option for Landau-Kleffner syndrome (LKS) for the past 6 years. The effect of this technique on language and cognitive ability, behaviour, seizures, and EEG abnormalities is analysed here. Five children (4 males, 1 female; aged 5.5 to 10 years) underwent MST with sufficiently detailed pre- and postoperative data for analysis. Behaviour and seizure frequency improved dramatically after surgery in all children. Improvement in language also occurred in all children, although none improved to an age-appropriate level. All five had electrical status epilepticus in sleep (ESES) before surgery, which was eliminated by the procedure. One child has had an extension of his MST due to the recurrence of ESES and accompanying clinical deterioration with good effect. An attempt is made to set the effect of MST against the natural history of the condition. MST is an important treatment modality in LKS, although the timing of this intervention and its effect on final language outcome remains to be defined.
We have considered multiple subpial transection (MST) as a treatment option for Landau‐Kleffner syndrome (LKS) for the past 6 years. The effect of this technique on language and cognitive ability, behaviour, seizures, and EEG abnormalities is analysed here. Five children (4 males, 1 female; aged 5.5 to 10 years) underwent MST with sufficiently detailed pre‐ and postoperative data for analysis. Behaviour and seizure frequency improved dramatically after surgery in all children. Improvement in language also occurred in all children, although none improved to an age‐appropriate level. All five had electrical status epilepticus in sleep (ESES) before surgery, which was eliminated by the procedure. One child has had an extension of his MST due to the recurrence of ESES and accompanying clinical deterioration with good effect. An attempt is made to set the effect of MST against the natural history of the condition. MST is an important treatment modality in LKS, although the timing of this intervention and its effect on final language outcome remains to be defined.
Abnormal EEG features are a hallmark of epilepsy, and abnormal frequency and network features are apparent in EEGs from people with idiopathic generalised epilepsy in both ictal and interictal states. Here, we characterise differences in the resting-state EEG of individuals with juvenile myoclonic epilepsy and assess factors influencing the heterogeneity of EEG features. We collected EEG data from 147 participants with juvenile myoclonic epilepsy through the Biology of Juvenile Myoclonic Epilepsy study. 95 control EEGs were acquired from two independent studies (Chowdhury et al. (2014) and EU-AIMS Longitudinal European Autism Project). We extracted frequency and functional network-based features from 10-20 s epochs of resting-state EEG, including relative power spectral density, peak alpha frequency, network topology measures and brain network ictogenicity: a computational measure of the propensity of networks to generate seizure dynamics. We tested for differences between epilepsy and control EEGs using univariate, multivariable and receiver operating curve analysis. Additionally, we explored the heterogeneity of EEG features within and between cohorts by testing for associations with potentially influential factors such as age, sex, epoch length and time, as well as testing for associations with clinical phenotypes including anti-seizure medication, and seizure characteristics in the epilepsy cohort. P-values were corrected for multiple comparisons. Univariate analysis showed significant differences in power spectral density in delta (2-5 Hz) (p = 0.0007, hedges’ g = 0.55) and low-alpha (6-9 Hz) (p = 2.9 × 10−8, g = 0.80) frequency bands, peak alpha frequency (p = 0.000007, g = 0.66), functional network mean degree (p = 0.0006, g = 0.48) and brain network ictogenicity (p = 0.00006, g = 0.56) between epilepsy and controls. Since age (p = 0.009) and epoch length (p = 1.7 × 10−8) differed between the two groups and were potential confounders, we controlled for these covariates in multivariable analysis where disparities in EEG features between epilepsy and controls remained. Receiver operating curve analysis showed low-alpha power spectral density was optimal at distinguishing epilepsy from controls, with an area under the curve of 0.72. Lower average normalized clustering coefficient and shorter average normalized path length were associated with poorer seizure control in epilepsy patients. To conclude, individuals with juvenile myoclonic epilepsy have increased power of neural oscillatory activity at low-alpha frequencies, and increased brain network ictogenicity compared to controls, supporting evidence from studies in other epilepsies with considerable external validity. In addition, the impact of confounders on different frequency-based and network-based EEG features observed in this study highlights the need for careful consideration and control of these factors in future EEG research in idiopathic generalised epilepsy particularly for their use as biomarkers.
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