Status epilepticus on the paediatric intensive care unit—the role of EEG monitoring

AbstractA pilot study was undertaken of the feasibility of continuous EEG monitoring of patients admitted to a Paediatric Intensive Care Unit (PICU) for management of status epilepticus or its immediate sequelae. Eight children were studied and seizure activity was recorded in four patients. Additional information influencing management was obtained: the bedside nurse considered decerebrate posturing in one patient to be a seizure: there were no epileptiform EEG changes. Another patient was considered to have seizures (clonic movements of both upper limbs) following cardiac arrest; the EEG showed electrocerebral silence, and thiopentone treatment was discontinued. In another patient, continuing epileptiform activity on EEG gave intensivists the confidence to use higher than usual doses of thiopentone. The problems encountered were delays in monitoring, once for a CT scan and once because of two admissions within hours of each other. We conclude that EEG monitoring on a PICU is feasible and provides clinically useful information

Investigating ataxia in childhood

© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ. Ataxia is a common presentation to an acute paediatric unit and it can often be difficult to determine the cause. It is important to distinguish between serious causes, for example, brain tumours and encephalitis, and more benign causes in order to guide investigations and treatment. In this review, we describe the different types of ataxia, the causes associated with them, the examination findings and what investigations to perform in order to make a diagnosis

Seizure characteristics and the use of anti-epileptic drugs in children and young people with brain tumours and epileptic seizures: analysis of regional paediatric cancer service population

PURPOSE: Epileptic seizures complicate the management of childhood brain tumours. There are no published standards for clinical practice concerning risk factors, treatment selection or strategies to withdraw treatment with antiepileptic drugs (AED). METHOD: We undertook a case note review of 120 patients with newly diagnosed brain tumours, referred to a regional paediatric cancer service. RESULTS: Data was available on 117/120 (98%) children <18 years: median age at tumour presentation was 8.1 years (IQR 25°-75°: 3.6-12.7), median follow up was 33 months (IQR 25°-75°: 24-56), and 35/117 (29%) experienced seizures. A cortical tumour location was associated with the highest risk of seizures (OR: 7.1; CI 95% 2.9-17.3). At a median follow up of 24 months (IQR25°-75°: 15-48), 22/35 (63%) with seizures, had a single seizure episode, 15/35 (43%) were seizure free (SF) on AEDs, 13/35 (37%) were SF off AEDs, and 7/35 (20%) experienced continuing epileptic seizures. Overall 34/35 (97%) were treated with AEDs after a seizure, of whom 12/35 (35%) withdrew from AED medication, and although 4/35 (12%) had seizure relapse, all were after further acute events. The median duration of AED before withdrawal was 11 months (IQR25°-75° 5-14 months), and the median follow up after withdrawal was 15 months (IQR25°-75° 5-34 months). CONCLUSIONS: Seizures affect about 1/3rd of children and young people presenting with and being treated for brain tumours particularly when the tumour is in the cerebral cortex. The low risk of recurrent seizures after AED treatment justifies consideration of early withdrawal of AED after seizure control

Pseudotumor cerebri syndrome in childhood : incidence, clinical profile and risk factors in a national prospective population-based cohort study

Aim To investigate the epidemiology, clinical profile and risk factors of pseudotumor cerebri syndrome (PTCS) in children aged 1-16 years. Methods A national prospective population-based cohort study over 25 months. Newly diagnosed PTCS cases notified via British Paediatric Surveillance Unit (BPSU) were ascertained using classical diagnostic criteria and categorised according to 2013 revised diagnostic criteria. We derived national age, sex and weight-specific annual incidence rates and assessed effects of sex and weight category. Results We identified 185 PTCS cases of which 166 also fulfilled revised diagnostic criteria. The national annual incidence (95% CI) of childhood PTCS aged 1-16 years was 0.71 (0.57- 0.87) per 100,000 population increasing with age and weight to 4.18 and 10.7 per 100,000 in obese 12-15 year old boys and girls respectively. Incidence rates under 7 years were similar in both sexes. From 7 years onwards, the incidence in girls was double that in boys, but only in overweight (including obese) children. In 12-15 year old children, an estimated 82% of the incidence of PTCS was attributable to obesity. Two subgroups of PTCS were apparent: 168 (91%) cases aged from 7 years frequently presented on medication and with headache, and were predominantly female and obese. The remaining 17 (9%) cases under 7 years often lacked these risk factors and commonly presented with new onset squint. Conclusions This uniquely largest population-based study of childhood PTCS will inform the design of future intervention studies. It suggests that weight reduction is central to the prevention of PTCS

What is new in migraine management in children and young people?

For this narrative review we found recent publications on the use and effectiveness of old therapies including nutraceuticals, such as riboflavin, vitamin D, magnesium, melatonin, and talking therapies. Recent large trials of established conventional pharmaceuticals such as propranolol, pizotifen, topiramate and amitriptyline for childhood migraine have failed, but the use of a quasi-placebo in future trials could help. We reviewed the evidence for angiotensin antagonists including candesartan in adults, but found a lack of evidence for their use in children.There have been new developments in pharmaceuticals recently, including a more selective 5-HT1F agonist, lasmiditan, an effective acute treatment with no vasoconstrictor activity in adults, currently being tested in children. Also, a number of new Calcitonin Gene-Related Peptide (CGRP) antibodies and antagonists, with proven efficacy in acute treatment and or prevention of migraine in adults, are undergoing trials in children.Peripheral nerve blocks and botulinum toxin are gaining popularity in adult practice, but we really need more good quality evidence for their effectiveness in children.Finally, electroceuticals, that is therapeutic electric devices, are now marketed for acute and or preventative treatment, including an external trigeminal nerve stimulator (e-TNS), a non-invasive vagal nerve stimulator (nVNS), a single-pulse transcranial magnetic stimulator (sTMS), and a remote electrical neuromodulation device (REN). At the moment, evidence for their effectiveness in children is still lacking.So, there has been much progress, but mostly for adults. We are in urgent need of more migraine trials in children

Protocol for a prospective observational study of adverse drug reactions of antiepileptic drugs in children in the UK

Background Epilepsy is a common chronic disease of children that can be treated with anti-epileptic drugs (AEDs). AEDs, however, have significant side effects. Newer AEDs are thought to have fewer side effects. There have, however, been few comparative studies of AED toxicity. The aim is to compare the safety profile of the most frequently used AEDs by performing a multicentre prospective cohort study. This protocol describes the planned study. Design A multicentre prospective cohort study of children on AED treatment in hospitals across the UK. Ethical approval will be obtained. Sample size Three thousand children on treatment for epilepsy will be recruited from paediatric clinics. It is expected that this sample size will have the potential to compare toxicity between the most frequently used AEDs. Duration of study 24 months. Outcome measure Adverse drug reactions (ADRs) to AEDs. These will be identified by the use of a validated questionnaire, the Paediatric Epilepsy Side Effect Questionnaire. They will be evaluated using the Naranjo algorithm. Preventability will be assessed using the Schumock and Thornton scale. Discussion Toxicity of individual AEDs when given as monotherapy and polytherapy will be determined. Additionally, discontinuation rates due to ADRs will be determined. The data will assist clinicians in choosing AEDs with the least toxicity

Safety of antiepileptic drugs in children and young people: a prospective cohort study

Purpose: This study aims to describe the incidence of adverse drug reactions (ADRs) in children receiving antiepileptic drugs (AEDs) and compare ADRs to the individual drugs when given as monotherapy. Method: Paediatric patients (≤18 years old) were enrolled for this prospective observational study over a 6-month period, between September 2015 and March 2016. Adverse reactions to antiepileptic drugs (AEDs) were elicited at the time of enrolment and after 3 months using the Paediatric Epilepsy Side Effects Questionnaire. Results: A total of 1139 suspected ADRs were reported in 124 participants. Eighteen different AEDs were prescribed. Sixty-six children (53%) were receiving AED monotherapy at the time of recruitment; 34/66 (52%) of whom received new generation AEDs. Levetiracetam was the most frequently prescribed AED (62/124, 50%). When only children receiving AED monotherapy were considered, fatigue, drowsiness, weight gain, dizziness were less likely with levetiracetam (p < .01). Slow thinking and decreased concentration were less likely with levetiracetam or carbamazepine than valproic acid (p < .05). Five patients (four on polytherapy) discontinued AED treatment due to ADRs and 2 had a dose reduction. Conclusions: Levetiracetam and carbamazepine were better tolerated than sodium valproate

The natural history of ataxia-telangiectasia (A-T): A systematic review

Background: Ataxia-telangiectasia is an autosomal recessive, multi-system, and life-shortening disease caused by mutations in the ataxia-telangiectasia mutated gene. Although widely reported, there are no studies that give a comprehensive picture of this intriguing condition. Objectives: Understand the natural history of ataxia-telangiectasia (A-T), as reported in scientific literature. Search methods: 107 search terms were identified and divided into 17 searches. Each search was performed in PubMed, Ovid SP (MEDLINE) 1946-present, OVID EMBASE 1980 -present, Web of Science core collection, Elsevier Scopus, and Cochrane Library. Selection criteria: All human studies that report any aspect of A-T. Data collection and analysis: Search results were de-duplicated, data extracted (including author, publication year, country of origin, study design, population, participant characteristics, and clinical features). Quality of case-control and cohort studies was assessed by the Newcastle-Ottawa tool. Findings are reported descriptively and where possible data collated to report median (interquartile range, range) of outcomes of interest. Main results: 1314 cases reported 2134 presenting symptoms. The most common presenting symptom was abnormal gait (1160 cases; 188 studies) followed by recurrent infections in classical ataxia-telangiectasia and movement disorders in variant ataxia-telangiectasia. 687 cases reported 752 causes of death among which malignancy was the most frequently reported cause. Median (IQR, range) age of death (n = 294) was 14 years 0 months (10 years 0 months to 23 years 3 months, 1 year 3 months to 76 years 0 months). Conclusions: This review demonstrates the multi-system involvement in A-T, confirms that neurological symptoms are the most frequent presenting features in classical A-T but variants have diverse manifestations. We found that most individuals with A-T have life limited to teenage or early adulthood. Predominance of case reports, and case series demonstrate the lack of robust evidence to determine the natural history of A-T. We recommend population-based studies to fill this evidence gap

Outcomes for children with acquired brain injury (ABI) admitted to acute neurorehabilitation

Aim:To evaluate an innovative paediatric neurorehabilitation model in relation to improving quality of neurorehabilitation and reducing length of stay (LOS) for children with acquired brain injury.Method:A process evaluation approach was conducted in line with Medical Research Council evaluation of complex interventions guidance. Analysis was conducted on routinely collected patient data from 2017 to 2018, including LOS and family feedback. Descriptive and inferential statistics were used for quantitative analysis and qualitative data was analysed thematically.Results:Outcomes for 70 children (0–16y, median age 5y, IQR 1–11y, 46 males, 24 females) referred to the service indicated improved function and reduced complexity of need. The mean LOS was 10.6 days compared to baseline mean LOS of 41 days (2011–2012). High satisfaction from the families was recorded; however, ongoing needs and service gaps regarding long‐term support were identified.Interpretation:This service model is effective in delivering quality paediatric neurorehabilitation, demonstrating a sustained impact on LOS, and positive patient outcome data and family feedback for this group of patients