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Morning report is an important clinical learning activity in many neurological institutions. A long experience of these meetings allows identification of several components to enhance its success. Meetings are best if brief (one or two cases) and held regularly, preferably daily and early in the working day, with full in-person team engagement. A senior clinician should lead the meeting and commit to a single interpretation, without fear of being wrong. Although the environment is relaxed (refreshments typically provided), it is a working meeting and with the essential focus on the patient rather than the learners. The rich learning experience is greatly enhanced by a subsequent confidential email summary and interpretation of the case(s) sent to all participants. [ABSTRACT FROM AUTHOR]

We have recently introduced a new item to our neurology Grand Rounds-the '1-3-5 presentation'. The format comprises a presentation on one topic, using three slides and lasting no more than 5 minutes. This a useful way of covering brief single topics and introducing and sparking discussion on more complex ones. '1-3-5s' have proven popular in our department and we have compiled a library of these presentations that is hosted on a YouTube channel. This article discusses the benefits and prospects for this format and encourages other units to provide similar opportunities for teaching and learning among all clinical grades. [ABSTRACT FROM AUTHOR]

Adeno-associated virus (AAV) gene therapies are generating much excitement in the rare disease field, particularly for previously untreatable neurological conditions. Efficacy has been claimed for several gene therapy products and the number of trials is rapidly increasing. However, reports of severe treatment-related adverse reactions are emerging, including death. There is still insufficient knowledge about their aetiology, prevention and treatment. We therefore undertook to systematically review publicly available data on AAV gene therapies in order to collate existing information on both safety and efficacy. Here, we review emerging efficacy reports of these novel therapies, many of which show promise. We also collate an increasing number of adverse reactions. Overwhelmingly, these results make a case for unified reporting of adverse events. This is likely to be critical for improving the safety of these promising treatments. [ABSTRACT FROM AUTHOR]

Good teamwork underpins excellent clinical services; a formal (typically annual) teambuilding event can help to foster a team's sense of purpose and ensure solidity and collaboration between team members. We have held several Epilepsy Unit teambuilding events and use this experience to identify their essential components and suggestions for various workplace- based and leisure activities to include. Other neurology teams might consider similar events to help develop their teamworking. [ABSTRACT FROM AUTHOR]

The investigation of children presenting with infantile and childhood epileptic encephalopathies (ICEE) is challenging due to diverse aetiologies, overlapping phenotypes and the relatively low diagnostic yield of MRI, electroencephalography (EEG) and biochemical investigations. Careful history and thorough examination remain essential as these may identify an acquired cause or indicate more targeted investigation for a genetic disorder. Whole exome sequencing (WES) with analysis of a panel of candidate epilepsy genes has increased the diagnostic yield. Whole genome sequencing (WGS), particularly as a trio with both parents' DNA, is likely to supersede WES. Modern genomic investigation impacts on the timing and necessity of other testing. We propose a structured approach for children presenting with ICEE where there is diagnostic uncertainty, emphasising the importance of WGS or, if unavailable, WES early in the investigative process. We note the importance of expert review of all investigations, including radiology, neurophysiology and biochemistry, to confirm the technique used was appropriate as well as the results. It is essential to counsel families on the risks associated with the procedures, the yield of the procedures, findings that are difficult to interpret and implication of 'negative' results. Where children remain without a diagnosis despite comprehensive investigation, we note the importance of ongoing multidisciplinary care. [ABSTRACT FROM AUTHOR]

Background: Timely management of non-convulsive status epilepticus (NCSE) is critical to improving patient outcomes. However, NCSE can only be confirmed using electroencephalography (EEG), which is either significantly delayed or entirely unavailable in emergency departments (EDs). We piloted the use of a new bedside EEG device, Rapid Response EEG (Rapid-EEG, Ceribell), in the ED and evaluated its impact on seizure management when used by emergency physicians.Methods: Patients who underwent Rapid-EEG to rule out NCSE were prospectively enrolled in a pilot project conducted at two ED sites (an academic hospital and a community hospital). Physicians were surveyed on the perceived impact of the device on seizure treatment and patient disposition, and we calculated physicians' sensitivity and specificity (with 95% CI) for diagnosing NCSE using Rapid-EEG's Brain Stethoscope function.Results: Of the 38 patients enrolled, the one patient with NCSE was successfully diagnosed and treated within minutes of evaluation. Physicians reported that Rapid-EEG changed clinical management for 20 patients (53%, 95% CI 37% to 68%), primarily by ruling out seizures and avoiding antiseizure treatment escalation, and expedited disposition for 8 patients (21%, 95% CI 11% to 36%). At the community site, physicians diagnosed seizures by their sound using Brain Stethoscope with 100% sensitivity (95% CI 5% to 100%) and 92% specificity (95% CI 62% to 100%).Conclusion: Rapid-EEG was successfully deployed by emergency physicians at academic and community hospitals, and the device changed management in a majority of cases. Widespread adoption of Rapid-EEG may lead to earlier diagnosis of NCSE, reduced unnecessary treatment and expedited disposition of seizure mimics. [ABSTRACT FROM AUTHOR]

Background: Cerebral palsy (CP) is a non-progressive disorder of posture and movement caused by prenatal or perinatal lesions of the brain. Children with CP are also at increased risk of other disabilities, for example, intellectual disability. Previous studies suggest the risk of intellectual disability varies in complex ways according to the type of motor impairment and perinatal factors such as gestational age.Objective: To determine the patterns of risk of intellectual disability in children with spastic CP.Design: Cross-sectional, population-based study using the Northern Ireland Cerebral Palsy Register.Participants: Persons born in 1981-2008 with congenital bilateral or unilateral spastic CP (N=1452).Outcome Measure: The outcome measure was severe intellectual disability (IQ <50), as reported by clinicians known to the child. Data pertaining to CP subtype, sex, gestational age, birth weight and functional level were included in analyses.Results: Severe intellectual disability was significantly more prevalent in children with bilateral spastic CP (BSCP) compared with children with unilateral spastic CP (χ² (2)=162.60, p<0.001). Compared with very preterm infants with BSCP, the risk of intellectual disability increased in moderately preterm (OR=3.97, 95% CI 1.04 to 15.23) and at-term (OR=2.51, 95% CI 1.16 to 5.44) children with BSCP.Conclusions: Children with BSCP are at increased risk of intellectual disability, with those born at term at the highest risk. The findings highlight the importance of early screening, particularly for children with BSCP born at term. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to conduct a randomised pilot trial to assess the feasibility of a randomised controlled trial (RCT) to investigate the effect of telephone-guided self-help for the treatment of mental health difficulties in children with neurological conditions.Design: Preliminary RCT. The primary outcome measure was the Strengths and Difficulties Questionnaire.Setting: Neurology clinics in a national tertiary paediatric hospital.Patients: Young people attending neurology clinics who met criteria for mental health difficulties according to the Development and Wellbeing Assessment.Interventions: 12 weeks of telephone-guided self-help based on a modular approach to psychological therapy for children delivered to children and/or their parents (n=17; eight males; mean age 12.04 years, SD=3.34) or a waiting list for telephone-guided self-help with no additional intervention over 12 weeks (n=17; nine males; mean age 10.53 years, SD=3.14).Results: 124 participants completed the DAWBA, and 34 children and young people were entered into the trial. 65% of those randomised to the intervention arm completed the full intervention, and the intervention was acceptable to those completing it. However, there were significant problems related to lack of data completion (38% data loss for primary outcome measure), choice of control comparator and outcome measures. Due to significant loss of data at follow-up, the effect size findings are considered unreliable.Conclusions: Further feasibility work should be conducted to improve data completeness before progression to a definitive trial of guided self-help for mental health problems in children with neurological conditions can be recommended.Trial Registration Number: ISRCTN21184717. [ABSTRACT FROM AUTHOR]

Objective: To determine the usefulness of video recordings for validating neonatal encephalopathy (NE) exams.Design: Population-based prospective cohort study. NE was assessed and recorded at 1, 3 and 5 hours after birth by the attending physician. Recordings were reviewed blindly after the recruitment period by two specialists. Outcome was assessed at 36 months of age.Setting: Twelve intensive care units in Spain.Patients: Infants of ≥35 weeks' gestational age with perinatal asphyxia.Main Outcomes Measures: Weighted kappa to measure disagreement between the two specialists and between the attending physician and the specialists' classification agreed on by consensus. Regression models to test the association of disagreement on NE assessment and outcome.Results: Of the 32 325 liveborn infants, 217 met the inclusion criteria. Video-recordings were not available for 43 infants (20%). Weighted kappa statistic was 0.74 (95% CI 0.67 to 0.81) between the specialists and the attending physicians. Disagreement occurred in 93 of the 417 (22%) videos, specifically in 39 (14%), 43 (47%), 11 (34%) and 0 exams categorised as no, mild, moderate and severe NE, respectively. According to the specialist consensus assessment, there was disagreement on the therapeutic hypothermia decision in 10 infants.When there was consensus among the specialists assessing a more severe NE degree compared with the attending physicians in 170 infants, those infants had lower cognitive scores with a median of -5.33 points (95% CI -9.85 to -8.16; p=0.02).Conclusions: This study supports the feasibility and benefit of using video recordings to identify NE in infants with perinatal asphyxia. [ABSTRACT FROM AUTHOR]

Background: The prevalence of and risk factors for neurological problems in childhood coeliac disease (CD) are unclear.Methods: We performed a cross-sectional, community-based audit of CD in children diagnosed from January 2010 to December 2016 in Lothian.Results: 79 (28%) of 284 children with CD (201, 70.8% female) (mean age 8.3 years, range of 1-16) had neurological problems. Fifteen (5.3%) had headaches/migraine, 10 (3.5%) anxiety, 8 (2.8%) motor/co-ordination problems / ataxia (there were no patients with ataxia), 7 (2.5%) had behavioural issues, 5 (1.8 %) with ASD, 5 (1.8%) low mood, 4 (1.4%) ADD/ ADHD, 3 (1.1%) seizures and 2 (0.7%) had neuropathy. Neurological problems were more common with later age at CD diagnosis (OR 1.07, 95% CI 1.01 to 1.14) and male gender (OR 1.69, 95% CI 0.96 to 2.95).Conclusion: Prevalence of neurological problems in children with CD in Lothian is lower than published adult CD studies and similar or lower to the reported prevalence in the general childhood population. [ABSTRACT FROM AUTHOR]

Background and Aim: Clinical centres have seen an increase in tic-like movements during the COVID-19 pandemic. A series of children and adolescents are described.Methods: A retrospective chart review of 34 consecutive paediatric patients presenting with sudden onset tic-like movements, seen over 6 months.Results: 94% of patients were female, with an average age of sudden onset or increase of tic-like movements of 13.7 years. 44% had a previous diagnosis of tics, and 47% initially presented to an emergency department. Comorbid psychiatric and neurodevelopmental disorders were reported in 91% with 68% reporting anxiety.Conclusion: We highlight a dramatic presentation of sudden onset functional tic-like movements in predominantly female adolescents to help inform identification and management. There is need to research the neurobiological underpinnings and environmental exacerbating factors leading to these presentations and to explore effective therapeutic strategies. [ABSTRACT FROM AUTHOR]