Publications by authors named "Christopher Verity"
Prednisolone or tetracosactide depot for infantile epileptic spasms syndrome? A prospective analysis of data embedded within two randomised controlled trials.
Eur J Paediatr Neurol
January 2023
Objective: To report a prospectively planned analysis of two randomised controlled trials with embedded comparisons of prednisolone versus tetracosactide depot for the treatment of infantile epileptic spasms syndrome (IESS).
Methods: Individual patient data from patients randomly allocated to prednisolone or tetracosactide depot were analysed from two trials (UKISS, ICISS). The comparison was embedded within trials in which some patients also received vigabatrin but only patients receiving monotherapy with randomly allocated hormonal treatments are included in this analysis.
New pediatric and adult subacute sclerosing panencephalitis cases between 1996 and 2020 were reported based on an established UK registry with no evidence of under-ascertainment using a separate pediatric surveillance system. After 15 years with no pediatric UK-acquired cases, 3 cases arose from 2017 after increased measles. Modeling suggested this was in line with measles notifications, underreporting of laboratory-confirmed measles or increased subacute sclerosing panencephalitis risk.
View Article and Find Full Text PDFAim: To report the differential diagnosis in children with progressive intellectual and neurological deterioration (PIND) in the UK.
Method: Since 1997 the PIND Study has searched for variant Creutzfeldt-Jakob disease (vCJD) in children, using the British Paediatric Surveillance Unit to perform prospective surveillance of those younger than 16 years with PIND.
Results: From May 1997 to October 2019, 2255 children meeting PIND criteria had been notified, of whom 2008 (1085 males, 923 females) had underlying diagnoses.
Objectives: Selective dorsal rhizotomy (SDR) has gained interest as an intervention to reduce spasticity and pain, and improve quality of life and mobility in children with cerebral palsy mainly affecting the legs (diplegia). We evaluated the cost-effectiveness of SDR in England.
Methods: Cost-effectiveness was quantified with respect to Gross Motor Function Measure (GMFM-66) and the pain dimension of the Cerebral Palsy Quality of Life questionnaire for Children (CPQOL-Child).
Article Synopsis
- A study examined the causes and treatment responses in 377 infants with infantile spasms, finding that 58% had a known underlying cause, with varying response rates to different treatments.* -
- Infants with strokes showed better treatment responses (82%) compared to other causes (56%), and those with Down syndrome had similar response rates across treatment types, indicating no significant advantage from adding vigabatrin.* -
- The findings highlight the importance of classification for comparing outcomes and suggest that specific underlying conditions may be linked to better or different responses to treatment in infantile spasms.*
Background: Selective dorsal rhizotomy (SDR) is an irreversible surgical procedure involving the division of selected sensory nerve roots, followed by intensive physiotherapy. The aim is to improve function and quality of life in children with cerebral palsy and a Gross Motor Function Classification System (GMFCS) level of II or III (walks with or without assistive devices, respectively). We assessed gross motor function before and after SDR and postoperative quality of life in a study commissioned by NHS England.
View Article and Find Full Text PDFObjectives: To report investigations performed in children with progressive neurodegenerative diseases reported to this UK study.
Design: Since 1997 paediatric surveillance for variant Creutzfeldt-Jakob disease (vCJD) has been performed by identifying children aged less than 16 years with progressive intellectual and neurological deterioration (PIND) and searching for vCJD among them.
Setting: The PIND Study obtains case details from paediatricians who notify via the British Paediatric Surveillance Unit.
Vigabatrin with hormonal treatment versus hormonal treatment alone (ICISS) for infantile spasms: 18-month outcomes of an open-label, randomised controlled trial.
Lancet Child Adolesc Health
October 2018
Article Synopsis
- The International Collaborative Infantile Spasms Study (ICISS) found that using a combination of vigabatrin and hormonal therapy was more effective than hormonal therapy alone in treating infantile spasms during the first 14 to 42 days of treatment.* -
- The follow-up study aimed to determine if this combination therapy also led to better developmental and epilepsy outcomes for infants at 18 months of age.* -
- The trial involved multiple hospitals across several countries, using a randomized controlled design to compare therapies, and assessed outcomes using measures such as the Vineland Adaptive Behaviour Scales and seizure frequency.*
Aim: To describe the cases of Niemann-Pick type C (NP-C) disease in a United Kingdom epidemiological study of progressive intellectual and neurological deterioration in childhood.
Method: Paediatricians notified cases via the British Paediatric Surveillance Unit between 1997 and 2015.
Results: Fifty-three NP-C patients were identified: 29 females, 24 males.
Safety and effectiveness of hormonal treatment versus hormonal treatment with vigabatrin for infantile spasms (ICISS): a randomised, multicentre, open-label trial.
Lancet Neurol
January 2017
Article Synopsis
- Infantile spasms is a severe epilepsy syndrome in infants, often hard to treat, with hormonal therapy and vigabatrin being common treatments.
- A trial involving 102 hospitals aimed to determine if combining hormonal therapy with vigabatrin was more effective than hormonal therapy alone in treating this condition.
- The study enrolled 766 infants, with 377 randomly assigned to receive either the combined treatment or hormonal therapy alone, measuring the primary outcome of cessation of spasms over a designated period.
Aim: To report on the epidemiology of the brain white matter disorders of children identified via a national prospective study.
Method: Since 1997 a study of UK children with progressive intellectual and neurological deterioration (PIND) has used the British Paediatric Surveillance Unit system to identify children with progressive neurodegenerative disease. This paper reports on children in the study with brain white matter disorders.
Aim: The aim of this study was to investigate whether children in England with narcolepsy who received the ASO3 adjuvanted pandemic A/H1N1 2009 influenza vaccine (Pandemrix) differed clinically from unvaccinated patients.
Method: A retrospective review was conducted in children with narcolepsy diagnosed by sleep centres and paediatric neurologists in 16 English hospitals. The inclusion criteria were patient age 4 to 18 years, onset of narcolepsy after January 2008, and diagnosis by the time of the key data-gathering visit in 2011.
An investigation into the relationship between vigabatrin, movement disorders, and brain magnetic resonance imaging abnormalities in children with infantile spasms.
Dev Med Child Neurol
September 2013
Article Synopsis
- The study investigated the connection between movement disorders, brain MRI changes, and vigabatrin therapy in children with infantile spasms.
- Out of 124 infants reviewed, 10 developed a movement disorder while on vigabatrin, with varying responses to dosage adjustments.
- The findings suggest that while vigabatrin may be associated with movement disorders in some cases, the majority of infants did not experience these issues, and MRI changes linked to vigabatrin do not specifically correlate with movement disorders.
Objective: To evaluate the risk of narcolepsy in children and adolescents in England targeted for vaccination with ASO3 adjuvanted pandemic A/H1N1 2009 vaccine (Pandemrix) from October 2009.
Design: Retrospective analysis. Clinical information and results of sleep tests were extracted from hospital notes between August 2011 and February 2012 and reviewed by an expert panel to confirm the diagnosis.
Aim: To report the demographic, phenotypic, and time-to-diagnosis characteristics of children with GM2 gangliosidosis referred to the UK study of Progressive Intellectual and Neurological Deterioration.
Method: Case notification is made via monthly surveillance card, administered by the British Paediatric Surveillance Unit to all UK-based paediatricians; children with GM2 gangliosidosis were identified from cases satisfying inclusion in the UK study of Progressive Intellectual and Neurological Deterioration and analysed according to phenotypic and biochemical categories.
Results: Between May 1997 and January 2010, 73 individuals with GM2 gangliosidoses were reported: 40 with Tay-Sachs disease, 31 with Sandhoff disease, and two with GM2 activator protein deficiency.
Objective: To determine the contribution of herpes simplex virus (HSV) to serious neurological disease.
Setting And Patients: A 3-year prospective survey of children aged 2-23 months in Britain and Ireland.
Results: 19 children had HSV central nervous system (CNS) infection; 13 aged 2-11 months had focal neuroimaging abnormalities and 11 long-term neurological sequelae.
Article Synopsis
- Infantile spasms, a severe seizure disorder in infants, is influenced by various factors including age of onset and how quickly treatment begins, which can significantly affect developmental outcomes.
- A study assessed 77 infants at 4 years using the Vineland Adaptive Behaviour Scales, revealing that younger age at onset and longer lead times to treatment were linked to poorer developmental scores.
- Findings suggest that early diagnosis and treatment are crucial in minimizing developmental delays, particularly emphasizing that younger infants might be at greater risk for severe consequences.
Article Synopsis
- The UK Infantile Spasms Study (UKISS) aimed to identify the causes of infantile spasms using the pediatric adaptation of ICD-10 for classification.
- Out of 207 infants studied, 61% had a proven etiology related to neurologic diseases, while 33% had no identifiable cause. Key causes included hypoxic-ischemic encephalopathy and chromosomal abnormalities.
- The classification method offers a structured approach for reporting results, avoids ambiguous terminology, and can adapt to account for emerging neurologic diseases, facilitating future comparisons and meta-analyses.
Article Synopsis
- Infantile spasms, a severe form of epilepsy in infants, was studied in the UKISS, which found that hormonal treatments led to better developmental outcomes compared to vigabatrin after 14 months, though epilepsy outcomes were similar for both treatments.* ! -
- A follow-up study at an average age of 4 years revealed that while developmental scores were higher for infants receiving hormonal treatments, the overall difference between the two groups was not statistically significant.* ! -
- For infants with no known cause of their condition, those treated with hormonal therapy maintained improved developmental scores, suggesting that these benefits may persist as they grow, despite similar epilepsy outcomes across both treatment groups.* !
Objective: To study the epidemiology of diseases that cause progressive intellectual and neurological deterioration (PIND) in UK children.
Design: Since May 1997, the authors have performed active surveillance to search for variant Creutzfeldt-Jakob Disease (vCJD) among the many diseases that cause neurological deterioration in children, using the monthly surveillance card sent to all UK consultant paediatricians by the British Paediatric Surveillance Unit. The authors obtain clinical details from reporting paediatricians by questionnaire or site visit, and an Expert Group then independently classifies the cases.
Aim: Our aim was to study the clinical presentation, mode of diagnosis, and epidemiology of mitochondrial disorders in children from the UK who have progressive intellectual and neurological deterioration (PIND).
Method: Since April 1997, we have identified patients aged 16 years or younger with suspected PIND through the monthly notification card sent to all UK consultant paediatricians by the British Paediatric Surveillance Unit. Clinical details obtained from reporting paediatricians are classified by an Expert Group.
Is there evidence of vertical transmission of variant Creutzfeldt-Jakob disease?
J Neurol Neurosurg Psychiatry
July 2011
Objectives: The possibility of vertical transmission of variant Creutzfeldt-Jakob disease (vCJD) has been raised because of the widespread distribution of infectivity in vCJD and the demonstration that this condition can be transmitted through blood transfusion. The aim of this study is to search for evidence of this type of transmission of vCJD.
Methods: A national surveillance system for CJD has been established in the UK since 1990.