Publications by authors named "Christopher M Verity"

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.

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  • 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.*
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  • 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.*
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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.

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  • 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.
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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.

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  • 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.
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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.

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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.

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  • 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.
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  • 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.
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  • 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.* !
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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.

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Objective: We sought to investigate the risk of serious neurologic disease after immunization in early childhood.

Methods: The results of a 3-year prospective study of children (2-35 months old) in Britain and Ireland with encephalitis and/or severe illness with convulsions and fever were linked to each child's vaccine history. Cases were reported via the British Paediatric Surveillance Unit's network.

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Background: Infantile spasms is a severe infantile seizure disorder that is difficult to treat and has a high morbidity. Absence of spasms on days 13 and 14 after randomisation is more common in infants allocated hormone treatments than in those allocated vigabatrin. We sought to assess whether early control of spasms is associated with improved developmental or epilepsy outcomes.

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Background: Infantile spasms, which comprise a severe infantile seizure disorder, have a high morbidity and are difficult to treat. Hormonal treatments (adrenocorticotropic hormone and prednisolone) have been the main therapy for decades, although little evidence supports their use. Vigabatrin has been recorded to have a beneficial effect in this disorder.

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