Prednisolone or tetracosactide depot for infantile epileptic spasms syndrome? A prospective analysis of data embedded within two randomised controlled trials.

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.

The metformin in tuberous sclerosis (MiTS) study: A randomised double-blind placebo-controlled trial.

Background: Tuberous Sclerosis Complex (TSC) is a genetic disorder characterised by the development of benign tumours secondary to loss of inhibitory regulation of the mTOR (mechanistic Target of Rapamycin) intracellular growth pathway. Metformin inhibits the mTOR pathway. We investigated whether metformin would reduce growth of hamartomas associated with tuberous sclerosis complex.

Epileptic spasms - 175 years on: Trying to teach an old dog new tricks.

Purpose: This text provides an overview of how the condition "infantile spasms" has evolved in the last 175 years.

Method: Key references are summarised to assimilate this review.

Results: Infantile spasms, first described by Dr West in 1841, has undergone extensive investigation to understand the pathogenesis, aetiologies, optimal intervention and most likely prognosis for the affected child.

Immunotherapy for arterial ischaemic stroke in childhood: a systematic review.

Background: There is little evidence about either prevention or treatment of childhood arterial ischaemic stroke (AIS). However, drugs that regulate the immune and inflammatory response could theoretically prevent occurrence or recurrence of AIS. Additionally, as an acute treatment, they may limit the neurological damage caused by AIS.

The outcome of surgical management of subependymal giant cell astrocytoma in tuberous sclerosis complex.

Objectives: The indications for surgery and outcomes of patients who underwent surgical removal of subependymal giant cell astrocytomas (SEGAs) in our institution between 2000 and 2011 were reviewed.

Methods: We reviewed the clinical details of 16 patients with a diagnosis of Tuberous Sclerosis Complex (TSC) who underwent surgery for SEGA in Bristol since 2000. We collected information on age, sex, epilepsy history and cognitive status.

Juvenile parkinsonism associated with heterozygous frameshift ATP13A2 gene mutation.

We report a case of levodopa-responsive juvenile parkinsonism (JP) associated with a heterozygous ATP13A2 gene frameshift mutation. The clinical phenotype of our case is more severe when compared with other published reports of symptomatic heterozygous ATP13A2 mutation carriers. To our knowledge, this is the youngest reported patient with JP associated with a heterozygous ATP13A2 mutation.

The epidemiology of childhood stroke.

This paper reviews the epidemiology of childhood stroke. Stroke is an important condition in children. It is one of the top ten causes of childhood death and there is a high risk of serious morbidity for the survivors.

Research governance delays for a multicentre non-interventional study.

Objectives: To evaluate the delay in research governance approval for a non-interventional, multicentre study in the United Kingdom.

Design: The times taken from application to the granting of research governance approval for an observational study of childhood stroke with ethical approval were prospectively recorded.

Setting: Ninety-two acute NHS Trusts in the United Kingdom.

Duration of breast feeding and cognitive function: Population based cohort study.

Some evidence suggests that breast feeding is weakly but positively associated with cognitive function. This association has been robust to adjustment for various confounders. The aim of this paper is to determine if duration of breast feeding is associated with cognitive function in late childhood.

The United Kingdom Infantile Spasms Study (UKISS) comparing hormone treatment with vigabatrin on developmental and epilepsy outcomes to age 14 months: a multicentre randomised trial.

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.

The United Kingdom Infantile Spasms Study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multicentre, randomised controlled trial.

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.