Drug resistant epilepsy with mesial temporal sclerosis as possible late neurological complication in two AML survivors after stem cell transplantation.

•Mesial temporal sclerosis (MTS) is a potential late complication of hematological malignancies.•Seizures are usually drug resistant with MTS yet seizure freedom may be achieved by surgery.•Early evaluation for epilepsy surgery is warranted for drug resistant seizures due to AML.

Efficacy, safety and immunogenicity of a human rotavirus vaccine (RIX4414) in Hong Kong children up to three years of age: a randomized, controlled trial.

BACKGROUND: A phase III, double-blind, randomized, controlled trial was conducted in Hong Kong to evaluate the efficacy, safety and immunogenicity of a human rotavirus vaccine, RIX4414 (Rotarix) against severe rotavirus gastroenteritis in children up to three years of age. METHODS: Healthy infants aged 6-12 weeks were enrolled between 08-December-2003 and 31-August-2005 and received two oral doses of either RIX4414 vaccine (N=1513) or placebo (N=1512) given 2 months apart. Vaccine efficacy was assessed from two weeks post-Dose 2 until the children were two and three years of age. Anti-rotavirus IgA seroconversion rate was calculated pre-vaccination and 1-2 months post-Dose 2 using ELISA (cut-off=20 U/mL) for 100 infants. Safety was assessed until the children were two years of age; serious adverse events (SAEs) were recorded throughout the study period. RESULTS: In children aged two and three years of life, vaccine efficacy against severe rotavirus gastroenteritis was 95.6% (95% CI: 73.1%-99.9%) and 96.1% (95% CI: 76.5%-99.9%), respectively. The seroconversion rate 1-2 months after the second dose of RIX4414 was 97.5% (95% CI: 86.8%-99.9%). At least one SAE was recorded in 439 and 477 infants who were administered RIX4414 and placebo, respectively (p-value=0.130). Six intussusception cases were reported (RIX4414=4; placebo=2) and none was assessed to be vaccine-related. CONCLUSION: RIX4414 was efficacious, immunogenic and safe in the prevention of rotavirus gastroenteritis for at least two years post-vaccination in Hong Kong children.

Revascularisation surgery for paediatric moyamoya: a review of the literature.

INTRODUCTION AND AIMS: The role of and optimal surgical revascularisation technique for paediatric moyamoya syndrome (MMS) are controversial. In this literature review our primary aim was to evaluate the evidence base for the efficacy of surgical revascularisation for the treatment of paediatric MMS. Secondary aims were to estimate the rate of peri-operative complications and to ascertain whether direct or indirect revascularisation techniques resulted in differences in clinical or radiological outcomes. METHODS: Papers describing surgical revascularisation and its outcome in the treatment of children with MMS were identified from the OVID Medline database (1966-2004). Only papers in English were reviewed. Data were abstracted using a standardised form. RESULTS: Fifty-seven studies, including data on 1,448 patients, were reviewed. Most were Japanese; 10% were from Western institutions. Indications for revascularisation were described in <15% of studies and varied between centres. Indirect procedures were most commonly performed (alone in 73% of cases, combined with direct procedures in 23%). The rates of peri-operative stroke and reversible ischaemic events were 4.4 and 6.1% respectively. Out of 1,156 (87%) patients, 1,003 derived symptomatic benefit from surgical revascularisation (complete disappearance or reduction in symptomatic cerebral ischaemia), with no significant difference between the indirect and direct/combined groups. Data on developmental and functional outcomes were limited and of uncertain significance as they were not related to pre-operative status. Good collateral formation was significantly more frequent in the direct/combined group than in the indirect group (chi(2), p<0.001). CONCLUSIONS: Data from the medical literature suggest that surgical revascularisation is a safe intervention for paediatric MMS and most treated patients derive some symptomatic benefit. However, paucity of data on selection criteria and more global outcome measures means that the impact of surgical revascularisation on natural history remains uncertain. Direct and/or combined procedures provide better revascularisation, but this is not associated with differences in symptomatic outcome. International standardisation of the clinical approach to the treatment of paediatric MMS is urgently needed to critically evaluate the optimal indications for and timing of surgical revascularisation.

Arteriovenous malformations presenting with papilloedema.

Cerebral arteriovenous malformations (AVMs) are fairly common and the majority of paediatric patients with this condition also present with intracranial haemorrhage. Two patients who had an incidental finding of an AVM associated with papilloedema are described here. The first was a 13-year-old male who presented after an accidental kick to the eyes. Examination revealed bilateral papilloedema. He gave a 2-year history of intermittent headache. Brain magnetic resonance imaging (MRI) showed an unruptured AVM in the temporal lobe. Lumbar puncture revealed elevated cerebrospinal fluid pressure. Visual acuity and visual fields were normal. He was treated with acetazolamide and improved within a few weeks. He subsequently underwent stereotactic radiosurgery to the AVM. He discontinued acetazolamide due to adverse side effects and there was no recurrence of headache and papilloedema. The second patient was a 14-year-old male who had polyarticular juvenile chronic arthritis and received low-dose steroids and methotrexate. Bilateral papilloedema was discovered during routine ophthalmology surveillance and he was otherwise asymptomatic neurologically. Brain MRI revealed an AVM in the posterior fossa. He had three embolization procedures, which have resulted in significant reduction in lesion size. The papilloedema resolved completely after the first two procedures, and visual acuity and fields remained normal. Here, possible underlying mechanism of raised intracranial pressure and importance of visual assessment in those with AVMs and their management are discussed.