Childhood Ataxia telangiectasia neuroimaging Assessment Project

  • Research type

    Research Study

  • Full title

    CATNAP - The Childhood Ataxia telangiectasia neuroimaging Assessment Project

  • IRAS ID

    149134

  • Contact name

    Robert Dineen

  • Contact email

    rob.dineen@nottingham.ac.uk

  • Duration of Study in the UK

    1 years, 11 months, 31 days

  • Research summary

    Ataxia-telangiectasia (AT) is a rare inherited condition. Children with AT develop neurological symptoms such as abnormal movements and difficulties with walking and speech. These neurological symptoms result from irreversible changes in the brain tissue structure termed neurodegeneration (or ND for short), particularly involving the back part of the brain called the cerebellum. Although there are treatments for some of the symptoms of AT, there is no effective treatment for the underlying progressive ND.

    State-of-the-art magnetic resonance imaging (MRI) scans can allow us to measure a variety of different biological processes in brain tissue, and we believe that some of these MRI measures (referred to as MRI biomarkers) have the potential to improve our ability to detect and monitor ND in children with AT. In this study, which is jointly funded by a grant from the Ataxia Telangiectasia Children’s Project and Action for A-T, and is supported by the Ataxia Telangiectasia Society, we will test how a panel of potential MRI biomarkers of neurodegeneration relate to neurological symptoms in AT. We will do this by performing a detailed clinical assessment and MRI scanning in a group of children with AT attending the National AT clinic run at the Nottingham University Hospitals NHS trust, and a group of healthy children for comparison. Once completed, this study will allow us to select the most promising MRI biomarkers of ND in AT to take forward into future MRI and treatment trials.

  • REC name

    East Midlands - Derby Research Ethics Committee

  • REC reference

    14/EM/1175

  • Date of REC Opinion

    3 Nov 2014

  • REC opinion

    Further Information Favourable Opinion