Article Text

Download PDFPDF
006 Quality improvement: management of myotonic dystrophy patients in tertiary care centres
  1. Sola Odunsi1,
  2. Mark Roberts2,
  3. James Lilleker2,
  4. Andrew Bentley1,
  5. Tim Felton1
  1. 1University Hospital South Manchester
  2. 2Salford Royal Hospital


Background Myotonic Dystrophy type 1 is an inherited multisystem disorder with a European prevalence of 3–15 per 100,000. Cardiorespiratory complications (e.g. arrythmia, respiratory failure) account for around 70% of early deaths. In many cases this could be prevented though periodic screening and timely intervention, including with pacemakers or non-invasive ventilation, where indicated.

Recently, international consensus-based care recommendations have been published with a view to reduce mortality by improving quality and standardising care for patients with Myotonic Dystophy type 1. We hypothesised that nationally, adherence to these recommendations is likely to be poor.

Methods We audited the care of patients with Myotonic Dystrophy type 1 at the Manchester Centre for Clinical Neuroscience (MCCN) and the University Hospital South Manchester (UHSM) Ventilation Clinic against consensus-based care recommendations. A data collection proforma was created based on the recent care recommendations. Retrospective case note review was performed.

Results and conclusion Data collection and analysis is ongoing. Full results will be presented at the conference along with a quality improvement action plan. These results will also feed in to a planned redevelopment of tertiary neuromuscular services in the region.

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.