117th ENMC Workshop: Ventilatory Support in Congenital Neuromuscular Disorders — Congenital Myopathies, Congenital Muscular Dystrophies, Congenital Myotonic Dystrophy and SMA (II) 4–6 April 2003, Naarden, The Netherlands

Abstract

Eighteen participants from Australia, Austria, Denmark, Finland, France, Germany, The Netherlands, the UK, and the USA met in Naarden, representing a variety of disciplines with experience in the respiratory management of patients with neuromuscular disorders (NMD). The aims of the workshop were to agree upon and report minimum recommendations for the investigation and treatment of respiratory involvement in congenital muscular disorders, and to identify areas where further research is needed. The workshop specifically excluded patients with Duchenne muscular dystrophy where evidence for the need for and efficacy of the treatment of respiratory failure is better established. All participants contributed to the review and assessment of published evidence in the field, and current practice amongst the group was also compared. Despite the individual rarity of the conditions under consideration in this workshop, the accumulated experience of the group represented the care of more than 545 patients with these disorders, of whom around one-third were receiving mechanical ventilation.