title: Respiratory function assessment and intervention in neuromuscular disorders.
abstract: OBJECTIVE
  Published research on neuromuscular respiratory failure has increased rapidly over the past decade. Progress in the management of respiratory complications and preventive care have improved outcomes and quality of life for many. In particular, noninvasive positive pressure ventilation (NIPPV) is effective in resolving respiratory failure and has dramatically changed the management of these patients. In this review we discuss recent studies assessing the course of respiratory failure, with a focus on non-Duchenne muscular dystrophy; investigating the impact NIPPV has when it is introduced before diurnal respiratory failure develops; and evaluating a device to treat insufficient cough--the mechanical insufflator--exsufflator (MI-E).
  
  
  RESULTS
  Studies in children with congenital muscular dystrophies and spinal muscular atrophies detected a high prevalence of respiratory complications and confirmed that respiratory failure can be predicted by measurement of vital capacity. NIPPV is an established treatment that is effective in the long-term management of end-stage diurnal respiratory failure. Additionally, recent studies showed improvement in symptoms, quality of life and prevention of respiratory complications when it is introduced for nocturnal hypoventilation before the development of diurnal respiratory failure. However, data on the efficiency of the MI-E are limited, and studies comparing it with other techniques of assisted coughing are needed.
  
  
  CONCLUSIONS
  Recent findings stress the importance of regular respiratory assessment in neuromuscular disease, including sleep studies; strongly support the introduction of NIPPV for management of symptomatic nocturnal hypoventilation; and suggest that prospective randomized trials are required to verify the usefulness of the MI-E.
authors.affiliation: University of Essen, Children's Hospital, Department of General Pediatrics and Neuropediatrics, Essen, Germany. Uwe.mellies@uni-essen.de
authors.forename: Uwe
authors.initials: U
authors.lastname: Mellies
authors.forename: Christian
authors.initials: C
authors.lastname: Dohna-Schwake
authors.forename: Thomas
authors.initials: T
authors.lastname: Voit
mesh_terms: D006801:Humans; D007385:Intermittent Positive-Pressure Ventilation; D009468:Neuromuscular Diseases / Q000503:physiopathology* / Q000628:therapy*; D012119:Respiration