Symptoms, clinical and physiological findings motivating home mechanical ventilation in patients with neuromuscular diseases

J Rehabil Med. 2006 Jul;38(4):250-4. doi: 10.1080/16501970600721033.


Objective: To clarify the relationship between symptoms, clinical signs and physiological abnormalities that were motivating the initiation of home mechanical ventilation in patients suffering from neuromuscular diseases.

Methods: From The Swedish Home Mechanical Ventilation Register we identified 352 patients with neuromuscular diseases and we looked at circumstances (acute vs elective) and clinical motives for starting ventilatory support.

Results: Home mechanical ventilation was commenced electively in 268 patients (76%) and among these daytime sleepiness was the most common motive, being reported in 56% of the patients. In the 24 children with spinal muscular atrophy, however, 96% started ventilation electively and cough insufficiency was the most common motive. The patients were moderately hypercapnic (PaCO(2): 7.0 kPa, SD 1.3). None of the clinical motives were related to the PaCO(2) level. Average PaO(2) was above 8 kPa in all groups, but lowest in the patients with post-polio and dystrophia myotonica. Mean vital capacity was close to 40% of predicted, but significantly lower in the Duchenne patients (26% of predicted).

Conclusion: Daytime sleepiness was the most common clinical symptom motivating home mechanical ventilation in this group of patients with chronic hypercapnic respiratory insufficiency secondary to neuro/myopathies. Respiratory function testing is therefore suggested to be included in the diagnostic work up of daytime sleepiness in these patients.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Amyotrophic Lateral Sclerosis / complications
  • Amyotrophic Lateral Sclerosis / physiopathology
  • Amyotrophic Lateral Sclerosis / therapy
  • Blood Gas Analysis
  • Child
  • Child, Preschool
  • Female
  • Home Care Services
  • Humans
  • Male
  • Middle Aged
  • Muscular Atrophy, Spinal / complications
  • Muscular Atrophy, Spinal / physiopathology
  • Muscular Atrophy, Spinal / therapy
  • Muscular Dystrophy, Duchenne / complications
  • Muscular Dystrophy, Duchenne / physiopathology
  • Muscular Dystrophy, Duchenne / therapy
  • Myotonic Dystrophy / complications
  • Myotonic Dystrophy / physiopathology
  • Myotonic Dystrophy / therapy
  • Neuromuscular Diseases / complications
  • Neuromuscular Diseases / diagnosis
  • Neuromuscular Diseases / physiopathology
  • Neuromuscular Diseases / therapy*
  • Partial Pressure
  • Postpoliomyelitis Syndrome / complications
  • Postpoliomyelitis Syndrome / physiopathology
  • Postpoliomyelitis Syndrome / therapy
  • Respiration, Artificial*
  • Respiratory Insufficiency / diagnosis
  • Respiratory Insufficiency / etiology
  • Respiratory Insufficiency / physiopathology
  • Respiratory Insufficiency / therapy*