Applicable Conditions
Conditions with Breathing Muscle Weakness that can benefit from
Noninvasive Ventilatory Support and Mechanical Insufflation-Exsufflation

People with the following conditions are conventionally treated with low-span bi-level positive airway pressure (PAP) and uncomfortable airway suctioning, which are inadequate to fully support the respiratory muscles. As their condition/symptoms worsen, they require invasive airway tubes (e.g. tracheostomy tubes). However, if you can understand and cooperate with basic instruction, and you have breathing or coughing muscle weakness (i.e. if you are too weak to breathe or cough effectively), then you do not need a tracheostomy tube. In fact, even people intubated for acute respiratory failure fall under this criteria.

Diagnostic categories:

Outcomes for amyotrophic lateral sclerosis (ALS)

Outcomes for chronic lung disease and debilitated elderly in critical care

Outcomes for Duchenne muscular dystrophy (DMD)

Outcomes for type 1 neuromuscular disorders (e.g. SMA type 1)

Outcomes for other neuromuscular disorders (e.g. SMA types 2 and 3)

Outcomes for obesity hypoventilation syndrome

Outcomes for post-poliomyelitis syndrome

Outcomes for spinal cord injury/myelopathies

Myopathies (Muscle Disorders)

Muscular dystrophies:














Walker-Warburg Syndrome


Other myopathies:

Steroid myopathy





Central core disease


Myotonia congenital

Paramyotonia congenita


Acid maltase deficiency (Pompe disease)


Cori disease (debrancher enzyme deficiency)

Finnish (tibial) distal myopathy

Inclusion-body myositis

ZASP-related myofibrillar

Nemaline rod

Andersen-Tawil syndrome

Mitochondrial myopathies


Kearns-Sayre syndrome

Leigh syndrome

Progressive external ophthalmoplegia


Fiber-type disproportion


Metabolic myopathies with respiratory dysfunction:

Lactate dehydrogenase deficiency

McArdle disease (phosphorylase mutase deficiency)

Myoadenylate deaminase deficiency

Periodic paralysis

Phosphofructokinase deficiency

Phosphoglycerate kinase deficiency


Acquired myopathies of systemic disease:

Carcinomatous myopathy

Cachexia/anorexia nervosa

Medication associated (e.g. glucocorticoid)

Inflammatory myopathies





Diseases of the myoneural junction:

Myasthenia gravis

Congenital myasthenic syndromes

Lambert-Eaton myasthenic syndrome


Motor Neuron, Spinal Cord, and Peripheral Nerve Disorders


Motor neuron diseases:

Amyotrophic lateral sclerosis (ALS)

Spinal muscular atrophy (SMA)

Kennedy disease



Spinal cord injury (SCI)

Myelopathies of rheumatoid, infectious, spondylitic, vascular, traumatic, or idiopathic origin

Tetraplegia associated with toxic metals, pancuronium bromide, or botulism



Hereditary sensory motor neuropathies

Familial hypertrophic interstitial polyneuropathy

Charcot-Marie-Tooth disease

Chronic inflammatory demyelinating polyneuropathy

Guillain-Barré syndrome

Phrenic neuropathies associated with cardiac hypothermia, surgical, or other trauma (including radiation, phrenic electrostimulation, familial, paraneoplastic or infectious), associated with lupus erythematosus or other connective tissue diseases, or idiopathic


Central Nervous System Diseases


Multiple sclerosis (MS)

Parkinson’s disease

Disorders of supraspinal tone

Friedreich's ataxia

Cerebral palsy

Static encephalopathies including those associated with Arnold Chiari malformation, syringomyelia, myelomeningocele, and encephalitis


Other Medical Conditions


Intensive care deconditioning and neuromyopathy (i.e. healthy elderly people weakened by long stays in intensive care)

Chronic obstructive pulmonary disease (COPD) or other lung disease with severe respiratory muscle dysfunction

Obesity hypoventilation syndrome

Central alveolar hypoventilation including Ondine's curse


Anyone with weak muscles at risk for respiratory complications of surgery

© 2020 by VentilaMed and

The information presented on this website is not to be reproduced unless granted express consent by its creators.


The information contained within this application is neither intended nor implied to be a substitute for professional medical advice and is provided for educational purposes only. You assume full responsibility for how you choose to use this information. Always seek the advice of your physician or other qualified healthcare provider before starting any new treatment or discontinuing an existing treatment. Talk with your healthcare provider about any questions you may have regarding a medical condition. If you think that you are having a medical emergency, immediately call 911 or the number for the local emergency ambulance service. The BreatheNVS website is an ongoing project and we will continue to bring you new resources and information.


  • Facebook App Icon
  • Twitter App Icon
  • Instagram Social Icon
  • YouTube Social  Icon