NEUROMUSCULAR DISEASE &

RESPIRATORY FAILURE
Shanojan Thiyagalingam
PATHOPHYSIOLOGY
CAUSES
CLINICAL MANIFESTATION
 Insufficient ventilation
 Dyspnea, orthopnea, rapid shallow breath, accessory
muscle use, thoracoabd paradox, hypercapnia,
hypoxia
 Nocturnal hypoventilation
 May be first sign of chronic neuromuscular disease
 Chocking, insomnia, daytime somnolence, morning
headache, fatigue, impaired cognition; like “OSA”
 Bulbar dysfunction
 Dysphagia, dysarthria, weak mastication, facial
weakness, nasal speech, protruding tongue
 Ineffective cough
 Aspiration, retention of secretion, pneumonia
EVALUATION
 Consider three domains:
 Ventilation
 FVC, MIP, CO2 level
 FEV1/FVC, TLC, RV, VC

 MVV, MIP, DLCO

 Hypoventilation symptoms – PSG study

 Upper airway-
 swallowing, malnutrition, speech/swallow therapist
 Cough
 PCF, MEP, expiratory cough flow tracing

 ABG, CXR, need for ventilation in acute care

settings
ASSESS NEED FOR VENTILATOR SUPPORT
 Indication
 Time of onset of symptoms to admission <7 days
 Rapid progression to disability
 Bulbar dysfunction
 Bilateral facial weakness
 Inability to stand; unable to lift head/elbow off bed
 Dysautonomia
 Abnormal vital capacity/MIP/MEP
 Demyelination on neurophys testing
 Raised LFTs
 Serial decline >30% ( FVC, MIP, MEP, VC)

 Mechanical ventilation: cardiopulm arrest, resp distress,

ABG marked abnrmality, severe bulbar dysf, impaired
consciousness
MONITORING
 Acute Neuromuscular Disorders

 Chronic Neuromuscular Disorders

 Serial VC, MIP, ABG for hypercapnia
 Freq depends on progression of muscle weakness
 Need for noninvasive ventilation

 Monitor symptoms
 Exertional dyspnea, orthopnea, fatigue, non-refreshing
sleep, morning headeaches
LONG TERM MANAGEMENT
 Referred to pulmonologist
 Respiratory muscle training

 Assisted cough techniques

 Non-invasive ventilation
 Decision depends on disease, rate of progression,
impact of quality of life
CURRENT RESEARCH
 Distribution of acute neuromuscular respiratory
failure in ICU not formally evaluated

 85 patients Mayo Clinic ICU 2003-2009

 Acute neuromuscular resp failure requiring
mechanical ventilation

 55% of admissions had no known neuromuscular

diagnosis
 Longer duration mech vent, longer ICU stay, worsse
outcome at discharge
 12% had unknown diagnosis on discharge
 higher rates of severe disability
REFERENCE
 Archives of Neurology Journal
 Causes and outcomes of acute neuromuscular respiratory
failure – retrospective study
 Practical Neurology Journal
 Neuromuscular disease and respiratory failure – review article
 Textbook of Respiratory Medicine
 The respiratory system and neuromuscular diseases - Chapter
 UpToDate
 Respiratory muscle weakness due to neuromuscular disease:
clinical manifestations and evaluation