ABSTRACT
Hypoventilation in neuromuscular disease is attributed to both respiratory muscle
weakness and reduced chemoreceptor sensitivity essential in ventilatory drive. Acute
or chronic respiratory failure is seen in a spectrum of neuromuscular disease; whereas
some are treatable others are progressive and devastating. Sleep is associated with
a reduction in ventilation and hence worsening hypoventilation. Problems with sleep
may be an early indicator of further muscle weakness and should prompt the clinician
for further investigation, though usefulness of pulmonary function testing, arterial
blood gas analysis, and other measures may not be universally predictive. The timing
of respiratory failure is variable, but knowledge of the clinical aspects, pathogenesis,
and treatment of respiratory failure and hypoventilation may be helpful in evaluating
the patient with neuromuscular disease. For those with progressive and terminal disease,
additional factors such as end of life care, especially ventilation and cough, may
be useful for the patient, caregivers, and treating medical personnel.
KEYWORDS
Hypoventilation - sleep - neuromuscular disease
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Shirley F JonesM.D.
Department of Internal Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine
Scott and White Memorial Hospital/Texas A&M Health Science Center, 2401 South 31st.
St., Temple, TX 76508
Email: shjones@swmail.sw.org