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Motor neuron disease presenting with respiratory failure
M de Carvalho1, T Matias, F Coelho
1Department of Neurology, EMG Laboratory, Hospital de Santa Maria, Lisbon, Portugal.
Journal of the Neurological Sciences
|August 1, 1996
Summary
Amyotrophic lateral sclerosis (ALS) can initially present as acute respiratory failure due to diaphragm weakness. Some patients may improve with rest and ventilatory support, avoiding permanent ventilator dependence.
Area of Science:
- Neurology
- Pulmonology
- Neuron science
Background:
- Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease.
- Respiratory failure is the primary cause of mortality in ALS patients.
- Diagnosis of ALS based on respiratory insufficiency is rare.
Observation:
- Four cases of ALS presenting with acute respiratory failure are reported.
- Electromyography (EMG) in three patients revealed severe denervation of the hemidiaphragms.
- A review of 25 previously described patients showed frequent upper limb weakness and diaphragm involvement, with less frequent bulbar dysfunction.
Findings:
- Rapidly progressive ventilatory failure can be a prominent early sign of ALS.
- Diaphragm weakness is a key factor in acute respiratory failure in these patients.
- Prognosis may not always involve permanent ventilator dependence.
Implications:
- This suggests that acute respiratory failure can be a presenting symptom of ALS.
- Early recognition of diaphragm dysfunction is crucial for diagnosis.
- Non-invasive ventilatory assistance and rest may lead to significant improvement, altering the disease course.