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Respiratory apraxia in amyotrophic lateral sclerosis
Susana Pinto1, Anabela Pinto, António Atalaia
1Neuromuscular Unit, Institute of Molecular Medicine, Lisbon, Portugal.
Summary
Amyotrophic lateral sclerosis (ALS) can cause respiratory apraxia, a condition affecting voluntary breathing control. Electromyography (EMG) of respiratory muscles can help detect this specific dysfunction in ALS patients.
Area of Science:
- Neurology
- Respiratory Medicine
Background:
- Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease primarily affecting motor neurons.
- Respiratory dysfunction is a common and critical complication in ALS patients, significantly impacting prognosis.
Observation:
- A case study of a 74-year-old female diagnosed with ALS presenting with progressive bulbar symptoms (dysarthria, dysphagia).
- The patient exhibited irregular volitional inspiratory movements upon command, characteristic of respiratory apraxia, while automatic breathing remained rhythmic.
- No signs of dementia or apraxia in limb or buco-facial movements were observed.
Findings:
- Electromyography (EMG) of respiratory muscles, including the diaphragm, revealed irregular phasic activity during volitional inspiration, confirming respiratory apraxia.
- Despite rapid clinical deterioration, no cognitive decline or other apraxic symptoms emerged.
Implications:
- This case highlights respiratory apraxia as a potential manifestation of ALS, particularly with bulbar onset.
- EMG of respiratory muscles is a valuable diagnostic tool for identifying respiratory apraxia in ALS.
- Understanding and identifying respiratory apraxia can aid in the comprehensive management of ALS patients.
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