Neurodevelopmental and Neuromuscular Disorders in Critical Care
Neuromuscular crises
Know Guillain-Barré syndrome (ascending weakness, areflexia, albuminocytologic dissociation), myasthenia gravis and crisis (fatigable weakness, ptosis, respiratory failure), and spinal muscular atrophy. Recognize that respiratory failure may precede limb weakness, so monitor vital capacity, negative inspiratory force, and cough strength rather than relying on oxygen saturation alone. Anticipate autonomic instability in Guillain-Barré and avoid succinylcholine and certain anesthetics in neuromuscular disease due to hyperkalemia and malignant hyperthermia risk.
Chronic and developmental conditions
Understand how cerebral palsy, epilepsy, mitochondrial disease, and metabolic disorders affect critical care: altered baseline exam, aspiration risk, and drug sensitivities. Recognize that children with developmental disability may have atypical presentations and communication needs. Consider baseline function when judging deterioration.
Supportive care
Focus on airway clearance, secretion management, nutrition, pressure injury prevention, and early rehabilitation. Coordinate with families who know the child's baseline. Monitor for complications such as aspiration pneumonia, autonomic dysreflexia-like instability, and thrombosis.
Traps
Questions test that pulse oximetry can be normal despite failing ventilation, that weakness from Guillain-Barré can ascend to bulbar muscles, and that medication reactions differ in neuromuscular disease.
Rule of thumb
In neuromuscular weakness, trend respiratory mechanics (vital capacity, inspiratory force) and intubate for declining trend, not for falling saturation alone.