Abstract
A patient with a diagnosis of intermediate or benign spinal muscular atrophy was severely incapacitated from respiratory complications and alveolar hypoventilation and was confined to mechanical ventilatory support. After extensive diagnostic evaluation of her pulmonary status, including transcutaneous stimulation of the phrenic nerves associated with nasogastric and surface electrode recording of the diaphragmatic response, this patient had a phrenic neurostimulation system implanted bilaterally for diaphragm pacing. Although spinal muscular atrophy has not been previously recognized as an indication for diaphragm pacing, her subsequent social, physical, and psychological improvement indicate that it may be significantly beneficial in selected cases of alveolar hypoventilation due to spinal muscular atrophy or neuromuscular disease.