RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164082414 T1 Progressive Bulbar Palsy of Childhood T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accessanesthesiology.mhmedical.com/content.aspx?aid=1164082414 RD 2024/04/19 AB A very rare inherited condition characterized by progressive degeneration of bulbar nuclei and anterior horn cells of the cranial nerves with little or no involvement of the spinal cord. Sensorineural deafness and respiratory compromise. Clinical features include significant atrophy of muscles innervated by cranial nerves and corticobulbar tracts, dysphagia, ptosis, bilateral facial weakness, absent gag reflex, and hyperreflexia.