TY - CHAP M1 - Book, Section TI - Progressive Bulbar Palsy of Childhood A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e 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. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=1164082414 ER -