TY - CHAP M1 - Book, Section TI - Urbach-Wiethe Syndrome A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - A congenital lipoid storage disease with multiple tissue infiltrations producing waxiness and thickening of the skin and mucous membranes of the mouth, pharynx, larynx, and hypopharynx. It causes hoarseness and an inability to cry, often from birth. Intracranial calcifications selectively affect the amygdala resulting in abnormalities of fear processing, affect and cognition, anxiety, and memory. Clinically patients demonstrate grand mal epilepsy, attacks of rage, and mental retardation. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=1164087194 ER -