TY - CHAP M1 - Book, Section TI - Dejerine-Sottas Syndrome A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - Hereditary degenerative neurological disorder characterized primarily by peripheral motor nerves damage causing progressive muscle wasting, and affecting also sensitive and autonomic nervous components. The onset of the disease occurs during infancy or early childhood, and surely before the age of 3 years. The neuromuscular progression tends to be slow until the teenage years to, subsequently, accelerate with severe generalized disability. It is generally presumed that clinical course is severe, leading to wheelchair dependency at an early age. This form of neurodegeneration is more severe than observed in the other forms of Charcot-Marie-Tooth Disease. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/04/18 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164067673 ER -