RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Engelhardt, Thomas
SR Print(0)
ID 1164067673
T1 Dejerine-Sottas Syndrome
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=1164067673
RD 2024/04/19
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.