RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Engelhardt, Thomas
SR Print(0)
ID 1164072579
T1 Hereditary Motor Sensory Neuropathies (HMSNs): An Overview
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=1164072579
RD 2024/04/19
AB Hereditary  Motor  Sensory  Neuropathy  (HMSN)  is  a  name  given  to  a  group  of  different  neuropathies  that  are  all  characterized  by  their  implications  on  both  the  afferent  and  efferent  neural  communication.  HMSNs  are  characterized  by  atypical  neural  development  and  degradation  of  neural  tissue.  The  two  common  forms  of  HMSNs  are  either  hypertrophic  demyelinated  nerves  or  complete  atrophy  of  neural  tissue.  Hypertrophic  condition  causes  neural  stiffness  and  a  demyelination  of  nerves  in  the  peripheral  nervous  system,  whereas  the  complete  atrophy  of  neural  tissues  causes  the  breakdown  of  axons  and  neural  cell  bodies.  Patients  affected  with  this  pathophysiological  condition  experience  progressive  muscle  atrophy  and  sensory  neuropathy  of  the  extremities.  Originally,  the  term  “hereditary  motor  and  sensory  neuropathy”  was  historically  used  to  denote  the  more  common  form  called  Charcot-Marie-Tooth  (CMT)  Disease.  With  the  identification  of  a  wide  number  of  genetically  and  phenotypically  distinct  forms  of  CMT,  the  term  HMSN  is  now  used  to  describe  a  larger  number  of  medical  conditions  (see  Table  H-3).