TY  - CHAP
M1  - Book, Section
TI  - The Patient With Neuromuscular, Skeletal Disease, or Movement Disorders
A1  - Easdown, L. Jane
A1  - Lee, Lorri A.
A2  - Longnecker, David E.
A2  - Mackey, Sean C.
A2  - Newman, Mark F.
A2  - Sandberg, Warren S.
A2  - Zapol, Warren M.
PY  - 2017
T2  - Anesthesiology, 3e
AB  - KEY  POINTSNeuromuscular  disorders  (NMDs)  are  rare,  are  difficult  to  study,  and  have  significant  anesthetic  implications  for  perioperative  management.  These  include  diseases  of  the  motor  neurons,  peripheral  and  autonomic  nerves,  neuromuscular  junction,  and  myopathies.Patients  with  NMDs  may  have  life-threatening  contraindications  to  hydrocarbon-based  volatile  anesthetics  and  neuromuscular  blocking  drugs,  particularly  succinylcholine,  with  risks  of  prolonged  muscle  weakness,  hyperkalemia,  rhabdomyolysis,  or  malignant  hyperthermia  (MH).The  use  of  regional  anesthesia,  spinal  or  epidural,  is  reported  in  case  series  in  most  types  of  NMDs.  There  is  concern  for  local  anesthetic  neurotoxicity  with  demyelinating  NMDs  such  as  multiple  sclerosis  and  Guillain-Barré  disease.The  leading  causes  of  death  for  patients  with  NMDs  are  respiratory  failure  and  aspiration  pneumonia.  Preoperative  consultation  with  a  neurologist  or  pulmonologist  to  optimize  these  patients,  depending  on  severity  of  disease,  may  improve  outcomes.  Coordination  of  the  perioperative  care  by  the  patient’s  primary  care  provider  should  facilitate  recovery.Some  patients  with  NMDs  have  cardiac  muscle  affected,  leading  to  dilated  cardiomyopathy,  arrhythmias,  and  valvular  regurgitation.  The  degree  of  skeletal  muscle  involvement  does  not  necessarily  correlate  with  the  severity  of  cardiac  involvement.Patients  with  NMDs  may  have  autonomic  dysfunction  with  delayed  gastrointestinal  emptying,  resting  tachycardia,  and  orthostatic  hypotension  and  may  exhibit  severe  hemodynamic  instability.Patients  with  NMDs,  particularly  those  with  advanced  disease,  should  have  a  discussion  of  advanced  directives.In  patients  with  myasthenia  gravis,  the  signs  and  symptoms  of  toxicity  with  a  cholinergic  crisis  include  salivation,  hyperhidrosis,  tearing,  bradycardia,  and  miosis,  and  it  can  be  difficult  to  differentiate  from  a  myasthenic  crisis;  however,  the  vast  majority  of  events  are  caused  by  a  myasthenic  crisis.The  presenting  signs  of  Duchenne  muscular  dystrophy  (MD)  in  pediatric  males  might  be  acute  hyperkalemia  and  asystole  with  succinylcholine  or  rhabdomyolysis  in  Becker  MD.The  hyperdynamic  reaction  noted  in  some  patients  with  Duchenne  or  Becker  MD  has  certain  aspects  that  are  similar  to  MH.  Some  authors  call  this  reaction  anesthesia-induced  rhabdomyolysis  (AIR).Exacerbations  of  multiple  sclerosis  during  the  perioperative  period  have  been  linked  to  stress,  delivery,  and  temperature  fluctuations.The  major  anesthetic-related  concerns  for  patients  with  skeletal  disorders  are  the  stability  of  the  cervical  spine  and  airway  management  and  safe  positioning.Patients  treated  for  Parkinson  disease  have  an  increased  incidence  of  dysphagia,  aspiration  pneumonia,  and  falls.  They  are  at  risk  for  neuroleptic  malignant  syndrome  and  serotonin  syndrome.Patients  with  Marfan  syndrome  should  have  an  assessment  of  the  ascending  aorta  and  cardiac  valves  prior  to  surgery.Patients  with  Ehlers-Danlos  syndrome  may  be  difficult  to  intubate  because  of  temporomandibular  joint  dislocation  or  atlantoaxial  laxity.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/24
UR  - accessanesthesiology.mhmedical.com/content.aspx?aid=1144112550
ER  -