RT Book, Section
A1 Amato, Anthony A.
A1 Russell, James A.
SR Print(0)
ID 1115657111
T1 Amyotrophic Lateral Sclerosis
T2 Neuromuscular Disorders, 2e
YR 2015
FD 2015
PB McGraw-Hill Education
PP New York, NY
SN 9780071752503
LK neurology.mhmedical.com/content.aspx?aid=1115657111
RD 2024/04/19
AB The  motor  neuron  diseases  (MNDs)  are  categorized  by  their  pathological  affinity  for  the  voluntary  motor  system  including  anterior  horn  cells,  certain  motor  cranial  nerve  nuclei,  and  corticospinal/bulbar  tracts.  Amyotrophic  lateral  sclerosis  (ALS),  also  known  as  Lou  Gehrig  disease,  is  the  most  notorious  of  these  disorders.  The  boundaries  of  what  is  and  what  is  not  ALS,  particularly  in  the  context  of  early  diagnosis  of  individual  patients,  remain  imprecise.  In  this  Chapter,  in  an  attempt  to  distinguish  ALS  from  other  MNDs,  we  consider  ALS  to  be  a  disorder  that  has  the  following  characteristics:  (1)  the  clinical  manifestations  are  dominated  by  signs  attributable  to  voluntary  motor  system  dysfunction,  (2)  the  disease  progresses  rapidly  both  within  and  between  different  body  regions,  (3)  that  life  expectancy  is  <5  years  from  clinical  onset  in  the  vast  majority  of  cases,  (4)  and  that  no  other  etiology  can  be  identified.