RT Book, Section
A1 Wells, Robert G.
SR Print(0)
ID 1148948978
T1 Autoimmune Disorders of the Brain
T2 Diagnostic Imaging of Infants and Children
YR 2015
FD 2015
PB McGraw-Hill Education
PP New York, NY
SN 9780071808392
LK accesspediatrics.mhmedical.com/content.aspx?aid=1148948978
RD 2024/04/20
AB Acute  disseminated  encephalomyelitis  (ADEM)  is  an  immune-mediated  central  nervous  system  (CNS)  disorder.  Patients  with  ADEM  have  multifocal  inflammatory  (but  noninfectious)  lesions  within  the  brain  or  spinal  cord.  There  is  usually  a  history  of  a  preceding  upper  respiratory  infection  or  immunization  within  a  few  weeks  of  onset  of  the  neurological  manifestations.  The  pathophysiology  likely  involves  an  excessive  immunological  response  that  attacks  myelin.  The  histological  features  include  infiltration  of  monocytoid  cells  and  perivenous  demyelination.  Patients  with  ADEM  usually  experience  a  sudden  onset  of  multifocal  neurological  signs  and  symptoms.  The  CNS  lesions  may  completely  subside  within  a  few  weeks,  or  progress  to  glial  scars.  Important  considerations  in  the  diagnosis  of  ADEM  include  the  clinical  presentation,  cerebrospinal  fluid  (CSF)  analysis,  neuroimaging  studies,  and  the  absence  of  another  definable  cause  of  the  symptoms.  The  clinical  differential  diagnosis  includes  multiple  sclerosis,  leukodystrophy,  and  mitochondrial  encephalomyopathy.  ADEM  is  a  monophasic  disorder.  Multiphasic  disseminated  encephalomyelitis  refers  to  a  relapsing  form.1–4