T his  report  present  a  rare  case  of  adult-onset  severe  Acute  Disseminated  Encephalomyelitis (ADEM)  complicated  by  uncal  herniation,  which  is  a  very  rare  complication  of  the  disease, requiring  lifesaving  decompressive  hemicraniectomy  with  complete  recovery.  This  is  a  27–year  -old female,  who  presented  with  headache,  right-sided  body  weakness,  dysarthria,  blurred  vision,  and progressive  altered  level  of  consciousness  over  a  course  of  2  days,  preceded  by  an  upper  respiratory tract  infection  7  days  prior  to  the  onset  of  the  above  -  mentioned  symptoms.  Brain  MRI  showed extensive  abnormal  white  matter  lesions  which  appear  hyperintense,  involving  occipital,  parietal, and  temporal  lobes,  more  on  the  left  side.  CSF  exam  showed  high  protein.  According  to  the  clinical and  MRI  findings,  she  was  diagnosed  as  severe  ADEM.  She  was  started  on  high  dose  steroids  and plasma  exchange.  Unfortunately,  her  condition  deteriorated  rapidly  with  sudden  drop  in  her  level  of consciousness  with  fixed  dilated  pupils.  Urgent  imaging  showed  uncal  herniation.  Lifesaving  urgent left  decompressive  hemicraniectomy  was  performed  with  subsequent  resolution  of  herniation radiologically  and  clinically.  She  was  kept  on  steroid  and  plasmapheresis,  followed  by  Intravenous Immunoglobulin  (IVIG)  with  gradual  improvement.  After  one  month,  she  completely  recovered and  became  independent  in  her  activities,  with  significantly  improved  MRI  lesions.  In  conclusions, severe  ADEM  complicated  by  uncal  herniation  is  an  extremely  rare  presentation  of  the  disease. Urgent  and  aggressive  interventions,  including  decompressive  surgeries,  can  save  lives  and  lead  to favorable outcomes.

Title

Annals of Clinical Case Reports

Publisher

Remedy Publications

Publisher Country

United States of America

Publication Type

Online only

Volume

6

Year

2021

Pages

3