RT Book, Section
A1 Douglas, Vanja C.
A1 Aminoff, Michael J.
A2 Papadakis, Maxine A.
A2 McPhee, Stephen J.
A2 Rabow, Michael W.
SR Print(0)
ID 1166171839
T1 Neuromyelitis Optica
T2 Current Medical Diagnosis and Treatment 2020
YR 2020
FD 2020
PB McGraw-Hill Education
PP New York, NY
SN 9781260455281
LK accessmedicine.mhmedical.com/content.aspx?aid=1166171839
RD 2022/05/18
AB This  disorder  is  characterized  by  optic  neuritis  and  acute  myelitis  with  MRI  changes  that  extend  over  at  least  three  segments  of  the  spinal  cord.  An  isolated  myelitis  or  optic  neuritis  may  also  occur.  Previously  known  as  Devic  disease  and  once  regarded  as  a  variant  of  multiple  sclerosis,  neuromyelitis  optica  is  associated  with  a  specific  antibody  marker  (NMO-IgG)  targeting  the  water  channel  aquaporin-4  in  80%  of  cases,  and  with  antibodies  to  myelin  oligodendrocyte  glycoprotein  (MOG-IgG)  in  approximately  33%  of  NMO-IgG  seronegative  patients.  MRI  of  the  brain  typically  does  not  show  widespread  white  matter  involvement,  but  such  changes  do  not  exclude  the  diagnosis.  Treatment  is  by  long-term  immunosuppression.  First-line  therapy  is  with  rituximab  (two  1  g  intravenous  infusions  spaced  by  2  weeks,  or  four  weekly  infusions  of  375  mg/m2;  re-dosing  may  occur  every  6  months  or  when  CD19/20-positive  or  CD27-positive  lymphocytes  become  detectable),  mycophenolate  mofetil  (500–1500  mg  orally  twice  daily,  titrated  until  the  absolute  lymphocyte  count  falls  below  1500/mcL),  or  azathioprine  (2.5–3  mg/kg  orally).  Acute  relapses  are  treated  with  corticosteroids  at  doses  similar  to  those  outlined  for  multiple  sclerosis  and  with  plasma  exchange  for  severe  relapses  unresponsive  to  corticosteroids.