PMID- 17052732
OWN - NLM
STAT- MEDLINE
DCOM- 20070222
LR  - 20220331
IS  - 0022-510X (Print)
IS  - 0022-510X (Linking)
VI  - 251
IP  - 1-2
DP  - 2006 Dec 21
TI  - Treatment of Susac syndrome with gamma globulin and corticosteroids.
PG  - 17-22
AB  - Susac syndrome is a rare vasculopathy characterized by visual, hearing, and 
      cognitive dysfunction. Optimal treatment is unknown, but many patients require 
      chemotherapy to control disease activity. We describe two patients with Susac 
      syndrome and their response to intravenous immune globulin (IVIg) and 
      corticosteroids. Both patients improved following acute treatment with IVIg and 
      intravenous methylprednisolone (IVMP), and no further relapses were observed. One 
      patient showed significant improvement in hearing and MRI lesions shortly 
      following acute treatment. Treatment with IVIg and corticosteroids provides a 
      therapeutic option that avoids the toxicities of chemotherapy and suggests the 
      possible importance of pathologic antibodies in the pathogenesis of Susac 
      syndrome.
FAU - Fox, Robert J
AU  - Fox RJ
AD  - Mellen Center, Department of Neurology, U-10, Cleveland Clinic Foundation, 
      Cleveland, OH 44195, USA. foxr@ccf.org
FAU - Costello, Fiona
AU  - Costello F
FAU - Judkins, Alexander R
AU  - Judkins AR
FAU - Galetta, Steven L
AU  - Galetta SL
FAU - Maguire, Albert M
AU  - Maguire AM
FAU - Leonard, Brian
AU  - Leonard B
FAU - Markowitz, Clyde E
AU  - Markowitz CE
LA  - eng
GR  - K23 NS 47211/NS/NINDS NIH HHS/United States
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
DEP - 20061017
PL  - Netherlands
TA  - J Neurol Sci
JT  - Journal of the neurological sciences
JID - 0375403
RN  - 0 (Adrenal Cortex Hormones)
RN  - 0 (Immunoglobulins, Intravenous)
SB  - IM
MH  - Adrenal Cortex Hormones/*therapeutic use
MH  - Adult
MH  - Agnosia/*drug therapy
MH  - Drug Therapy, Combination
MH  - Female
MH  - Functional Laterality
MH  - Hearing Disorders/complications/*drug therapy/pathology
MH  - Hearing Tests/methods
MH  - Humans
MH  - Immunoglobulins, Intravenous/*therapeutic use
MH  - Magnetic Resonance Imaging
MH  - Male
MH  - Retinal Artery Occlusion/complications/*drug therapy/pathology
MH  - Syndrome
EDAT- 2006/10/21 09:00
MHDA- 2007/02/23 09:00
CRDT- 2006/10/21 09:00
PHST- 2006/05/31 00:00 [received]
PHST- 2006/08/11 00:00 [revised]
PHST- 2006/08/16 00:00 [accepted]
PHST- 2006/10/21 09:00 [pubmed]
PHST- 2007/02/23 09:00 [medline]
PHST- 2006/10/21 09:00 [entrez]
AID - S0022-510X(06)00362-5 [pii]
AID - 10.1016/j.jns.2006.08.007 [doi]
PST - ppublish
SO  - J Neurol Sci. 2006 Dec 21;251(1-2):17-22. doi: 10.1016/j.jns.2006.08.007. Epub 
      2006 Oct 17.