Document Detail

IgG4-related inflammatory pseudotumor of the central nervous system responsive to mycophenolate mofetil.
MedLine Citation:
PMID:  22546342     Owner:  NLM     Status:  MEDLINE    
Orbital apex and skull base masses often present with neuro-ophthalmic signs and symptoms. Though the localization of these syndromes and visualization of the responsible lesion on imaging is typically straightforward, definitive diagnosis usually relies on biopsy. Immunohistochemistry is important for categorization and treatment planning. IgG4-related disease is emerging as a pathologically defined inflammatory process that can occur in multiple organ systems. We present two patients with extensive inflammatory mass lesions of the central nervous system with immunohistochemistry positive for IgG4 and negative for ALK-1 as examples of meningeal based IgG4-related inflammatory pseudotumors. In both patients, there was treatment response to mycophenolate mofetil.
Heather E Moss; Luis J Mejico; Gustavo de la Roza; Thomas M Coyne; Steven L Galetta; Grant T Liu
Publication Detail:
Type:  Case Reports; Journal Article; Research Support, N.I.H., Extramural     Date:  2012-04-28
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  318     ISSN:  1878-5883     ISO Abbreviation:  J. Neurol. Sci.     Publication Date:  2012 Jul 
Date Detail:
Created Date:  2012-06-01     Completed Date:  2013-03-28     Revised Date:  2014-09-08    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  31-5     Citation Subset:  IM    
Copyright Information:
Copyright © 2012 Elsevier B.V. All rights reserved.
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MeSH Terms
Central Nervous System / immunology,  pathology*
Granuloma, Plasma Cell / drug therapy*,  pathology*
Immunoglobulin G / biosynthesis*
Immunosuppressive Agents / administration & dosage
Meningitis / drug therapy*,  pathology
Middle Aged
Mycophenolic Acid / administration & dosage,  analogs & derivatives*
Grant Support
K12 EY021475/EY/NEI NIH HHS; K12 EY021475-01/EY/NEI NIH HHS; K12EY021475/EY/NEI NIH HHS
Reg. No./Substance:
0/Immunoglobulin G; 0/Immunosuppressive Agents; 9242ECW6R0/mycophenolate mofetil; HU9DX48N0T/Mycophenolic Acid

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