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IgG4-related inflammatory pseudotumor of the central nervous system responsive to mycophenolate mofetil

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posted on 20.06.2012 by Heather E. Moss, Luis Mejico, Gustavo de la Roza, Thomas M. Coyne, Steven L. Galetta, Grant T. Liu
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.

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NOTICE: this is the author’s version of a work that was accepted for publication in Journal of the Neurological Sciences. Changes resulting from the publishing process, such as peer review, editing, corrections, structural formatting, and other quality control mechanisms may not be reflected in this document. Changes may have been made to this work since it was submitted for publication. A definitive version was subsequently published in Journal of the Neurological Sciences, [Vol 318, Issue 1-2, (15 July 2012)] http://dx.doi.org/10.1016/j.jns.2012.04.010

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Elsevier

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en_US

issn

0022-510X

Issue date

01/04/2012

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