Abstract
IgG4-related disease is characterized by histologic fibrosis with IgG4-positive plasma cell infiltration. Our study evaluated MR imaging features of IgG4-related disease in the head and neck and brain. Images from 15 patients were retrospectively evaluated for the location, signal intensity, and enhancement patterns of lesions. Lacrimal gland enlargement was observed in 8 cases. Other lesions included orbital pseudotumor in 5, pituitary enlargement in 5, and cranial nerve enlargement in 7; the infraorbital nerve was involved in 4. All lesions were hypointense on T2-weighted images, which is typical for IgG4-related lesions. Multiple sites were involved in the head and neck and brain in 11 patients. The diagnosis of IgG4-related disease should be considered in a patient presenting with T2 hypointense lacrimal gland, pituitary, or cranial nerve enlargement, or a T2 hypointense orbital mass, especially if multiple sites in the head and neck are involved in the presence of elevated serum IgG4.
Highlights
In 11 of the 15 patients, multiple sites were involved in the HN and brain
We observed enlargement of the lacrimal gland in 8 cases of MD; according to the 2010 criteria for IgG4-RD, we recognize that these patients probably had IgG4-related disease of the HN.[18]
Thickening of branches of the trigeminal nerve was a common periorbital finding, with preferential involvement of the infraorbital nerve noted in our cases
Summary
This study evaluated MR imaging features of IgG4-RD in the HN and brain, using data collected from multiple institutions. Symptoms associated with the HN or brain lesions in the 15 patients were exophthalmos (n ϭ 10), visual acuity loss (n ϭ 2), headache (n ϭ 2), 2136 Toyoda AJNR 33 ͉ Dec 2012 ͉ www.ajnr.org
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