Imaging CNS autoimmune and inflammatory disease
Masslike inflammatory disease
This is the third lecture in a case based review of imaging of the brain and spine for autoimmune and inflammatory conditions. We will cover the MRI findings of some of the common conditions and some potential pitfalls and mimics.
This lecture covers two masslike inflammatory diseases which can occur in the orbit and brain. These don’t really fit well into other categories with the exception that they are more likely to have a mass and adjacent expansion.
Orbital inflammatory disease
Orbital inflammatory disease is an infiltrative disease most often seen in the orbit and anterior skull base. It is characterized by masslike replacement of the orbital contents, sometimes with extension in the orbits. It is often T2 intermediate to dark and has avid enhancement. Many cases are associated with IgG4 abnormalities, while other cases are purely idiopathic. This disease was previously called orbital pseudotumor, but it is more exact now to call it IgG4 orbital disease or idiopathic orbital inflammation. The differential diagnosis for this appearance includes metastatic disease, lymphoma, and sarcoidosis.
Sarcoidosis
Sarcoid can also affect the brain, orbits, and spine, and when it does is referred to as neurosarcoidosis. The most common manifestation is leptomeningeal enhancement, but it can be accompanied by parenchymal nodules. It preferentially affects the basal cisterns and skull base. When you have a basilar meningitis, the differential includes metastatic disease (leptomeningeal carcinomatosis), unusual infections (such as fungi, tuberculosis, and other atypical organisms), and sarcoidosis, as was the case here.
Summary and Conclusion
In summary, when you have nodular masslike enhancement centered in the skull base or orbits, there is a pretty limited differential diagnosis. The inflammatory differential includes sarcoidosis and orbital inflammatory disease. Other things such as metastatic disease and atypical infections are also considerations.
The level of this lecture is appropriate for radiology residents, radiology fellows, and trainees in other specialties, such as neurology, who have an interest in neuroradiology or may see patients with CNS demyelinating or inflammatory conditions.