Objective:

To characterize the clinical and radiographic features of neurosarcoidosis of the cavernous sinus.

Background:

Neurosarcoidosis has been reported as a cause of cavernous sinus pathology in case reports, but the full scope of its manifestations remains unknown.

Design/Methods:

Clinical details, radiographic findings, and responsiveness to treatment are reported in cases of biopsy-proven sarcoidosis involving the cavernous sinus.

Results:

Ten biopsy-proven (5/10 neurologic) patients (median age of onset 53 years; female 6/10; Black 8/10) were included. Cavernous sinus disease was an inaugural manifestation in 7/10 with chronic presentations (median 90 days) most common (7/10). Symptoms referable to cavernous sinus involvement included pain (8/10), diplopia (3/10), facial sensory loss (3/10), and ischemic stroke (3/10). On MRI, disease was unilateral in 7/10 and homogeneously enhancing in all (10/10). Extension to adjacent structures was common, including Meckel’s cave (7/10), pituitary gland/infundibulum (6/10), pachymeninges (5/10), optic canal (5/10), and orbital apex (2/10). Adjacent bony destruction or lesional diffusion restriction was not seen. Non-cavernous lesions were present intracranially in 10/10 and in the spine in 2/5. Serum ACE level was elevated in 2 cases. At least one routine CSF parameter (nucleated cell count, protein, and/or glucose) was abnormal in 4/5 tested. Most were treated with corticosteroids (IV methylprednisolone 5/10, PO prednisone 8/10) and disease modifying treatments (one line 3/10, two lines 3/10, three lines 1/10). After a median follow-up of 45 months, 3/10 relapsed, the last EDSS was 4.5, and most were clinically improving (6/10).

Conclusions:

Neurosarcoidosis of the cavernous sinus presents chronically with pain, diplopia, and facial sensory loss, with the potential for strokes to be superimposed. It is rarely isolated to the cavernous sinus, often extending intracranially to adjacent structures on MRI, and is usually associated with markers of inflammation in the CSF. Deficits are often debilitating but can respond to treatment.