Objective:

To clinically characterize perivascular disease in neurosarcoidosis.

Background:

Granulomas in neurosarcoidosis occur commonly in the perivascular Virchow-Robin spaces and may result in perivascular enhancement on MRI, a finding that has previously been associated with ischemic stroke and intraparenchymal hemorrhage.

Design/Methods:

Adult patients with probable or definite neurosarcoidosis were included if their MRIs demonstrated perivascular enhancement, and their clinical presentations, serum and CSF testing results, MRI findings, treatment responsiveness, and outcomes were examined.

Results:

Sixteen patients were included (average age 41.8 years; 9 female; 13 Black, 2 White, 1 unknown race; 11 probable and 5 definite diagnoses). Perivascular disease was the dominant disease manifestation in 4 patients. Common symptoms included headache (13), extremity numbness (7), memory loss (6), and seizures (5). Perivascular enhancement on MRI most frequently affected the deep medullary veins (13), lenticulostriate arteries (8), and cortical pial vessels (5). Larger cerebral arteries were enhancing in 2. Ischemic stroke and intraparenchymal hemorrhage occurred in 6 and 1 patients, respectively. Co-existing MRI findings included leptomeningitis (12), brain parenchymal disease (9), and cerebral white matter disease (7). CSF was obtained in 13 patients: lymphocytic pleocytosis in 10/10, elevated protein in 12/13, and hypoglycorrhachia in 2/13. Thirteen patients were treated acutely with either IV or oral corticosteroids. Nonsteroidal maintenance treatments were used in sixteen patients, with eight patients requiring 3 or more lines of treatment. The average modified Rankin scale score and Expanded Disability Status Scale score at onset were 0.60 and 1.57, respectively, which worsened to 1.08 and 1.85, respectively, by last follow-up (average follow-up 107 months).  

Conclusions:

Perivascular disease in neurosarcoidosis principally affects the deep medullary veins and lenticulostriate arteries and presents with headache, cognitive impairment, and seizures. Disease may be treatment refractory and complicated by ischemic stroke and intracranial hemorrhage.