Neurosarcoidosis: presentations and management

Sarcoidosis affects the central nervous system more frequently than previously appreciated. The diagnosis of neurosarcoidosis is often delayed, potentially leading to serious complications. Symptoms, when present, are not specific, may be subtle and resemble those of other neurologic diseases.

During the past decade, significant progress has been made in understanding the epidemiology and pathophysiology of neurosarcoidosis, as well as the ability to diagnose and treat this disease. Studies have shown that the optimal diagnostic imaging modality for neurosarcoidosis is magnetic resonance imaging with gadolinium as it enhances visualization of granulomatous infiltration in neural tissue. Subclinical neurosarcoidosis may not be uncommon in patients with sarcoidosis. It is now evident that neurosarcoidosis does not invariably present as a catastrophic event. Adverse effects associated with high-dose systemic corticosteroids, the standard therapy, have discouraged practitioners from initiating treatment in the absence of significant symptomatic neurologic disease. However, other immunosuppressive agents as well newer biologic agents have emerged as an effective, well-tolerated therapeutic alternative to corticosteroids, which are often effective in corticosteroid-recalcitrant cases.

Neurologists should be aware of the varying presentations of neurosarcoidosis since early recognition of neurologic involvement in patients with undiagnosed or proven sarcoidosis is currently possible and critical to the prevention of disabling complications.