Sarcoidosis and the Nervous System

Sarcoidosis is a multisystem noninfectious granulomatous disorder that is immune mediated, reflecting the response to an as-yet unidentified antigen or antigens. Neurosarcoidosis refers to neurologic involvement due to sarcoidosis that clinically manifests in 5% of cases of sarcoidosis, with asymptomatic involvement in as many as another one in five patients with sarcoidosis. Sarcoid granulomas can occur in any anatomic substrate in the nervous system, causing protean manifestations that have earned neurosarcoidosis the sobriquet the great mimic. Nevertheless, central nervous system sarcoidosis occurs in well-defined presentations that can be classified as cranial neuropathies, meningeal disease, brain parenchymal (including pituitary-hypothalamic) disease, and spinal cord disease. In addition, the peripheral nervous system is affected in the form of peripheral neuropathy and myopathy. Glucocorticoids are the cornerstone of treatment, especially in the acute stage, whereas steroid-sparing agents such as methotrexate, mycophenolate mofetil, and azathioprine are used for prolonged therapy to minimize steroid toxicity. Anti-tumor necrosis factor agents may help in refractory cases.