[Central nervous system involvement of sarcoidosis: diagnosis and management]

Sarcoidosis is a multisystem inflammatory granulomatous disease that affects the central nervous system (CNS). CNS Involvement occurs in a relatively small number of patients with sarcoidosis but is potentially serious manifestation. Diagnostic criteria usually include histologic identification of a noncaseating epithelioid granuloma, supportive laboratory or neuroimaging tests or both, and a compatible clinical course. Diagnostic criteria proposed by "The Diagnosis Guideline for Sarcoidosis in Japan -2006" can distinguish definite, probable, and possible neurosarcoidosis, and may be now commonly used. Patients with a definite or probable diagnosis of CNS sarcoidosis should start treatment promptly. The pharmacologic regimens for the treatment of neurosarcoidosis are not standardized since no prospective, randomized, controlled trials have been performed in the World. Corticosteroids, however, are typically the first line or therapy and approximately half of patients have substantial benefit. For patients who are refractory to or intolerant of corticosteroid therapy, second-line agents include azathioprine, methotrexate, cyclosporine, cyclophosphamide, and mycophenolate mofetil (MMF). Treatment regimens vary in term of doing, maintenance, and tapering. Radiotherapy or neurosurgical treatment is indicated when medications fail or when life-threatening emergences, such as severe hydrocephalus and elevated intracranial pressure, arise. The combination of infliximab, an anti-TNF-alpha neutralizing antibody, and MMF has recently been used to treat neurosarcoidosis. Treatment option will likely evolve as well-designed studies are undertaken.