Neurosarcoidosis

Involvement of the nervous system by sarcoidosis (or neurosarcoidosis) is not an infrequent extrapulmonary manifestation of sarcoidosis and a driver of morbidity and mortality in the disease. Any part of the nervous system can be affected, resulting in heterogeneity of clinical presentation. Diagnosis of neurosarcoidosis can be challenging due to inaccessibility of tissue confirmation. Possible and probable neurosarcoidosis are often diagnosed based on the presence of extraneural sarcoidosis and evidence of CNS inflammation from imaging study and cerebrospinal fluid analysis. Spontaneous remission for moderate-to-severe neurosarcoidosis is rare, and therefore, treatment is normally required. High-dose glucocorticoids followed by a prolonged taper is the backbone treatment. Concomitant use of steroid-sparing agents and/or biologic agents are usually required due to risk of relapse during/after taper and to limit long-term side effects of glucocorticoids.