A pragmatic approach to diagnosing and treating neurosarcoidosis in the 21st century.

Objective: Neurosarcoidosis may be a serious complication of sarcoidosis. As the presentation of neurosarcoidosis is manifold, solitary nervous system sarcoidosis without systemic activity remains a difficult diagnosis. Appropriate treatment may be a dilemma.

Results: Most neurosarcoidosis patients present with neurological symptoms as the first manifestation. Whole-body fluorodeoxyglucose positron emission tomography has been found useful in neurological patients suspected of sarcoidosis. Small-fiber neuropathy is commonly associated with sarcoidosis and can cause significant morbidity to afflicted patients. New drugs such as antitumor necrosis factor alpha have been proven valuable in the treatment of neurosarcoidosis in different locations. Progressive multifocal leucencephalopathy should be considered in neurosarcoid patients, especially when treatment fails.

Conclusions: In this paper an update on clinical manifestations of neurosarcoidosis, diagnostic dilemmas, and therapeutic options is provided.