This is a thorough review of the state-of-the-art in 2005.

The cause(s) and mechanism(s) of neurosarcoidosis, a multi- system granulomatous disease, remain unknown. Nervous system involvement is rare but serious. The paper reviews the features of lymphocytic meningitis, psychiatric disorders, diabetes insipidus and cranial nerves palsies which are the most frequently encountered central manifestations.

CSF examination and and gadolinium enhances MRimaging of the brain and cervical cord are the initial examinations that need to be performed. If possible, but in some cases only, histological evidence of a non-caseating granuloma must be obtained by neuromuscular, meningeal or cerebral biopsy. Early corticosteroid therapy is indicated for life threatening situations and in cases of focal dysfunction. In worsening cases or in those that do not respond to therapy or have a poor tolerance to corticosteroids, other immunosuppressive agents should be considered.

Maintenance therapy, often life-long, prevents relapse and maintains treatment derived benefits.

148 references.