[Neurosarcoidosis - inflammatory disease that affects the nervous system].

Sarcoidosis is a rare granulomatous inflammatory disease of unknown etiology that affects the nervous system in 5-10% of cases, which is referred to as neurosarcoidosis. The clinical symptoms depend on the localization and the extent of the lesions. Cranial nerves and leptomeninges are the most common sites affected. In rare cases, the disease can also affect the peripheral nervous system. In the updated 2018 classification, the disease is defined as »definite neurosarcoidosis« when confirmed by a biopsy from the nervous system. »Probable neurosarcoidosis« is defined by typical clinical findings and positive pathological findings in biopsy from systemic manifestations of sarcoidosis. »Possible neurosarcoidosis« is diagnosed when clinical observations suggest sarcoidosis, exclude alternative diagnoses, and there is no pathological confirmation. The clinical evaluation includes a thorough medical history, MRI of the central nervous system (CNS) with contrast, blood tests, and cerebrospinal fluid (CSF) analysis to detect signs of inflammation in the nervous system. There are no specific blood or CSF markers for the disease. High-resolution computed tomography (HRCT) of the chest and whole-body FDG-PET scans are used to search for systemic manifestations and find appropriate biopsy sites. A comprehensive differential diagnostic work-up should be part of the disease investigation. The cornerstone of the treatment is initially high doses of steroids, followed by tapering oral steroid doses, usually in combination with other immunosuppressive agents, including TNF-alpha inhibitors.