Neurosarcoïdose : état des connaissances

The origins of neurosarcoidosis, a multisystemic granulomatous disease, remain unknown. Nervous system localizations remain rare, but severe. Lymphocytic meningitis, psychiatric disorders, diabetes insipidus and cranial nerve palsy are the most frequent signs. Cerebral fluid test and cervical medullar and cerebral MRI with gadolinium have to be performed first. In some cases, histological evidence of granuloma have to be obtained with neuromuscular, meningeal or cerebral biopsies. Functional impairment and life-threatening conditions require early corticosteroid therapy. In worsening cases or in the event of no therapeutic response or poor tolerance to corticosteroids, other immunosuppressive agents should be associated. Maintenance therapy and most often life long maintenance therapy allow a continuous success while avoiding relapse.