Introduction
Sarcoidosis is a chronic, granulomatous disease of unknown etiology1,2 which mainly affects the respiratory and lymphatic systems. Rarely (5-13% of sarcoidosis patients), the central nervous system (CNS) is involved 3, or is the only affected system (1%) 4. Although, post-mortem studies suggest that ante-mortem diagnosis is only made in 50% of patients with sarcoidosis with nervous-system involvement 5. The first report of neurosarcoidosis was published in 19056,7. Cranial nerve deficits are the most common clinical presentation, particularly a facial nerve palsy. Other symptoms include endocrine dysfunction, seizures, encephalopathy, peripheral neuropathy, meningitis, spinal cord dysfunction or myopathy8. Neurosarcoidosis can present with granulomas at the surface of the brain, preferably at the skullbase , with a perivascular distribution with parenchymal involvement, or even as a tumefactive mass9.
Contrast-enhanced MRI is the preferred examination when a granulomatous disease is suspected 10,11, and can be used to monitor the course of the disease. A final diagnosis of sarcoidosis requires histological confirmation of a noncaseating granuloma in radiologically involved tissue 9. The differential diagnosis however is extensive due to the variation in presenting symptoms and affected organs, other pathologies thus need to be ruled out.
We describe a unique case with symptomatic hydrocephalus as the first sign of sarcoidosis. The patient’s recent history of ischemic cerebrovascular disease could possibly be attributed to the previous undetected sarcoidosis.