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.