Vasculitis confined to the central nervous system (CNS) is a rare dise
ase usually characterized by headache and focal neurologic symptoms. P
atients with primary vasculitis of the CNS may have symptoms and labor
atory findings of systemic disease such as fatigue and elevated erythr
ocyte sedimentation rate, but by definition, focal inflammation should
not be present outside the CNS. We describe 3 patients with uveitis i
n association with this diagnosis. The recognition of this association
adds to the complex differential diagnosis of uveitis in association
with CNS disease, and indicates that ''isolated'' angiitis of the CNS
may display clinical features outside the brain and spinal cord.