Cerebral vasculitis is a serious but uncommon condition which presents
considerable difficulties in recognition, diagnosis and treatment. We
studied eight consecutive patients in whom this diagnosis was made. D
espite the great diversity of symptoms and signs, we noted three clini
cal patterns: (i) acute or sub-acute encephalopathy, (ii) a picture wi
th some similarities to multiple sclerosis ('MS-plus'), and (iii) feat
ures of a rapidly progressive space-occupying lesion. The identificati
on of these patterns may help recognition of cerebral vasculitis. The
diagnostic value of four investigative procedures not previously studi
ed in cerebral vasculitis was assessed: ophthalmological examination u
sing low-dose fluorescein angiography with slit-lamp video microscopy
of the anterior segment (abnormal in 4/5 patients); spinal fluid oligo
clonal band analysis (abnormal in 3/6 patients); anti-neutrophil cytop
lasmic antibody assay (abnormal in 3/8 patients); and indium-labelled
white-cell cerebral imaging (positive in only one patient). Treatment
was with steroid alone (n = 2) or steroid with cyclophosphamide (n = 6
). Seven patients responded clinically.