Background Serial cerebral angiograms, computed tomography, and magnet
ic resonance imaging are among the proposed methods for monitoring dis
ease activity and response to therapy in isolated angiitis of the cent
ral nervous system. Cerebrospinal fluid has not proved to be useful in
monitoring clinical course. Case Description We describe a 45-year-ol
d man with histological diagnosis of isolated angiitis of the central
nervous system that was treated with prednisone plus azathioprine and
monitored for 2 years. Samples of the cerebrospinal fluid were obtaine
d for cytological and routine chemical examination, as well as albumin
and immunoglobulin content. Before treatment, cerebrospinal fluid sho
wed marked plasmatic transudation of albumin and intrathecal synthesis
of immunoglobulins. During the first year of immunosuppression no eve
nts were noticed, and the previously abnormal aspects of the cerebrosp
inal fluid showed improvement. During the weaning of azathioprine, a n
ew stroke occurred in conjunction with a marked deterioration of cereb
rospinal fluid parameters. Immunosuppression was resumed at previous l
evels, and during the following year no further events occurred. Once
again, abnormal cerebrospinal fluid values improved significantly. Con
clusions We report a case of isolated angiitis of the central nervous
system in which the serial cerebrospinal fluid examinations (albumin a
nd immunoglobulin content) showed a close correlation with clinical co
urse. This method may be useful in monitoring response to therapy.