Humoral and cellular immunologic study of cerebrospinal fluid in a patient with Behcet encephalitis
P. J. Jongen, H. E. Daelmans, B. Bruneel and M. R. den Hartog
Department of Neurology, University Hospital Nijmegen, The Netherlands.
To study the immunopathogenesis of neuro-Behcet syndrome, we performed
serial cerebrospinal fluid (CSF) examinations in a patient with Behcet's
syndrome and involvement of the central nervous system. Before and after
immunosuppressive treatment, we measured the CSF indexes of immunoglobulins
(Ig), and the third (C3) and the fourth (C4) component of complement, and
quantified immune complexes and lymphocyte subsets in CSF and peripheral
blood. During active encephalitis, humoral abnormalities were intrathecal
production of IgM and, to a lesser degree, IgG and IgA, presence of immune
complexes in CSF but not in peripheral blood, intrathecal C3 production,
and elevated CSF C3 and C4 concentrations; lymphocyte subset analysis
showed an increased CSF CD8+ T-cell percentage, in combination with
slightly increased PB CD3+ and CD8+ T-cell subsets. After effective
immunosuppressive treatment, humoral and cellular CSF values were normal.
We conclude that intrathecally produced immunoglobulins, immune complexes,
and C3 as well as CD8+ T cells are likely to participate in the development
of Behcet encephalitis.