The authors report high intrathecal release of tumor necrosis factor alpha
(TNF-alpha) and interleukin (TL)-1 beta in five patients with sporadic or n
ew-variant Creutzfeldt-Jakob disease (CJD) without activation of the humora
l or lymphocytic immune responses. Increased release of TNP-alpha and IL-1
beta was also detected in some patients with progressive dementias. CJD is
associated with a local cerebral host response that involves the release of
proinflammatory cytokines.