The development of a diagnostic marker for earlier and more accurate clinical diagnosis of Alzheimer's disease (AD) is essential to identify AD patients during life unequivocally. The purpose of this study was to investigate the basic performance and clinical significance of tau level measurement in the cerebrospinal fluid (CSF) using an enzyme-linked immunosorbent assay (ELISA) developed by Innogenetics. The ELISA system showed reliable reproducibility and good linearity. For clinical studies, the CSF samples from a variety of patients (n = 332) were examined. They were classified into the four groups: Alzheimer's disease (AD); neurodegenerative disease (ND); cerebrovascular diseases (VD); and a neurological control (NC) group. The CSF-tau levels for AD, ND, VD and NC were 426 +/- 234 pg/ml, 239 +/- 157 pg/ml, 216 +/- 136 pg/ml, and 188 +/- 103 pg/ml, respectively. The CSF-tau level of the AD group was significantly higher than that of the other groups (p < 0.001). The CSF-tau levels increased during follow-up. The measurement of the tau level in CSF is shown to be a useful marker to confirm a clinical diagnosis of AD.