Cellular immune activation markers neopterin and β2-microglobulin are not elevated in the cerebrospinal fluid of patients with Creutzfeldt-Jakob disease.

In prion diseases, neuroimmunological responses include activation of microglia, astrocytosis and release of pro- and anti-inflammatory cytokines, which might substantially contribute to the neurodegenerative process. In this study we investigated neopterin and beta(β)2-microglobulin, as markers of cellular immune activation, in the cerebrospinal fluid (CSF) of patients with Creutzfeldt-Jakob disease (CJD) and of patients with other neurological and non-neurological diseases. CSF samples from CJD patients were collected in the framework of the German CJD Surveillance study. Concentrations of neopterin and β2-microglobulin were determined in CSF using ELISA. We could not obtain significant changes in CSF levels of neopterin and β2-microglobulin in CJD patients when compared to other neurological and non-neurological controls. In a subanalysis of CJD patients only, we could find significant elevated neopterin levels in patients with MV genotype, potentially reflecting a distinct disease pathology. Since autoimmune inflammatory disorders are important differential diagnoses in CJD, additional biomarker might be helpful in clinical setting.