Cystatin C and cathepsin B in CSF from patients with inflammatory neurologic diseases

Background: In CSF, proteolytic enzymes are believed to have crucial roles in the initiation and progression of inflammatory neurologic diseases (IND) . Cystatin C, a major cysteine protease inhibitor in CSF, is tightly bound to cathepsin B and H. Objective: To determine if cystatin C is involved in the disease process of IND, the authors measured the cystatin C concentrati on by ELISA method and cathepsin B and H activities in the CSF of patients with acute IND. Methods: Cystatin C concentration and cathepsin B and H act ivities were measured in CSF samples taken from patients during the acute p hase of their disease. Subjects studied were 8 patients with Guillain-Barre syndrome (GBS), 5 with chronic inflammatory demyelinating polyneuropathy ( CIDP), 12 with MS, 16 with aseptic meningitis, 15 with neurodegenerative di seases as disease controls, and 35 healthy controls. Results: A significant decrease in CSF cystatin C level was seen in the patients with GBS, CIDP, and MS compared to the control subjects. High cathepsin B activity, but not cathepsin H activity, was also observed in the patients with GBS, CIDP, an d MS. Conclusion: Cystatin C levels in CSF measured by ELISA may help the p hysician recognize GBS, CIDP, and MS. Decreased levels of cystatin C may be related to the high levels of cathepsin B activity seen in the CSF of pati ents with GBS, CIDP, and MS.