Increased concentration of C4d complement protein in the cerebrospinal fluids in progressive supranuclear palsy

Acta Neurol Scand. 1994 Jan;89(1):42-6. doi: 10.1111/j.1600-0404.1994.tb01631.x.


Plasma and CSF levels of C4d and the circulating immune complex (CIC) to C1q were measured in 27 patients with progressive supranuclear palsy (PSP), Parkinson's disease (PD) and cervical spondylosis (CS). There was no significant difference among groups in plasma C4d or in plasma or CSF CIC to C1q. However, the PSP group had significantly higher CSF levels of C4d than the PD and CS groups. Higher CSF C4d index in the PSP group was also shown compared with PD and CS groups. These results suggest that augmented complement activation in the wide areas of the central nervous system occurs in PSP. CSF levels of C4d or C4d index may serve as a basis for differentiating PSP from PD.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Brain / immunology
  • Cervical Vertebrae
  • Complement C4 / cerebrospinal fluid*
  • Complement C4b*
  • Complement Pathway, Classical / physiology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Parkinson Disease / diagnosis
  • Parkinson Disease / immunology
  • Peptide Fragments / cerebrospinal fluid*
  • Reference Values
  • Spinal Osteophytosis / diagnosis
  • Spinal Osteophytosis / immunology
  • Supranuclear Palsy, Progressive / diagnosis
  • Supranuclear Palsy, Progressive / immunology*


  • Complement C4
  • Peptide Fragments
  • Complement C4b
  • complement C4d