Primary arteritis of the central nervous system


INTRODUCTION


Primary arteritis of the central nervsous sytem (PACNS) is a relatively rare disease that can be devastating and very challenging to pinpoint. It causes multiple strokes or loss of blood flow in the brain.  

EPIDEMIOLOGY 


PACNS is rare, with a reported prevalence of to cases per million persons. The majority of patients (to percent) are men, and most patients are diagnosed between the ages of and years (often in their or ), though the disease has been reported in women as young as years and as old as years. The incidence of PACNS 

Laboratory testing

Common laboratory test abnormalities in patients with PACNS include
  • Spinal fluid
    • Analysis of the CSF is a crucial part of the evaluation of patients with potential PACNS and should be performed in all patients unless there are contraindications. The importance of CSF analysis in excluding any infectious or malignant process and the performance of adequate stains and cultures of the CSF cannot be overemphasized. The CSF is abnormal in 80 to 90 percent of patients with pathologically documented disease. Normal CSF findings can occur with localized disease and in patients presenting with mass-like lesions. There are no specific abnormalities of the CSF in PACNS; however, the CSF findings in the majority of patients show an aseptic meningitis pattern with modest lymphocytic pleocytosis, normal glucose levels, elevated protein level, and occasionally the presence of oligoclonal bands and elevated IgG synthesis. By contrast, in the RCVS, the most common mimic of PACNS, the CSF analysis is usually normal or reflects findings of subarachnoid hemorrhage if this is present. [1]
  • high sed rate
  • High CRP

References

[1]R Hajj-Ali, L Calabrese. PACNS. Uptodate.