Primary angiitis of the central nervous system

Abstract

The primary angiitis of the central nervous system (PACNS) is a rare disorder with unknown etiology witch
affects the small and the medium vessels of CNS. It appears with a wide range of clinical patterns from
the CNS and its severity and development seems to be different among the patients. The diagnosis and
management of PACNS is a challenge for the clinician. There aren’t efficient non-invasive modalities for
diagnosis and no randomized trials of treatments have been done. The cerebral angiography appears
limited specificity and sensitivity. Define diagnosis of the disease and exclusion of other disorders witch
presented with similar findings, can be done with brain and leptomeninges biopsy. As the disease is partial,
normal biopsy can’t exclude the diagnosis. PACNS demand immediate and aggressive confrontation with
strong immunosuppressive therapy. It is recommended steroids and cyclophosphamide in monotherapy or
in combination. In resistant cases it has been tried anti-TNF factors with good results.
The purpose of this article is to review the literature concerning the clinical presentation, diagnosis and
treatment approach of PACNS.