The diagnosis of PACNS depends on a combination of clinical, imaging (MRI and then angiography) and histological features. Laboratory exclusion of systemic diseases and infections is essential, and CSF findings are supportive. Table 20.2 summarizes the modified criteria for the diagnosis of PACNS, suggested by Moore.12 This set of criteria, which covers a step-by-step use of diagnostic methods, is aimed at excluding other possibilities which may mimic CNS vasculitis, and finally confirm the diagnosis by biopsy.

Table 20.2 Criteria for the diagnosis of PACNSa

Clinical features consistent with a multifocal or diffuse CNS disease with a recurrent or progressive coursea (clinical evidence)

Exclusion of an underlying systemic inflammatory process or infection by appropriate laboratory studies (laboratory/blood evidence)

A CSF study consistent with CNS inflammation (elevated protein and pleocytosis) and excluding infection and neoplasia (laboratory/CSF evidence)

An MRI study suggestive of CNS vasculitis and excluding other alternative diagnoses; followed by a cerebral angiogram that is consistent with vasculitis (imaging evidence)

A brain biopsy confirming the presence of vascular inflammation and excluding infection, neoplasia or alternative causes of vasculopathy (histological evidence)

For a definite clinical diagnosis of PACNS, patients must fulfill all of the above. a Modified from Moore12

b Clinical features may suggest focal CNS involvement in some patients, but imaging studies are expected to reveal more widespread disease

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