CNS Vasculitis

Abstract

Central nervous system vasculitis (CNSV) is a complex disease that poses considerable diagnostic and therapeutic challenges. It is divided into primary angiitis of the CNS (PACNS), or secondary angiitis of the CNS when associated with systemic conditions. Clinical presentation can be extremely variable and there is no classic presentation. In addition, there is no single diagnostic test and the sensitivity/specificity of all currently available tests is suboptimal. As such, an exhaustive approach with thorough historical data, physical examination, and corroborating investigations should be performed to exclude or confirm a diagnosis of CNSV. Infectious, neoplastic, and autoimmune conditions should be carefully evaluated. Knowledge of CNSV mimics is crucial given the therapeutic and prognostic implications. Reversible cerebral vasoconstriction syndrome is now recognized as the most common mimicker of PACNS. Much progress has been made in the understanding of PACNS histopathology, and specific clinical subsets and their response to treatment. Contrary to historical reports, recent PACNS cohorts achieved favorable clinical outcomes when early diagnosis and prompt treatment was initiated. Successful treatment with newer drugs has also been reported in PACNS cases. Newer imaging modalities hold promise for distinguishing inflammatory from noninflammatory vascular lesions. In this review, the authors provide a broad overview with updates on the types of adult CNSV along with its clinical assessment, diagnosis, and treatment.

Intracranial Atherosclerosis Versus Primary Angiitis of the Central Nervous System: a Case Report

“…According to John and Hajj-Ali recent PACNS cohorts have experienced favorable outcomes when early diagnosis and prompt treatment were initiated [ 3 ]. Given that a speedy diagnosis and aggressive treatment help to avoid permanent neurological damage, it is imperative to be familiar with the many mimics of PACNS that may delay proper diagnosis.…”

Infection, Immunodeficiency, and Inflammatory Diseases in Autoimmune Neurology

“…60 PACNS should be considered in patients with recurrent episodes of ischemia in multiple vascular territories, an inflammatory CSF profile, subacute or chronic headaches with cognitive decline, and aseptic meningitis or chronic meningitis; alternate etiologies such as infections, malignancy, and other inflammatory disorders must be excluded. 61 Secondary CNS vasculitis, albeit still rare, is much more common compared with PACNS and has broad range of possible etiologies. Presentation is often dependent on the type of vessel involvement, for example, small versus large vessel vasculitis.…”

“…Almost any systemic vasculitis (►Table 2) can involve the CNS, although PNS involvement with mononeuritis multiplex and peripheral neuropathy is more common than CNS involvement. 61 CNS involvement is more common with the antineutrophil cytoplasmic autoantibody (ANCA)-associated small vessel vasculitis (includes microscopic polyangiitis, granulomatous polyangiitis, and eosinophilic granulomatous with polyangiitis) compared with the other systemic large or medium vessel vasculitides.…”

New onset refractory status epilepticus due to primary angiitis of the central nervous system

“…PACNS is a disease of substantial diagnostic and therapeutic challenges to clinicians [14] . Despite the efforts made to assist clinicians in the diagnosis, the decision of biopsy in a patient suspected to have PACNS remains a challenge.…”