Diagnosis and follow-up evaluation of central nervous system vasculitis: an evaluation of vessel-wall MRI findings

Abstract

Objective To approach the clinical value of MRI with vessel wall imaging (VWI) in patients with central nervous system vasculitis (CNSV), we analyzed patterns of VWI findings both at the time of initial presentation and during follow-up. Methods Stenoocclusive lesions, vessel-wall contrast enhancement (VW-CE) and diffusion-restricted lesions were analyzed in patients with a diagnosis of CNSV. On available VWI follow-up, progression, regression or stability of VW-CE were evaluated and correlated with the clinical status. Results Of the 45 patients included, 28 exhibited stenoses visible on MR angiography (MRA-positive) while 17 had no stenosis (MRA-negative). VW-CE was found in 2/17 MRA-negative and all MRA-positive patients (p < 0.05). 79.1% (53/67) of stenoses showed VW-CE. VW-CE was concentric in 88.3% and eccentric in 11.7% of cases. Diffusion-restricted lesions were found more frequently in relation to stenoses with VW-CE than without VW-CE (p < 0.05). 48 VW-CE lesions in 23 patients were followed over a median time of 239.5 days. 13 VW-CE lesions (27.1%) resolved completely, 14 (29.2%) showed partial regression, 17 (35.4%) remained stable and 4 (8.3%) progressed. 22/23 patients received immunosuppressive therapy for the duration of follow-up. Patients with stable or progressive VW-CE were more likely (p < 0.05) to have a relapse (14/30 cases) than patients with partial or complete regression of VW-CE (5/25 cases). Conclusion Concentric VW-CE is a common finding in medium/large-sized vessel CNSV. VW-CE might represent active inflammation in certain situations. However, follow-up VWI findings proved ambiguous as persisting VW-CE despite immunosuppressive therapy and clinical remission was a frequent finding.

Vasculitic Moyamoya syndrome in a patient with paroxysmal nocturnal hemoglobinuria: A case report and literature review

“…Except one little boy who received eculizumab treatment and had a good prognosis, the remaining three patients all presented with recurrent cerebral infarction and responded differently to antiplatelet therapy, which were detailed depicted in For the treatment of PNH associated vasculitic moyamoya syndrome, complement inhibitor such as Eculizumab to cope with PNH and reduce PNH relevant complications is the first choice, 9 corticosteroid or immunosuppressive therapy may be an alternative strategy. 10 Surgical revascularization seems to be a more advanced and effective option for ischemic moyamoya syndrome.…”

“…For the treatment of PNH associated vasculitic moyamoya syndrome, complement inhibitor such as Eculizumab to cope with PNH and reduce PNH relevant complications is the first choice, 9 corticosteroid or immunosuppressive therapy may be an alternative strategy. 10 Surgical revascularization seems to be a more advanced and effective option for ischemic moyamoya syndrome.…”

Vessel Wall Magnetic Resonance Imaging in Cerebrovascular Diseases

“…In a prospective study, VWE regressed in seven of nine PACNS patients after treatment, at a median follow-up period of 8 months, with three of these who also showed regression of vessel stenosis [ 61 ]. Persistent or progressive VWE is associated with a higher risk of relapses in patients with PANCS and secondary CNS vasculitis [ 62 ]. Nevertheless, persistent VWE must be interpreted cautiously because it can be of uncertain clinical significance and can be seen in patients with clinical remission even at long term follow-up.…”

“…Nevertheless, persistent VWE must be interpreted cautiously because it can be of uncertain clinical significance and can be seen in patients with clinical remission even at long term follow-up. In these cases, it probably represents post-inflammatory arterial wall changes, like mural fibrosis with or without neovascularization [ 62 ].…”

Sensitivity and specificity of vessel wall MRI sequences to diagnose central nervous system angiitis

“…In particular, CSF analysis were deemed abnormal if leucocyte count exceeded 5 cells/mL, and/or total protein level >0.45 mg/mL, and/or intrathecal oligoclonal bands were present (Destrebecq et al, 2020). Clinical outcome was defined as either "stable/remission" or "progression" according to Salvarani et al (2015b) and Patzig et al (2022). Brain MRI protocol has been previously described (Destrebecq et al, 2020).…”