Subtypes of primary angiitis of the CNS identified by MRI patterns reflect the size of affected vessels

Schuster, Simon F.; Bachmann, H; Thom, Vivien; Kaufmann-Buehler, A.-K.; Matschke, Jakob; Siemonsen, Susanne; Glatzel, Markus; Fiehler, Jens; Gerloff, Christian; Magnus, Tim; Thomalla, Götz

doi:10.1136/jnnp-2017-315691

Cited by 37 publications

(29 citation statements)

References 27 publications

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“…Included patients with inflammatory vasculopathies encompassed: (1) 25 ; and (5) PACNS (imaging or biopsy proven), diagnosed according to Schuster et al 13…”

Section: Patientsmentioning

confidence: 99%

“…11,12 In addition, the current standard for diagnosis of inflammatory vasculopathies is brain/meningeal biopsy or angiography, 11,12 but these, unlike MRI, are substantially invasive procedures and are not always diagnostic. 13 The physical relationship between WM lesions and venules can now be visualized by susceptibility-based MRI sequences, taking advantage of the T2*-shortening effect of deoxyhemoglobin 14,15 in venous blood. 16 Several studies conducted with 3T and 7T MRI clearly showed that in MS, the association between brain WM venules and lesions (perivenular lesions), also named the "central vein sign," 17 can be efficiently visualized; in MS, the proportion of lesions that have clear central veins is high.…”

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confidence: 99%

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Central vein sign differentiates Multiple Sclerosis from central nervous system inflammatory vasculopathies

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et al. 2018

Annals of Neurology

166

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ObjectivesIn multiple sclerosis (MS), magnetic resonance imaging (MRI) is a sensitive tool for detecting white matter lesions, but its diagnostic specificity is still suboptimal; ambiguous cases are frequent in clinical practice. Detection of perivenular lesions in the brain (the “central vein sign”) improves the pathological specificity of MS diagnosis, but comprehensive evaluation of this MRI biomarker in MS‐mimicking inflammatory and/or autoimmune diseases, such as central nervous system (CNS) inflammatory vasculopathies, is lacking. In a multicenter study, we assessed the frequency of perivenular lesions in MS versus systemic autoimmune diseases with CNS involvement and primary angiitis of the CNS (PACNS).MethodsIn 31 patients with inflammatory CNS vasculopathies and 52 with relapsing–remitting MS, 3‐dimensional T2*‐weighted and T2–fluid‐attenuated inversion recovery images were obtained during a single MRI acquisition after gadolinium injection. For each lesion, the central vein sign was evaluated according to consensus guidelines. For each patient, lesion count, volume, and brain location, as well as fulfillment of dissemination in space MRI criteria, were assessed.ResultsMS showed higher frequency of perivenular lesions (median = 88%) than did inflammatory CNS vasculopathies (14%), without overlap between groups or differences between 3T and 1.5T MRI. Among inflammatory vasculopathies, Behçet disease showed the highest median frequency of perivenular lesions (34%), followed by PACNS (14%), antiphospholipid syndromes (12%), Sjögren syndrome (11%), and systemic lupus erythematosus (0%). When a threshold of 50% perivenular lesions was applied, central vein sign discriminated MS from inflammatory vasculopathies with a diagnostic accuracy of 100%.InterpretationThe central vein sign differentiates inflammatory CNS vasculopathies from MS at standard clinical magnetic field strengths. Ann Neurol 2018;83:283–294

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“…Included patients with inflammatory vasculopathies encompassed: (1) 25 ; and (5) PACNS (imaging or biopsy proven), diagnosed according to Schuster et al 13…”

Section: Patientsmentioning

confidence: 99%

mentioning

confidence: 99%

Central vein sign differentiates Multiple Sclerosis from central nervous system inflammatory vasculopathies

Maggi

Absinta

Grammatico

et al. 2018

Annals of Neurology

166

186

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“…Twenty-seven patients (n = 27) suffered from PACNS, whereas in 15 of them the diagnosis was biopsyproven and 12 patients (n = 12) had a probable PACNS (see Supplementary Information: "Patients with probable PACNS"). Of note, all patients with a probable PACNS were classified to the medium vessel variant (MVV) of the disease with multiple irregular alternating stenosis or occlusions and dilatations in either conventional angiography or MR angiography (MRA) (17,18). Patients with PACNS were allocated to two subgroups according to disease activity.…”

Section: Patients and Healthy Controlsmentioning

confidence: 99%

Circulating Endothelial Cells as Promising Biomarkers in the Differential Diagnosis of Primary Angiitis of the Central Nervous System

et al. 2020

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Background: Diagnosis of primary angiitis of the central nervous system (PACNS) and discrimination of PACNS from its mimics, e. g., reversible cerebral vasoconstriction syndrome (RCVS) or moyamoya disease (MMD) as non-inflammatory vasculopathies, still remain challenging. Circulating endothelial cells (CEC) are well-established markers for endothelial damage and potential biomarkers in PACNS. This study aimed to investigate if CECs may also help to distinguish an active PACNS from its important differentials (RCVS, MMD). Methods: CECs were assessed in 47 subjects. Twenty-seven patients with PACNS were included, seven with an active disease (aPACNS), 20 in remission (rPACNS). Seven patients with RCVS/MMD were analyzed. Thirteen healthy subjects served as controls (HC). CECs were measured by immunomagnetic isolation from peripheral venous blood. Mann-Whitney-U-Tests were applied for between-group comparisons. The Benjamini-Hochberg-procedure was applied to adjust for multiple comparisons. Results: In aPACNS, CECs were significantly elevated compared to HC (480 vs. 40 CEC/ml, p < 0.001) and rPACNS (54 CEC/ml, p < 0.001). RCVS/MMD patients showed higher CEC levels (288 CEC/ml) than HC (p < 0.001), but lower than those in aPACNS (p = 0.017). An adjustment for multiple comparisons confirmed prior significant differences. An increased CEC value (cut-off 294 CEC/ml) is indicative for an active PACNS [sensitivity 100%, 95% confidence interval (CI) 63-100%; specificity 93%, CI 81-98%]. Conclusions: CECs may serve as biomarkers for diagnosis, treatment monitoring, and also for differential diagnosis of PACNS. CECs seem to be a marker of endothelial injury with higher levels in inflammatory than non-inflammatory vasculopathies. Larger patient samples are required to corroborate these findings.

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“…No better explanation for the presenting complaint should be found. Peng et al, 2017 [27] Harsha et al, [28] ( ≥ 44.4% [8] 50% [6] 83% [10] 40.5% 6660% [28] ≥47.4% [9] 83.9% [26] 100% [ 100% 5100% (12) 100% (16) [79% [30]]…”

Section: Black Blood Mrimentioning

confidence: 99%

“When should primary angiitis of the central nervous system (PACNS) be suspected?”: literature review and proposal of a preliminary screening algorithm

et al. 2020

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Background Primary angiitis of the CNS (PACNS) is a process causing variously combined neurological disturbances. Its rarity and kaleidoscopic presentation make it difficult to diagnose and even to suspect. Objective (1) To provide an up-to-date review on PACNS and (2) to create a preliminary screening algorithm based on clinical and radiological first-level data, useful to suspect PACNS and guide further investigations. Methods Review of PUBMED case series on PACNS, published from 2002 to 2017, collection of frequencies of clinical and neuroimaging features and calculation of median values. Classification of features as “major” or “minor” if frequency was higher or lower than median value. Combination of features in sets of criteria represented by all possible combinations of major and minor clinical and neuroradiological features. Application of criteria to published PACNS case reports and selection of the ones best identifying patients with definite PACNS. Results We reviewed 24 case series. “Major” clinical features were headache, stroke, cognitive impairment, focal neurological deficits; “minor” were seizures, altered consciousness, psychiatric disorders. “Major” neuroradiological features were multiple parenchymal lesions, parenchymal/meningeal contrast enhancement, magnetic resonance angiography vessel abnormalities, vessel wall enhancement; “minor” were parenchymal/subarachnoid hemorrhage, single parenchymal lesion. The selected sets of criteria able to identify all PACNS patients were (1) one clinical (major/minor) + one major neuroradiological feature; and (2) Two clinical (≥ 1 major) + one minor neuroradiological feature. Conclusion Our review provides a detailed clinical/neuroradiological picture of PACNS. The proposed algorithm should be regarded as a preliminary screening tool to move the first steps towards PACNS diagnosis that needs validation.

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Subtypes of primary angiitis of the CNS identified by MRI patterns reflect the size of affected vessels

Cited by 37 publications

References 27 publications

Central vein sign differentiates Multiple Sclerosis from central nervous system inflammatory vasculopathies

Central vein sign differentiates Multiple Sclerosis from central nervous system inflammatory vasculopathies

Circulating Endothelial Cells as Promising Biomarkers in the Differential Diagnosis of Primary Angiitis of the Central Nervous System

“When should primary angiitis of the central nervous system (PACNS) be suspected?”: literature review and proposal of a preliminary screening algorithm

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