Lesion morphology at 7 Tesla MRI differentiates Susac syndrome from multiple sclerosis

Wuerfel, Jens; Sinnecker, Tim; Eb, Ringelstein; Jarius, Sven; Schwindt, Wolfram; Niendorf, Thoralf; Paul, Friedemann; Kleffner, Ilka; Dörr, Jan

doi:10.1177/1352458512441270

Cited by 137 publications

(134 citation statements)

References 27 publications

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“…This perivenous configuration of MS lesions has been recently demonstrated using T2*‐weighted ultrahigh‐field 7T MRI,13, 15 and in studies that have included non‐MS populations, the quantification of CVs has predicted a diagnosis of MS 12, 14, 17, 27, 28, 29. Several studies reported detection of CVs in lesions of MS patients more frequently than other populations, including patients with Susac syndrome29 and neuromyelitis optica spectrum disorder,17 and controls with high risk for vascular disease 28. A number of studies have suggested that a finding of >40% of lesions with CVS may predict MS 12, 14.…”

Section: Discussionmentioning

confidence: 80%

“Central vessel sign” on 3T FLAIR* MRI for the differentiation of multiple sclerosis from migraine

Solomon

Schindler

Howard³

et al. 2015

Ann Clin Transl Neurol

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ObjectiveThe diagnosis of multiple sclerosis (MS) presently relies on radiographic assessments of imperfect specificity. Recent data using T2* methodology for the detection of the “central vessel sign” (CVS) in MS lesions suggests this novel MRI technique may distinguish MS from other disorders. Our aim was to determine if evaluation for CVS on 3T FLAIR* MRI differentiates MS from migraine.MethodsPatients with MS or migraine and a prior brain MRI demonstrating at least two hyperintense lesions ≥3 mm were recruited. Exclusion criteria included any additional comorbidity known to cause brain MRI abnormalities. 3T MRI was performed in each participant with administration of gadopentetate dimeglumine, and FLAIR* images were generated in postprocessing. The total number of discrete ovoid lesions ≥3 mm were counted on FLAIR, per participant, and subsequently evaluated for presence of CVS on FLAIR*. An exploratory method evaluating for CVS in a maximum of 12 lesions per subject was also completed.ResultsTen participants with MS and 10 with migraine completed the study. The median percentage (quartiles) of lesions in MS participants with CVS was 84 (79, 94) compared to 22 (15, 54) in migraine (P = 0.008). In a subanalysis by brain region, in the subcortical and deep white matter, the median percentage (quartiles) of lesions in MS participants with CVS was 88 (81, 100) compared to 19 (11, 54) in migraine (P = 0.004). This difference was not identified in juxtacortical, periventricular, or infratentorial regions.InterpretationIdentification of CVS using FLAIR* on 3T MRI helps differentiate MS from migraine, particularly in the subcortical and deep white matter.

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Section: Discussionmentioning

confidence: 80%

“Central vessel sign” on 3T FLAIR* MRI for the differentiation of multiple sclerosis from migraine

Solomon

Schindler

Howard³

et al. 2015

Ann Clin Transl Neurol

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“…Susac syndrome is believed to be an autoimmune vasculopathy that causes occlusion of small vessels in the brain, retina and inner ear. A study of five patients with this very rare disease found that a blood vessel was detectable in 54% of 148 white matter lesions 21 . Interestingly, the identified blood vessels were most commonly located at the lesion periphery.…”

Section: The Central Vein In Ms Discussionmentioning

confidence: 99%

“…The 'central vein sign' (CVS) has been investigated in various neurological conditions by several groups, and evidence has accumulated that the CVS may have the ability to accurately differentiate MS from its mimics [15][16][17][18][19][20][21] . As a consequence, recent guidelines from the Magnetic Resonance Imaging in MS (MAGNIMS) group 1,4 and the Consortium of MS Centers (CMSC) task force 22 have acknowledged the potential of the CVS and its dedicated MRI acquisitions for the differential diagnosis of MS, while calling for further research before considering a possible modification of the diagnostic criteria.…”

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confidence: 99%

The central vein sign and its clinical evaluation for the diagnosis of multiple sclerosis: a consensus statement from the North American Imaging in Multiple Sclerosis Cooperative

et al. 2016

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At present, the diagnosis of multiple sclerosis (MS) relies heavily on the use of MRI, which can demonstrate disease dissemination in space and time [1][2][3][4] . The current 2010 McDonald criteria have enabled earlier diagnosis 5,6 and initiation of disease-modifying treatment, with substantial benefits for disease outcome 7,8 , but they still have imperfect sensitivity and specificity 9,10 . The limited accuracy of the criteria results in challenging cases and misdiagnosis, which are prevalent problems in MS 11,12 . Therefore, more-accurate and pathologically specific MRI criteria are still needed to exclude other disorders that can mimic MS 13,14 .The MRI-detectable central vein inside white matter lesions has recently been proposed as a biomarker of inflammatory demyelination and, thus, may aid the diagnosis of MS 15 . The 'central vein sign' (CVS) has been investigated in various neurological conditions by several groups, and evidence has accumulated that the CVS may have the ability to accurately differentiate MS from its mimics [15][16][17][18][19][20][21] . As a consequence, recent guidelines from the Magnetic Resonance Imaging in MS (MAGNIMS) group 1,4 and the Consortium of MS Centers (CMSC) task force 22 have acknowledged the potential of the CVS and its dedicated MRI acquisitions for the differential diagnosis of MS, while calling for further research before considering a possible modification of the diagnostic criteria. However, the lack of standardization for the definition and imaging of the CVS, as well as a dearth of large-scale prospective studies evaluating the CVS for MS diagnosis, are currently preventing the clinical validation of this potential biomarker 1,23 .This Consensus Statement aims to provide recommendations for the definition, standardization and clinical evaluation of the CVS in the diagnosis of MS. These statements are based on a thorough review of the existing literature on the CVS and the consensus opinion of the members of the North American Imaging in Multiple Sclerosis (NAIMS) Cooperative. E X P E RT C O N S E N S U S D O C U M E N T on behalf of the NAIMS CooperativeAbstract | Over the past few years, MRI has become an indispensable tool for diagnosing multiple sclerosis (MS). However, the current MRI criteria for MS diagnosis have imperfect sensitivity and specificity. The central vein sign (CVS) has recently been proposed as a novel MRI biomarker to improve the accuracy and speed of MS diagnosis. Evidence indicates that the presence of the CVS in individual lesions can accurately differentiate MS from other diseases that mimic this condition. However, the predictive value of the CVS for the development of clinical MS in patients with suspected demyelinating disease is still unknown. Moreover, the lack of standardization for the definition and imaging of the CVS currently limits its clinical implementation and validation. On the basis of a thorough review of the existing literature on the CVS and the consensus opinion of the members of the North American Imaging in Mult...

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“…These signs are particularly visible with MRI systems that operate at high magnetic field strengths (≥3.0 T). [42][43][44][45][46][47][48][49] Susceptibilityweighted imaging (SWI), a sequence first described in 2004, 50 has also shown high sensitivity for detecting ironcontaining tissue and small veins because of their paramagnetic properties. This capability has conferred added value to MRI for diagnostic purposes, particularly when SWI is co-registered and mixed with standard pulse sequences such as T2 fluid-attenuated inversion recovery (FLAIR), which is termed FLAIR*.…”

Section: Differential Diagnosis Via Detection Of Iron and Veinsmentioning

confidence: 99%

MAGNIMS consensus guidelines on the use of MRI in multiple sclerosis—clinical implementation in the diagnostic process

et al. 2015

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| The clinical use of MRI in patients with multiple sclerosis (MS) has advanced markedly over the past few years. Technical improvements and continuously emerging data from clinical trials and observational studies have contributed to the enhanced performance of this tool for achieving a prompt diagnosis in patients with MS. The aim of this article is to provide guidelines for the implementation of MRI of the brain and spinal cord in the diagnosis of patients who are suspected of having MS. These guidelines are based on an extensive review of the recent literature, as well as on the personal experience of the members of the MAGNIMS (Magnetic Resonance Imaging in MS) network. We address the indications, timing, coverage, reporting and interpretation of MRI studies in patients with suspected MS. Our recommendations are intended to help radiologists and neurologists standardize and optimize the use of MRI in clinical practice for the diagnosis of MS.

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Lesion morphology at 7 Tesla MRI differentiates Susac syndrome from multiple sclerosis

Cited by 137 publications

References 27 publications

“Central vessel sign” on 3T FLAIR* MRI for the differentiation of multiple sclerosis from migraine

“Central vessel sign” on 3T FLAIR* MRI for the differentiation of multiple sclerosis from migraine

The central vein sign and its clinical evaluation for the diagnosis of multiple sclerosis: a consensus statement from the North American Imaging in Multiple Sclerosis Cooperative

MAGNIMS consensus guidelines on the use of MRI in multiple sclerosis—clinical implementation in the diagnostic process

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