Ivy sign, misery perfusion, and asymptomatic moyamoya disease: FLAIR imaging and 15O-gas positron emission tomography

Vuignier, Sandra; Ito, Masaki; Kurisu, Kota; Kazumata, Ken; Nakayama, Naoki; Shichinohe, Hideo; Shiga, Tohru; Kiss, József Zoltán; Tamaki, Nagara; Houkin, Kiyohiro

doi:10.1007/s00701-013-1860-4

Cited by 21 publications

(27 citation statements)

References 23 publications

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“…Although ivy sign and white matter lesions were not selected in multivariate analysis, the odds ratio revealed high values that were significant. The ivy sign is associated with low cerebral perfusion or misery perfusion [ 37 , 38 ]; therefore, high EPVS might be associated with lower cerebral blood flow. However, this is not true in pediatric cases with moyamoya disease, which can exhibit linear hyperintensities that extend into the perivascular space of the deep white matter in FLAIR imaging [ 39 ].…”

Section: Discussionmentioning

confidence: 99%

Prevalence of and risk factors for enlarged perivascular spaces in adult patients with moyamoya disease

et al. 2017

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BackgroundEnlarged perivascular spaces (EPVS) are often observed with magnetic resonance imaging in patients with small vessel disease. However, the risk factors, radiological features, and clinical relevance of EPVS in patients with moyamoya disease are poorly understood. The purpose of this study was to evaluate EPVS, the risk factors of many EPVS, and the pathophysiology of EPVS in adult patients with moyamoya disease.MethodsOne hundred cerebral hemispheres of 50 adult patients with moyamoya disease were examined. The control group consisted of 50 age/sex-matched patients without ischemic disease. The numbers of EPVS at the level of the centrum semiovale per hemisphere were compared between the moyamoya disease and control groups. In each hemisphere, the total numbers of EPVS were categorized into five grades (0–4), and the clinical and radiological characteristics of the predictive factors in patients in the high EPVS grade group (EPVS grade = 4) were assessed.ResultsThe EPVS counts and grades were significantly higher in the moyamoya disease group. Analyses of the background characteristics of the patients with moyamoya disease revealed that significantly higher prevalence of high EPVS grades were associated with the female sex, hypertension, high magnetic resonance angiography scores, high numbers of flow voids in the basal ganglia, high brain atrophy scores, ivy signs, and white matter lesions. A logistic multivariate analysis of the patients with high EPVS grades revealed significant associations with the female sex, hypertension, and flow voids in the basal ganglia.ConclusionsIncreased EPVS were confirmed in adult patients with moyamoya disease, and the associated clinical and radiological factors were identified. The presence of hypertension, the female sex, and flow voids in the basal ganglia were important for predicting high EPVS grades in patients with moyamoya disease. Reductions in arterial pulsations with steno-occlusive changes can inhibit the flow of interstitial fluid, which can increase the number of EPVS in patients with moyamoya disease. Other clinical factors, such as the female sex and hypertension, may promote secondary brain damage in patients with moyamoya disease. Further evaluations of EPVS in patients with moyamoya disease are needed to better understand their pathophysiological importance.

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Section: Discussionmentioning

confidence: 99%

Prevalence of and risk factors for enlarged perivascular spaces in adult patients with moyamoya disease

et al. 2017

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“…4,13 Linear hyperintensity along the sulci on fluid-attenuated inversion recovery (FLAIR) imaging is referred to as the "leptomeningeal ivy sign," is considered to indicate retrograde slow flow of the leptomeningeal anastomosis produced by the stenotic or occlusive change in the major vessels, 20 and is indicative of misery perfusion, which is hemodynamic compromise due to impaired cerebral blood flow. 15,24,36 In some pediatric cases, the linear hyperintensity on FLAIR imaging extends to the perivascular space of the deep white matter, 16,20,31,38 is referred to as "medullary streaks," and is considered to be associated with changes in vasculature. 34 The aim of this study was to investigate the characteristics of medullary streaks on FLAIR imaging in pediatric moyamoya disease and to explore the pathophysiology of the findings.…”

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confidence: 99%

Pathophysiological consideration of medullary streaks on FLAIR imaging in pediatric moyamoya disease

Suzuki¹,

Mikami²,

Kuribara³

et al. 2017

Journal of Neurosurgery: Pediatrics

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OBJECTIVEMedullary streaks detected on fluid-attenuated inversion recovery (FLAIR) imaging have been considered to be reflected ischemic regions in pediatric moyamoya disease. The purpose of this study was to evaluate these medullary streaks both clinically and radiologically and to discuss associated pathophysiological concerns.METHODSThe authors retrospectively reviewed data from 14 consecutive pediatric patients with moyamoya disease treated between April 2009 and June 2016. Clinical and radiological features and postoperative imaging changes were analyzed. In 4 patients, hyperintense medullary streaks on FLAIR imaging (HMSF) at the level of the centrum semiovale were detected.RESULTSThe HMSF were coincident with hyperintense medullary streaks on a T2-weighted image, though they were not completely coincident with the vasculature on either a T2*-weighted image or contrast-enhanced CT. Analysis revealed significantly higher values in terms of MR angiography scores, number of flow voids of the basal ganglia, and the presence of the medullary artery in the group with HMSF than in those without. In contrast, the presence of white matter damage was significantly less frequent in the HMSF group. All HMSF disappeared after surgery, and the mean apparent diffusion coefficient at the same level was significantly reduced postoperatively.CONCLUSIONSAlthough HMSF should be associated with collateral circulation in moyamoya disease, other factors may be involved, including stagnated cerebrospinal fluid or vasogenic edema that is relevant to the impaired state of the white matter. Findings in this study provide insight into the pathophysiological basis of the perivascular space in moyamoya disease.

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“…20,24,31 By using positron emission tomography, Vuignie et al also found that ivy sign was significantly correlated with the increase OEF. 23 Consistently, the improvement of CVR after direct or indirect bypass surgery brought about a decrease in ivy sign. 19,20,22 Thus, all the above results were considered as critical evidence that the ivy sign originates from slow retrograde flow of engorged pial arteries via leptomeningeal anastomosis compensating for decreased perfusion pressure.…”

Section: Angiographymentioning

confidence: 78%

“…The incidence of ivy sign in MMD on the term "patients" ranged from 30.3% to 100%. However, if we exclude the three studies 23,28,31 that reported incidence less than 40%, the remaining nine studies all reported a very high incidence: more than 80%.…”

Section: Incidencementioning

confidence: 99%

What and why: the current situation and future prospects of “ivy sign” in moyamoya disease

Xie³

et al. 2020

Therapeutic Advances in Chronic Disease

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“Ivy sign” is a special imaging manifestation of moyamoya disease (MMD), which shows continuous linear or punctate high intensity along the cortical sulci and subarachnoid space on magnetic resonance images. Ivy sign was reported to reflect the development of compensatory collaterals, and to be closely related to hemodynamic changes and clinical symptoms, and to indicate the postoperative prognosis, in MMD patients. It is a unique and critical marker for MMD. However, due to the lack of consistent criteria, such as definition, grading, and identification standards, ivy sign has not received much attention. We undertook a comprehensive literature search and summarized the current situation regarding ivy sign in MMD in terms of baseline characteristics, detection methods, definition, regional division and distribution patterns, grading criterions, incidence, related factors, the mechanism of ivy sign, and the effects of treatments. We also provided related concerns raised and future prospects relevant to studies about ivy sign in MMD.

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Ivy sign, misery perfusion, and asymptomatic moyamoya disease: FLAIR imaging and 15O-gas positron emission tomography

Cited by 21 publications

References 23 publications

Prevalence of and risk factors for enlarged perivascular spaces in adult patients with moyamoya disease

Prevalence of and risk factors for enlarged perivascular spaces in adult patients with moyamoya disease

Pathophysiological consideration of medullary streaks on FLAIR imaging in pediatric moyamoya disease

What and why: the current situation and future prospects of “ivy sign” in moyamoya disease

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