The “Ivy-Sign” in Moyamoya Disease—From MRI Pattern to Diagnosis

2020 ◽  
Vol 51 (04) ◽  
pp. 241-244 ◽  
Author(s):  
Janina Gburek-Augustat ◽  
Ina Sorge ◽  
Andreas Merkenschlager

AbstractMoyamoya disease (MMD) is characterized by bilateral, chronic progressive stenosis at the terminal portions of the internal carotid arteries and their proximal branches. The “smoke-like” appearance of the arterial collaterals in angiography gives the disease its name. The “ivy-sign” is the less-known magnetic resonance imaging (MRI) pattern of this disease. The leptomeningeal collaterals present as diffuse signal enhancement at the brain surface in contrast-enhanced T1-weighted image and fluid-attenuated inversion recovery sequences “as if overgrown with ivy.”We report on three patients with MMD in whom the “ivy-sign” was already present but misinterpreted in the initial MRI of the brain. The correct diagnosis was made only after repeated MRI.Using three case studies, we describe the difficulties in the interpretation of the “ivy-sign” as an MRI pattern. Knowledge of the “ivy-sign” can be helpful, especially in diseases predisposing to MMD. If this MRI pattern is present, MMD should be considered and MR angiography should be added.

2013 ◽  
Vol 127 (2) ◽  
pp. 196-199 ◽  
Author(s):  
L-S Tseng ◽  
S-D Luo

AbstractObjective:We describe a rare case of sudden onset of unilateral sensorineural hearing loss occurring as the first symptom of moyamoya disease, which is characterised by progressive stenosis of the intracranial internal carotid arteries and their proximal anterior cerebral arteries and middle cerebral arteries.Method:Case report and review of the world literature regarding moyamoya disease with hearing loss.Results:The reported patient had moyamoya disease that initially presented as sudden, unilateral sensorineural hearing loss. Magnetic resonance imaging showed occlusion of the anterior cerebral, middle cerebral and distal internal carotid arteries bilaterally. The possible mechanism of this patient's sudden sensorineural hearing loss may have been vascular occlusion resulting from thrombotic narrowing or blockage by plaque.Conclusion:The described patient represents the first reported case of sudden onset, unilateral sensorineural hearing loss occurring as the first symptom of moyamoya disease. The possibility of a vascular lesion such as moyamoya disease should be considered in patients with sudden sensorineural hearing loss, especially children, young adults and Asian patients. Due to this disease's poor outcome, early diagnosis and treatment are important to prevent stroke.


2014 ◽  
Vol 6 (2) ◽  
Author(s):  
Sarmad Said ◽  
Chad J. Cooper ◽  
Haider Alkhateeb ◽  
Juan M. Galvis ◽  
German T. Hernandez ◽  
...  

Moyamoya disease was first described in 1957 as <em>hypoplasia of the bilateral internal carotid arteries</em>, the characteristic appearance of the associated network of abnormally dilated collateral vessels on angiography was later likened to something hazy, like a puff of cigarette smoke, which, in Japanese, is <em>moyamoya</em>. This paper describes two cases of moyamoya presentations, including moyamoya disease and moyamoya syndrome. Moyamoya may rarely occur in North American Hispanic patients. The presentation can vary significantly and ranges bwtween fulminant outcome and prolonged survival. Awareness about moyamoya and its different presentations may be beneficial for the patients and can improve the outcome.


Author(s):  
Yasuo Murai ◽  
Koji Adachi ◽  
Fumihiro Matano ◽  
Kojiro Tateyama ◽  
Akira Teramoto

Abstract:Objective:We present herein the intraoperative indocyanin green videoangiography (ICGVAG) findings for three cases of cerebellar hemangioblastoma (HB).Cases:Cerebellar HB was detected in three patients presenting with symptoms of vertigo and/or headaches and diagnosed on the basis of preoperative magnetic resonance imaging (MRI) and cerebral angiographic findings. Preoperative embolization of the tumor feeding artery was not performed in any of the patients. None of the patients underwent any procedure prior to ICGVAG that would affect the ICG findings, such as perilesional hemostatic coagulation or ablation. In each patient, it was possible to judge the approximate location of the tumor in relation to the brain surface and to distinguish the feeding and draining vessels. Following resection of the tumor, ICGVAG images confirmed that the mural nodule had been eliminated. None of the patients required blood transfusion, either during or after the surgery. For each patient, the lesion was pathologically confirmed as HB, postoperative contrast-enhanced MRI confirmed the absence of residual tumor, and diffusion-weighted MRI revealed no ischemic changes.Results:Differentiation of feeding and draining vessels in the region of the lesion is particularly important for successful surgical removal of HB. In the present three patients, ICGVAG findings enabled easy vascular differentiation and were also useful for confirming that there was no residual tumor. Indocyanin green videoangiography was concluded to be useful for safe resection of HB.


2009 ◽  
Vol 26 (4) ◽  
pp. E7 ◽  
Author(s):  
Ali A. Baaj ◽  
Siviero Agazzi ◽  
Zafar A. Sayed ◽  
Maria Toledo ◽  
Robert F. Spetzler ◽  
...  

Moyamoya disease (MMD) is a progressive, occlusive disease of the distal internal carotid arteries associated with secondary stenosis of the circle of Willis. Symptoms include ischemic infarcts in children and hemorrhages in adults. Bypass of the stenotic vessel(s) is the primary surgical treatment modality for MMD. Superficial temporal artery-to-middle cerebral artery bypass is the most common direct bypass method. Indirect techniques rely on the approximation of vascularized tissue to the cerebral cortex to promote neoangiogenesis. This tissue may be in the form of muscle, pericranium, dura, or even omentum. This review highlights the surgical options available for the treatment of MMD.


2010 ◽  
Vol 5 (3) ◽  
pp. 297-301 ◽  
Author(s):  
Ho Jun Seol ◽  
Duk Chul Shin ◽  
Yoo Seok Kim ◽  
Eun Bo Shim ◽  
Seung-Ki Kim ◽  
...  

Object Bilateral intimal thickening of the distal internal carotid arteries (ICAs) and the development of many collateral vessels in the base of the brain characterize moyamoya disease (MMD). Although the etiology of and the reason why MMD is limited to the major intracranial vessels remain unclear, flow dynamics, such as shear stress, may be related to its smooth-muscle cell migration. Therefore, this study was performed to determine the local hemodynamic factor, which concerns the predominance of specific anatomical sites, such as the distal ICA in the early stage and the proximal posterior cerebral artery (PCA) in the advanced stage of MMD. Methods The authors simulated the hemodynamics in the circle of Willis using computational models of 2D geometries of the distal ICA and PCA. A finite-element commercial package, automatic dynamics incremental nonlinear analysis (ADINA), was used to simulate blood flow in these arteries. Results Numerical results demonstrated that shear stress was relatively low at the ICA region. The distribution of shear stress was related to the predisposing area of MMD. Conclusions Diminished shear stress may promote stenosis of the distal ICA, which is a major pathological region in MMD.


2020 ◽  
Vol 55 (4) ◽  
pp. 203-209
Author(s):  
Seiichiro Eguchi ◽  
Yasuo Aihara ◽  
Kentaro Chiba ◽  
Koji Yamaguchi ◽  
Akitsugu Kawashima ◽  
...  

<b><i>Objective:</i></b> Moyamoya disease is a chronic but progressive obliterative cerebrovascular disease of bilateral internal carotid arteries (ICAs) causing hemorrhagic or ischemic cerebral strokes. Surgical revascularization has the potential for resolving the capillary vessels, but the effect on the occlusive ICA and the moyamoya vessels after a direct bypass remains unclear. <b><i>Patient:</i></b> A 2-year-old girl with a history of repeated transient ischemic attacks and direct bypasses but demonstrating improvement and associated anomaly is reported. A year and a half later, after a bilateral revascularization, an intracerebral capsulized hematoma growth was identified, and it was removed surgically. Neovascularization including many microvessels similar to capillary telangiectasia were identified by pathological investigation despite the reduction of moyamoya vessels on the repeated angiograms after the revascularization surgeries. In the present case, proliferation of capillary vessels was clearly confirmed by direct bypasses. <b><i>Conclusion:</i></b> There is no doubt that direct bypasses prevent further ischemic stroke by improving cerebral blood flow. However, they may result in failure in reducing the load of moyamoya vessels, albeit decreasing the potential risk of hemorrhagic strokes.


2021 ◽  
Vol 10 (13) ◽  
pp. 2815
Author(s):  
Yudai Hirano ◽  
Satoru Miyawaki ◽  
Hideaki Imai ◽  
Hiroki Hongo ◽  
Yu Teranishi ◽  
...  

Moyamoya disease is characterized by severe stenosis at the ends of the bilateral internal carotid arteries and the development of collateral circulation. The disease is very diverse in terms of age at onset, onset patterns, radiological findings, and genetic phenotypes. The pattern of onset is mainly divided into ischemic and hemorrhagic onsets. Recently, the opportunity to identify asymptomatic moyamoya disease, which sometimes manifests as nonspecific symptoms such as headache and dizziness, through screening with magnetic resonance imaging has been increasing. Various recent reports have investigated the associations between the clinical features of different onset patterns of moyamoya disease and the corresponding imaging characteristics. In this article, we have reviewed the natural history, clinical features, and imaging features of each onset pattern of moyamoya disease.


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