(A) Left common carotid angiogram demonstrating moderate stenosis of the proximal portion of the left external carotid artery. (B) Left common carotid angiogram after stent and balloon angioplasty revealing almost normal caliber of the external carotid artery.

(A) Left common carotid angiogram demonstrating moderate stenosis of the proximal portion of the left external carotid artery. (B) Left common carotid angiogram after stent and balloon angioplasty revealing almost normal caliber of the external carotid artery.

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A 55-year-old woman presented with moyamoya disease manifesting as recurrent transient ischemic attacks despite taking aspirin and antihypertensive agent. Angiography showed the characteristic angiographic appearance with bilateral internal carotid artery occlusion and abnormal collateral vessels. Left external carotid angiography demonstrated mode...

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Context 1
... circulation from the left MMA. Brain technetium-99m exametazime (Ceretec; Amersham, Buckinghamshire, U.K.) single photon emission computed tomography revealed a region of decreased perfusion in the left frontoparietal cortex (Fig. 3). Left external carotid angiography demonstrated moderate stenosis of the proximal portion of the left ECA (Fig. 4A). Vertebral angiography showed adequate leptomeningeal collateral circulation from the bilateral posterior cerebral arteries. The extracranial vertebral arteries had normal ...
Context 2
... Co., Natick, Mass., U.S.A.) was introduced from the ECA to the common carotid artery. After deployment of the stent, left carotid angiography showed persistent narrowing of the lumen. Therefore, balloon angioplasty was performed with a balloon catheter. After angioplasty, final carotid angiography showed almost normal caliber of the ECA (Fig. 4B). Heparin administration was discontinued after the procedure. The postoperative course was uneventful and the patient was discharged at 4 days after the procedure. The patient received aspirin (100 mg per day) and clopidogrel (75 mg per day) for 3 months. Follow-up angiography at 1 year revealed patency of the stented artery with mild ...

Citations

... Regarding autopsy findings, hypertensive change was also observed in the vertebrobasilar artery of patients with moyamoya disease. 2) Lee et al. 5) reported a patient with moyamoya disease who had a favorable outcome after stenting for external carotid artery stenosis and suggested that external carotid artery stenosis was likely to have been atherosclerotic change. ...
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Objective: We report a case of acute internal carotid artery occlusion in a patient with adult-onset moyamoya disease who underwent mechanical thrombectomy and had a good outcome. Case Presentation: A 73-year-old woman was diagnosed with moyamoya disease by asymptomatic right middle cerebral artery occlusion at 59 years of age. The patient was transported for stroke symptoms. Magnetic resonance imaging (MRI) demonstrated left terminal internal carotid artery occlusion and low-intensity signal on T2*-weighted imaging at the occlusion site. Alteplase was administered and endovascular treatment was subsequently performed. A small-diameter microcatheter was guided to the distal end of the occlusion and angiography after deployment of a stent retriever revealed irregular stenosis. Severe stenosis remained after thrombectomy, and balloon angioplasty was added. The treatment resulted in recanalization and good outcome. Conclusion: Adults with moyamoya disease may have accompanying atherosclerotic intracranial artery occlusion. Angiography after deployment of a stent retriever was useful for clarifying the etiology of occlusion. It is important to determine the etiology of occlusion based on the medical history or imaging findings and to select an appropriate treatment.
... The idiopathic form of MMD has been linked to several genetic loci in the Asian population, including the RNF213 gene in chromosome 17 [2]. Patients with moyamoya syndrome (MMS) have angiographic findings similar to those of MMD patients but with well-known associated medical conditions, such as atherosclerosis [3], diabetes, infection [4], sickle cell disease [5][6][7], neurofibromatosis type 1 [8], tuberous sclerosis [9], Sturge-Weber syndrome [10], Down syndrome [11], and Turner syndrome [12]. ...
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Background and purpose: Cerebral microbleeds (CMB) are reported to be frequent in moyamoya disease (MMD) and moyamoya syndrome (MMS) in the Asian population. It is associated with an increased risk of intracerebral hemorrhage. The significance of CMB in MMD/MMS in non-Asian populations has not been well established. Our study aimed to investigate the prevalence of CMB in MMD/MMS in a moymoya cohort with a majority of non-Asians and to identify risk factors for developing a CMB and its predictive value for subsequent vascular events. Methods: The moyamoya database was compiled by screening for MMD/MMS among patients admitted to the Zale-Lipshy University Hospital at the University of Texas Southwestern Medical Center. We identified and analyzed data of 67 patients with MMD or MMS. Patients were characterized as CMB+ or CMB- based on MRI findings. In CMB+ patients, the total number and location of CMB were identified. Univariate and multivariate logistic regression were used to identify risk factors for developing CMB and whether CMB are associated with the development of subsequent vascular events. Results: Out of a total of 67 patients, 11 (16%) had CMB. Males had significantly higher odds of having CMB as compared to females (OR 1.76; 95% CI 1.40-24.3, p = 0.021). The incidence of CMB was also associated with age at diagnosis (mean age of CMB+ patients vs. CMB- patients: 44 vs. 34 years, respectively, p = 0.024), smoking (p = 0.006), and hemorrhagic stroke at presentation (p = 0.034). Logistic regression with multivariate analysis found that gender and age at diagnosis remained statistically significant. New ischemic events occurred in 2 (20%) out of 10 CMB+ patients and 13 (23%) out of 55 CMB- patients, respectively (p = 0.79). While 2 (3%) CMB- patients had a new cerebral hemorrhage during follow-up, none of the CMB+ patients did. Conclusions: CMB are less prevalent in MMD/MMS in the USA than in Asia. An older age at diagnosis and male gender were associated with CMB. The presence of CMB was not associated with an increased risk of a subsequent ischemic or hemorrhagic stroke.
... Table 1 shows reports and reviews in which patients received ECA intervention, including endarterectomy or stenting. [3][4][5][6][7][8][9][10][11][12][13][14] The majority of patients receiving surgery or angioplasty had concurrent symptomatic ipsilateral ICA occlusion from underlying atherosclerotic disease. Presentation included signs and symptoms of ischemic stroke, transient ischemic attack, and amaurosis fugax. ...
Article
Background Moyamoya disease is characterized by progressive narrowing of the internal carotid artery (ICA). Symptomatic patients typically undergo cerebrovascular intervention via extracranial–intracranial (EC–IC) bypass, most often with the use of the superficial temporal artery. This case of Moyamoya disease is of particular interest as the patient presented with a unilateral atherosclerotic external carotid artery (ECA) stenosis after EC–IC bypass that eliminated the benefit of his original surgery, resulting in a symptomatic presentation. Clinical presentation A 53-year-old man presenting with Moyamoya disease and known left ICA occlusion had received a bilateral encephaloduroarteriosynangiosis (EDAS) bypass 10 years previously. He re-presented complaining of right-sided tingling, weakness, and numbness radiating up the arm. CT angiography indicated significant stenosis of the left ECA. ECA angioplasty and stenting with a distal protection device resulted in resolution of his symptoms. Conclusions This case illustrates that a patient presenting with Moyamoya disease and concurrent symptomatic ECA stenosis post-EDAS can be effectively and safely treated with ECA stenting.
... All reports describing angioplasty and/or stenting of stenotic intracranial vessels in a patient with moyamoya were collected. One report that described stenting of an atherosclerotic, stenosed external carotid artery in a patient with moyamoya was thus excluded [15]. ...
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Moyamoya is a rare though important source of neurological morbidity as a result of both ischemic and hemorrhagic sequelae. Although a litany of series detailing the endovascular management of cerebral ischemia is present in the literature, only a paucity of such reports exists for moyamoya. A systematic review of the literature was performed for patients with moyamoya managed with endovascular techniques in addition to the contribution of an additional case managed at our institution. We evaluated treatment approach (angioplasty and/or stent), complications, and both angiographic and clinical outcomes at last follow-up. Results from a total of 28 endovascular procedures were collected (11 stenting, 17 angioplasty alone). Procedural success, defined as a lack of both angiographic and clinical recurrence at follow-up, was achieved after seven procedures (25 %). This rate did not significantly differ between disease type (moyamoya disease vs moyamoya syndrome, p = 1.0) and treatment approach (angioplasty alone vs stenting, p = 1.0). The overall monthly angiographic and clinical recurrence rates were 9.3 and 8.0 %, respectively. Clinically devastating intracerebral hemorrhage was seen after two procedures (7 %), and in an additional three procedures, the treated vessel could not be effectively dilated (11 %). There is no evidence that angioplasty or stenting improves the natural history of moyamoya. Both are associated with significant rates of early angiographic and/or clinical recurrence of symptoms. Taken with the risk of procedural complications, the current limited data should advise against attempted endovascular treatment of moyamoya.
... Intraoperative and patho-logical observations have shown that the outer diametersof the relevant ICA terminus is markedly reduced due tointimal fibrocellular thickening, an uneven waving of theinternal elastic lamina and attenuation of the media; then, pathognomonic collateral arterial networks areextensively developed and seem to be closely associatedwith the onset of infarction and hemorrhage (3) . In addition, an essential arterial changes could occur to the ver-tebrobasilar system due to the compensatory verte-brobasilar hypertension secondary to the ICA occlusionor stenosis (9,12) . With our patient, the basilar arterial arte-riosclerosis (Fig. B) is suggestive of hemodynamicchanges and consequent tissue responses secondary tothe ICA terminus stenosis. ...
Article
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Purpose: The oldest patients diagnosed with moyamoya disease (MMD) in the USA may have been as old as 85+ years, and 68 years in Taiwan; therefore, MMD is generally thought not to occur in extremely old patients in Taiwan. Herein, we report this case to revise the common thinking. Case report: An 82-year-old woman had suffered twice from a right cerebral infarction. A digital subtraction angiogram demonstrated abundant collateral arterial networks from the bilateral proximal middle cerebral arteries despite the absence of arteriosclerotic stenosis of the bilateral extracranial internal carotid arteries. Aspirin has effectively controlled her symptom since diagnosis and, as a result, a direct or indirect revascularization procedure is unnecessary. Conclusion: MMD does occur in extremely old Taiwanese, as old as over 80 years old, and thus should be a differential diagnosis of cerebral infarction in an extremely old patient. Digital subtraction angiography is the gold standard for the diagnosis.
Article
Moyamoya disease (MMD) is a rare disease characterized by progressive stenosis of the terminal part of the internal carotid arteries (ICA) and the proximal part of their branches, which is accompanied by the formation of collateral network that look like smoke clouds on angiography (Japanese terminology - moyamoya). If the disease is comorbid to other diseases, usually associated with acute or chronic inflammation, including autoimmune processes, it is designated as moyamoy syndrome (MMS). MMD and MMS are one of the causes of ischemic stroke and chronic cerebrovascular insufficiency in young and middle age, less often they lead to hemorrhages. The review presents data on epidemiology, morphology, pathogenesis (the role of genetic predisposition, inflammation, proangiogenic factors and immune disorders), clinical manifestations, instrumental diagnostics and treatment.
Article
Objective To assess the safety and efficacy of endovascular treatment for basilar tip aneurysms associated with moyamoya disease. Methods Seven patients with basilar tip aneurysms associated with moyamoya disease were treated by endovascular embolization in our department between Oct. 2006 and Jul. 2013. Two patients were treated by pure coiling and the others were treated by stent-assisted coiling. The immediate angiographic result was evaluated by Raymond scale. Postoperative angiographic and clinical follow-ups were obtained in all the 7 cases, and the clinical outcomes were evaluated by the modified Rankin score (mRS). Results All the 7 patients successfully received endovascular embolization without procedure-related complications. Immediate angiographic results showed complete occlusion in 3 cases, neck residual in 2, and partial occlusion in 2. Postoperative angiographic follow-ups were obtained for a mean of (10. 4 ± 3. 5) months, with complete occlusion found in 6 patients and stable in 1 patient. No progressive thrombosis or in-stent occlusion were detected by follow-up angiograms in the stent group. Clinical follow-ups were obtained for a mean of (21. 1 ± 15. 8) months and all the patients reported good outcomes (mRS 0-2). Conclusion Our data suggest that endovascular embolization is a safe and efficient treatment for basilar tip aneurysms associated with moyamoya disease. Stent-assistance might be a safe and effective option for the large or wide-necked aneurysms, though the long-term safety still remains to be confirmed. © 2015, Second Military Medical University Press. All rights reserved.
Article
Carotid artery stenting (CAS) is a validated option in the treatment of selected extracranial carotid artery stenosis. Carotid artery dissection during CAS is a rare but potentially devastating complication. We report a case of acute dissection and thrombosis of the left internal carotid artery during filter tip wire engaging maneuvers, complicated by intraoperative complete blindness of the left eye. Immediate conversion to carotid endarterectomy was performed under general anesthesia with electroencephalographic monitoring. The patient was discharged home symptomless and remains asymptomatic eight months after the operation, with normal left internal carotid patency and fully recovered eyesight. In conclusion, the management of acute carotid occlusion during CAS requires emergent evaluation and definitive endovascular or open surgical repair to minimize neurologic morbidity. We advocate that all endovascular procedures are carried out in a well-established surgical environment.
Article
A 58-year-old Caucasian lady presented for severe left internal carotid artery (ICA) stenosis. Two months before she was operated for right carotid endarterectomy (CEA) in another Institution, complicated with internal carotid artery thrombosis and development of transient hemiplegia and aphasia. Postoperative selective DSA showed the development of an abnormal basal meshwork of collateral vessels with typical evidence for moyamoya disease. Preoperative workup in our Institution included cerebral MRI witch showed two ischemic right frontal and parietal lesions. The patient underwent successful stenting of the left ICA. This is the first report of extracranial ICA stenting in a patient with moyamoya syndrome.