scholarly journals Moyamoya syndrome in an adult with essential thrombocythemia

2011 ◽  
Vol 3 (1) ◽  
pp. 3 ◽  
Author(s):  
Marc Lazzaro ◽  
Elizabeth J. Cochran ◽  
Demetrius K. Lopes ◽  
Shyam Prabhakaran

Moyamoya syndrome is a rare cerebrovascular disorder characterized by progressive occlusion of the supraclinoid internal carotid artery and proximal portions of the anterior and middle cerebral arteries resulting in an extensive network of collateralized blood vessels and producing a characteristic angiographic appearance. Although the pathophysiology is unclear, hematologic disorders have been associated with development of the moyamoya syndrome. A case report is presented. A 29 year-old female with a history of essential thrombocythemia developed progressive ischemic strokes. Angiography revealed characteristic moyamoya changes and pathologic examination showed intimal hyperplasia with scant collagen fibers and myxoid change. This is the first reported case of moyamoya syndrome in an adult patient with essential thrombocythemia demonstrating histological findings that suggest a shared pathophysiology with moyamoya syndrome in sickle cell anemia.

Author(s):  
Pietro Fiaschi ◽  
Marcello Scala ◽  
Gianluca Piatelli ◽  
Domenico Tortora ◽  
Francesca Secci ◽  
...  

Abstract Moyamoya vasculopathy is a rare chronic cerebrovascular disorder characterized by the stenosis of the terminal branches of the internal carotid arteries and the proximal tracts of anterior and middle cerebral arteries. Although surgical revascularization does not significantly change the underlying pathogenic mechanisms, it plays a pivotal role in the management of affected individuals, allowing to decrease the risk of ischemic and hemorrhagic complications. Surgical approaches may be direct (extracranial-intracranial bypass), indirect, or a combination of the two. Several indirect techniques classifiable according to the tissue (muscle, periosteum, galea, dura mater, and extracranial tissues) or vessel (artery) used as a source of blood supply are currently available. In this study, we reviewed the pertinent literature and analyzed the advantages, disadvantages, and pitfalls of the most relevant indirect revascularization techniques. We discussed the technical aspects and the therapeutical implications of each procedure, providing a current state-of-the-art overview on the limits and pitfalls of indirect revascularization in the treatment of moyamoya vasculopathy.


Author(s):  
Novikova I.N. ◽  
Popova T.F. ◽  
Gribacheva I.A. ◽  
Petrova E.V. ◽  
Marushchak A.A. ◽  
...  

Moya-Moya disease is a rare progressive chronic cer-ebrovascular disease characterized by a narrowing of the lumen of the intracranial segments of the internal carotid arteries, as well as the initial segments of the anterior and middle cerebral arteries with the devel-opment of a network of small vascular anastomoses. Violations of blood supply due to occlusion lead to the development of ischemic strokes in the correspond-ing pools, and ruptures of vascular anastomoses - to the development of hemorrhagic strokes, causing a variety of neurological disorders. The article presents a clinical case of Moya-Moya disease in a 31-year-old patient. The disease was manifested by acute disorders of cerebral circulation in ischemic and hemorrhagic types. The diagnosis was made in accordance with the diagnostic criteria of the disease based on the data of endovascular cerebral angiography.


1980 ◽  
Vol 53 (3) ◽  
pp. 300-304 ◽  
Author(s):  
Toshisuke Sakaki ◽  
Kazuhiko Kinugawa ◽  
Tatsuo Tanigake ◽  
Seiji Miyamoto ◽  
Kikuo Kyoi ◽  
...  

✓ Embolism from an aneurysm is one of the mechanisms involved in the pathogenesis of ischemic symptoms associated with intracranial aneurysms. Four cases are reported in which aneurysms of the internal carotid arteries and middle cerebral arteries were the source of emboli resulting in cerebral infarction. In the treatment of these aneurysms, it is best to clip the neck of the aneurysm with great care to avoid embolism due to extrusion of clot into the distal artery.


2015 ◽  
Vol 123 (4) ◽  
pp. 897-905 ◽  
Author(s):  
Daniel H. Sahlein ◽  
Mohammad Fouladvand ◽  
Tibor Becske ◽  
Isil Saatci ◽  
Cameron G. McDougall ◽  
...  

OBJECT Neuroophthalmological morbidity is commonly associated with large and giant cavernous and supraclinoid internal carotid artery (ICA) aneurysms. The authors sought to evaluate the neuroophthalmological outcomes after treatment of these aneurysms with the Pipeline Embolization Device (PED). METHODS The Pipeline for Uncoilable or Failed Aneurysms (PUFS) trial was an international, multicenter prospective trial evaluating the safety and efficacy of the PED. All patients underwent complete neuroophthalmological examinations both before the PED procedure and at a 6-month follow-up. All examinations were performed for the purpose of this study and according to study criteria. RESULTS In total, 108 patients were treated in the PUFS trial, 98 of whom had complete neuroophthalmological follow-up. Of the patients with complete follow-up, 39 (40%) presented with a neuroophthalmological baseline deficit that was presumed to be attributable to the aneurysm, and patients with these baseline deficits had significantly larger aneurysms. In 25 of these patients (64%), the baseline deficit showed at least some improvement 6 months after PED treatment, whereas in 1 patient (2.6%), the deficits only worsened. In 5 patients (5%), new deficits had developed at the 6-month follow-up, while in another 6 patients (6%), deficits that were not originally assumed to be related to the aneurysm had improved by that time. A history of diabetes was associated with failure of the baseline deficits to improve after the treatment. The aneurysm maximum diameter was significantly larger in patients with a new deficit or a worse baseline deficit at 6 months postprocedure. CONCLUSIONS Patients treated with the PED for large and giant ICA aneurysms had excellent neuroophthalmological outcomes 6 months after the procedure, with deficits improving in most of the patients, very few deficits worsening, and few new deficits developing.


2020 ◽  
Vol 15 (1) ◽  
pp. 110-113
Author(s):  
Md Abdur Razzak ◽  
Ghulam Kawnayn ◽  
Fateha Naznin ◽  
Quazi Audry Arafat Rahman

Moyamoya disease is a disease in which certain arteries in the brain are constricted. Blood flow is blocked by the constriction, and also by blood clots (thrombosis). A collateral circulation develops around the blocked vessels to compensate for the blockage, but the collateral vessels are small, weak, and prone to bleeding, aneurysm and thrombosis which may result in TIA, recurrent ischemic or hemorrhagic stroke or seizure. The disease may manifest in pediatric age or young adults. In May 2019 we have diagnosed a young lady with Moyamoya disease who presented with right sided hemiplegia, motor aphasia and dysphagia. She was labeled as hypertensive 6 months prior to this event and used to take anti-hypertensive irregularly and gave past history of occasional headache. Her CT scan and MRI of brain revealed left sided ischemic infarct involving frontotemporoparietal region and cerebral angiogram revealed narrowing of left MCA and non-visualization of distal part. There is extensive fine collaterals (Moyamoya vessels) giving the appearance of puffed smoke. The right ACA and MCA were also narrowed with appearance of early collateral vessels. She was treated with aspirin, PPI, NG feeding, antihypertensive medication, physiotherapy, rehabilitation therapy and other supportive care. His condition gradually improved and discharged on 2.7.19. He was referred to Department of Neurosurgery for cerebral revascularization by STA-MCA (superficial temporal and middle cerebral arteries) bypass surgery after stabilization and MR perfusion study. Journal of Armed Forces Medical College Bangladesh Vol.15 (1) 2019: 110-113


2014 ◽  
Vol 59 (No. 7) ◽  
pp. 343-348 ◽  
Author(s):  
W. Brudnicki ◽  
B. Skoczylas ◽  
R. Jablonski ◽  
W. Nowicki ◽  
A. Brudnicki ◽  
...  

The brain arteries derived from 50 adult degu individuals of both sexes were injected with synthetic latex introduced with a syringe into the left ventricle of the heart under constant pressure. After fixation in 5% formalin and brain preparation, it was found that the sources of the brain’s supply of blood are vertebral arteries and the basilar artery formed as a result of their anastomosis. The basilar artery gave rise to caudal cerebellar arteries and then divided into two branches which formed the arterial circle of the brain. The internal carotid arteries in degus, except for one case, were heavily reduced and did not play an important role in the blood supply to the brain. The arterial circle of the brain in 48% of the cases was open from the rostral side. Variation was identified in the anatomy and the pattern of the arteries of the base of the brain in the degu which involved an asymmetry of the descent of caudal cerebellar arteries (6.0%), rostral cerebellar arteries (8%) as well as middle cerebral arteries (12%). In 6% of the individuals double middle cerebral arteries were found. In one out of 50 cases there was observed a reduction in the left vertebral artery and the appearance of the internal carotid artery on the same side. In that case the left part of the arterial circle of the brain was supplied with blood by an internal carotid artery, which was present only in that animal.


2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Le H. Hua ◽  
Robert L. Dodd ◽  
Neil E. Schwartz

Moyamoya disease is a stenoocclusive disease involving the intracranial carotid and proximal middle cerebral arteries. There are rarely any additional extracranial stenoses occurring concurrently with moyamoya. The pathophysiology of moyamoya remains obscure, but hematologic disorders, notably sickle-cell anemia, have been associated in some cases. We describe the novel case of polycythemia vera associated with severe steno-occlusive disease of both intracranial and extracranial large arteries. A 47-year-old woman with polycythemia vera had multiple transient ischemic attacks, and noninvasive vessel imaging revealed steno-occlusive disease of bilateral supraclinoid internal carotid arteries with moyamoya-type collaterals, proximal left subclavian artery, right vertebral artery origin, bilateral renal arteries, superior mesenteric artery, and right common iliac artery. Laboratory workup for systemic vasculitis was negative. She required bilateral direct external carotid to internal carotid bypass procedures and percutaneous balloon angioplasty of her right VA origin stenosis. This case suggests that hematologic disorders can lead to vessel stenoses and occlusion. The pathophysiology may be due to a prothrombotic state leading to repeated endothelial injury, resultant intimal hyperplasia, and progressive steno-occlusion.


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