DIFFICULTIES IN DIAGNOSING MOYA-MOYA DISEASE (CLINICAL CASE)

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.


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.


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.


1991 ◽  
Vol 69 (12) ◽  
pp. 1889-1895 ◽  
Author(s):  
Yasuaki Kawai ◽  
Shin Yokoyama ◽  
Toshio Ohhashi

The time course of contractile responses to α-adrenoceptor agonists was investigated using various arteries isolated from dogs and monkeys. The contractile response to norepinephrine was increased during the time course of the experiment in canine basilar and internal carotid arteries, whereas the response of isolated canine external carotid arteries and monkey internal carotid arteries did not change significantly. Treatment with 10−7 M propranolol, 5 × 10−6 M cocaine plus 10−5 M hydrocortisone, or 5 × 10−5 M acetylsalicylic acid did not significantly affect the time-dependent potentiation of the norepinephrine-induced contraction in canine internal carotid arteries. The time-dependent enhancement in the response to norepinephrine was also observed in the arterial preparations from which the endothelial cells were removed. The contractile response of canine internal carotid arteries to phenylephrine did not alter significantly throughout the experiments. On the other hand, the responses to clonidine and xylazine were markedly enhanced with time. Significant potentiation of the norepinephrine-induced contraction was observed in canine internal carotid arteries treated with 10−8 M prazosin, whereas 10−8 M yohimbine attenuated the time-dependent potentiation. These results suggest that the contractile responses of isolated canine basilar and internal carotid arteries to norepinephrine are potentiated during the course of the experiment, which is likely to be related, in part, to an enhancement in α2-adrenoceptor mediated contraction.Key words: α2-adrenoceptor, dog, cerebral artery, enhancement of contraction, vascular smooth muscle.


1954 ◽  
Vol 32 (1) ◽  
pp. 539-547 ◽  
Author(s):  
Robert C. Buck ◽  
James C. Paterson ◽  
Roger J. Rossiter

The concentration of total cholesterol, free cholesterol, total phospholipid, nonlipid phosphorus, calcium, and fat-free dry residue was determined in the internal carotid and cerebral arteries obtained at autopsy from a series of 14 male subjects ranging in age from 40 to 85 yr. From these figures the concentration of cholesterol ester and percentage of the total cholesterol in the ester form was calculated.In terms of wet weight, there was, in general, no significant difference between the mean concentration of lipids in the internal carotid arteries and that in the cerebral arteries. On the other hand, the mean concentration of calcium and nonlipid phosphorus was much greater in the internal carotid arteries than in the cerebral arteries.By the method of rank correlation it was found that for both vessels the concentration of total, free, and ester cholesterol was significantly correlated with both age and the severity of the atherosclerosis. Ester cholesterol expressed as a percentage of total cholesterol was also significantly correlated with age and the severity of the disease process.For the internal carotid artery, the concentration of calcium and nonlipid phosphorus was correlated with both age and the severity of the atherosclerosis. This was in contrast to the cerebral arteries in which the concentration of calcium and nonlipid phosphorus was extremely low and was found to be correlated neither with age nor the severity of the disease process.


2018 ◽  
Vol 39 (7) ◽  
pp. 1204-1214 ◽  
Author(s):  
Baraa K Al-Khazraji ◽  
Leena N Shoemaker ◽  
Joseph S Gati ◽  
Trevor Szekeres ◽  
J Kevin Shoemaker

The larger intracranial conduit vessels contribute to the total cerebral vascular resistance, and understanding their vasoreactivity to physiological stimuli is required when attempting to understand regional brain perfusion. Reactivity of the larger cerebral conduit arteries remains understudied due to a need for improved imaging methods to simultaneously assess these vessels in a single stimulus. We characterized reactivity of basal intracranial conduit arteries (basilar, right and left posterior, middle and anterior cerebral arteries) and the right and left internal carotid arteries, to manipulations in end-tidal CO2 (PetCO2). Cross-sectional area changes (%CSA) were evaluated from high-resolution (0.5 mm isotropic) images collected at 7 T using a T1-weighted 3D SPACE pulse sequence, providing high contrast between vessel lumen and surrounding tissue. Cerebrovascular reactivity was calculated as %CSA/ΔPetCO2 in eight healthy individuals (18–23 years) during normocapnia (41 ± 4 mmHg), hypercapnia (48 ± 4 mmHg; breathing 5% CO2, balance oxygen), and hypocapnia (31 ± 8 mmHg; via hyperventilation). Reactivity to hypercapnia ranged from 0.8%/mmHg in the right internal carotid artery to 2.7%/mmHg in the left anterior cerebral artery. During hypocapnia, vasoconstriction ranged from 0.9%/mmHg in the basilar artery to 2.6%/mmHg in the right posterior cerebral artery. Heterogeneous cerebrovascular reactivity to hypercapnia and hypocapnia was characterized across basal intracranial conduit and internal carotid arteries.


2020 ◽  
pp. 1-3
Author(s):  
Ankita Shah

The moyamoya syndrome is a cerebrovascular condition that predisposes affected patients to stroke due to progressive stenosis of the intracranial internal carotid arteries and their proximal branches. It can be seen in association with several inherited disorders. However, its association with thalassemia is very rare.


1954 ◽  
Vol 32 (5) ◽  
pp. 539-547 ◽  
Author(s):  
Robert C. Buck ◽  
James C. Paterson ◽  
Roger J. Rossiter

The concentration of total cholesterol, free cholesterol, total phospholipid, nonlipid phosphorus, calcium, and fat-free dry residue was determined in the internal carotid and cerebral arteries obtained at autopsy from a series of 14 male subjects ranging in age from 40 to 85 yr. From these figures the concentration of cholesterol ester and percentage of the total cholesterol in the ester form was calculated.In terms of wet weight, there was, in general, no significant difference between the mean concentration of lipids in the internal carotid arteries and that in the cerebral arteries. On the other hand, the mean concentration of calcium and nonlipid phosphorus was much greater in the internal carotid arteries than in the cerebral arteries.By the method of rank correlation it was found that for both vessels the concentration of total, free, and ester cholesterol was significantly correlated with both age and the severity of the atherosclerosis. Ester cholesterol expressed as a percentage of total cholesterol was also significantly correlated with age and the severity of the disease process.For the internal carotid artery, the concentration of calcium and nonlipid phosphorus was correlated with both age and the severity of the atherosclerosis. This was in contrast to the cerebral arteries in which the concentration of calcium and nonlipid phosphorus was extremely low and was found to be correlated neither with age nor the severity of the disease process.


2019 ◽  
Vol 20 (2) ◽  
pp. 239-242
Author(s):  
Finat Airatovich Utyashev ◽  
◽  
Liana Raisovna Iskhakova ◽  
Elina Mansurovna Saydasheva

2001 ◽  
Vol 176 (1) ◽  
pp. 263-264 ◽  
Author(s):  
Rodolfo A. Ibarra ◽  
Prabhakar Kesava ◽  
John L. Fewins

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