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1.
Radiol. bras ; 55(1): 31-37, Jan.-Feb. 2022. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1360664

ABSTRACT

Abstract Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, often clinically manifesting as ischemic or hemorrhagic stroke with high rates of morbidity and mortality. On cerebral angiography, the formation of collateral vessels has the appearance of a puff of smoke (moyamoya in Japanese), which became more conspicuous with the refinement of modern imaging techniques. When there is associated disease, it is known as moyamoya syndrome. Treatments are currently limited, although surgical revascularization may prevent ischemic events and preserve quality of life. In this review, we summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies.


RESUMO A doença de moyamoya, ou doença cerebrovascular oclusiva crônica, é uma afecção não inflamatória e não aterosclerótica, caracterizada por hiperplasia endotelial e fibrose dos segmentos intracranianos das artérias carótidas internas e da porção proximal de seus ramos. Isso provoca estenose progressiva e oclusão, frequentemente manifestada clinicamente como isquemia cerebral ou hemorragia intracraniana, com alta morbimortalidade. A formação compensatória de vasos colaterais produz, na angiografia encefálica, um aspecto de nuvem de fumaça (moyamoya, em japonês). Quando existe doença subjacente que possa estar relacionada, a doença recebe o nome de síndrome de moyamoya. Embora a incidência esteja aumentando graças aos novos métodos diagnósticos, as estratégias terapêuticas ainda são limitadas. O diagnóstico precoce permite cirurgias de revascularização cerebral que podem evitar novos acidentes vasculares e melhorar a qualidade de vida. Nesta revisão são apresentados os avanços recentes sobre a doença de moyamoya, citando aspectos de epidemiologia, etiologia, apresentação, exames diagnósticos e tratamento.

2.
Arq. neuropsiquiatr ; 79(4): 321-333, Apr. 2021. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1278384

ABSTRACT

ABSTRACT Background: Pediatric arterial ischemic stroke (AIS), which was thought to be a rare disorder, is being increasingly recognized as an important cause of neurological morbidity, thanks to new advances in neuroimaging. Objective: The aim of this study was to review the main etiologies of stroke due to arteriopathy in children. Methods: Using a series of cases from our institution, we addressed its epidemiological aspects, physiopathology, imaging findings from CT, MR angiography, MR conventional sequences and MR DWI, and nuclear medicine findings. Results: Through discussion of the most recent classification for childhood AIS (Childhood AIS Standardized Classification and Diagnostic Evaluation, CASCADE), we propose a modified classification based on the anatomical site of disease, which includes vasculitis, varicella, arterial dissection, moyamoya, fibromuscular dysplasia, Takayasu's arteritis and genetic causes (such as ACTA-2 mutation, PHACE syndrome and ADA-2 deficiency). We have detailed each of these separately. Conclusions: Prompt recognition of AIS and thorough investigation for potential risk factors are crucial for a better outcome. In this scenario, neurovascular imaging plays an important role in diagnosing AIS and identifying children at high risk of recurrent stroke.


RESUMO Introdução: O acidente vascular cerebral (AVC) pediátrico, considerado um distúrbio raro, está sendo cada vez mais reconhecido como importante causa de morbidade neurológica, graças aos novos avanços na neuroimagem. Objetivo: Revisar as principais etiologias do AVC por arteriopatia em crianças. Métodos: Utilizando-se de uma série de casos de nossa instituição, abordamos seus aspectos epidemiológicos, fisiopatológicos e de imagem na angiotomografia computadorizada e angiorressonância magnética, sequências convencionais e avançadas de ressonância magnética e medicina nuclear. Resultados: Com base na classificação mais recente de AVC na infância (Classificação Padronizada e Avaliação Diagnóstica do AVC na Infância - CASCADE) propusemos uma classificação modificada com base no local anatômico da doença, que inclui vasculite, varicela, dissecção arterial, Moyamoya, displasia fibromuscular, arterite de Takayasu e causas genéticas (como mutação ACTA-2, síndrome PHACE e deficiência de ADA-2), detalhando cada uma separadamente. Conclusões: O reconhecimento imediato do AVC na infância e a investigação minuciosa de possíveis fatores de risco são cruciais para um melhor resultado. Nesse cenário, a imagem neurovascular desempenha papel importante no diagnóstico de AVC e na identificação de crianças com alto risco de recorrência.

3.
J. vasc. bras ; 20: e20200216, 2021. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1279379

ABSTRACT

Abstract Moyamoya disease is a rare disorder that involves the cerebrovascular system. Usually, it leads to occlusion of the arteries of the cerebral system and causes cerebral circulatory complaints. A 48-year-old female patient was admitted to our clinic with intermittent claudication in both legs. Biphasic and monophasic waveform patterns were detected bilaterally in distal (trifurcation arteries) lower extremities with Doppler sonography. The patient therefore underwent systemic vascular examination. Computed tomography angiography revealed bilateral carotid occlusion at the level of supraclinoid segments, and opacifications were detected at the distal segments of the bilateral anterior cerebellar and middle cerebellar arteries. The patient was diagnosed with moyamoya disease, and anticoagulant treatment was started. In conclusion, most previous reports have presented the cerebrovascular involvement of moyamoya disease. However, this disease can involve different peripheral vascular systems and careful and systemic vascular examination is necessary for an exact diagnosis.


Resumo A doença de moyamoya é um distúrbio raro que envolve o sistema cerebrovascular. Normalmente, leva à oclusão das artérias do sistema cerebral e causa problemas circulatórios no cérebro. Uma mulher de 48 anos foi admitida em nossa clínica com claudicação intermitente em ambas as pernas. Na ultrassonografia com Doppler, foram detectados padrões de formato de onda bifásico e monofásico nas extremidades inferiores distais (artérias da trifurcação) de forma bilateral. Portanto, realizou-se o exame vascular sistêmico na paciente. A angiografia por tomografia computadorizada revelou oclusão carotídea bilateral no nível dos segmentos supraclinoides, e opacificações foram detectadas nos segmentos distais das artérias cerebelares anteriores e médias de forma bilateral. A paciente foi diagnosticada com doença de moyamoya, e o tratamento anticoagulante foi iniciado. Em conclusão, a maioria dos relatos anteriores apresentou o envolvimento cerebrovascular da doença de moyamoya. No entanto, essa doença pode envolver diferentes sistemas vasculares periféricos, e um exame vascular sistêmico minucioso é necessário para um diagnóstico exato.

4.
Article in Chinese | WPRIM | ID: wpr-911298

ABSTRACT

Objective:To identify the risk factors for early neurological complications after revascularization in adult patients with moyamoya disease.Methods:The medical records of patients of both sexes with moyamoya disease, aged 18-65 yr, of American Society of Anesthesiologists physical status Ⅱ or Ⅲ, who underwent revascularization in our hospital from January 2017 to June 2019, were retrospectively collected.According to the occurrence of early postoperative neurological complications, patients were divided into early postoperative neurological complication group and non-early postoperative neurological complication group.The factors such as patient′s age, gender, preoperative clinical symptoms, previous history of hypertension, history of diabetes, history of coronary heart disease, American Society of Anesthesiologists physical status, methods of anesthesia, type of operation, anesthesia time, time for start of operation, operation time, intraoperative urine volume, times of intraoperative vasoactive drugs used, and time of the post-anaesthesia observation room (PACU) stay were collected.Logistic regression analysis was used to identify the risk factors for postoperative early neurological complications.Results:A total of 510 adult patients with moyamoya disease underwent revascularization were enrolled in this study, and the incidence of early postoperative neurological complications was 9.0%.The results of logistic regression analysis showed that preoperative ischemia, intraoperative use of vasoactive drugs more than 3 times and PACU stay time>90 min were risk factors for postoperative neurological complications ( P<0.05). Conclusion:Preoperative ischemia, intraoperative use of vasoactive drugs >3 times and PACU stay time>90 min are risk factors for early neurological complications after revascularization in the patients with moyamoya disease.

5.
Rev. argent. neurocir ; 34(3): 223-225, sept. 2020.
Article in Spanish | LILACS, BINACIS | ID: biblio-1120955

ABSTRACT

Introducción: En 1957, Takeuchi y Shimizu describen una vasculopatía oclusiva que involucra la arteria carótida interna bilateral, con la formación de vasos colaterales. En 1969, Suzuki y Takaku denominan a la conexión vascular colateral en las imágenes de angiografía "moyamoya" que significa nube de humo.2,3 Objetivos: El propósito del siguiente video es la descripción detallada de una cirugía de revascularización directa a través de un bypass temporosilviano en paciente con enfermedad Moyamoya. Materiales y Métodos: Se describe el caso de un paciente masculino de 27 años de edad que presentó de accidente vascular cerebral hemorrágico derecho. En la angiografía se diagnosticó estenosis del 70% de la arteria carótida interna supraclinoidea derecha, acompañado de estenosis de la arteria cerebral media y cerebral anterior homolateral. Se realizó cirugía de revascularización cerebral directa con bypass temporosilviano derecho.4,5 Resultados: Luego de realizado el bypass se confirmó adecuada permeabilidad del mismo y en la angiografía postoperatoria se observó el desarrollo de circulación colateral a través de la anastomosis. El paciente no presentó déficit en el periodo postoperatorio. Conclusión: Aunque la incidencia de enfermedad de Moyamoya no es elevada, es una causa probable de stroke isquémico o hemorrágico en niños y adultos. El manejo adecuado es fundamental para mejorar el pronostico a largo plazo de los pacientes con esta rara patología.


Introduction: In 1957, Takeuchi and Shimizu describes an occlusive vasculopathy involving the bilateral internal carotid arteries, with the formation of collateral vessels. In 1969, Suzuki and Takaku designate the collateral vascular connections in the angiographical images "moyamoya" which means puff of smoke.2,3 Objectives: The purpose of the following video is the detailed description of a direct revascularization surgery through a temporosilvian bypass in a patient with Moyamoya disease. Materials and methods: We present a case of a 27-year-old male patient with a history of right hemorrhagic cerebral vascular accident. In the angiography, 70% stenosis of the right supraclinoid internal carotid artery was diagnosed, accompanied by stenosis of the middle and anterior homolateral cerebral artery. Direct cerebral revascularization surgery was performed with right temporosilvian bypass.4,5 Results: After performing the bypass, adequate permeability is confirmed and in the postoperative angiography the development of collateral circulation through the anastomosis was observed. The patient did not present a deficit in the postoperative period. Conclusion: Although the incidence of Moyamoya disease is not high, it is a probable cause of ischemic or hemorrhagic stroke in children and adults. Proper management is essential to improve the long-term prognosis of patients with this rare pathology.


Subject(s)
Humans , Male , Moyamoya Disease , General Surgery , Cerebral Revascularization
6.
Braz. j. med. biol. res ; 53(11): e9974, 2020. graf
Article in English | ColecionaSUS, LILACS, ColecionaSUS | ID: biblio-1132490

ABSTRACT

Moyamoya disease (MMD) is currently thought to involve endothelial progenitor cells (EPCs). We investigated whether superparamagnetic iron oxide (SPIO) can be used to label EPCs. Mononuclear cells from 10 moyamoya disease patients were isolated, and cluster of differentiation 133 (CD133) positive cells sorted by magnetic-activated cell sorting were cultured in vitro. The positive rates of CD133, vascular endothelial growth factor receptor (VEGFR)-2, and cluster of differentiation 34 (CD34) were detected by flow cytometry. The cells were co-cultured with fluorescence labeled Dil-acetylated-low-density lipoprotein (Dil-ac-LDL) and Ulex europaeus agglutinin-1 (UEA-1) to observe the endocytosis of Dil-ac-LDL and binding to UEA-1. Prussian blue staining and transmission electron microscopy were used to observe the endocytosis of different SPIO concentrations in EPCs, and CCK-8 was used to detect proliferation of cells transfected with different concentrations of SPIO. T2 weighted imaging (T2WI) signals from magnetic resonance imaging after SPIO endocytosis were compared. Positive rates of CD133, VEGFR-2, and CD34 on sorted mononuclear cells were 68.2±3.8, 57.5±4.2, and 36.8±6.5%, respectively. The double-positive expression rate of CD34 and VEGFR-2 was 19.6±4.7%, and 83.1±10.4% of cells, which showed the uptake of Dil-ac-LDL and binding with UEA-1. The labeling efficiencies of SPIO at concentrations of 25 and 50 μg/mL were higher than for 12.5 μg/mL. The proliferation of cells was not influenced by SPIO concentrations of 12.5 and 25 μg/mL. After labeling, the T2WI of EPCs was reduced. The concentration of 25 μg/mL SPIO had high labeling efficiency detected by magnetic resonance imaging (MRI) without decreased EPCs viability.


Subject(s)
Humans , Male , Adult , Middle Aged , Magnetite Nanoparticles , Endothelial Progenitor Cells , Moyamoya Disease/diagnostic imaging , Magnetic Resonance Imaging , Ferric Compounds , Cells, Cultured , Vascular Endothelial Growth Factor A , Metal Nanoparticles
7.
Rev. neuropsiquiatr ; 82(4): 285-292, oct.-dic 2019.
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1144851

ABSTRACT

Se reporta el caso de un paciente de 32 años de edad que desde los 17 y hasta la actualidad, ha experimentado cefaleas constrictivas de minutos de duración, desencadenadas por actividad o esfuerzos físicos. En los últimos siete años ha presentado progresiva declinación cognitiva y cambios acentuados de personalidad con creciente dependencia para el desarrollo de actividades elementales. A los 27 años desarrolló hemiplejía izquierda total con recuperación completa en dos semanas. Muestra asimismo episodios de estado confusional severo y conducta infantil. Los exámenes de laboratorio descartaron cardiopatía, ateroesclerosis y enfermedades autoinmunes sistémicas en el paciente. Estudios neuroimagenológicos del cerebro (TAC, Angio - TEM y RM cerebralcontrastada) pusieron en evidencia seis infartos cerebrales, en estadios subagudos y crónicos, severa estenosis de las arterias cerebrales anteriores y presencia de un microaneurisma en el segmento M de la arteria cerebral media izquierda. Una limitación del presente reporte es la ausencia de estudios con angiografía digital, debida a dificultades económicas. En conclusión, el caso corresponde a un paciente con cefalea crónica asociada a demencia progresiva, en el que estudios de neuroimágenes y pruebas neuropsicológicas sustentan el diagnóstico de deterioro cognitivo asociado a la enfermedad de Moyamoya.


The case of a 32 years old male patient who since age 17 has experienced constrictive headaches of several minutes duration, triggered by physical effort is reported. For the last seven years there, he has presented a progressive cognitive decline cognitive, and marked personality changes, having become extremely dependent even for conducting elemental activities. At the age of 27, he experienced total left hemiplegia from which he completely recovered in two weeks. He also shows severe confusional states and child-like behavior. Laboratory tests ruled out heart disease, atherosclerosis and systemic autoimmune diseases. Brain neuroimaging tests (CT, Angio - TEM and contrasted cerebral MRI) revealed the occurrence of six cerebral infarcts, in subacute and chronic stages, severe stenosis of the anterior cerebral arteries, and presence of microaneurysm in the M segment of the left middle brain artery. A limitation of this report is the absence of a study with digital angiography, due to economic difficulties. In short, the case of a patient with chronic headache associated with progressive dementia is presented, with neuroimaging and neuropsychological tests that support the diagnosis of of cognitive impairment associated with Moyamoya disease.

8.
Article | IMSEAR | ID: sea-204270

ABSTRACT

Moyamoya cerebral angiopathy is characterized by progressive stenosis or occlusion of the internal carotid artery or its branches with subsequent development of basilar collaterals. It is commonly seen in Asian population. Authors present a case report of a 12 year-old boy with Multidrug resistant tuberculosis with Moyamoya disease. Moyamoya disease rarely coexists with tuberculosis. However, we can infer that tuberculosis may coexist in a patient in a patient of Moyamoya disease.

9.
Article in English | WPRIM | ID: wpr-788769

ABSTRACT

Moyamoya disease (MMD) is a progressive cerebrovascular disease with unknown etiology, characterized by bilateral stenoocclusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network formation at the base of the brain. MMD has an intrinsic nature to convert the vascular supply for the brain from internal carotid (IC) system to the external carotid (EC) system, as indicated by Suzuki’s angiographic staging. Insufficiency of this ‘IC-EC conversion system’ could result not only in cerebral ischemia, but also in intracranial hemorrhage from inadequate collateral anastomosis, both of which represent the clinical manifestation of MMD. Surgical revascularization prevents cerebral ischemic attack by improving cerebral blood flow, and recent evidence further suggests that extracranial-intracranial bypass could powerfully reduce the risk of re-bleeding in MMD patients with posterior hemorrhage, who were known to have extremely high re-bleeding risk. Although the exact mechanism underlying the hemorrhagic presentation in MMD is undetermined, most recent angiographic analysis revealed the characteristic angio-architecture related to high re-bleeding risk, such as the extension and dilatation of choroidal collaterals and posterior cerebral artery involvement. We sought to update the current management strategy for hemorrhagic MMD, including the outcome of surgical revascularization for hemorrhagic MMD in our institute. Further investigations will clarify the optimal surgical strategy to prevent hemorrhagic manifestation in patients with MMD.


Subject(s)
Angiography , Brain , Brain Ischemia , Carotid Artery, Internal , Cerebrovascular Circulation , Cerebrovascular Disorders , Choroid , Dilatation , Hemorrhage , Humans , Intracranial Hemorrhages , Moyamoya Disease , Posterior Cerebral Artery
10.
Article in Chinese | WPRIM | ID: wpr-800701

ABSTRACT

Compared with traditional cerebral angiography, magnetic resonance angiography has the advantages of non-invasive, convenient, and no adverse effects of contrast agents. In many cases, it can be used as an alternative examination or an important supplement to digital subtraction angiography. This article reviews the application progress of magnetic resonance angiography in the diagnosis and treatment of moyamoya disease.

11.
Article in Chinese | WPRIM | ID: wpr-791707

ABSTRACT

Objective To evaluate the effect of desflurane-remifentanil anesthesia on balance between cerebral oxygen supply and demand during cerebral revascularization in the patients with moyamoya disease.Methods Forty patients of both sexes with moyamoya disease,aged 18-64 yr,with body mass index of 18-25 kg/m2,undergoing superficial temporal artery-middle cerebral artery anastomosis,were allocated into 2 groups using a random number table method:desflurane-remifentanil group (D group) and propofol-remifentanil group (P group),with 20 cases in each group.Anesthesia was induced by intravenously injecting etomidate 0.3 mg/kg,sufentanil 0.4-0.5 μg/kg,and cis-atracurium 0.15-0.2 mg/kg.The patients were mechanically ventilated after tracheal intubation,and the end-tidal pressure of carbon dioxide was maintained at 35-45 mmHg.Anesthesia was maintained with propofol 4-6 mg · kg-1 · h-1 (group P),4%-6% desflurane (group D),remifentanil 0.1-0.3 μg· kg-1 · min-1,remifentanil 0.1-0.3 μg · kg-1 · min-1 and intermittent intravenous boluses of cis-atracurium,and BIS value was maintained at 40-60.At 15 min after intubation (T1),30 min after skin incision (T2),immediately after opening the dura mater (T3),immediately after vascular bypass and patency (T4),and at the end of surgery (T5),blood samples were obtained from the radial artery and internal jugular bulb for blood gas analysis,jugular venous oxygen saturation (SjvO2) was recorded,and arteriovenous blood O2 content difference (Da-jvO2) and cerebral O2 extraction rate (CERO2) were calculated.Results Compared with group P,Da-jvO2 at T3-6 and CERO2 at T4-6 were significantly decreased,and SjvO2 was increased at T4-6 in group D (P<0.05).Compared with the value at T1,Da-jvO2 was significantly decreased,and SjvO2 was increased at T5 in group D (P<0.05).CERO2 was significantly lower,and SjvO2 was higher at T5 than at T3 in group P (P<0.05).Compared with the values at T4,CERO2 was significantly decreased,and SjvO2 was increased at T5 in P and D groups (P< 0.05).Conclusion Compared with propofol-remifentanil anesthesia,desflurane-remifentanil anesthesia can maintain the balance between cerebral oxygen supply and demand better during cerebral revascularization in the patients with moyamoya disease.

12.
Article in English | WPRIM | ID: wpr-765340

ABSTRACT

Moyamoya disease (MMD) is a progressive cerebrovascular disease with unknown etiology, characterized by bilateral stenoocclusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network formation at the base of the brain. MMD has an intrinsic nature to convert the vascular supply for the brain from internal carotid (IC) system to the external carotid (EC) system, as indicated by Suzuki’s angiographic staging. Insufficiency of this ‘IC-EC conversion system’ could result not only in cerebral ischemia, but also in intracranial hemorrhage from inadequate collateral anastomosis, both of which represent the clinical manifestation of MMD. Surgical revascularization prevents cerebral ischemic attack by improving cerebral blood flow, and recent evidence further suggests that extracranial-intracranial bypass could powerfully reduce the risk of re-bleeding in MMD patients with posterior hemorrhage, who were known to have extremely high re-bleeding risk. Although the exact mechanism underlying the hemorrhagic presentation in MMD is undetermined, most recent angiographic analysis revealed the characteristic angio-architecture related to high re-bleeding risk, such as the extension and dilatation of choroidal collaterals and posterior cerebral artery involvement. We sought to update the current management strategy for hemorrhagic MMD, including the outcome of surgical revascularization for hemorrhagic MMD in our institute. Further investigations will clarify the optimal surgical strategy to prevent hemorrhagic manifestation in patients with MMD.


Subject(s)
Angiography , Brain , Brain Ischemia , Carotid Artery, Internal , Cerebrovascular Circulation , Cerebrovascular Disorders , Choroid , Dilatation , Hemorrhage , Humans , Intracranial Hemorrhages , Moyamoya Disease , Posterior Cerebral Artery
13.
Article in Chinese | WPRIM | ID: wpr-742988

ABSTRACT

Objective To investigate the application value of CT perfusion (CTP) imaging for the revascularization treatment in adult patients with Moyamoya disease.Methods Adult patients with Moyamoya disease underwent revascularization in the Department of Neurosurgery,Wuhan No.1 Hospital from July 2009 to December 2016 were analyzed retrospectively.CTP and clinical evaluation were performed before and after 3-6 months of procedure.The modified Rankin Scale (mRS) was used to assess the functional outcomes.Results A total of 20 patients were enrolled in the study,including 9 females and 11 males,aged 29 to 73 years,with an average of 53.5 years.The initial symptom was ischemic stroke in 10 patients,transient isehemic attack in 7 patients,and hemorrhagic stroke in 3 patients.All patients underwent superficial temporal artery-middle cerebral artery bypass grafting plus encephalomyo-synangiosis under general anesthesia.All patients have different degrees of improvement in cerebral blood flow after procedure,and the CTP parameters were significantly improved compared with those before procedure (all P <0.05).The clinical symptoms were significantly improved in 3 cases (15%) and recovered in 13 cases (65%) at 6 months after procedure.The proportion of the mRS score 0-2 was significantly higher than that before procedure (90.0% [18/20] 对 50.0% [10/20];x2 =7.619,P =0.006).Conclusion CTP can evaluate the cerebral perfusion status in various vascular areas through hemodynamic parameters in early stage,which can effectively guide the operation mode of Moyamoya disease,and evaluate the changes of cerebral perfusion status after procedure as a means of follow-up of the disease.

14.
Article in Chinese | WPRIM | ID: wpr-856041

ABSTRACT

Moyamoya disease ( MMD) is a progressive cerebrovascular disease with characteristics of ethnic susceptibility and familial aggregation. The pathological changes of slenosed vessels in MMD includc intima thickening,tunica media thinning,irregular undulation of the internal elastic laminae and lymphocytic infiltration The etiology and pathogenesis arc not completely clarified. It is presently thought that this disease is related to endothelial cells,smooth muscle cells,abnormal metabolism of extracellular matrix and inflammatory responses. This review will discuss,from genetic.cellular environmental and immunological perspectives,the specific roles of several genes in the development of MMD, including endothelial cell susceptibility gene ring finger protein 213,smooth muscle ccll susceptibility genes,extracellular matrix genes,angiogenesis factors and immune-related genes.

15.
Article in Chinese | WPRIM | ID: wpr-823506

ABSTRACT

Objective To study on the role of histone methylation enzyme enhancer of zeste homolog 2 (EHZ2) and vascular endothelial growth factor 165 (VEGF165) in momymoya disease. Methods The animal model of moyamoya disease was established by ear vein injection of horse serum in New Zealand rabbits. VEGF165 was over-expressed in situ by packaging lentivirus. Real-time quantitative PCR and Western Blot were used to detect the expression of VEGF165, EZH2 and H3K27me3 in the brain tissues of the animal models. Results Compared with the normal control group, the expression levels of mRNA and protein of EZH2 in the moyamoya disease model group were increased (EZH2 mRNA:P<0.01), and the level of histone H3K27me3 was increased. After overexpression of VEGF165 in the moyamoya disease model group, the expression levels of mRNA and protein of EZH2 was further increased (EZH2 mRNA: P<0.01), and the level of histone H3K27me3 was also increased. Conclusions EZH2 plays a certain role in the pathogenesis of moyamoya disease, and the expression of EZH2 is regulated by VEGF 165, which provides a theoretical basis for the study of the pathogenesis of moyamoya disease.

16.
Article in Korean | WPRIM | ID: wpr-766745

ABSTRACT

Reversible cerebral vasoconstriction syndrome (RCVS) is a disease characterized by reversible and multiple stenoses of cerebral blood vessels that improve within 3 months, accompanied by thunderclap headache. Here, we report an interesting case of RCVS initially misdiagnosed as Moyamoya disease with transient ischemic attack. A 45-year-old woman visited the Neurology Department of Eulji University Hospital. The patient was initially diagnosed with Moyamoya disease with transient ischemic attack. However, follow-up magnetic resonance angiography performed 12 months after the patient was appropriately diagnosed as having RCVS.


Subject(s)
Blood Vessels , Constriction, Pathologic , Female , Follow-Up Studies , Headache Disorders, Primary , Humans , Ischemic Attack, Transient , Magnetic Resonance Angiography , Middle Aged , Moyamoya Disease , Neurology , Vasoconstriction
17.
Article in Korean | WPRIM | ID: wpr-766555

ABSTRACT

Moyamoya disease (MMD) refers to a chronic progressive steno-occlusive disease at the distal portion of the internal carotid artery with abnormal collateral vessel formation of unknown etiology. The definite diagnosis of MMD requires cerebral angiography or magnetic resonance angiography and/or magnetic resonance imaging after excluding other underlying diseases, particularly in adult patients. The treatment aims to improve regional cerebral blood flow to prevent cerebral ischemic events and alleviate hemodynamic instability that can provoke cerebral hemorrhage. Although various surgical revascularization methods have been introduced, combined revascularization surgery including direct revascularization is preferred over indirect revascularization only in adult MMD patients. Several recent studies have shown that surgical treatment has better outcomes and prognosis for symptomatic hemodynamically unstable MMD patients with both ischemic and hemorrhagic presentations. For asymptomatic patients, follow up with appropriate imaging is recommended. Surgery should be considered when new symptoms emerge with hemodynamic aggravation.


Subject(s)
Adult , Carotid Artery, Internal , Cerebral Angiography , Cerebral Hemorrhage , Cerebrovascular Circulation , Diagnosis , Follow-Up Studies , Hemodynamics , Humans , Magnetic Resonance Angiography , Magnetic Resonance Imaging , Moyamoya Disease , Prognosis
18.
Rev. bras. cir. cardiovasc ; 33(3): 309-311, May-June 2018. tab, graf
Article in English | LILACS | ID: biblio-958415

ABSTRACT

Abstract Moyamoya disease is a rare, idiopathic, progressive, occlusive disease of the internal carotid artery characterized by the development of collateral vasculature in the brain base. In patients with accompanying coronary artery disease, cardiopulmonary bypass posses a potential risk for perioperative cerebral ischemic complication. Herein, we report a 53-year-old male case of Moyamoya disease and coronary artery disease who was treated with off-pump coronary artery bypass grafting.


Subject(s)
Humans , Male , Middle Aged , Coronary Stenosis/complications , Coronary Artery Bypass, Off-Pump/methods , Moyamoya Disease/surgery , Angiography, Digital Subtraction/methods , Risk Factors , Treatment Outcome , Coronary Angiography/methods , Ultrasonography, Doppler/methods , Coronary Stenosis/diagnostic imaging , Moyamoya Disease/diagnostic imaging
19.
Article in Chinese | WPRIM | ID: wpr-706268

ABSTRACT

Objective To observe the value of three-dimensional arterial spin labeling (3D-ASL) PWI in evaluating postoperative cerebral perfusion changes in patients with Moyamoya disease.Methods Totally 19 patients of Moyamoya disease confirmed with DSA were enrolled.All the patients received revascularization.Before and after operation,3D-ASL PWI and dynamic susceptibility contrast perfusion weighted imaging (DSC-PWI) were performed.ROI was located in the region with obvious perfusion changes supplied by middle cerebral artery on the operating side.Then the cerebral blood flow (CBF) was measured on 3D-ASL images,and time to peak (TTP) was measured on DSC-PWI images before and after operation.The differences of CBF and TTP before and after operation were compared,as well as the improvement rate of CBF,TTP and clinical symptoms.Results Before and after operation,CBF was (41.40±11.36) ml/(100 g · min) and (54.10±16.69) ml/(100 g · min),respectively,and the difference was statistically significant (t=-4.273,P<0.01).TTP was (28.66 ± 3.21) s and (26.44 ± 3.93) s,respectively,and the difference was also statistically significant (t =-2.936,P<0.01).The improvement rate of clinical symptoms was 84.21% (16/19),of CBF was 78.95% (15/19) and of TTP was 68.42% (13/19),the differences of improvement rate had no statistically significant (P=0.625).Conclusion 3D-ASL PWI is noninvasive,no contrast agent need to be used,and can be used to evaluate perfusion changes after operation of revascularization in patients with Moyamoya disease.

20.
Article in English | WPRIM | ID: wpr-715682

ABSTRACT

BACKGROUND AND PURPOSE: Sudden neurological deterioration which cannot be explained by structural change, ischemia or seizure is often observed among neurosurgical patients. We aimed to provide new insight into the pathophysiology of postoperative transient neurologic dysfunction. METHODS: We describe prolonged but fully reversible focal neurologic dysfunction of unknown origin based on the initial evaluation in 8 patients who had received encephalo-duro-arterio-synangiosis for moyamoya disease. We performed brain imaging, including diffusion weighted imaging and perfusion magnetic resonance imaging or single photon emission computed tomography, and electroencephalography (EEG) during the episodes and after resolution of the symptoms. RESULTS: The symptoms consisted of dysarthria, hemiparesis, or hemiparesthesia of limbs contralateral to the operated side. These symptoms developed between 12 hours and 8 days after surgery and lasted between 12 hours and 17 days. Structural imaging did not show any significant interval change compared with the immediate postoperative images. Perfusion imaging showed increased cerebral blood flow in the symptomatic hemisphere. EEG revealed low amplitude arrhythmic slowing in the corresponding hemisphere. Follow-up imaging and EEG after recovery did not show any abnormalities. CONCLUSIONS: Transient neurologic dysfunction can occur during the postoperative period of brain surgery. Although this may last more than usual transient ischemic attack or seizure, it eventually resolves regardless of treatment. Based on our observation, we propose that this is the manifestation of the transient cortical depression triggered by mechanical stimulation, analogous to migraine aura associated with cortical spreading depression.


Subject(s)
Brain , Cerebrovascular Circulation , Cortical Spreading Depression , Depression , Diffusion , Dysarthria , Electroencephalography , Epilepsy , Extremities , Follow-Up Studies , Humans , Ischemia , Ischemic Attack, Transient , Magnetic Resonance Angiography , Migraine Disorders , Moyamoya Disease , Neuroimaging , Neurologic Manifestations , Paresis , Perfusion Imaging , Postoperative Period , Seizures , Tomography, Emission-Computed, Single-Photon
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