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1.
J Stroke Cerebrovasc Dis ; 28(11): 104328, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31471213

RESUMO

OBJECTIVE: Present study was aimed to precisely evaluate the angio-architectures in patients with asymptomatic moyamoya disease (MMD) by comparing with those with hemorrhagic stroke. METHODS: This study used the data set of cerebral angiography in Asymptomatic Moyamoya Registry (AMORE) Study and Japan Adult Moyamoya (JAM) Trial at enrollment. The development of 3 subtypes of collateral vessels, including lenticulostriate, thalamic, and choroidal anastomosis, was evaluated on cerebral angiography. Suzuki's angiographical stage and posterior cerebral artery (PCA) involvement were also assessed. These findings were compared between asymptomatic (AMORE) and hemorrhagic (JAM) groups. RESULTS: This study included 55 hemispheres of 35 patients in asymptomatic group and 75 hemispheres of 75 patients in hemorrhagic group. In asymptomatic group, thalamic anastomosis was less developed than in hemorrhagic group (P = .011), but there were no significant differences in the development of lenticulostriate and choroidal anastomosis between the 2 groups (P = .077 and P = .26, respectively). Suzuki's stage was more progressed and the prevalence of PCA involvement was significantly higher in hemorrhagic group than in asymptomatic group (P = .0033 and P = .016, respectively). CONCLUSIONS: This study reveals no significant differences in the development of choroidal anastomoses between asymptomatic and hemorrhagic-onset MMD. On the other hand, disease stage and PCA involvement were less advanced in asymptomatic MMD than in hemorrhagic-onset MMD. These findings strongly suggest a certain subgroup of asymptomatic patients with MMD is at potential risk for hemorrhagic stroke.


Assuntos
Angiografia Cerebral/métodos , Artérias Cerebrais/diagnóstico por imagem , Hemorragias Intracranianas/diagnóstico por imagem , Angiografia por Ressonância Magnética , Doença de Moyamoya/diagnóstico por imagem , Acidente Vascular Cerebral/diagnóstico por imagem , Adolescente , Adulto , Idoso , Doenças Assintomáticas , Artérias Cerebrais/fisiopatologia , Circulação Cerebrovascular , Circulação Colateral , Estudos Transversais , Feminino , Humanos , Hemorragias Intracranianas/etiologia , Hemorragias Intracranianas/fisiopatologia , Japão , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/complicações , Doença de Moyamoya/fisiopatologia , Valor Preditivo dos Testes , Prognóstico , Sistema de Registros , Fatores de Risco , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/fisiopatologia , Adulto Jovem
2.
Neurology ; 93(4): e388-e397, 2019 07 23.
Artigo em Inglês | MEDLINE | ID: mdl-31239360

RESUMO

OBJECTIVE: To identify independent predictors of clinical or cerebral lesion progression in a large sample of adult patients with moyamoya angiopathy (MMA) prior to decisions regarding revascularization surgery. METHODS: Ninety participants (median age, 37.5 years) were assessed at baseline and followed for a median time of 42.8 months. Incident ischemic and hemorrhagic strokes, death, as well as any incident ischemic and hemorrhagic lesions on MRI were recorded. Multiple demographic, clinical, and cerebral imaging measures at baseline were considered as potential predictors of clinical or cerebral tissue change at follow-up. Data were analyzed based on the Andersen-Gill counting process model, followed by internal validation of the prediction model. RESULTS: Among multiple potential predictive measures considered in the analysis, Asian origin, a history of TIAs, and a reduction in hemodynamic reserve, as detected by imaging, were found to be significantly associated with an increased risk of combined clinical and imaging events. While the model estimated the risk of clinical or cerebral lesion progression to be approximately 0.5% per year when none of these factors was present, this risk exceeded 20% per year when all factors were present. CONCLUSION: A simple combination of demographic, clinical, and cerebral perfusion imaging measures may aid in predicting the risk of incident stroke and cerebral lesion progression in adult patients with MMA. These results may help to improve therapeutic decisions and aid in the design of future trials in adults with this rare condition.


Assuntos
Hemorragias Intracranianas/etiologia , Ataque Isquêmico Transitório/etiologia , Doença de Moyamoya/fisiopatologia , Acidente Vascular Cerebral/etiologia , Adulto , Grupo com Ancestrais do Continente Africano/estatística & dados numéricos , Grupo com Ancestrais do Continente Asiático/estatística & dados numéricos , Revascularização Cerebral , Circulação Cerebrovascular , Tomada de Decisão Clínica , Progressão da Doença , Grupo com Ancestrais do Continente Europeu/estatística & dados numéricos , Feminino , França , Humanos , Masculino , Mortalidade , Doença de Moyamoya/complicações , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/cirurgia , Prognóstico , Medição de Risco , Adulto Jovem
3.
Cerebrovasc Dis ; 47(3-4): 178-187, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31121577

RESUMO

OBJECTIVE: Superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis is the standard surgical management for adult moyamoya disease (MMD) patients, but local cerebral hyperperfusion (CHP) and cerebral ischemia are potential complications of this procedure. Recent hemodynamic analysis of the acute stage after revascularization surgery for MMD revealed a more complex and unique pathophysiological condition, the so-called "watershed shift (WS) phenomenon," which is defined as a paradoxical decrease in the cerebral blood flow (CBF) at the adjacent cortex near the site of local CHP. The objective of this study was to clarify the exact incidence, clinical presentation, and risk factors of the WS phenomenon after direct revascularization surgery for adult MMD. PATIENTS AND METHODS: Among 74 patients with MMD undergoing STA-MCA anastomosis for 78 affected hemispheres, 60 adult patients comprising 64 hemispheres underwent serial quantitative CBF analysis by N-isopropyl-p-[123I] iodoamphetamine single-photon emission computed tomography after revascularization surgery. The local CBF was quantitatively measured at the site of anastomosis and the adjacent cortex before surgery, as well as on 1 and 7 days after surgery. Then, we investigated the incidence, clinical presentation, and risk factors of the WS phenomenon. RESULTS: The WS phenomenon was evident in 7 patients (7/64 hemispheres; 10.9%) after STA-MCA anastomosis for adult MMD. None of the patients developed neurological deterioration due to the WS phenomenon, but 1 patient developed reversible ischemic change on diffusion-weighted imaging at the site of the WS phenomenon. Multivariate analysis revealed that a lower preoperative CBF value was significantly associated with the occurrence of the WS phenomenon (20.3 ± 7.70 mL/100 g/min in WS-positive group vs. 31.7 ± 8.81 mL/100 g/min in WS-negative group, p= 1.1 × 10-2). CONCLUSIONS: The incidence of the WS phenomenon was as high as 10.9% after STA-MCA anastomosis for adult MMD. The clinical outcome of the WS phenomenon is generally favorable, but there is a potential risk for perioperative cerebral infarction. Thus, we recommend routine CBF measurement in the acute stage after revascularization surgery for adult MMD to avoid surgical complications, such as local CHP and cerebral ischemia, caused by the WS phenomenon. Concomitant detection of the WS phenomenon with local CHP is clinically important because blood pressure reduction to counteract local CHP may have to be avoided in the presence of the WS phenomenon.


Assuntos
Córtex Cerebral/irrigação sanguínea , Revascularização Cerebral/efeitos adversos , Circulação Cerebrovascular , Artéria Cerebral Média/cirurgia , Doença de Moyamoya/cirurgia , Complicações Pós-Operatórias/epidemiologia , Artérias Temporais/cirurgia , Adolescente , Adulto , Idoso , Anastomose Cirúrgica/efeitos adversos , Velocidade do Fluxo Sanguíneo , Feminino , Humanos , Incidência , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Artéria Cerebral Média/diagnóstico por imagem , Artéria Cerebral Média/fisiopatologia , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/fisiopatologia , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/fisiopatologia , Fluxo Sanguíneo Regional , Estudos Retrospectivos , Fatores de Risco , Artérias Temporais/diagnóstico por imagem , Artérias Temporais/fisiopatologia , Resultado do Tratamento , Adulto Jovem
4.
World Neurosurg ; 127: e137-e141, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30862600

RESUMO

BACKGOUND: Asymmetric cortical vessel sign (ACVS) on susceptibility-weighted imaging (SWI) indicates elevated concentration of deoxyhemoglobin and elevated oxygen extraction fraction in patients with cerebral ischemia. This study aimed to clarify whether ACVS is associated with impaired hemodynamics and hyperperfusion syndrome in patients with moyamoya disease (MMD). METHODS: Consecutive adult patients with MMD were enrolled. ACVS data on SWI and perfusion data using dynamic perfusion computed tomography were obtained and evaluated preoperatively and on postoperative days 2 and 180. RESULTS: A total of 24 patients with MMD were enrolled. Of 11 (45.83%) patients showing positive ACVS before surgery, 8 turned negative on postoperative day 2 and 9 showed absence of ACVS 180 days after surgery. Regions of interest showing positive ACVS had lower cerebral blood flow (CBF, P<0.001), increased cerebral blood volume (P = 0.021), prolonged time to peak (P<0.001), and mean transit time (P = 0.009). No patients with hemorrhagic symptoms showed positive ACVS(P = 0.041) and patients with positive ACVS showed more increase in CBF (P<0.004). CONCLUSIONS: In patients with MMD, ACVS on SWI indicates severe impairment in hemodynamics and is associated with more increase in CBF after bypass surgery. Hence, ACVS on SWI might be considered as a neuroimaging marker for the evaluation of hemodynamics in patients with MMD.


Assuntos
Hemodinâmica , Imagem por Ressonância Magnética , Doença de Moyamoya/diagnóstico por imagem , Neuroimagem , Tomografia Computadorizada por Raios X , Adulto , Isquemia Encefálica/diagnóstico por imagem , Isquemia Encefálica/etiologia , Isquemia Encefálica/mortalidade , Revascularização Cerebral , Circulação Cerebrovascular , Feminino , Hemoglobinas/análise , Humanos , Masculino , Doença de Moyamoya/complicações , Doença de Moyamoya/fisiopatologia , Doença de Moyamoya/cirurgia , Oxigênio/análise , Estudos Prospectivos , Fatores de Risco , Adulto Jovem
5.
J Neurol ; 266(6): 1421-1428, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30868219

RESUMO

INTRODUCTION: Moyamoya angiopathy (MMA) is a rare vasopathy, especially among European Caucasians. Data about demographics, clinical presentation, comorbid conditions, radiological findings as well as laboratory and cerebral spinal fluid (CSF) data are sparse. METHODS: Patients with MMA treated in the Alfried Krupp Hospital, Essen, Germany, between 2010 and 2017 with focus on demographic, clinical, radiological and laboratory as well as CSF data were evaluated retrospectively. Patients with non-Caucasian family background were excluded from this study. RESULTS: Altogether 200 European Caucasian patients with MMA were identified. There was a female predominance of 3.2:1. The mean age at first presentation was 32.9 years and the mean age of diagnosis was 36.0 years. Eleven of 194 index patients (5.7%) showed a familial presentation. In 11.6% posterior cerebral artery was additionally involved, in 4% additionally cerebral aneurysm and in 2.5% dysgenesis of corpus callosum was found. Most patients suffered from transient ischemic attacks (71.5%) and stroke (82%). Cerebral hemorrhage was found in 9.5%. Livedo racemosa was an associated symptom in 12.8% of patients and thyroid diseases were found in 23.8%. CONCLUSIONS: Compared with Asian data, cerebral hemorrhages are infrequent and female predominance is accentuated among European Caucasians. Some former unknown rare features like associated livedo racemosa, dysgenesis of corpus callosum and associated syncope have been discovered systematically for the first time in this huge European Caucasian cohort.


Assuntos
Grupo com Ancestrais do Continente Europeu/estatística & dados numéricos , Doença de Moyamoya/epidemiologia , Doença de Moyamoya/fisiopatologia , Adolescente , Adulto , Idoso , Agenesia do Corpo Caloso/epidemiologia , Criança , Pré-Escolar , Comorbidade , Feminino , Alemanha/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/complicações , Estudos Retrospectivos , Fatores Sexuais , Dermatopatias Vasculares/epidemiologia , Dermatopatias Vasculares/etiologia , Síncope/epidemiologia , Síncope/etiologia , Adulto Jovem
6.
Neuroimage Clin ; 22: 101713, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30743136

RESUMO

BACKGROUND AND PURPOSE: Patients with Moyamoya Disease (MMD) need hemodynamic evaluation of vascular territories at risk of stroke. Today's investigative standards include H215O PET/CT with pharmacological challenges with acetazolamide (ACZ). Recent developments suggest that CO2-triggered blood­oxygen-level-dependent (BOLD) functional MRI might provide comparable results to current standard methods for evaluation of territorial hemodynamics, while being a more widely available and easily implementable method. This study examines results of a newly developed quantifiable analysis algorithm for CO2-triggered BOLD MRI in Moyamoya patients and correlates the results with H215O PET/CT with ACZ challenge to assess comparability between both modalities. METHODS: CO2-triggered BOLD MRI was performed and compared to H215O PET/CT with ACZ challenge in patients with angiographically proven MMD. Images of both modalities were analyzed retrospectively in a blinded, standardized fashion by visual inspection, as well as with a semi-quantitative analysis using stimuli-induced approximated regional perfusion-weighted data and BOLD-signal changes with reference to cerebellum. RESULTS: 20 consecutive patients fulfilled the inclusion criteria, a total of 160 vascular territories were analyzed retrospectively. Visual analysis (4-step visual rating system) of standardized, color-coded cerebrovascular reserve/reactivity maps showed a very strong correlation (Spearman's rho = 0.9, P < 0.001) between both modalities. Likewise, comparison of approximated regional perfusion changes across vascular territories (normalized to cerebellar change) reveal a highly significant correlation between both methods (Pearson's r = 0.71, P < 0.001). CONCLUSIONS: The present analysis indicates that CO2-triggered BOLD MRI is a very promising tool for the hemodynamic evaluation of MMD patients with results comparable to those seen in H215O PET/CT with ACZ challenge. It therefore holds future potential in becoming a routine examination in the pre- and postoperative evaluation of MMD patients after further prospective evaluation.


Assuntos
Imagem por Ressonância Magnética/métodos , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/fisiopatologia , Neuroimagem/métodos , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons/métodos , Adulto , Feminino , Hemodinâmica/fisiologia , Humanos , Hipercapnia , Masculino , Radioisótopos de Oxigênio , Compostos Radiofarmacêuticos , Estudos Retrospectivos
7.
Stroke ; 50(2): 373-380, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30636572

RESUMO

Background and Purpose- Noninvasive imaging of brain perfusion has the potential to elucidate pathophysiological mechanisms underlying Moyamoya disease and enable clinical imaging of cerebral blood flow (CBF) to select revascularization therapies for patients. We used hybrid positron emission tomography (PET)/magnetic resonance imaging (MRI) technology to characterize the distribution of hypoperfusion in Moyamoya disease and its relationship to vessel stenosis severity, through comparisons with a normative perfusion database of healthy controls. Methods- To image CBF, we acquired [15O]-water PET as a reference and simultaneously acquired arterial spin labeling (ASL) MRI scans in 20 Moyamoya patients and 15 age-matched, healthy controls on a PET/MRI scanner. The ASL MRI scans included a standard single-delay ASL scan with postlabel delay of 2.0 s and a multidelay scan with 5 postlabel delays (0.7-3.0s) to estimate and account for arterial transit time in CBF quantification. The percent volume of hypoperfusion in patients (determined as the fifth percentile of CBF values in the healthy control database) was the outcome measure in a logistic regression model that included stenosis grade and location. Results- Logistic regression showed that anterior ( P<0.0001) and middle cerebral artery territory regions ( P=0.003) in Moyamoya patients were susceptible to hypoperfusion, whereas posterior regions were not. Cortical regions supplied by arteries with stenosis on MR angiography showed more hypoperfusion than normal arteries ( P=0.001), but the extent of hypoperfusion was not different between mild-moderate versus severe stenosis. Multidelay ASL did not perform differently from [15O]-water PET in detecting perfusion abnormalities, but standard ASL overestimated the extent of hypoperfusion in patients ( P=0.003). Conclusions- This simultaneous PET/MRI study supports the use of multidelay ASL MRI in clinical evaluation of Moyamoya disease in settings where nuclear medicine imaging is not available and application of a normative perfusion database to automatically identify abnormal CBF in patients.


Assuntos
Bases de Dados Factuais , Imagem por Ressonância Magnética , Artéria Cerebral Média , Doença de Moyamoya , Tomografia por Emissão de Pósitrons , Adolescente , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Artéria Cerebral Média/diagnóstico por imagem , Artéria Cerebral Média/fisiopatologia , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/fisiopatologia , Marcadores de Spin
8.
J Stroke Cerebrovasc Dis ; 28(4): 1113-1125, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30679013

RESUMO

BACKGROUND AND PURPOSE: Chronic ischemia may induce brain microstructural damage and lead to neurocognitive dysfunction in patients with Moyamoya disease (MMD). We applied neurite orientation dispersion and density imaging (NODDI) and 15O-gas positron emission tomography (PET) to elucidate the specific ischemic brain microstructural damage of MMD in the cortex and the white matter. MATERIALS AND METHODS: Thirty-one patients (16-63years old, 9 males) and 20 age- and sex-matched normal controls were enrolled in this study. NODDI evaluates quantitative parameters reflecting neurite and axonal density, network complexity and the interstitial fluid in all participants. Of 31 patients, 12 newly diagnosed patients were evaluated with PET, also. We evaluated correlations between the microstructural parameters of NODDI and the hemodynamic and metabolic parameters of PET, the relationship between NODDI and clinical severity of each hemisphere (Normal, Asymtpomatic, Symptomatic, and Infarcted) as well as neurocognitive performance. RESULTS: All NODDI parameters significantly correlated with PET parameters (absolute r = 0.46-0.83, P ≤ .048) and clinical severity (P < .001), suggesting that neurite and axonal density and network complexity decreased, and the interstitial fluid increased, as the ischemic burden became severe. NODDI parameters reflecting neurite and axonal density and network complexity significantly correlated with neurocognitive profiles (r = 0.36-0.64, P ≤ .048), but the interstitial fluid component did not. CONCLUSIONS: Chronic ischemia in patients with MMD may induce decreased neurite and axonal density, simplified network complexity, and may lead to neurocognitive dysfunction. The increased interstitial fluid accompanying hemodynamic impairment may not be identical to the decreased neurite density and might be driven by another mechanism.


Assuntos
Isquemia Encefálica/diagnóstico por imagem , Encéfalo/irrigação sanguínea , Imagem de Difusão por Ressonância Magnética , Microvasos/diagnóstico por imagem , Doença de Moyamoya/diagnóstico por imagem , Tomografia por Emissão de Pósitrons , Adolescente , Adulto , Axônios/patologia , Isquemia Encefálica/patologia , Isquemia Encefálica/fisiopatologia , Isquemia Encefálica/psicologia , Estudos de Casos e Controles , Angiografia Cerebral/métodos , Circulação Cerebrovascular , Cognição , Feminino , Hemodinâmica , Humanos , Angiografia por Ressonância Magnética , Masculino , Microcirculação , Microvasos/fisiopatologia , Pessoa de Meia-Idade , Doença de Moyamoya/patologia , Doença de Moyamoya/fisiopatologia , Doença de Moyamoya/psicologia , Neuritos/patologia , Testes Neuropsicológicos , Valor Preditivo dos Testes , Índice de Gravidade de Doença , Adulto Jovem
10.
World Neurosurg ; 125: e313-e318, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30685378

RESUMO

OBJECTIVE: Primary intraventricular hemorrhage (PIVH) is a rare condition in adult patients. PIVH occurs frequently in adult hemorrhagic Moyamoya disease (MMD). Idiopathic PIVH is defined as PIVH without cerebrovascular abnormalities. This study is aimed to compare the baseline characteristics and outcomes of acute MMD-related and idiopathic PIVH. METHODS: Adult patients with acute MMD-related or idiopathic PIVH were retrospectively included. Baseline characteristics and outcomes at discharge were obtained and compared. Chi-square test, Student's t-test, or rank-sum test were used in statistical analyses. RESULTS: This study finally included 32 patients with acute MMD-related PIVH and 112 with acute idiopathic PIVH. Patients with acute MMD-related PIVH were significantly younger (53.3 ± 15.8 vs. 42.8 ± 12.2 years, P < 0.001). The admission systolic blood pressure in patients with acute idiopathic PIVH was significantly higher (161.7 ± 30.9 vs. 134.6 ± 24.6 mm Hg, P < 0.001). Patients with acute idiopathic PIVH had significantly higher admission serum urea (5.68 ± 2.66 vs. 4.34 ± 1.62 mmol/L, P = 0.008), cystatin C (0.97 ± 0.72 vs. 0.68 ± 0.16 mg/L, P = 0.023), and uric acid (309.01 ± 105.97 vs. 242.24 ± 77.65 µmol/L, P = 0.001). In patients with acute MMD-related PIVH, only one (3.1%) patient was dead at discharge. In contrast, a total of 22 (19.6%) patients with acute idiopathic patients died at discharge (P = 0.027). CONCLUSIONS: Compared with patients with acute idiopathic PIVH, patients with acute MMD-related PIVH have younger age, lower blood pressure, and better renal function. Moreover, patients with acute MMD-related PIVH have lower short-term mortality.


Assuntos
Hemorragia Cerebral Intraventricular/diagnóstico por imagem , Doença de Moyamoya/diagnóstico por imagem , Doença Aguda , Idoso , Angiografia Digital , Pressão Sanguínea/fisiologia , Hemorragia Cerebral Intraventricular/fisiopatologia , Hemorragia Cerebral Intraventricular/cirurgia , Feminino , Humanos , Rim/fisiologia , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/fisiopatologia , Doença de Moyamoya/cirurgia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Resultado do Tratamento
11.
J Cereb Blood Flow Metab ; 39(1): 163-172, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-28901822

RESUMO

Quantification of regional cerebral blood flow (CBF) using [15O]H2O positron emission tomography (PET) requires the use of an arterial input function. Arterial sampling, however, is not always possible, for example in ill-conditioned or paediatric patients. Therefore, it is of interest to explore the use of non-invasive methods for the quantification of CBF. For validation of non-invasive methods, test-retest normal and hypercapnia data from 15 healthy volunteers were used. For each subject, the data consisted of up to five dynamic [15O]H2O brain PET studies of 10 min and including arterial sampling. A measure of CBF was estimated using several non-invasive methods earlier reported in literature. In addition, various parameters were derived from the time-activity curve (TAC). Performance of these methods was assessed by comparison with full kinetic analysis using correlation and agreement analysis. The analysis was repeated with normalization to the whole brain grey matter value, providing relative CBF distributions. A reliable, absolute quantitative estimate of CBF could not be obtained with the reported non-invasive methods. Relative (normalized) CBF was best estimated using the double integration method.


Assuntos
Circulação Cerebrovascular/fisiologia , Radioisótopos de Oxigênio , Compostos Radiofarmacêuticos , Algoritmos , Artérias Cerebrais/diagnóstico por imagem , Criança , Simulação por Computador , Feminino , Substância Cinzenta/irrigação sanguínea , Substância Cinzenta/diagnóstico por imagem , Humanos , Cinética , Imagem por Ressonância Magnética , Masculino , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/fisiopatologia , Tomografia por Emissão de Pósitrons , Reprodutibilidade dos Testes , Água
12.
J Cereb Blood Flow Metab ; 39(2): 342-351, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-28925802

RESUMO

Stroke is a dramatic complication of sickle cell disease (SCD), which is associated with cerebral vasculopathies including moya moya, intravascular thrombi, cerebral hyperemia, and increased vessel tortuosity. The spontaneous occurrence of these pathologies in the sickle cell mouse model has not been described. Here, we studied Townes humanized sickle cell and age-matched control mice that were 13 months old. We used in vivo two-photon microscopy to assess blood flow dynamics, vascular topology, and evidence of cerebral vasculopathy. Results showed that compared to controls, sickle cell mice had significantly higher red blood cell (RBC) velocity (0.73 mm/s vs. 0.55 mm/s, p = 0.013), capillary vessel diameter (4.84 µM vs. 4.50 µM, p = 0.014), and RBC volume flux (0.015 nL/s vs. 0.010 nL/s, p = 0.021). Also, sickle cell mice had significantly more tortuous capillary vessels ( p < 0.0001) and significantly shorter capillary vessel branches ( p = 0.0065) compared to controls. Sickle cell mice also had significantly higher number of capillary occlusive events (3.4% vs. 1.9%, p < 0.0001) and RBC stalls (3.8% vs. 2.1%, p < 0.0001) in the cerebral capillary bed. In post-mortem immunohistochemical analyses, sickle cell mice had a 2.5-fold higher frequency of cortical microinfarcts compared to control mice. Our results suggest that aged Townes sickle cell mice spontaneously develop SCD-associated cerebral vasculopathy.


Assuntos
Anemia Falciforme , Infarto Cerebral , Doença de Moyamoya , Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia , Anemia Falciforme/patologia , Anemia Falciforme/fisiopatologia , Animais , Infarto Cerebral/epidemiologia , Infarto Cerebral/etiologia , Infarto Cerebral/patologia , Infarto Cerebral/fisiopatologia , Modelos Animais de Doenças , Camundongos , Doença de Moyamoya/epidemiologia , Doença de Moyamoya/etiologia , Doença de Moyamoya/patologia , Doença de Moyamoya/fisiopatologia , Prevalência
14.
J Stroke Cerebrovasc Dis ; 28(2): 392-398, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30409746

RESUMO

BACKGROUND: There are a variety of collateral routes to compensate persistent cerebral ischemia in moyamoya disease. However, there is no report presenting the persistent primitive olfactory artery (POA) as a spontaneous collateral route to the anterior cerebral artery (ACA) in moyamoya disease. METHODS: We precisely examined cerebral angiography in 84 patients with moyamoya disease to identify the collateral channel through the persistent POA. Its anatomy was evaluated on pre- and postoperative angiography. RESULTS: Of 84 patients, four (4.8%) had spontaneous collateral channel through the persistent POA. All of these four hemispheres were categorized into Stage 5. In all four patients, the collateral blood flow arose from the ophthalmic artery and run to the persistent POA through the ethmoidal moyamoya. The persistent POA provided collateral blood flow from the ophthalmic artery to the ACA in all four patients. Superficial temporal artery to middle cerebral artery anastomosis and encephalo-duro-myo-arterio-pericranial synangiosis was performed in three of four patients. After surgery, the collateral channel through the persistent POA completely disappeared or markedly regressed, suggesting a significant improvement of cerebral hemodynamics in the territory of not only the MCA but also the ACA. CONCLUSION: The persistent POA can potentially provide collateral blood flow to the ACA in about 5% of patients with moyamoya disease, and should be recognized as a novel collateral channel in moyamoya disease. The persistent POA may be useful to evaluate therapeutic effects of surgical revascularization on the ACA territory.


Assuntos
Artéria Cerebral Anterior/fisiopatologia , Artérias Cerebrais/fisiopatologia , Circulação Cerebrovascular , Circulação Colateral , Doença de Moyamoya/fisiopatologia , Adolescente , Adulto , Artéria Cerebral Anterior/diagnóstico por imagem , Artéria Cerebral Anterior/cirurgia , Angiografia Cerebral , Artérias Cerebrais/anormalidades , Artérias Cerebrais/diagnóstico por imagem , Feminino , Humanos , Masculino , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/cirurgia , Imagem de Perfusão , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Resultado do Tratamento
15.
Stroke ; 49(12): 2837-2843, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30571396

RESUMO

Background and Purpose- The study goal was to evaluate cerebral perfusion in moyamoya patients with a novel staging system and investigate the association between differences of perfusion status and clinical outcomes in patients treated with revascularization. Methods- About 506 consecutive patients from 2009 to 2015 were enrolled. The perfusion status was evaluated by a staging system-the stage of preinfarction period based on the result of computed tomography perfusion. Hemisphere in different perfusion stage was compared between hemorrhagic patients (n=155) and ischemic patients (n=351). The modified Rankin Scale was applied to evaluate the prognosis of patients. Results- In the enrolled 506 patients: 229 hemispheres (22.6%) with normal perfusion, 72 hemispheres (7.1%) in stage I, 205 hemispheres (20.3%) in stage II, 308 hemispheres (30.4%) in stage III, and 198 hemispheres (19.6%) in stage IV. Significant difference was observed in stage distribution between hemorrhagic patients and ischemic patients ( P<0.01). The ratio of hemispheres with normal perfusion in hemorrhagic group is more than the ischemic group ( P<0.05; odds ratio, 1.440; 95% CI, 1.144-1.811). The ratio of hemispheres in stage III in ischemic group is more than the hemorrhagic group ( P<0.01; odds ratio, 0.618, 95% CI, 0.487-0.783). In the prognosis-related analysis, the stage I group has the highest improved ratio (73.9%) and the normal perfusion group has the lowest improved ratio (33.3%). The improved ratio has a decreasing tendency from stage I to stage IV. Conclusions- The novel preinfarction staging system is a valuable assessment tool to evaluate cerebral perfusion status in moyamoya patients and predict the efficacy of revascularization. Ischemic patients suffer more from hypoperfusion. Patients in stage I and stage II are more likely to obtain improvement after revascularization. This is a retrospective study.


Assuntos
Isquemia Encefálica/diagnóstico por imagem , Circulação Cerebrovascular , Hemorragias Intracranianas/diagnóstico por imagem , Doença de Moyamoya/diagnóstico por imagem , Adolescente , Adulto , Angiografia Digital , Isquemia Encefálica/fisiopatologia , Angiografia Cerebral , Revascularização Cerebral/métodos , Criança , Pré-Escolar , Feminino , Humanos , Hemorragias Intracranianas/fisiopatologia , Angiografia por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/fisiopatologia , Doença de Moyamoya/cirurgia , Seleção de Pacientes , Imagem de Perfusão , Prognóstico , Estudos Retrospectivos , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X , Adulto Jovem
16.
Cerebrovasc Dis ; 46(3-4): 161-171, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30300893

RESUMO

BACKGROUND: Missense mutations in the gene that codes for smooth muscle actin, ACTA2, cause diffuse smooth muscle dysfunction and a distinct cerebral arteriopathy collectively known as multisystemic smooth muscle dysfunction syndrome (MSMDS). Until recently, ACTA2 cerebral arteriopathy was considered to be a variant of moyamoya disease. However, recent basic science and clinical data have demonstrated that the cerebral arteriopathy caused by mutant ACTA2 exhibits genetic loci, histopathology, neurological sequelae, and radiographic findings unique from moyamoya disease. We conducted a literature review to provide insight into the history, clinical significance, and neurosurgical management of this recently described novel cerebral arteriopathy. SUMMARY: We performed a literature search using PubMed with the key words "ACTA2 mutation," "ACTA2 cerebral arteriopathy," and "multisystemic smooth muscle dysfunction syndrome." Case reports with confirmed ACTA2 mutations and cerebral arteriopathy were included in our review. Our literature search revealed 15 articles (58 cases) of confirmed ACTA2 cerebral arteriopathy. Distinctive features of this arteriopathy included an aberrant internal carotid circulation with dilatation of the proximal segments, occlusive disease at the distal segments, and dolichoectasia. As such, mutant ACTA2 predisposed patients to ischemic strokes as children. Direct and indirect cerebral revascularization procedures are the mainstay treatment options with varying degrees of success. Key Messages: ACTA2 cerebral arteriopathy is a recently described novel cerebrovascular disease seen in patients with MSMDS. Patients currently diagnosed with moyamoya disease who also have dysfunction of smooth muscle organs may benefit from reevaluation by a medical geneticist and ACTA2 genotyping.


Assuntos
Actinas/genética , Doenças Arteriais Cerebrais/genética , Doença de Moyamoya/genética , Músculo Liso Vascular , Mutação de Sentido Incorreto , Adolescente , Adulto , Angiografia Digital , Angiografia Cerebral/métodos , Doenças Arteriais Cerebrais/diagnóstico por imagem , Doenças Arteriais Cerebrais/fisiopatologia , Doenças Arteriais Cerebrais/cirurgia , Artérias Cerebrais/fisiopatologia , Artérias Cerebrais/cirurgia , Criança , Pré-Escolar , Feminino , Estudos de Associação Genética , Predisposição Genética para Doença , Humanos , Lactente , Recém-Nascido , Angiografia por Ressonância Magnética , Masculino , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/fisiopatologia , Doença de Moyamoya/cirurgia , Músculo Liso Vascular/diagnóstico por imagem , Músculo Liso Vascular/fisiopatologia , Músculo Liso Vascular/cirurgia , Procedimentos Neurocirúrgicos , Fenótipo , Prognóstico , Fatores de Risco
17.
AJNR Am J Neuroradiol ; 39(11): 2037-2044, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30361434

RESUMO

BACKGROUND AND PURPOSE: Impaired cerebrovascular reactivity has been associated with decreased cortical thickness in patients with arterial occlusive diseases. This study tests the hypothesis that severe hemodynamic impairment, indicated by increased oxygen extraction fraction ratios on positron-emission tomography with 15O tracers, is associated with decreased cortical thickness in patients with Moyamoya phenomenon. MATERIALS AND METHODS: Patients with unilateral or bilateral idiopathic Moyamoya phenomenon were recruited. Oxygen extraction fraction ratio maps were generated from cerebral images of O[15O] counts divided by H2[15O] counts with normalization by corresponding cerebellar counts. The normal range of the oxygen extraction fraction ratio was estimated from historically available healthy control subjects. Cortical thickness was estimated from T1-weighted MR imaging and FreeSurfer. Regional samples of oxygen extraction fraction ratios and cortical thicknesses were drawn using FreeSurfer parcellations, retaining only parcellations from the vascular territory of the middle cerebral artery. RESULTS: Complete MR imaging and PET datasets were available in 35 subjects, including 23 women; the mean age at scanning was 44 years. Patients with Moyamoya phenomenon had a significantly increased regional oxygen extraction fraction ratio compared with 15 healthy control subjects (P < .001). Regional oxygen extraction fraction ratio and age were significant predictors of cortical thickness (P < .001 for each) in a generalized linear mixed-effects model. Using hemisphere averages and patient averages, we found that only age was a significant predictor of cortical thickness (P < .001). CONCLUSIONS: Chronic hemodynamic impairment, as indicated by a higher regional oxygen extraction fraction ratio, was significantly predictive of reduced cortical thickness in mixed-effects analysis of FreeSurfer regions. This phenomenon may be related to reversible metabolic down-regulation.


Assuntos
Córtex Cerebral/patologia , Hemodinâmica/fisiologia , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/patologia , Adulto , Córtex Cerebral/irrigação sanguínea , Córtex Cerebral/diagnóstico por imagem , Circulação Cerebrovascular/fisiologia , Feminino , Humanos , Imagem por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/fisiopatologia , Tomografia por Emissão de Pósitrons/métodos
18.
Stroke ; 49(10): 2504-2507, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30355113

RESUMO

Background and Purpose -Microstructural damage in the brain induced by chronic ischemia is suggested to play a pivotal role in the neurocognitive dysfunction of adults with Moyamoya disease (MMD). We investigated specific changes in the brain microstructure and their correlations with neurocognitive dysfunction in patients with MMD using a multishell diffusion magnetic resonance imaging technique called neurite orientation dispersion and density imaging. Methods- We evaluated 26 patients with MMD (16-63 years old, 20 females) and 20 age- and sex-matched normal volunteers using neurite orientation dispersion and density imaging and neuropsychological batteries. Neurite orientation dispersion and density imaging calculates 2 parameters: the intracellular volume fraction (Vic), which reflects the axon density in the white matter and dendrite density in the cortex, and the orientation dispersion index (OD), which reflects the network complexity. The microstructural damage and its correlation with neurocognitive performance were evaluated by performing a whole-brain analysis using SPM12 and correlation analysis with regional values. Results- Patients with MMD had significantly lower Vic in the white matter and a lower OD mainly in the cortex than those of the controls ( P<0.001, family-wise error corrected). Of all neuropsychological scores, Processing Speed Index (PS) exhibited the strongest correlation with Vic in the white matter ( P<0.001, family-wise error corrected). The Vic and OD values for regions with group differences, including both temporoparietal and frontal areas, correlated with neurocognitive performance (absolute r=0.37-0.64; P<0.01). Conclusions- Chronic ischemia in MMD may decrease the axon density in the white matter and dendrite density in the cortex (Vic) and simplify network complexity (OD), leading to neurocognitive dysfunction. Processing Speed Index may be the most sensitive index used to evaluate the ischemic burden, and the posterior part of the brain may play an important role in neurocognitive function. Clinical Trial Registration- URL: http://www.umin.ac.jp/ctr/ . Unique identifier: UMIN000023082.


Assuntos
Encéfalo/cirurgia , Disfunção Cognitiva/cirurgia , Doença de Moyamoya/cirurgia , Neuritos/patologia , Adolescente , Adulto , Axônios/patologia , Encéfalo/patologia , Disfunção Cognitiva/patologia , Imagem de Difusão por Ressonância Magnética/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/fisiopatologia , Resultado do Tratamento , Adulto Jovem
19.
Medicine (Baltimore) ; 97(41): e12768, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30313089

RESUMO

The aim of this study was to explore the correlation between multi-inversion time arterial spin labeling (mTI-ASL) and dynamic susceptibility contrast-enhanced perfusion weighted imaging (DSC-PWI) in assessment of hemodynamics of patients with Moyamoya disease (MMD).In this study, 24 MMD patients and 21 healthy subjects were enrolled between June 2017 and December 2017. The images of mTI-ASL and DSC-PWI in the week before revascularization surgery were retrospectively analyzed. The parameters of cerebral blood flow (CBF), time to peak (TTP), and bolus arrival time (BAT) were measured in regions of interest (ROIs) of lateral middle cerebral artery (MCA) territories, basal ganglia (BG), and cerebellum, and relative perfusion parameters (rCBF-ASL, rBAT-ASL, rCBF-DSC, and rTTP-DSC) were calculated by dividing by cerebellum value. One-way analysis of variance and Student-Newman-Keuls tests were performed to compare rCBF-ASL and rCBF-DSC in the MMD group and the control group. Unpaired t test was used to compare rBAT-ASL and rTTP-DSC in the MMD group and the control group. And we assessed the correlation between rCBF-ASL and rCBF-DSC and between rBAT-ASL and rTTP-DSC using Pearson correlation analysis.All the relative parameters were significantly different between the MMD group and the control group (all P<.05). Meanwhile, there was significant difference between rCBF-ASL and rCBF-DSC (P<.05), and there was strong correlation between rCBF-ASL and rCBF-DSC (r = 0.839, P<.001), and moderate correlation between rBAT-ASL and rTTP-DSC (r = 0.519, P<.001).Both mTI-ASL and DSC-PWI could be used to assess perfusion state in MMD patients before revascularization surgery effectively. As a noninvasive imaging technique, mTI-ASL could provide perfusion parameters without contrast medium injection, and the results were quite correlative with DSC-PWI.


Assuntos
Circulação Cerebrovascular/fisiologia , Imagem por Ressonância Magnética/métodos , Doença de Moyamoya/diagnóstico por imagem , Adulto , Meios de Contraste , Feminino , Hemodinâmica/fisiologia , Humanos , Angiografia por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/fisiopatologia , Estudos Retrospectivos , Marcadores de Spin , Adulto Jovem
20.
World Neurosurg ; 120: e1301-e1309, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30240869

RESUMO

OBJECTIVE: To investigate hemodynamic changes in moyamoya disease (MMD) via two-dimensional cine phase-contrast magnetic resonance imaging and computational fluid dynamics. METHODS: In 18 patients with MMD and 10 healthy control subjects, phase-contrast magnetic resonance imaging was performed to quantify flow rate of main supplying arteries, including internal carotid arteries (ICAs) and vertebral arteries. Mean flow rate in these vessels was adopted as the patient-specific boundary condition for computational fluid dynamics simulation of the circle of Willis in MMD and control groups. Pressure drop in both ICAs and their difference, wall shear stress and secondary flow in the carotid siphon of ICAs, and flow rate and size of posterior communicating arteries (PComAs) were compared between MMD and control groups. Four patients with MMD underwent follow-up scans for longitudinal comparison. RESULTS: Phase-contrast magnetic resonance imaging data revealed significantly different flow rate in the left ICA and right vertebral arteries between MMD and control groups. Computational fluid dynamics simulation demonstrated similar wall shear stress and similar secondary flow of both ICAs but significantly higher pressure drop in left ICA, higher pressure drop difference between left ICA and right ICA, and higher flow rate in PComAs in patients with MMD compared with control subjects. Significantly increased size of left PComA in patients with MMD was also found. Follow-up results confirmed that the combination of pressure drop difference, flow rate, and size of PComAs can potentially assist long-term prognosis after surgery. CONCLUSIONS: Pressure drop difference, flow rate, and size of PComAs can be used to evaluate impairments in cerebrovascular reserve and indicate long-term prognosis in MMD.


Assuntos
Hemodinâmica , Imagem por Ressonância Magnética , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/fisiopatologia , Adulto , Artérias/diagnóstico por imagem , Artérias/fisiopatologia , Simulação por Computador , Meios de Contraste , Feminino , Seguimentos , Humanos , Hidrodinâmica , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Modelos Cardiovasculares , Modelos Neurológicos
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