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1.
Neurology ; 96(13): e1783-e1791, 2021 03 30.
Artigo em Inglês | MEDLINE | ID: mdl-33568546

RESUMO

OBJECTIVE: To test the hypothesis that de novo genetic variants are responsible for moyamoya disease (MMD) in children with unaffected relatives, we performed exome sequencing of 28 affected children and their unaffected parents. METHODS: Exome sequencing was performed on 28 trios of affected patients with MMD and unaffected parents. RESULTS: We identified 3 novel rare de novo RNF213 variants, 1 in the RING domain and 2 in a highly conserved region distal to the RING domain (4,114-4,120). These de novo cases of MMD present at a young age with aggressive MMD and uniquely have additional occlusive vascular lesions, including renal artery stenosis. Two previously reported cases had de novo variants in the same limited region and presented young with aggressive MMD, and 1 case had narrowing of the inferior abdominal aorta. CONCLUSIONS: These results indicate a novel syndrome associated with RNF213 rare variants defined by de novo mutations disrupting highly conserved amino acids in the RING domain and a discrete region distal to the RING domain delimited by amino acids 4,114 to 4,120 leading to onset of severe MMD before 3 years of age and occlusion of other arteries, including the abdominal aorta, renal, iliac, and femoral arteries.


Assuntos
Adenosina Trifosfatases/genética , Doença de Moyamoya/genética , Ubiquitina-Proteína Ligases/genética , Adulto , Idade de Início , Doenças da Aorta/genética , Doenças da Aorta/fisiopatologia , Arteriopatias Oclusivas/genética , Arteriopatias Oclusivas/fisiopatologia , Pré-Escolar , Feminino , Artéria Femoral , Humanos , Artéria Ilíaca , Masculino , Doença de Moyamoya/fisiopatologia , Mutação , Obstrução da Artéria Renal/genética , Obstrução da Artéria Renal/fisiopatologia
2.
J Stroke Cerebrovasc Dis ; 30(4): 105624, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33516067

RESUMO

OBJECT: Abnormal collateral channels, so-called moyamoya vessels, play a critical role to compensate cerebral ischemia, but carry the risk for hemorrhagic stroke in moyamoya disease (MMD). The present study was aimed to clarify if superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis and encephalo-myo-duro-arterio-pericranial synangiosis (EDMAPS) can efficiently regress the abnormal collateral channels in MMD patients. METHODS: This study included 92 hemispheres of 61 MMD patients who underwent STA-MCA anastomosis combined with EDMAPS between 2013 and 2019. There were 17 children and 44 adults. We retrospectively analyzed the findings on cerebral angiography before and 3 to 6 months after surgery, including Suzuki's angiographical stage, the development of surgical collaterals, and the extent of abnormal collateral channels such as lenticulostriate, thalamic, and choroidal channels. RESULTS: Following surgery, no pediatric and adult patients experienced any stroke during follow-up periods (40.2±25.5 and 54.9±19.7 months, respectively). Suzuki's stage significantly advanced in both adult and pediatric patients after surgery (P=0.042 and P<0.001). In adult patients, all of the lenticulostriate, thalamic, and choroidal channels significantly regressed after surgery (P<0.001, P=0.012, and P=0.004, respectively). In pediatric patients, however, lenticulostriate and choroidal channels significantly regressed (P=0.005 and P=0.034, respectively). Correlation analysis revealed that the development of surgical collaterals determined the postoperative regression of choroidal channels (P<0.001). CONCLUSION: STA-MCA anastomosis and EDMAPS may be one of the most effective procedures to widely provide surgical collaterals to the operated hemispheres and prevent not only ischemic but also hemorrhagic stroke by regressing the hemorrhage-prone abnormal collateral channels in MMD.


Assuntos
Revascularização Cerebral , Circulação Cerebrovascular , Circulação Colateral , Artéria Cerebral Média/cirurgia , Doença de Moyamoya/cirurgia , Artérias Temporais/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , /fisiopatologia , Humanos , /fisiopatologia , Masculino , Pessoa de Meia-Idade , Artéria Cerebral Média/diagnóstico por imagem , Artéria Cerebral Média/fisiopatologia , Doença de Moyamoya/complicações , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/fisiopatologia , Estudos Retrospectivos , Fatores de Risco , Artérias Temporais/diagnóstico por imagem , Artérias Temporais/fisiopatologia , Fatores de Tempo , Resultado do Tratamento
3.
J Stroke Cerebrovasc Dis ; 29(11): 105154, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33066884

RESUMO

OBJECTIVE: Transcranial color-duplex sonography (TCCS) is a promising method in evaluating the hemodynamics in patients with moyamoya disease (MMD). This study aimed to explore the feasibility of preoperative TCCS in predicting the outcome of revascularization surgery in MMD patients. METHODS: We retrospectively analysed 64 cases of MMD patients receiving revascularization surgery from January 2012 to January 2014. We utilized TCCS to perform comprehensive hemodynamic examination on the hemodynamics of bilateral intracranial and extracranial cerebrovascular flow and assessed the surgical outcomes and prognosis through the longitudinal comparison of the preoperative and postoperative cerebrovascular hemodynamics. Occurrence of bypass blockage was regarded as surgical failure. RESULTS: We established a prediction model for bypass blockage among MMD patients with an AUC of 0.858 (95% CI: 0.666-1). The parameters, EDV of ECA and PSV of MA obtained by the model are the main preoperative predictors for bypass blockage. CONCLUSIONS: TCCS could preoperatively determine the degree of MMD and evaluate the outcome of revascularization surgery. It also is a feasible tool to predict the curative effect by providing preoperative hemodynamic information.


Assuntos
Revascularização Cerebral , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/cirurgia , Ultrassonografia Doppler em Cores , Ultrassonografia Doppler Transcraniana , Adulto , Revascularização Cerebral/efeitos adversos , Circulação Cerebrovascular , Estudos de Viabilidade , Feminino , Hemodinâmica , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/fisiopatologia , Valor Preditivo dos Testes , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do Tratamento , Adulto Jovem
4.
J Stroke Cerebrovasc Dis ; 29(10): 105075, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32912572

RESUMO

We report an extremely rare case of a 27-year-old woman presenting with ischemic stroke as an initial manifestation of moyamoya disease in the first trimester of pregnancy. We conducted an artificial abortion when her neurological symptoms rapidly became refractory to optimal antithrombotic treatments. The progression of neurologic deficits stopped immediately after abortion, resulting in recovery to independence, with slight motor aphasia and right hemiparesis due to improved cerebral flow. We highlight rapid artificial abortion combined with antithrombotic treatment for patients of moyamoya disease with pregnancy-associated ischemic stroke as an appropriate treatment to correct hemodynamic instability and suppress the progression of neurological symptoms.


Assuntos
Aborto Terapêutico , Isquemia Encefálica/terapia , Fibrinolíticos/uso terapêutico , Doença de Moyamoya/terapia , Complicações Cardiovasculares na Gravidez/terapia , Acidente Vascular Cerebral/terapia , Adulto , Isquemia Encefálica/diagnóstico por imagem , Isquemia Encefálica/fisiopatologia , Circulação Cerebrovascular , Feminino , Hemodinâmica , Humanos , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/fisiopatologia , Gravidez , Complicações Cardiovasculares na Gravidez/diagnóstico por imagem , Complicações Cardiovasculares na Gravidez/fisiopatologia , Primeiro Trimestre da Gravidez , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/fisiopatologia , Resultado do Tratamento
5.
BMC Neurol ; 20(1): 302, 2020 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-32799829

RESUMO

BACKGROUND: Ischemic Moyamoya disease is one of the important causes of stroke, which leads to severe impairment in cognitive functions. This cognitive impairment occurs prior to stroke. However, the cognitive functions that are impaired and the mechanisms of these impairments have not been determined. METHODS: We analyzed 12 patients with Moyamoya disease and 12 controls. All participants underwent cognitive tests and magnetic resonance imaging (MRI) scans. The diffusion tensor imaging (DTI) data was processed using Tract-Based Spatial Statistics (TBSS). Significantly different white matter areas were correlated with different cognitive functions. RESULTS: There were significant differences in intelligence and subtraction between the patients and controls (p < 0.05). The parameters of DTI such as fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) have different changes in anterior thalamic radiation, inferior fronto-occipital fasciculus (IFO), superior longitudinal fasciculus (SLF), uncinate fasciculus (UF), inferior longitudinal fasciculus, forceps minor, and other regions between the two groups. CONCLUSION: Left UF and IFO may be the key brain regions affecting arithmetic function, while bilateral IFO has an effect on intelligence. RD and AD may be better indicators for early prediction of chronic white matter damage than FA, while MD tends to have a comprehensive indirect change. There is cognitive impairment in ischemic MMD, which is closely related to white matter impairment. TRIAL REGISTRATION: Clinical Trial Registration, Unique identifier: ChiCTR1900023610 . Registered 4 June 2019 - Prospective study registered.


Assuntos
Disfunção Cognitiva , Doença de Moyamoya , Substância Branca , Disfunção Cognitiva/diagnóstico por imagem , Disfunção Cognitiva/etiologia , Disfunção Cognitiva/fisiopatologia , Imagem de Tensor de Difusão , Humanos , Doença de Moyamoya/complicações , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/epidemiologia , Doença de Moyamoya/fisiopatologia , Substância Branca/diagnóstico por imagem , Substância Branca/fisiopatologia
6.
J Stroke Cerebrovasc Dis ; 29(9): 104830, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32807407

RESUMO

BACKGROUND AND PURPOSE: Nitric oxide (NO) plays a key role in ischemia and shows potential as a biomarker for ischemia. We measured mixed venous nitrite (NO2-) as a proxy for NO, during controlled cerebral ischemia in patients with moyamoya disease (MMD) during direct extracranial/intracranial (EC/IC) bypass surgery with temporary occlusion of the M4 branch of the middle cerebral artery (MCA) to permit anastomosis with the superficial temporal artery (STA). This small, focal ischemic event is not reliably detected using cerebral oximetry, somatosensory evoked potentials (SSEPs) or electroencephalography (EEG). METHODS: We enrolled nine adult MMD patients (n=8 female, n=1 male) undergoing direct EC/IC bypass surgery. Nitrite was measured at least one hour prior to MCA occlusion, and before, during and after anastomosis. Cortical function was monitored using either multi-lead EEG and SSEPs, or frontal EEG activity. RESULTS: Mixed venous NO2- was significantly elevated (p<0.05) within 12 min following arterial occlusion vs. baseline. An M4 branch of the MCA was cross clamped for a median duration of 18 (IQR = 5) minutes during anastomosis. One patient with elevated NO2- showed a transient neurologic deficit that resolved 3 days post-operatively. CONCLUSIONS: Mixed venous NO2- was significantly elevated shortly following cerebral artery occlusion vs. baseline in a majority of the study subjects, suggesting that NO2- is a potential biomarker for ischemia. Since all patients received identical burst suppression anesthesia and vasopressors, the fact that NO2- was not elevated during cross-clamp in all patients supports the conclusion that the NO2- elevation is likely due to ischemia.


Assuntos
Isquemia Encefálica/diagnóstico , Revascularização Cerebral , Artéria Cerebral Média/cirurgia , Doença de Moyamoya/cirurgia , Nitritos/sangue , Artérias Temporais/cirurgia , Oclusão Terapêutica , Adulto , Biomarcadores/sangue , Isquemia Encefálica/sangue , Isquemia Encefálica/etiologia , Isquemia Encefálica/fisiopatologia , Revascularização Cerebral/efeitos adversos , Circulação Cerebrovascular , Circulação Colateral , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Artéria Cerebral Média/diagnóstico por imagem , Artéria Cerebral Média/fisiopatologia , Doença de Moyamoya/sangue , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/fisiopatologia , Valor Preditivo dos Testes , Fatores de Risco , Artérias Temporais/diagnóstico por imagem , Artérias Temporais/fisiopatologia , Oclusão Terapêutica/efeitos adversos , Fatores de Tempo , Resultado do Tratamento , Regulação para Cima , Vasodilatação
7.
J Stroke Cerebrovasc Dis ; 29(9): 105058, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32807463

RESUMO

OBJECTIVES: Arterial spin labeling (ASL) is a magnetic resonance imaging (MRI) technique used to assess cerebral perfusion. When tissue perfusion is impaired, such as in Moyamoya disease, a hyperintense band called the arterial transit artifact (ATA) may occur, which interferes with accurate measurements on ASL-MRI. In this study, we evaluated the correlation of ATAs with magnetic resonance angiography (MRA) and single-photon emission computed tomography (SPECT) imaging results in Moyamoya disease. The aim of our study was to elucidate the pathophysiology of ATAs and risk factors for high ATA scores. MATERIALS AND METHODS: This retrospective study included 28 patients (56 hemispheres) with Moyamoya disease treated at our institution. MRI, MRA, ASL perfusion, and N-isopropyl-[123I] b-iodoamphetamine (123I-IMP) SPECT were performed. In order to semi-quantitatively evaluate the degree of ATA, the ATA scores were measured according to the number of hyperintense signal bands in the cerebral cortex. The relationship between the ATA scores and clinical and radiological factors were analyzed. RESULTS: Regional cerebral blood flow (rCBF) determined with ASL weakly correlated with that determined by 123I-IMP SPECT (ρ=0.31, p=0.027). There was no significant association between the ATA scores and rCBF values determined with 123I-IMP SPECT (p=0.872, 0.745, 0.743 at PLD1000 (post-labeling delay), 1500, and 2000, respectively). However, there was a significant correlation between ATA scores and MRA scores (ρ=0.427 p=0.001; ρ=0.612 p=0.001; ρ=0.563 p=0.001 at PLD1000, 1500, and 2000, respectively). An analysis of patient background characteristics revealed a significantly higher incidence of high ATA scores in female patients, patients with high MRA scores, and patients with a distinguishable ivy sign. A multivariate analysis confirmed that female sex, high MRA score, and presence of an ivy sign were risk factors for high ATA scores. CONCLUSION: ATA scores were moderately correlated with MRA scores, and presence of an ivy sign was the most predictive factor for high ATA scores. A high ATA score determined using ASL in a patient with Moyamoya disease might suggest an advanced disease stage and a reduction in cerebrovascular reserve capacity.


Assuntos
Artefatos , Angiografia Cerebral/métodos , Artérias Cerebrais/diagnóstico por imagem , Circulação Cerebrovascular , Angiografia por Ressonância Magnética , Doença de Moyamoya/diagnóstico por imagem , Imagem de Perfusão do Miocárdio/métodos , Marcadores de Spin , Tomografia Computadorizada de Emissão de Fóton Único , Adulto , Velocidade do Fluxo Sanguíneo , Artérias Cerebrais/fisiopatologia , Feminino , Humanos , Iofetamina/administração & dosagem , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/fisiopatologia , Valor Preditivo dos Testes , Compostos Radiofarmacêuticos/administração & dosagem , Reprodutibilidade dos Testes , Estudos Retrospectivos
8.
Cerebrovasc Dis ; 49(4): 396-403, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32829323

RESUMO

INTRODUCTION: Superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis is an effective surgical procedure for adult patients with moyamoya disease (MMD) and is known to have the potential to prevent cerebral ischemia and/or hemorrhagic stroke. Cerebral hyperperfusion (CHP) syndrome is one of the serious complications of this procedure that can result in deleterious outcomes, such as delayed intracerebral hemorrhage, but the prediction of CHP before revascularization surgery remains challenging. The present study evaluated the diagnostic value of preoperative three-dimensional (3D)-time-of-flight (TOF) magnetic resonance angiography (MRA) for predicting CHP after STA-MCA anastomosis for MMD. MATERIALS AND METHODS: The signal intensity of the peripheral portion of the intracranial major arteries, such as the anterior cerebral artery (ACA), MCA, and posterior cerebral artery (PCA) ipsilateral to STA-MCA anastomosis, on preoperative MRA was graded (0-2 in each vessel) according to the ability to visualize each vessel on 97 affected hemispheres in 83 adult MMD patients. Local cerebral blood flow (CBF) at the site of anastomosis was quantitatively measured by N-isopropyl-p-[123I]-iodoamphetamine single-photon emission computed tomography 1 and 7 days after surgery, in addition to the preoperative CBF value at the corresponding area. Then, we investigated the correlation between the preoperative MRA score and the development of CHP. RESULTS: The CHP phenomenon 1 day after STA-MCA anastomosis (local CBF increase over 150% compared with the preoperative value) was evident in 27 patients (27/97 hemispheres; 28%). Among them, 8 (8 hemispheres) developed CHP syndrome. Multivariate analysis revealed that the hemispheric MRA score (0-6), the summed ACA, MCA, and PCA scores for the affected hemisphere, was significantly associated with the development of CHP syndrome (p = 0.011). The hemispheric MRA score was also significantly correlated with the CHP phenomenon, either symptomatic or asymptomatic (p < 0.001). CONCLUSION: The signal intensity of the intracranial major arteries, including the ACA, MCA, and PCA, on preoperative 3D-TOF MRA may identify adult MMD patients at higher risk for CHP after direct revascularization surgery.


Assuntos
Angiografia Cerebral/métodos , Revascularização Cerebral/efeitos adversos , Circulação Cerebrovascular , Transtornos Cerebrovasculares/etiologia , Angiografia por Ressonância Magnética , Artéria Cerebral Média/cirurgia , Doença de Moyamoya/cirurgia , Artérias Temporais/cirurgia , Adolescente , Adulto , Idoso , Anastomose Cirúrgica , Transtornos Cerebrovasculares/diagnóstico por imagem , Transtornos Cerebrovasculares/fisiopatologia , 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 , Valor Preditivo dos Testes , Medição de Risco , Fatores de Risco , Síndrome , Artérias Temporais/diagnóstico por imagem , Artérias Temporais/fisiopatologia , Resultado do Tratamento , Adulto Jovem
9.
Cerebrovasc Dis Extra ; 10(2): 66-75, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32726777

RESUMO

INTRODUCTION: Moyamoya disease (MMD) is a rare cerebrovascular disease associated with cerebral infarction or hemorrhage. Hyperperfusion is the most significant complication of direct bypass surgery. Previous research has shown that an increase in cerebral blood flow (CBF) is strongly related to symptomatic hyperperfusion and highlighted the importance of postoperative assessment of CBF. OBJECTIVE: The principal aims of this study were to quantitatively analyze the relationship between intraoperative graft flow and increase in CBF and to evaluate the effectiveness of intraoperative graft flow measurement during bypass surgery for patients with MMD. METHODS: This study included 91 surgeries in 67 consecutive adult patients with MMD who underwent direct revascularization surgery at our institution between November 2013 and September 2018. Intraoperative graft flow of the branches and main trunk was measured in all patients, after anastomosis had been established. Postoperative CBF measurements were performed under sedation, immediately after surgery. Radiological hyperperfusion was defined as focal high uptake, as determined by CBF imaging immediately after surgery. Patients were divided into two groups (radiological hyperperfusion and nonradiological hyperperfusion groups), and the relationship between intraoperative graft flow and radiological hyperperfusion was analyzed. RESULTS: Significant differences were observed between the radiological hyperperfusion and nonradiological hyperperfusion groups in terms of intraoperative graft flow of both the branch (median 72 vs. 42 mL/min, respectively; p < 0.01) and main trunk (median 113 vs. 68 mL/min, respectively; p < 0.01). A receiver-operating characteristic analysis was performed to test the utility of intraoperative flow as a quantitative measure. We set the cutoff values for the intraoperative branch and main trunk flow at 57 mL/min (sensitivity: 0.707, specificity: 0.702; area under the curve [AUC]: 0.773; 95% confidence interval [CI]: 0.675-0.871) and 84 mL/min (sensitivity: 0.667, specificity: 0.771; AUC: 0.78; 95% CI: 0.685-0.875), respectively. CONCLUSIONS: Measuring intraoperative graft flow during bypass surgery may be an effective means of predicting hyperperfusion and could serve to facilitate early therapeutic intervention such as strict blood pressure control.


Assuntos
Angiografia Cerebral , Revascularização Cerebral/efeitos adversos , Circulação Cerebrovascular , Angiografia por Ressonância Magnética , Monitorização Intraoperatória , Doença de Moyamoya/cirurgia , Complicações Pós-Operatórias/diagnóstico por imagem , Tomografia Computadorizada de Emissão de Fóton Único , Adolescente , Adulto , Idoso , Velocidade do Fluxo Sanguíneo , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/fisiopatologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/fisiopatologia , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
10.
Cerebrovasc Dis ; 49(4): 361-368, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32674110

RESUMO

INTRODUCTION: Cerebral hyperperfusion syndrome (CHS) is one of the most serious complications after revascularization surgery in patients with moyamoya disease (MMD). However, there are few effective measures to prevent the occurrence of CHS. OBJECTIVE: The present study aims to examine the effect of the method about matching selection of donor-recipient vessels during revascularization surgery on the incidence of postoperative CHS in adult MMD patients. METHODS: 216 Chinese adult patients with MMD received surgery treatment between January 2018 and December 2019 in our hospital were enrolled in this study. 191 out of 216 patients were included in this study. Matching selection method was defined as follows: (1) blood flow: the direction of blood flow and speed of the donor artery and potential receptor arteries were measured by flow 800 indocyanine green video angiography; (2) vascular diameter: the diameters of the donor artery and potential receptor arteries by a miniature ruler. Only the artery with antegrade flow and with smallest difference in flow speed and diameter with the donor artery will be selected as the receptor artery to perform anastomosis. Matching selection was performed from January 2019. Digital subtraction angiography was performed in all patients for initial MMD diagnosis. Perioperative cerebral perfusion and related clinical symptoms were monitored. Clinical characteristics, contralateral progression, and risk factors were reviewed. The incidence of CHS and the correlation of CHS with baseline characteristics or clinical conditions were analyzed. RESULTS: Of these 191 patients, 82 patients received matching selection of donor-recipient vessels during revascularization surgery and 109 patients without. The postoperative CHS incidence in the matching group was 3.66%, which was much lower than that in the nonmatching group (15.60%). Multivariate analysis did not reveal a significant risk factor between the progression group and the nonprogression group. Correlation analysis revealed only the matching selection method was significantly associated with the reduced postoperative CHS incidence in MMD patients. CONCLUSIONS: The matching selection of donor-recipient vessels during revascularization surgery effectively reduces the incidence of postoperative CHS in adult patients with MMD.


Assuntos
Revascularização Cerebral , Circulação Cerebrovascular , Transtornos Cerebrovasculares/prevenção & controle , Doença de Moyamoya/cirurgia , Adulto , Velocidade do Fluxo Sanguíneo , Revascularização Cerebral/efeitos adversos , Transtornos Cerebrovasculares/diagnóstico por imagem , Transtornos Cerebrovasculares/epidemiologia , China/epidemiologia , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/fisiopatologia , Estudos Retrospectivos , Fatores de Risco , Síndrome , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
11.
J Stroke Cerebrovasc Dis ; 29(8): 104957, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32689603

RESUMO

INTRODUCTION: Clinical spectrum of Moyamoya angiopathy (MMA) differs across populations with different ethnicity. This study, the largest one done among Indian population was undertaken to assess clinico-radiological profile of MMA patients in eastern India. METHODS: A single centre cross-sectional study was undertaken among 76 MMA cases. Each patient was evaluated for epidemiological, clinical and radiological characteristics. SPSS 25 was used for statistical analysis. P < 0.05 was taken as statistically significant. RESULTS: 36 (47.4%) were children without gender preponderance. There were female predominance among adults (male:female = 1:2.33). Mean age at onset of first neurological symptoms for children was 4.2 ± 2.0years, followed by 34.9 ± 58.2months of latency with final diagnosis at the mean age of 7.4 ± 3.5years. For adults, mean age of onset of first neurological symptoms was 31.5 ± 12.3years, followed by 14.7 ± 41.7months time gap and diagnosed at the mean age of 33.5 ± 12.5years. There was a statistically significant difference between child and adult regarding the diagnostic latency (p = 0.035). Fixed motor weakness (FMW) was the predominant symptom across the whole disease course. Among children predominant first neurological symptom was fixed motor weakness (FMW) (52.8%), followed by seizures (22.2%). FMW was predominant (55%) first neurological complaint, followed by headache (22.5%) among adults. Seizure was more prevalent among children both as first (p = 0.002) and presenting symptom at the time of diagnosis (p = 0.048). Over the course of the disease seizure was more common among children (p = 0.001), while headache was more common among adults (p = 0.017). Recurrence of symptoms was more common among children (p = 0.059). Infarcts were more common among children (91.7%) than adults (72.5%), while hemorrhage was seen only among adults (25%) (p = 0.004). Isolated cerebral cortex was involved more commonly among children (59.4%) than adults (36.1%), while isolated subcortical involvement was seen only among adults (19.4%) (p = 0.016). Majority of the MMA cases were of Suzuki stage 4 (39.5%) and 5 (27.6%). Brain atrophy was associated with diagnostic latency (p = 0.009). CONCLUSION: Indian Moyamoya presents similar to disease presentation in Caucasian and Japanese patients. It is a frequently overlooked cause of stroke in young, often with various non-motor presentations, failure to recognize which leads to delay in diagnosis. Radiological burden disproportionate to number of acute vascular events, with subtle neurological manifestations like headache or seizure, often with cognitive decline, should raise suspicion of MMA.


Assuntos
Doença de Moyamoya/diagnóstico , Sistema Nervoso/fisiopatologia , Exame Neurológico , Avaliação de Sintomas , Adolescente , Adulto , Idade de Início , Criança , Pré-Escolar , Estudos Transversais , Diagnóstico Tardio , Feminino , Humanos , Índia/epidemiologia , Lactente , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/epidemiologia , Doença de Moyamoya/fisiopatologia , Sistema Nervoso/diagnóstico por imagem , Valor Preditivo dos Testes , Prevalência , Prognóstico , Fatores de Risco , Adulto Jovem
13.
J Stroke Cerebrovasc Dis ; 29(8): 104846, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32439351

RESUMO

BACKGROUND: To determine the functional outcomes in each period for adult ischemic and hemorrhagic Moyamoya disease (MMD) patients and identify prognostic factors. METHODS: The current retrospective study reviewed consecutive adult MMD patients surgically treated from January 2012 to June 2017. Perioperative clinical data were collected and follow-up was conducted via telephone interviews. Functional outcomes and prognostic factors were analyzed. RESULTS: A total of 219 ischemic MMD patients (268 hemispheres) and 157 hemorrhagic patients (193 hemispheres) were included. The median follow-up time was 18 months (3-69 months). MMD type had no significant effect on the modified Rankin Scale(mRS) score at discharge. Perioperative complications (P = 0.004) and the mRS score at baseline (P < 0.001) were risk factors correlated with the short-term functional outcomes in both groups, while diabetes mellitus (DM, P = 0.022) also played a role in the ischemic group. During the follow-up period, functional outcomes obviously improved in both groups, but two groups showed nonproportional cumulative curves for favorable functional outcomes (log-rank test, P = 0.483). Stroke recurrence (P < 0.001) and mRS at discharge (P < 0.001) were common factors related with long-term functional outcomes in two types of MMD patients. Particularly, female patients with ischemic MMD were more likely to have higher mRS scores (P = 0.028) and Suzuki stage was positively associated with long-term functional outcomes in hemorrhagic group (P = 0.044). CONCLUSIONS: MMD type had no significant effect on prognosis while both types of MMD patients showed overall significant improvements in functional outcomes after surgery. Different types of MMD patients have distinct prognostic factors for short-term and long-term functional outcomes.


Assuntos
Revascularização Cerebral , Doença de Moyamoya/cirurgia , Adolescente , Adulto , Idoso , Revascularização Cerebral/efeitos adversos , Avaliação da Deficiência , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/diagnóstico , Doença de Moyamoya/fisiopatologia , Recuperação de Função Fisiológica , Recidiva , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
14.
Stroke Vasc Neurol ; 5(1): 97-102, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32411414

RESUMO

Background: Evidence on the natural angiographic course of moyamoya disease (MMD) is lacking. It takes about 6 months for waiting for revascularisation surgery. The issue of when to perform subtraction angiography (DSA) for follow-up remains unclear. We investigated the natural course of MMD by DSA and attempted to determine the best interval to perform the follow-up DSA. Methods: This is a single-centre cohort study of Chinese MMD inpatients treated from 1 January 2015 to 31 August 2019. Their angiographic findings were evaluated on Suzuki stage and collateral circulation between two follow-ups of the same hemisphere. Results: A total of 110 patients who met the criteria were enrolled in this study. After a median 6 months follow-up, five patients (4.5%) had progression, four females and one male. Time interval of progression ranged from 4 to 137 months with a mean of 61.4 months. Of five patients with progression, four had unilateral lesion (two ipsilateral and two contralateral) and one had bilateral lesions. Collateral circulation was changed in three of five patients. Conclusions: The angiographic evidence of progression in MMD was rare in the short-term follow-up, and most patients with progression had initial unilateral involvement. DSA re-examination may be not needed in patients with bilateral MMD, but needed in unilateral MMD.


Assuntos
Angiografia Digital , Angiografia Cerebral , Doença de Moyamoya/diagnóstico por imagem , Adulto , Pequim , Circulação Cerebrovascular , Circulação Colateral , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/fisiopatologia , Valor Preditivo dos Testes , Estudos Retrospectivos , Fatores de Tempo
15.
J Stroke Cerebrovasc Dis ; 29(6): 104811, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32312630

RESUMO

AIM: The purpose of this study was to compare the 5-year prognosis of combined superficial temporal artery- middle cerebral artery (STA-MCA) bypass and Encephalodurosynangiosis (EDAS) and EDAS alone in hemorrhagic moyamoya disease (MMD). METHODS: This study included 123 adult patients admitted to Beijing Tiantan Hospital with hemorrhagic MMD between 2010 and 2015. The surgical procedures included combined revascularization of STA-MCA anastomosis with EDAS (n = 79) or EDAS alone (n = 44). We recorded basic demographic data as well as several risks factors, and used multivariate regression analysis to evaluate the predictive factor of overall survival and rebleeding-free survival. RESULTS: Of the 123 patients with hemorrhagic MMD, the mean age was 37.97 ± 11.04 years old and the mean follow-up period was 65.9 months (ranging from 12 to 100 months). A total of 21 rebleeding events occurred in 19 patients, yielding an annual incidence of rebleeding of 3.1%. Of the 19 patients with rebleeding, 11 (57.8%) patients died of rebleeding and one patient experience 3 rebleeding events. In the combined revascularization group, 9 (11.3%) patients experienced rebleeding, of which 5 (6.3%) died. This incidence was lower than in the indirect group, where 22.7% of patients experienced rebleeding events and 13.6% died. However, no significant difference was found between these 2 groups. In Kaplan-Meier survival analysis, the combined revascularization group had a better prognosis than the EDAS alone group, and multivariate regression analysis revealed that the combined revascularization procedure was associated with a better outcome. CONCLUSIONS: Both combined revascularization and EDAS alone can reduce the risk of rebleeding in hemorrhagic MMD. Combined revascularization was found to be superior to EDAS alone in terms of preventing rebleeding events.


Assuntos
Revascularização Cerebral , Hemorragias Intracranianas/cirurgia , Artéria Cerebral Média/cirurgia , Doença de Moyamoya/cirurgia , Artérias Temporais/cirurgia , Adulto , Pequim , Revascularização Cerebral/efeitos adversos , Revascularização Cerebral/mortalidade , Feminino , Humanos , Hemorragias Intracranianas/diagnóstico por imagem , Hemorragias Intracranianas/mortalidade , Hemorragias Intracranianas/fisiopatologia , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/mortalidade , Doença de Moyamoya/fisiopatologia , Recidiva , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento
16.
Sci Rep ; 10(1): 3700, 2020 02 28.
Artigo em Inglês | MEDLINE | ID: mdl-32111936

RESUMO

Moyamoya disease (MMD) is a rare cerebro-occlusive disease with unknown etiology that can cause both ischemic and hemorrhagic stroke. MMD is characterized by progressive stenosis of the terminal internal carotid artery (ICA) and development of basal brain collaterals. Early-stage MMD is known to cause hemodynamic insufficiency despite mild or moderate stenosis of the intracranial arteries, but the exact mechanism underlying this pathophysiological condition is undetermined. We used high-resolution Large Eddy Simulations to investigate multiple complex hemodynamic phenomena that led to cerebral ischemia in five patients with early-stage MMD. The effects of transitional flow, coherent flow structures and blood shear-thinning properties through regions of tortuous and stenosed arteries were explored and linked to symptomatology. It is evidently shown that in some cases complex vortex structures, such as Rankine-type vortices, redirects blood flow away from some arteries causing significant reduction in blood flow. Moreover, partial blood hammer (PBH) phenomenon was detected in some cases and led to significant hemodynamic insufficiency. PBH events were attributed to the interaction between shear-thinning properties, transitional flow structures and loss of upstream pressure-velocity phase lag. We clearly show that the hemodynamic complexities in early-stage MMD could induce ischemia and explain the non-responsiveness to antiplatelet therapy.


Assuntos
Estenose das Carótidas/fisiopatologia , Hemodinâmica , Ataque Isquêmico Transitório/fisiopatologia , Doença de Moyamoya/fisiopatologia , Adolescente , Adulto , Idoso , Estenose das Carótidas/tratamento farmacológico , Feminino , Humanos , Ataque Isquêmico Transitório/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/tratamento farmacológico , Inibidores da Agregação de Plaquetas/administração & dosagem
17.
Jpn J Nurs Sci ; 17(3): e12332, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32153137

RESUMO

AIM: To understand the daily lived experiences of adult moyamoya disease patients. METHODS: This qualitative study involved a purposive sample of 14 adult moyamoya disease patients diagnosed after 19 years or older at one university hospital in Seoul. Interviews conducted with patients included open-ended questions about the experience of living with moyamoya disease. The data were analyzed using Colaizzi's seven-step method, which derives the theme. RESULTS: Participants' experiences were divided into three themes and eight sub-themes. "Having an unexpected disease that suddenly struck my life" refers to confusion and depression due to the diagnosis of the unexpected illness; "being occasionally anxious about the illness" describes patients' uncertainty about the disease and worrying about passing the disease on to their child; and "living with the disease by going through the disease experience" refers to the process of accepting and adapting to the illness. CONCLUSIONS: The findings provide a better understanding of the life changes and lived experiences of adult patients with moyamoya disease. Nurses should consider various aspects when providing care to adult moyamoya disease patients.


Assuntos
Doença de Moyamoya/fisiopatologia , Adaptação Psicológica , Adulto , Ansiedade , Família , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/psicologia , Pesquisa Qualitativa
18.
BMC Neurol ; 20(1): 30, 2020 Jan 20.
Artigo em Inglês | MEDLINE | ID: mdl-31959138

RESUMO

BACKGROUND: In Moyamoya Angioplasty (MMA), increased apparent diffusion coefficient (ADC) in frontal white matter (WM) with a normal appearance has been associated with frontal hypoperfusion and executive dysfunction. Multiple burr-hole surgery enables the revascularization of large frontal areas. GOAL: To assess the effect of multiple burr-hole surgery on the ADC and cognitive functions in adults with MMA. METHODS: ADC was measured in 26 brain hemispheres of 14 consecutive adults with MMA (9 women, mean age ± SD: 38.1 ± 10.7 years) prior to and 6 months after burr-hole surgery. ADC was obtained from regions of interest located in frontal and posterior (temporo-occipital) normal-appearing WM. Ten patients had neuropsychological assessment that focused on executive and attentional functions before and after surgery. RESULTS: Anterior and posterior ADC values did not differ before surgery (815.8 ± 60.1 vs. 812.1 ± 35.3 mm2/s, p = 0.88). After surgery, frontal ADC was lower than prior to surgery (789.9 ± 64.5 vs. 815.8 ± 60.1 mm2/s; p <0.001) whereas no change occurred in posterior ADC (p = 0.31). Trail-making test part B median z-score increased from - 1.47 to - 0.21 (p = 0.018), suggesting improved cognitive flexibility. CONCLUSION: In adults with MMA, indirect revascularization with burr-hole is followed by a decrease of ADC in normal-appearing frontal WM and may have improved some executive functions in the flexibility process. Change in ADC may reflect the improvement in cerebral perfusion after surgery. The measuring of ADC may be a promising tool in exploring potentially reversible microstructural WM damage related to hypoperfusion and cognitive change in MMA.


Assuntos
Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/cirurgia , Trepanação/métodos , Substância Branca/diagnóstico por imagem , Adulto , Encéfalo/irrigação sanguínea , Encéfalo/fisiopatologia , Cognição , Imagem de Difusão por Ressonância Magnética/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/fisiopatologia , Substância Branca/fisiopatologia
19.
J Stroke Cerebrovasc Dis ; 29(4): 104625, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31952980

RESUMO

We report an adult moyamoya disease (MMD) patient who developed persistent local vasogenic edema with dynamic change in the regional cerebral blood flow after left superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis. A 49-year-old woman with ischemic-onset MMD underwent left STA-MCA anastomosis. Magnetic resonance (MR) imaging of fluid-attenuated inversion recovery 1 day after surgery revealed an asymptomatic local high-signal-intensity lesion at the site of anastomosis, and MR angiography demonstrated apparently patent STA-MCA bypass. Due to the increased apparent diffusion coefficient value, we diagnosed the lesion as vasogenic edema. A significant increase in focal cerebral blood flow (CBF) at the site of the anastomosis was observed on N-isopropyl-p-[123I] iodoamphetamine single-photon emission computed tomography (123I-IMP-SPECT) (139.8%; compared with the preoperative value). Under strict blood pressure control (systolic blood pressure under 130 mmHg), the patient remained asymptomatic during the entire peri-operative period, but the 123I-IMP-SPECT 7 days after surgery suggested paradoxical CBF decrease (72.9%). Based on this finding, we allow the patient to be maintained under normotensive condition (∼160 mmHg), which recovered the CBF (115.0%) 14 days after surgery. Vasogenic edema remained during the entire peri-operative period, but completely disappeared 83 days after surgery. Local vasogenic edema formation due to cerebral hyperperfusion is not uncommon after STA-MCA anastomosis for adult MMD, but dynamic CBF change at the site of persistent local vasogenic edema after STA-MCA anastomosis is extremely rare. We recommend serial CBF measurement in the acute stage after revascularization surgery for MMD, especially when MR imaging demonstrates local signal intensity change.


Assuntos
Edema Encefálico/etiologia , Revascularização Cerebral/efeitos adversos , Circulação Cerebrovascular , Artéria Cerebral Média/cirurgia , Doença de Moyamoya/cirurgia , Artérias Temporais/cirurgia , Edema Encefálico/diagnóstico por imagem , Edema Encefálico/fisiopatologia , Feminino , Humanos , Pessoa de Meia-Idade , Artéria Cerebral Média/diagnóstico por imagem , Artéria Cerebral Média/fisiopatologia , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/fisiopatologia , Artérias Temporais/diagnóstico por imagem , Artérias Temporais/fisiopatologia , Fatores de Tempo , Resultado do Tratamento
20.
Stroke ; 51(3): 775-783, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31856692

RESUMO

Background and Purpose- In unilateral moyamoya disease, altered endothelial shear stress on the intact-side terminal internal carotid artery might trigger the progression to bilateral disease. We analyzed the endothelial shear stress parameters of the normally appearing terminal internal carotid artery in unilateral moyamoya disease and its association with the progression to bilateral disease. Methods- This retrospective cohort study included patients diagnosed with unilateral moyamoya disease by cerebral angiography and followed-up with regular magnetic resonance imaging/magnetic resonance angiography evaluations for >1 year. Endothelial shear stress parameters acquired were mean and maximum signal intensity gradients (SIG) and SIG SD at the vessel boundary in time-of-flight sequences in initial brain magnetic resonance imaging/magnetic resonance angiography. Contralateral disease progression defined as the detection of newly developed vessel steno-occlusion with an magnetic resonance angiography steno-occlusive stage of ≥2, in the previously intact side of the brain on follow-up magnetic resonance imaging/magnetic resonance angiography evaluation. Results- Among 146 patients (66 males [45.2%] and 80 females [54.8%]; 76 pediatric [52.1%]), contralateral disease progression was detected in 43 patients (29.5%) after a mean follow-up of 4.3±2.4 years. Multivariate analysis showed that SIG SD was significantly associated with this progression (odds ratio, 13.001 [95% CI, 1.764-95.794], P=0.012). In receiver operating characteristic curve analysis, SIG SD predicted the contralateral progression with area under the curve values of 0.803 (95% CI, 0.726-0.880, P<0.001). The regression model was reproduced in the external cohort of 31 patients. Conclusions- Increased spatial variability of the endothelial shear stress around the normally appearing terminal internal carotid artery, as measured by SIG SD in time-of-flight sequences, may predict the contralateral progression of unilateral moyamoya disease.


Assuntos
Endotélio Vascular/diagnóstico por imagem , Doença de Moyamoya/diagnóstico por imagem , Adolescente , Adulto , Encéfalo/diagnóstico por imagem , Artéria Carótida Interna/diagnóstico por imagem , Artéria Carótida Interna/fisiopatologia , Criança , Pré-Escolar , Estudos de Coortes , Progressão da Doença , Endotélio Vascular/fisiopatologia , Feminino , Seguimentos , Humanos , Lactente , Angiografia por Ressonância Magnética , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/fisiopatologia , Curva ROC , Estudos Retrospectivos , Estresse Mecânico , Tomografia Computadorizada de Emissão de Fóton Único , Adulto Jovem
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