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1.
Medicine (Baltimore) ; 99(21): e20229, 2020 May 22.
Artigo em Inglês | MEDLINE | ID: mdl-32481296

RESUMO

RATIONALE: The incidence of pure arterial malformations is relatively low, and few cases have been reported. Only 2 cases with pure arterial malformation have been reported to receive surgery or endovascular treatment. PATIENT CONCERNS: We report 3 cases and review the relevant literatures. The head examinations of the patients suggested the presence of high-density shadows in front of the pons and midbrain, the dilation of the supraclinoid segment of the right internal carotid artery, and moyamoya in the left brain with an aneurysm-like expansion located on the left posterior communicating artery respectively. After admission, head digital subtraction angiography (DSA) was performed. DIAGNOSES: Digital subtraction angiography (DSA) for these 3 patients showed that the left posterior communicating artery, the supraclinoid segment of the right internal carotid artery, and the left posterior communicating artery appeared dilated, tortuous, and spirally elongated. In addition, the lesions in the latter 2 patients were accompanied with local aneurysmal changes. INTERVENTIONS: Two patients were given conservative treatment, and another patient was given endovascular treatment. A head DSA was reviewed 6 months after therapy. OUTCOMES: The prognosis status of the 3 patients was good. Two patients in the conservative treatment group showed no changes in the lesions on head DSA examination. The DSA examination of the third patient indicated that the vascular remodeling of the diseased vessels was good, the blood vessels were unobstructed, and the aneurysms had disappeared. LESSONS: Pure arterial malformations mostly occur in young women and may involve any blood vessels in the brain. It can be accompanied with local aneurysms and calcification. The patients are often given conservative treatment but need to be reviewed regularly. However, it is beneficial to give endovascular treatment to the patients with local aneurysms.


Assuntos
Aneurisma/diagnóstico por imagem , Angiografia Digital/métodos , Mesencéfalo/diagnóstico por imagem , Doença de Moyamoya/diagnóstico por imagem , Ponte/diagnóstico por imagem , Adolescente , Adulto , Aneurisma/patologia , Aneurisma/terapia , Vasos Sanguíneos/anormalidades , Artéria Carótida Interna/anormalidades , Artéria Carótida Interna/diagnóstico por imagem , Angiografia Cerebral/métodos , Criança , Pré-Escolar , Tratamento Conservador/métodos , Embolização Terapêutica/métodos , Procedimentos Endovasculares/métodos , Feminino , Humanos , Lactente , Doenças Arteriais Intracranianas/diagnóstico por imagem , Imagem por Ressonância Magnética/métodos , Masculino , Mesencéfalo/irrigação sanguínea , Mesencéfalo/patologia , Pessoa de Meia-Idade , Doença de Moyamoya/terapia , Ponte/irrigação sanguínea , Ponte/patologia , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Adulto Jovem
2.
Rev Med Liege ; 75(2): 75-77, 2020 Feb.
Artigo em Francês | MEDLINE | ID: mdl-32030929

RESUMO

Moyamoya disease is a rare cerebral vasculopathy. Disease onset is mainly sudden presenting as an ischemic stroke but also sometimes as a brain hemorraghe. Cerebral angiography is the gold standard to confirm the diagnosis. Different therapeutic approaches have been described such as conservative management or endoscopic and surgical approaches. We report the case of a young patient who was diagnosed with a brain hemorraghe following a sudden loss of consciousness.


Assuntos
Doença de Moyamoya , Acidente Vascular Cerebral , Encéfalo/diagnóstico por imagem , Angiografia Cerebral , Humanos , Doença de Moyamoya/complicações , Doença de Moyamoya/diagnóstico por imagem , Acidente Vascular Cerebral/etiologia
3.
Medicine (Baltimore) ; 99(7): e19168, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32049846

RESUMO

Ischemic moyamoya disease (MMD) can be treated with the revascularization of superficial temporal artery to middle cerebral artery (STA-MCA) bypass combined with encephalo-duro-arterio-myo-synangiosis (EDAMS) effectively. The purpose of the present study was to quantify the revascularization of STA-MCA bypass combined with EDAMS via whole-brain computed tomography perfusion (WB-CTP).Seventy-nine consecutive patients with ischemic MMD who admitted to our hospital from August 2012 to October 2018 were carried out STA-MCA bypass combined with EDAMS. WB-CTP was performed at 24 hours prior to operation and 3 months following bypass with a follow-up WB-CTP, respectively. Both automatic analysis of WB-CTP (MIStar, Apollo Medical imaging Technology, Melbourne, Australia) for analyzing values of brain volume in delayed time (DT) >3 seconds and DT > 6 seconds, relative cerebral blood flow (γCBF) < 30% and its mismatch ratio or percentage and diffusion-weighted imaging of magnetic resonance imaging in the ischemic penumbra and the infarct core at the 2 time points were studied for verifying the effectiveness of the combined revascularization. Changes in DT values at MCA-terminal territory after revascularization had been investigated. The dynamic data were with reference to the individual cerebellar arteries.All patients with ischemic MMD underwent STA-MCA bypass combined with EDAMS successfully. The preoperative brain volume in DT > 3 seconds in MCA-terminal territory was significantly larger than that of postoperative one (P < .05) in the ischemic penumbra in ischemic MMD. The mismatch ratio in brain volume of 24 hours prior to revascularization in MCA-terminal territory was significantly lower than that of 3 months (P < .05) following combined revascularization. The percentage of mismatch in brain volume of 24 hours prior to revascularization vs that of 3 months and the value of γCBF < 30% were similar to the above mismatch ratio (P < .05). The ratio of postoperative brain volume in DT > 3 seconds vs DT > 6 seconds indicated no significant differences compared with that of preoperative one (P > .05).The WB-CTP can be regarded as a choice for quantifying the combined revascularization in the ischemic penumbra and the infarct core in ischemic MMD. As proposed methods, brain volume in DT > 3 seconds, the value of γCBF < 30% and mismatch ratio in brain volume in MCA-terminal territory should be paid more attention in assessing the validity of STA-MCA bypass combined with EDAMS in ischemic MMD.


Assuntos
Angiografia Cerebral/métodos , Revascularização Cerebral , Doença de Moyamoya/diagnóstico por imagem , Adolescente , Adulto , Idoso , Imagem de Difusão por Ressonância Magnética , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/cirurgia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Adulto Jovem
4.
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
5.
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
7.
World Neurosurg ; 134: e991-e1000, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31734419

RESUMO

OBJECTIVE: Various experimental studies have reported neuroprotective effects of sevoflurane postconditioning against cerebral ischemia-reperfusion injury. We therefore investigated its neuroprotective effects on hyperperfusion-related transient neurologic deterioration, called symptomatic cerebral hyperperfusion (SCH), and also identified predictive factors for SCH in patients with moyamoya disease after revascularization surgery. METHODS: A total of 152 adult patients with moyamoya disease undergoing anastomosis of the superficial temporal artery to middle cerebral artery were randomly allocated into 2 groups. The postconditioning group (group S, n = 76) inhaled sevoflurane of 1 minimum alveolar concentration for 15 minutes immediately after reperfusion and then washed it out slowly for 15 minutes. The control group (group C, n = 76) received no intervention. The incidence of SCH was compared between the 2 groups. RESULTS: The incidence of SCH was not significantly different between groups S and C (53.3% vs. 43.4%, respectively; P = 0.291). The incidence of postoperative complications and the Glasgow Outcome Scale score at hospital discharge also did not differ significantly. Predictive factors for SCH included a decreased vascular reserve in preoperative single-photon emission computed tomography scan (odds ratio [OR], 7.18; 95% confidence interval [CI], 1.78-29.02; P = 0.006), an operation performed on the dominant hemisphere (OR, 3.32; 95% CI, 1.57-6.98; P = 0.002), temporal occlusion time (OR, 1.06; 95% CI, 1.01-1.11; P = 0.017), and intraoperative minimum partial pressure of carbon dioxide (PaCO2) (OR, 0.86; 95% CI, 0.78-0.94; P = 0.001). CONCLUSIONS: Sevoflurane postconditioning did not reduce the incidence of SCH after revascularization surgery in patients with moyamoya disease. Rather, a decreased vascular reserve, operation on the dominant hemisphere, increased temporal occlusion time, and decreased intraoperative minimum PaCO2 were associated with SCH in these patients.


Assuntos
Anestésicos Inalatórios/uso terapêutico , Isquemia Encefálica/prevenção & controle , Revascularização Cerebral/métodos , Pós-Condicionamento Isquêmico/métodos , Artéria Cerebral Média/cirurgia , Doença de Moyamoya/cirurgia , Complicações Pós-Operatórias/prevenção & controle , Sevoflurano/uso terapêutico , Artérias Temporais/cirurgia , Adulto , Isquemia Encefálica/epidemiologia , Isquemia Encefálica/fisiopatologia , Dióxido de Carbono , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/diagnóstico por imagem , Razão de Chances , Pressão Parcial , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/fisiopatologia , Fatores de Tempo , Tomografia Computadorizada de Emissão de Fóton Único
8.
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
9.
J Clin Neurosci ; 72: 258-263, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31843438

RESUMO

It is unclear whether the visual assessment of noninvasive arterial spin labeling magnetic resonance imaging (ASL) can identify instances of hemodynamic compromise including an elevated oxygen extraction fraction (OEF) measured by 15O-gas positron emission tomography (PET). Here we evaluated the relationship between a four-point visual assessment system referred to as 'ASL scores' using ASL with two postlabeling delays (PLDs; 1525 ms and 2525 ms) and some quantitative hemodynamic parameters measured by PET. We retrospectively evaluated the cases of 18 Japanese patients with moyamoya disease who underwent ASL and PET. We compared the patients' regional ASL scores on two ASL images to the regional values of PET parameters, and we observed a significant trend in accord with the presumed clinical severity among all PET parameters and ASL scores (p < .003). The ASL score of the long PLD (2525 ms) showed the highest specificity (98.5%) for elevated OEF. Our results suggest that hemodynamic impairment (including elevated OEF) in patients with moyamoya disease may be grossly assessed by a visual assessment of noninvasive ASL images, which can be easily obtained in clinical settings.


Assuntos
Hemodinâmica , Imagem por Ressonância Magnética/métodos , Doença de Moyamoya/diagnóstico por imagem , Tomografia por Emissão de Pósitrons/métodos , Adulto , Circulação Cerebrovascular , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/fisiopatologia , Marcadores de Spin
10.
Cardiovasc Pathol ; 45: 107182, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31869771

RESUMO

A case is reported of a 40-year-old woman clinically diagnosed as moyamoya disease with associated fibromuscular dysplasia of intrapulmonary bronchial arteries incidentally revealed during autoptic examination. Moyamoya disease represents an idiopathic noninflammatory and nonatherosclerotic arterio-occlusive process of intracranial arteries. Prolonged brain ischemia leads to formation of tiny and fragile collaterals. Clinically, patients with moyamoya angiopathy commonly present with severe neurological symptoms caused by brain infarction or hemorrhage. Histologically, the steno-occlusive process is based on fibrocellular thickening of intima and intimal smooth muscle cell proliferation. In the literature, extracranial arterial involvement, i.e. fibromuscular dysplasia of renal or pulmonary arteries, has been described in several cases of moyamoya disease. Our aim is to show a unique case of moyamoya disease associated with fibromuscular dysplasia affecting an uncommon site.


Assuntos
Artérias Brônquicas/patologia , Artérias Carótidas/patologia , Artérias Cerebrais/patologia , Displasia Fibromuscular/patologia , Doença de Moyamoya/patologia , Adulto , Angiografia Digital , Autopsia , Biópsia , Artérias Carótidas/diagnóstico por imagem , Artérias Carótidas/cirurgia , Angiografia Cerebral , Artérias Cerebrais/diagnóstico por imagem , Artérias Cerebrais/cirurgia , Evolução Fatal , Feminino , Displasia Fibromuscular/complicações , Humanos , Doença de Moyamoya/complicações , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/cirurgia
11.
World Neurosurg ; 136: e158-e164, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31870818

RESUMO

BACKGROUND: Indirect bypass surgery is used to improve the hemodynamic status of pediatric patients with moyamoya disease (MMD). Symptomatic cerebral infarction during the early postoperative period may be the most frustrating complication. This study was conducted to investigate the factors associated with early postoperative symptomatic cerebral infarction. METHODS: Between January 2000 and February 2014, we performed 1241 indirect bypass surgeries in 659 pediatric MMD patients. Symptomatic infarction during the early postoperative period was diagnosed in 63 operations in 61 patients. RESULTS: The overall incidence of symptomatic cerebral infarction after indirect bypass surgery was 5.1%. The median age of the patients with postoperative infarction was 6 years (mean, 6.4 years; range, 1-15 years). The performance of 2 craniotomies in single operation was associated with a higher rate of cerebral infarction. Moreover, the incidence was higher in young patients (age <6 years) compared with older patients. In a matched analysis, an immediate postoperative hemoglobin level >13 g/dL was associated with decreased risk of infarction (odds ratio, 0.144; P = 0.003). Mutation of the methylenetetrahydrofolate reductase (MTHFR) gene occurred in a relatively high proportion of our infarction cohort. CONCLUSIONS: Postoperative symptomatic infarctions can occur despite a unified surgical method and formulaic perioperative management protocol. Patient-centered factors, such as young age, genetic background of MTHFR, and certain medical conditions, including hyperthyroidism, renovascular hypertension, and hemolytic uremic syndrome, as well as management-related factors, including 2 craniotomies and low immediate postoperative hemoglobin level, could be risk factors for early postoperative symptomatic cerebral infarction.


Assuntos
Encéfalo/cirurgia , Infarto Cerebral/etiologia , Revascularização Cerebral/efeitos adversos , Doença de Moyamoya/cirurgia , Adolescente , Encéfalo/diagnóstico por imagem , Infarto Cerebral/diagnóstico por imagem , Infarto Cerebral/epidemiologia , Revascularização Cerebral/métodos , Criança , Pré-Escolar , Craniotomia/efeitos adversos , Craniotomia/métodos , Feminino , Humanos , Incidência , Lactente , Imagem por Ressonância Magnética , Masculino , Doença de Moyamoya/diagnóstico por imagem , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Resultado do Tratamento
12.
Cerebrovasc Dis ; 48(3-6): 217-225, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31812964

RESUMO

OBJECTIVE: Superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis is a standard surgical procedure for adult patients with moyamoya disease (MMD) and plays a role in preventing ischemic and/or hemorrhagic stroke. Cerebral hyperperfusion (CHP) syndrome is a potential complication of this procedure that can result in deleterious outcomes, such as delayed intracerebral hemorrhage, but the exact threshold of the pathological increase in postoperative cerebral blood flow (CBF) is unclear. Thus, we analyzed local CBF in the acute stage after revascularization surgery for adult MMD to predict CHP syndrome under modern perioperative management. MATERIALS AND METHODS: Fifty-nine consecutive adult MMD patients, aged 17-66 years old (mean 43.1), underwent STA-MCA anastomosis with indirect pial synangiosis for 65 affected hemispheres. All patients were perioperatively managed by strict blood pressure control (systolic pressure of 110-130 mm Hg) to prevent CHP syndrome. Local CBF at the site of anastomosis was quantitatively measured using the autoradiographic method 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. We defined CHP phenomenon as a local CBF increase over 150% compared to the preoperative value. Then, we investigated the correlation between local hemodynamic change and the development of CHP syndrome. RESULTS: After 65 surgeries, 5 patients developed CHP syndrome, including 2 patients with delayed intracerebral hemorrhage (3.0%), 1 with symptomatic subarachnoid hemorrhage (1.5%), and 2 with focal neurological deterioration without hemorrhage. The CBF increase ratio was significantly higher in patients with CHP syndrome (270.7%) than in patients without CHP syndrome (135.2%, p = 0.003). Based on receiver operating characteristic analysis, the cutoff value for the pathological postoperative CBF increase ratio was 184.5% for CHP syndrome (sensitivity = 83.3%, specificity =  94.2%, area under the curve [AUC] value  =  0.825) and 241.3% for hemorrhagic CHP syndrome (sensitivity =  75.0%, specificity =  97.2%, AUC value  =  0.742). CONCLUSION: Quantitative measurement of the local CBF value in the early postoperative period provides essential information to predict CHP syndrome after STA-MCA anastomosis in patients with adult MMD. The pathological threshold of hemorrhagic CHP syndrome was as high as 241.3% by the local CBF increase ratio, but 2 patients (3.0%) developed delayed intracerebral hemorrhage in this series that were managed following the intensive perioperative management protocol. Thus, we recommend routine CBF measurement in the acute stage after direct revascularization surgery for adult MMD and satisfactory blood pressure control to avoid the deleterious effects of CHP.


Assuntos
Revascularização Cerebral/efeitos adversos , Circulação Cerebrovascular , Artéria Cerebral Média/cirurgia , Doença de Moyamoya/cirurgia , Imagem de Perfusão/métodos , Complicações Pós-Operatórias/diagnóstico por imagem , Artérias Temporais/cirurgia , Tomografia Computadorizada de Emissão de Fóton Único , Adolescente , Adulto , Idoso , Anastomose Cirúrgica , Velocidade do Fluxo Sanguíneo , Diagnóstico Precoce , Feminino , Humanos , Iofetamina/administração & dosagem , 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/etiologia , Complicações Pós-Operatórias/fisiopatologia , Valor Preditivo dos Testes , Compostos Radiofarmacêuticos/administração & dosagem , Fatores de Risco , Artérias Temporais/diagnóstico por imagem , Artérias Temporais/fisiopatologia , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
13.
Medicine (Baltimore) ; 98(46): e16525, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31725599

RESUMO

To evaluate the clinical value of NeuroGam software in assessing the brain foci perfusion changes by TC-ECD single photon emission computed tomography/computed tomography (SPECT/CT) brain imaging in patients with Moyamoya Disease (MMD).Seventy-two patients with MMD who underwent superficial temporal artery-middle cerebral artery (STA-MCA) bypass combined with encephalo-duro-myo-synangiosis (EDMS) surgical revascularization were included. Baseline and follow-up TC-ECD SPECT/CT brain scans were performed on all patients at least twice before and after operation. Pre- and post-SPECT dicom images were reoriented into Talairach space using NeuroGam Software package. Additional visual analysis was performed. Differences mean pixel value between pre- and post- operation brain perfusion were assessed with paired t test and McNemar test.Significant differences in the number of hypoperfusion foci were found between visual assessment and NeuroGam aided assessment. More hypoperfusion foci were found by NeuroGam software aided assessment in the frontal, parietal, temporal, occipital lobe, thalamus, basal ganglia and cerebellum before and after surgery (P < .0001). According to NeuroGam software assessment, the perfusion of frontal, parietal, temporal lobe, anterior and middle cerebral regions on the operative side significantly improved before and after surgery (t = -3.734, t = -3.935, t = -5.099, t = -4.006, t = -5.170, all P < .001). However, no significant differences were found in the occipital lobe (t = -1.962, P = .054), thalamus (t = 1.362, P = .177), basal ganglia (t = -2.394, P = .019), and cerebellum (t = 1.383, P = .171) before and after surgery.The NeuroGam software provides a quantitative approach for monitoring surgical effect of MMD in a variable time (3-12 months after surgery). It could discover the perfusion changes that are neglected in conventional visual assessment.


Assuntos
Cisteína/análogos & derivados , Interpretação de Imagem Assistida por Computador/métodos , Doença de Moyamoya/diagnóstico por imagem , Compostos de Organotecnécio , Compostos Radiofarmacêuticos , Tomografia Computadorizada com Tomografia Computadorizada de Emissão de Fóton Único/estatística & dados numéricos , Adolescente , Adulto , Encéfalo/diagnóstico por imagem , Encéfalo/cirurgia , Revascularização Cerebral/métodos , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Artéria Cerebral Média/cirurgia , Doença de Moyamoya/cirurgia , Período Pós-Operatório , Período Pré-Operatório , Tomografia Computadorizada com Tomografia Computadorizada de Emissão de Fóton Único/métodos , Software , Artérias Temporais/cirurgia , Resultado do Tratamento , Adulto Jovem
14.
Cerebrovasc Dis ; 48(1-2): 77-84, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31578010

RESUMO

OBJECT: To investigate the association between p.R4810K variant and postoperative collateral formation (PCF) in patients with moyamoya disease. METHODS: The p.R4810K variant was detected in 254 Chinese moyamoya patients. Surgically treated 273 hemispheres with preoperative and postoperative digital subtraction angiography were included. PCF was evaluated on lateral and anteroposterior views using angiography. Univariate and multivariate logistic regression analyses were performed to determine the influence factors for PCF. RESULTS: Among 254 patients, 191 (75.2%) patients carried wild-type p.R4810K variant (GG) and 63 patients (24.8%) carried the heterozygous p.R4810K variant (GA). PCF was better in patients with GA than in patients with GG both on lateral views and anteroposterior views (p < 0.001 and p < 0.001). Over the median 7 months follow-up after discharge, good PCF was observed in 201 hemispheres (73.6%), and poor PCF was observed in 72 hemispheres (26.4%). The univariable logistic regression showed that patients with GA (OR 4.681; 95% CI 1.925-11.383; p = 0.001) was associated with good PCF. On the other hand, the increasing age (OR 0.971; 95% CI 0.952-0.989; p = 0.002) and the presence of hemorrhage (OR 0.189; 95% CI 0.096-0.374; p = 0.000) were associated with poor PCF. Multivariate logistic regression analyses of p.R4810K variant and clinical variables showed that GA (OR 3.671; 95% CI 1.452-9.283; p = 0.006) was associated with a good PCF, while the presence of hemorrhage (OR 0.258; 95% CI 0.065-0.362; p = 0.000) was identified as a predictor of poor PCF. CONCLUSIONS: The heterozygous p.R4810K variant was associated with better PCF.


Assuntos
Adenosina Trifosfatases/genética , Circulação Cerebrovascular , Circulação Colateral , Variação Genética , Doença de Moyamoya/genética , Ubiquitina-Proteína Ligases/genética , Adolescente , Adulto , Revascularização Cerebral/efeitos adversos , China , Feminino , Estudos de Associação Genética , Predisposição Genética para Doença , Heterozigoto , Humanos , Masculino , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/fisiopatologia , Doença de Moyamoya/cirurgia , Fenótipo , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Resultado do Tratamento , Adulto Jovem
15.
Pediatr Neurosurg ; 54(6): 436-440, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31590166

RESUMO

BACKGROUND: Moyamoya disease, an uncommon chronic intra-cerebral arteriopathy asymmetrically affecting the proximal vasculature, is rarely associated with clinical features pertaining to movement disorders. CASE DESCRIPTION: A 5-year-and-9-month-old boy developed repetitive episodic involuntary winking of the right eye along with ipsilateral shoulder shrugging movements in an absolutely conscious state, associated with paroxysmal shouts and loud laughs and punctuated with abusive verbal expressions (coprolalia). These episodic features, over the course of the next 1.5 years, got progressively accentuated by situations which evoked stress. In addition, there was progressive regression of verbal and cognitive milestones, emotional lability and aspects of attention deficit hyperkinetic disorder. The child was evaluated by a neurologist with magnetic resonance imaging of the brain, which showed characteristic ischaemic areas involving the basal ganglia and fronto-parietal cortical areas along the middle cerebral artery territory, predominantly on the left side. Subsequent cerebral angiography revealed extensive stenosis of bilateral (predominantly left-sided) internal cerebral arteries and middle cerebral arteries with evidence of diffuse leptomeningeal collaterals. The electroencephalography was reported to be normal. He was eventually diagnosed to be suffering from Moyamoya disease with associated Tourette's syndrome. Subsequently, the child underwent left-sided superficial temporal artery to middle cerebral artery anastomosis along with encephalo-duro-arterio-myo-synangiosis. Significant clinico-radiological improvement was noted after 3 months. The clinical deficiencies had dramatically resolved. There was evidence of excellent development of both direct and indirect surgical collaterals along the left middle cerebral artery territory. He could go back to school. CONCLUSION: Ours is probably the first case reporting an association of paediatric Moyamoya disease with Tourette's syndrome, which significantly resolved after cerebral revascularisation surgery.


Assuntos
Revascularização Cerebral , Doença de Moyamoya/cirurgia , Síndrome de Tourette/cirurgia , Angiografia Digital , Pré-Escolar , Deficiências do Desenvolvimento/etiologia , Humanos , Imagem por Ressonância Magnética , Masculino , Doença de Moyamoya/diagnóstico por imagem , Síndrome de Tourette/diagnóstico
16.
Neurol Res ; 41(12): 1097-1103, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31608819

RESUMO

Background: Management of hemorrhagic moyamoya disease (MMD) is one of the most challenging issues in neurosurgical practice. Recently, surgical revascularization by superficial temporal artery-middle cerebral artery anastomosis was reported to significantly reduce the risk of re-bleeding in hemorrhagic-onset MMD patients in the chronic state, but the optimal treatment strategy in the acute phase of hemorrhagic MMD is undetermined. Thus, we retrospectively analyzed our surgical results for hemorrhagic MMD in the acute stage, focusing on the efficacy of neuro-endoscopic management for hematoma removal. Materials and methods: Among 26 consecutive hemorrhagic MMD patients who were managed at our institution in the acute stage, 8 patients with intracerebral hemorrhage underwent surgical evacuation of the hematoma. All patients were diagnosed with MMD before surgery by magnetic resonance angiography and/or catheter angiography. Results: The initial surgical procedure was neuro-endoscopic hematoma removal in seven patients and microsurgical hematoma removal by craniotomy in one patient. One patient treated by the neuro-endoscopic procedure required subsequent small craniotomy due to difficulty in hemostasis. Satisfactory hematoma evacuation was achieved in all eight patients, as demonstrated by post-operative computed tomography, although one patient who was deeply comatose before surgery died in the early post-operative period. Conclusion: Neuro-endoscopic hematoma removal may be the optimal management method for hemorrhagic MMD in the acute stage in terms of the maximum preservation of pre-existing collateral anastomosis without affecting the potential donor vessels used for future revascularization surgery. Abbreviations: MMD: Moyamoya disease; STA: Superficial temporal artery; MCA: Middle cerebral artery; JAM: Japan Adult Moyamoya; ICH: Intracerebral hemorrhage; IVH: Intraventricular hemorrhage; SAH: Subarachnoid hemorrhage; CT: Computed tomography; TE: Total evacuation; PE: Partial evacuation; SMR: Light mass reduction; MR: Magnetic resonance; mRS: Modified Rankin scale; GCS: Glasgow Coma Scale; ICP: Intracranial pressure.


Assuntos
Hemorragia Cerebral/cirurgia , Doença de Moyamoya/cirurgia , Neuroendoscopia , Adulto , Idoso , Encéfalo/diagnóstico por imagem , Hemorragia Cerebral/diagnóstico por imagem , Hemorragia Cerebral/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/complicações , Doença de Moyamoya/diagnóstico por imagem , Estudos Retrospectivos , Resultado do Tratamento
17.
Ann Cardiol Angeiol (Paris) ; 68(5): 375-381, 2019 Nov.
Artigo em Francês | MEDLINE | ID: mdl-31471042

RESUMO

Moyamoya disease is a rare angiopathy characterized by a progressive distal occlusion of the internal carotid arteries and their branches. Extracerebral involvement, including coronary arteries, has been described. We report the case of a patient with moyamoya disease who suffered an out-of-hospital cardiac arrest associated with coronary spasm. We discussed the possible links between coronary spasm and moyamoya, as well as the contribution of multimodal cardiac imaging, combining conventional and intracoronary imaging, cardiac MRI, provocative tests for spasm, in the exploration of out-of-hospital cardiac arrest without obvious electrocardiographic and angiographic cause.


Assuntos
Vasoespasmo Coronário/diagnóstico por imagem , Doença de Moyamoya/diagnóstico por imagem , Imagem Multimodal , Parada Cardíaca Extra-Hospitalar/diagnóstico por imagem , Vasoespasmo Coronário/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/complicações , Parada Cardíaca Extra-Hospitalar/complicações
18.
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
19.
J Comput Assist Tomogr ; 43(4): 539-546, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31268874

RESUMO

OBJECTIVE: The objective of this study was to investigate the changes in the blood oxygen level-dependent (BOLD) response in the ipsilateral primary somatosensory cortex (SI) and thalamus of patients with moyamoya disease (MMD) during sensory stimulation. METHODS: Sixty-four MMD patients, and 15 healthy volunteers were enrolled. Thirty-three MMD patients exhibited paroxysmal numbness or hypoesthesia in the unilateral limbs. Fifteen patients with acroparesthesia underwent unilateral encephaloduroarteriosynangiosis (EDAS). All volunteers underwent BOLD functional magnetic resonance imaging (BOLD-fMRI) under median nerve electrical stimulation (MNES). Blood oxygen level-dependent fMRI data were processed to obtain time-signal intensity curves in the activation areas of the bilateral SI and thalamus. Processed dynamic susceptibility contrast-enhanced magnetic resonance imaging data were used to measure the time to peak of the BOLD response in the regions of interest, including the bilateral SI, thalamus, and cerebellum. Changes in the time-signal intensity curve-related hemodynamic parameters in the ipsilateral SI and thalamus were examined between healthy controls, nonacroparesthesia patients, and asymptomatic and symptomatic sides of unilateral acroparesthesia patients during MNES. Changes in these parameters in MMD patients before and after EDAS were examined. RESULTS: Compared with healthy volunteers, 3 groups of MMD patients exhibited an increased peak of the positive BOLD response in the ipsilateral thalamus during MNES (0.65 ± 0.24 vs 0.79 ± 0.35, 0.94 ± 0.57, and 0.89 ± 0.50; P = 0.0335). The positive response peak in the ipsilateral SI markedly increased in MMD patients with acroparesthesia during MNES on the asymptomatic side (0.56 ± 0.37 vs 0.38 ± 0.27, P = 0.0243). The time to peak negative response in the ipsilateral SI was prolonged during MNES on the symptomatic side after EDAS (12.14 ± 8.90 seconds vs 18.86 ± 9.20 seconds, P = 0.0201). CONCLUSIONS: During sensory stimulation treatment, BOLD response changes occurred in the ipsilateral SI and thalamus of MMD patients. These changes enabled the contralateral hemisphere of the brain to better deal with sensory stimuli.


Assuntos
Estimulação Elétrica/métodos , Imagem por Ressonância Magnética/métodos , Doença de Moyamoya/diagnóstico por imagem , Oxigênio/sangue , Córtex Somatossensorial , Tálamo , Adulto , Feminino , Humanos , Nervo Mediano/diagnóstico por imagem , Nervo Mediano/fisiologia , Plasticidade Neuronal , Córtex Somatossensorial/irrigação sanguínea , Córtex Somatossensorial/diagnóstico por imagem , Tálamo/irrigação sanguínea , Tálamo/diagnóstico por imagem
20.
World Neurosurg ; 131: e277-e283, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31351211

RESUMO

OBJECTIVE: To explore the feasibility of computed tomography perfusion imaging (CTP) for evaluating hemodynamics in hemorrhagic moyamoya disease (MMD). METHODS: The retrospective analysis included 25 patients with hemorrhagic MMD who underwent brain CTP examination. Two experienced radiologists manually delineated regions of interest (ROIs) in the bilateral frontal lobe, temporal lobe, brain tissue adjacent to the hemorrhagic foci, and brainstem as a control region. The perfusion values for all ROIs were extracted, including cerebral blood flow (CBF), cerebral blood volume (CBV), mean transit time (MTT), and time to peak (TTP). Subsequently, the differences in perfusion values for different brain tissues were compared between the hemorrhagic side and the nonhemorrhagic side. For patients who underwent revascularization surgery, differences in perfusion values from before to after surgery were determined in brain tissues on the ipsilateral side. RESULTS: CBF in the area around the hematoma and the lateral temporal lobe on the hemorrhage hemisphere was lower than that on the contralateral side, whereas TTP and MTT were higher. Among the 14 patients who underwent revascularization, CBF and CBV in the postoperative temporal lobes were higher than the preoperative values, whereas TTP and MTT were lower. CBF and CBV in the frontal lobe were higher after the operation. CONCLUSIONS: Hemorrhagic MMD results in cerebral ischemia, and CTP could be used to localize such ischemic brain tissue and objectively evaluate the changes in cerebral hemodynamics with revascularization.


Assuntos
Hemorragia Cerebral/diagnóstico por imagem , Revascularização Cerebral , Doença de Moyamoya/diagnóstico por imagem , Adulto , Angiografia Cerebral , Hemorragia Cerebral/etiologia , Hemorragia Cerebral/cirurgia , Circulação Cerebrovascular , Angiografia por Tomografia Computadorizada , Feminino , Hemodinâmica , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Moyamoya/complicações , Doença de Moyamoya/cirurgia , Imagem de Perfusão , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
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