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Introduction: Revascularization surgery is recommended for all pediatric patients with moyamoya disease (MMD) with ischemic symptoms because the brains of such patients are still developing. By contrast, no clear guidelines for selective revascularization surgery in adult patients (30 years or more) with ischemic presentation have been established. Regarding the age of initial onset of ischemic MMD, patients in their 20s are at the bottom of the distribution and this age group may share features with both adult and pediatric patients. The present prospective study aimed to clarify the clinical features and treatment outcomes of patients in their 20s (younger patients) with ischemic MMD compared with patients aged 30-60 years (older patients)., Methods: While patients with misery perfusion in the symptomatic cerebral hemisphere on 15O-positron emission tomography underwent combined surgery including direct and indirect revascularizations in the first study period and indirect revascularization alone in the second study period, patients without misery perfusion in that hemisphere received pharmacotherapy alone through the two study periods. Cerebral angiography via arterial catheterization and neuropsychological testing were performed before and after surgery., Results: During 12 years, 12 younger patients were included and comprised 6% of all adult patients (194 patients). The incidence of misery perfusion in the affected hemisphere was significantly higher in younger (12/12 [100%]) than in older patients (57/182 [31%]) (p < 0.0001). No difference in the incidence of cerebral hyperperfusion syndrome and postoperatively declined cognition was seen between younger (2/5 [40%] and 2/5 [40%], respectively) and older (11/36 [31%] and 15/36 [42%], respectively) cerebral hemispheres undergoing combined revascularization surgery. No difference in the incidence of postoperatively formed collateral flows feeding more than one-third of the middle cerebral artery cortical territory on angiograms and postoperatively improved cognition was seen between younger (9/10 [90%] and 6/10 [60%], respectively) and older (18/22 [83%] and 14/22 [64%], respectively) cerebral hemispheres undergoing indirect revascularization surgery alone., Conclusion: Patients in their 20s with ischemic MMD always exhibit misery perfusion in the affected hemisphere, unlike older patients, and sometimes develop cerebral hyperperfusion syndrome after combined revascularization surgery, leading to cognitive decline, similar to older patients. Moreover, indirect revascularization surgery alone forms sufficient collateral circulation and restores cognitive function in patients in their 20s, similar to older patients., (© 2024 The Author(s). Published by S. Karger AG, Basel.)

Objective: The present prospective study aimed to determine whether the addition of 123 I-iomazenil (IMZ) single-photon emission computed tomography (SPECT) to brain perfusion SPECT could improve the detection accuracy of misery perfusion on positron emission tomography (PET) in adult patients with ischemic moyamoya disease (MMD)., Methods: Oxygen extraction fraction (OEF) and brain perfusion were assessed using 15 O gas PET and N-isopropyl-p-[ 123 I]-iodoamphetamine (IMP) SPECT, respectively, in 137 patients. IMZ SPECT was also performed. Regions of interest (ROIs) were automatically placed in the five middle cerebral artery (MCA) territories ipsilateral to the symptomatic cerebral hemisphere and in the contralateral posterior cerebral artery territory using a three-dimensional stereotaxic ROI template. The radioactive count of the MCA ROI to the contralateral posterior cerebral artery ROI was calculated on IMP SPECT (relative SPECT-IMP uptake) and IMZ SPECT (relative SPECT-IMZ uptake). The relative SPECT-IMZ uptake to the relative SPECT-IMP uptake was also calculated (relative SPECT-IMZ/IMP uptake). Of the five MCA ROIs in the symptomatic cerebral hemisphere in each patient, the ROI with the highest PET-OEF value (one ROI per patient) was selected for analysis., Results: Significant correlations were observed between the PET-OEF and relative SPECT-IMP uptake (correlation coefficient, - 0.683) and relative SPECT-IMZ/IMP uptake (correlation coefficient, 0.875). The area under the receiver operating characteristic curve for detecting misery perfusion (PET-OEF > 51.3%) was significantly greater for the relative SPECT-IMZ/IMP uptake than for the relative SPECT-IMP uptake (difference between areas, 0.080; p = 0.0004). The sensitivity, specificity, and positive- and negative-predictive values for the relative SPECT-IMZ/IMP uptake for detecting misery perfusion were 100%, 92%, 81%, and 100%, respectively. The specificity and positive-predictive value were significantly greater for the relative SPECT-IMZ/IMP uptake than for the relative SPECT-IMP uptake., Conclusions: The addition of IMZ SPECT to brain perfusion SPECT improves the detection accuracy of misery perfusion on PET in adult patients with ischemic MMD., (© 2023. The Author(s) under exclusive licence to The Japanese Society of Nuclear Medicine.)

Periventricular anastomosis in moyamoya disease (MMD) is an unusual angiographic finding that arises from perforating arteries such as the lenticulostriate artery (LSA), thalamic artery (THA), and anterior choroidal artery (AChA). This anastomosis is associated with increased hemorrhagic risk in MMD and can be corrected by direct revascularization surgery. The present supplementary analysis on a prospective cohort aimed to elucidate changes in periventricular anastomosis after indirect revascularization surgery alone for adult patients with misery perfusion due to ischemic MMD. Twenty-two patients with misery perfusion in the symptomatic cerebral hemisphere who underwent indirect revascularization surgery alone also underwent six-vessel cerebral angiography via arterial catheterization before and at 6 months after surgery. Before surgery, two patients (9%) had positive periventricular anastomosis from the LSA and another (5%) from the AChA; all three of these periventricular anastomoses regressed after surgery, but these changes were not statistically significant (p = 0.0833). The degree of formation of collateral vessels from the LSA significantly decreased after surgery (p = 0.0143), but the degree of collateral vessels from the THA or AChA did not differ between pre- and postoperative conditions. Eight patients with postoperative regression of the collateral vessels from any perforating artery exhibited postoperative rich collateral flow from indirect revascularization. Periventricular anastomosis tended to regress after indirect revascularization surgery alone for adult patients with misery perfusion due to ischemic MMD. Collateral vessels formed from the LSA likely regressed after indirect revascularization surgery alone for such patients, but those vessels from the THA or AChA seldom changed., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: De novo cerebral microbleeds (CMBs) on T2*-weighted magnetic resonance imaging (MRI) develop over time in adult moyamoya disease (MMD) and are generally associated with a decline in global cognitive function. The present supplementary analysis of a 5-year prospective cohort aimed to elucidate the incidence of an interval increase in CMBs in adult patients receiving medical management alone for ischemic MMD and its impact on cognitive function., Methods: Sixty-four patients without misery perfusion in the symptomatic cerebral hemispheres at inclusion who did not experience any further ischemic symptoms or new hemorrhagic events during a 5-year follow-up period underwent T2*-weighted MRI and five kinds of neuropsychologic tests at inclusion and the end of the 5-year follow-up., Results: When T2*-weighted MRI was compared between inclusion and the end of the 5-year follow-up, 10 patients (15%) had an interval increase in CMBs in the symptomatic cerebral hemisphere at inclusion. The scores from two kinds of neuropsychologic tests significantly deteriorated at the end of the 5-year follow-up compared with those at inclusion in patients with an interval increase in CMBs, whereas the scores of four kinds of neuropsychologic tests significantly improved at the end of the 5-year follow-up compared with those at inclusion in patients without interval increases in CMBs, asymptomatic ischemic lesions, or angiographic disease progression., Conclusions: The incidence of an interval increase in CMBs was 15% per 5 years in adult patients receiving medical management alone for ischemic MMD, and this increase was associated with a decline in cognitive decline.

Objective: Brain 123 I-iomazenil single-photon emission computed tomography (SPECT) can assess the distribution of the binding potential of central benzodiazepine receptors in the cerebral cortex. This binding potential may reflect neuronal function in viable tissues. The present prospective study using brain 123 I-iomazenil SPECT aimed to determine whether improvements in cognitive function after indirect revascularization surgery alone are associated with postoperative recovery in neurotransmitter receptor function in the affected cerebral hemisphere among adult patients with moyamoya disease accompanied by ischemic presentation due to misery perfusion., Methods: Twenty-two patients who underwent indirect revascularization surgery alone also underwent brain SPECT scanning at 180 minutes after 123 I-iomazenil administration and neuropsychological testing before and at 6 months after surgery. The affected-to-contralateral cerebral hemispheric asymmetry of tracer uptake before and after surgery was then calculated., Results: The asymmetry of tracer uptake was significantly increased after surgery (P < 0.0001). A significant difference between the preoperative and postoperative asymmetry of tracer uptake was seen in patients with improved cognition compared with those with unchanged cognition (P = 0.0001). The area under the receiver operating characteristic curve was 0.99 for the difference between the preoperative and postoperative asymmetry of tracer uptake to assess the ability to discriminate patients with improved cognition from those with unchanged cognition., Conclusions: Improvements in cognitive function after indirect revascularization surgery alone are associated with postoperative recovery in the binding potential of central benzodiazepine receptors in the affected cerebral hemisphere in adult patients with moyamoya disease accompanied by ischemic presentation due to misery perfusion., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objectives: Although revascularization surgery is recommended for adult patients with moyamoya disease (MMD) who present with ischemic symptoms due to hemodynamic compromise, the clinical course of such patients who are treated with medical management alone remains unclear. Here, we report outcomes of adult patients with cerebral misery perfusion due to ischemic MMD who received medical management alone., Materials and Methods: We prospectively followed up patients who showed misery perfusion in the symptomatic cerebral hemisphere on 15 O gas positron emission tomography (PET) and received strict medical management alone after refusing revascularization surgery., Results: Of 57 patients who showed symptomatic misery perfusion on 15 O gas PET, three (5%) were included into the present study. Two of these patients suffered further ischemic events at 7 and 8 months after inclusion, after which, their modified Rankin disability scale scores deteriorated. In the remaining patient, fatal intracerebral hemorrhage developed at 10 months after inclusion., Conclusions: These findings suggest that receiving medical management alone is associated with considerably poor outcomes for adult patients with cerebral misery perfusion due to ischemic MMD., Competing Interests: Declaration of Competing Interest Kuniaki Ogasawara declares the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Consigned research fund from Nihon Medi-Physics Co., Ltd., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Revascularization surgery for adult patients with ischemic moyamoya disease (MMD) may improve both cognitive function and cerebral perfusion., Objective: To determine angiographic, cerebral hemodynamic, and cognitive outcomes of indirect revascularization surgery alone for adult patients with misery perfusion due to ischemic MMD (IDR group) and to test the superiority of indirect revascularization surgery for cognitive improvement by conducting comparisons with historical control patients who had undergone direct revascularization surgery (DR group) through prospective cohort study with historical controls., Methods: Twenty adult patients with cerebral misery perfusion underwent encephalo-duro-myo-arterio-pericranial-synangiosis alone. Cerebral angiography through arterial catheterization, brain perfusion single-photon emission computed tomography, and neuropsychological testing were performed preoperatively and at 6 months postoperatively., Results: In 17 patients of the IDR group, collateral flows that were newly formed after surgery on angiograms fed more than one-third of the middle cerebral artery (MCA) cortical territory. In the IDR group, perfusion in the MCA territory was significantly increased after surgery (P < .0001), and the difference in MCA perfusion between before and after surgery was significantly greater (P = .0493) compared with the DR group. Improved cognition was significantly more frequent in the IDR group (65%) than in the DR group (31%, P = .0233)., Conclusion: Indirect revascularization surgery alone forms sufficient collateral circulation, improves cerebral hemodynamics, and recovers cognitive function in adult patients with misery perfusion due to ischemic MMD. The latter 2 beneficial effects may be higher when compared with patients undergoing direct revascularization surgery., (Copyright © Congress of Neurological Surgeons 2022. All rights reserved.)

Angiographic disease progression reportedly develops in adult moyamoya disease (MMD). However, more than half of patients analyzed underwent revascularization surgery. The present supplementary analysis of a 5-year prospective cohort with follow-up using magnetic resonance angiography (MRA) and cerebral blood flow (CBF) measurements was to elucidate the incidence and clinical features of angiographic disease progression in adult patients receiving medical management alone for ischemic MMD. Sixty-eight patients without misery perfusion in the symptomatic cerebral hemispheres underwent MRA and CBF measurement using brain perfusion single-photon emission computed tomography at inclusion and at the end of the 5-year follow-up. When neurological symptoms recurred or newly developed during the 5-year follow-up period, additional MRA and CBF measurements were also performed at that time. All four patients with further ischemic events during the 5-year follow-up period exhibited angiographic disease progression on MRA at such events. Of the remaining 64 patients without further events during the 5-year follow-up period, four exhibited angiographic disease progression on MRA at the end of the 5-year follow-up. CBF was significantly lower at the time of further ischemic events or at the end of the 5-year follow-up than at inclusion in eight patients with angiographic disease progression (p = 0.0117). The incidence of angiographic disease progression was 12% for 5 years in medically treated adult patients with ischemic MMD without cerebral misery perfusion. Patients with further ischemic events always exhibited angiographic disease progression. Cerebral perfusion was reduced in patients with angiographic disease progression even when further ischemic events did not occur., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Adult patients with moyamoya disease (MMD) occasionally exhibit cerebral hyperperfusion after arterial bypass surgery, leading to persistent cognitive decline. The present supplementary analysis of a prospective 5-year cohort study aimed to determine whether cerebral hyperperfusion after arterial bypass surgery for adult patients with misery perfusion due to ischemic MMD causes cerebral atrophy, and whether the development of cerebral atrophy is related to persistent cognitive decline., Methods: In total, 31 patients who underwent arterial bypass surgery also underwent fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) and neuropsychological testing before surgery and at the end of a 5-year follow-up. The development of cerebral hyperperfusion and hyperperfusion syndrome after surgery was defined based on brain perfusion single-photon emission computed tomography (SPECT) findings and clinical symptoms. Univariate and multivariate logistic regression analyses of factors related to the development of cerebral atrophy on FLAIR MRI or cognitive decline on neuropsychological testing at the end of the 5-year follow-up were performed., Results: Eleven patients (35%) developed cerebral atrophy in the frontal lobe where the superficial temporal artery was anastomosed. Cerebral hyperperfusion on brain perfusion SPECT (odds ratio [OR], 50.6; p = 0.0008) or cerebral hyperperfusion syndrome (OR, 41.8; p = 0.0026) was independently associated with the development of cerebral atrophy, and cerebral atrophy development was significantly associated with cognitive decline (OR, 47.7; p = 0.0010)., Conclusions: Cerebral hyperperfusion after arterial bypass surgery for adult patients with misery perfusion due to ischemic MMD can cause cerebral atrophy related to persistent cognitive decline., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Objectives: Adult patients with moyamoya disease (MMD) occasionally develop cognitive decline due to cerebral hyperperfusion following direct revascularization surgery. However, how the hyperperfusion phenomenon contributes to declines in cognitive function remains unclear. The present supplementary analysis of a prospective study aimed to determine whether cerebral hyperperfusion following direct revascularization surgery for adult MMD with ischemic presentation and misery perfusion leads to development of de novo cerebral microbleeds (CMBs) and whether postoperative cognitive decline is related to these CMBs., Materials and Methods: In total, 32 patients who underwent direct revascularization surgery also underwent T2*-weighted magnetic resonance imaging (T2*WI) and neuropsychological testing before and 2 months after surgery. Development of cerebral hyperperfusion and hyperperfusion syndrome following surgery was defined based on brain perfusion single-photon emission computed tomography (SPECT) findings and clinical symptoms., Results: Cerebral hyperperfusion on brain perfusion SPECT (95% confidence interval [CI], 1.1-10.8; p = 0.0175) or cerebral hyperperfusion syndrome (95%CI, 1.3-15.3; p = 0.0029) was significantly associated with postoperatively increased CMBs on T2*WI. Postoperatively increased CMBs were significantly associated with postoperative cognitive decline (95%CI, 1.8-20.4, p = 0.0041). For patients with cerebral hyperperfusion on brain perfusion SPECT, the incidence of postoperative cognitive decline was significantly greater in patients with than in those without postoperatively increased CMBs (p = 0.0294)., Conclusions: Cerebral hyperperfusion following direct revascularization surgery for adult MMD with ischemic presentation and misery perfusion contributes to the development of de novo CMBs and postoperative cognitive decline is related to these CMBs., Competing Interests: Declaration of Competing Interest Kuniaki Ogasawara declares the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Consigned research fund from Nihon Medi-Physics Co., Ltd., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Introduction: No clear guidelines for treating adult patients with ischemic moyamoya disease (MMD) without cerebral hemodynamic compromise such as misery perfusion have been established. Our previous prospective cohort study of adult patients with MMD without misery perfusion who were treated with medical management alone, including an antiplatelet drug, showed a recurrent ischemic event rate of 3% per 2 years. The present prospective study aimed to elucidate the 5-year clinical, cerebral perfusion, and cognitive outcomes of medical management alone for Japanese adult patients with ischemic MMD without cerebral misery perfusion by following the same patients for another 3 years., Methods: In total, 68 patients without recurrent events at a 2-year follow-up were prospectively followed up for another 3 years. Cerebral blood flow (CBF) in the symptomatic cerebral hemisphere was measured using brain perfusion single-photon emission computed tomography at inclusion and at the end of the subsequent 3-year follow-up. Neuropsychological testing was performed at inclusion and at the end of the initial 2- and subsequent 3-year follow-ups., Results: During the subsequent 3-year follow-up, 2 patients (3%) developed further ischemic events. In patients without further ischemic events, CBF was significantly greater at the end of the subsequent 3-year follow-up than at inclusion (p = 0.0037), and all neuropsychological test scores improved or remained unchanged at the end of initial 2- and subsequent 3-year follow-ups compared with that at inclusion., Conclusion: In adult patients receiving medical management alone for ischemic MMD without cerebral misery perfusion, the incidence of further ischemic events was 6% per 5 years and did not change between the initial 2 years after the last is-chemic event and the subsequent 3 years. In patients without further ischemic events, CBF and cognitive function had not deteriorated at 5 years after the last ischemic event., (© 2021 S. Karger AG, Basel.)

Introduction: Revascularization surgery for adult moyamoya disease (MMD) with ischemic presentation changes cognitive function and prevents further cerebral ischemic events. Most studies however repeated neuropsychological evaluation within 1 year after surgery. Our previous prospective cohort study of adult patients with MMD with misery perfusion who underwent direct revascularization surgery showed cognitive improvement and decline in 31% and 44%, respectively, of the patients 2 months after surgery. The present prospective study aimed to elucidate the 5-year cognitive changes after direct revascularization surgery in adult patients with cerebral misery perfusion due to ischemic MMD by following the same patients., Methods: In total, 31 patients were prospectively followed up for 5 years after direct revascularization surgery. Five types of neuropsychological tests were performed preoperatively, 2 months after surgery, and at the end of the 5-year follow-up. Cerebral blood flow (CBF) in the symptomatic cerebral hemisphere relative to that in the ipsilateral cerebellar hemisphere (hemispheric relative CBF [RCBF]) was measured using brain perfusion single-photon emission computed tomography preoperatively and at the end of the 5-year follow-up., Results: Based on results of pre- and postoperative neuropsychological tests, 11, 10, and 10 patients showed cognitive improvement, no change in cognitive function, and cognitive decline, respectively, at the end of the 5-year follow-up. These ratios were not significantly different compared with those 2 months after surgery (cognitive improvement, no change in cognitive function, and cognitive decline in 10, 8, and 13 patients, respectively). Although hemispheric RCBF was significantly greater at the end of the 5-year follow-up than before surgery in patients with cognitive improvement (80.7 ± 6.1% vs. 92.9 ± 5.5%; p = 0.0033) and in those showing no change in cognitive function (85.6 ± 3.5 vs. 91.5 ± 5.2%; p = 0.0093), this value was significantly lower at the end of the 5-year follow-up than before surgery in patients with cognitive decline (83.8 ± 3.7 vs. 81.0 ± 5.8%; p = 0.0367)., Conclusion: One-third of adult patients with cerebral misery perfusion due to ischemic MMD who underwent direct revascularization surgery exhibited cognitive improvement, and one-third exhibited decline at the end of the 5-year follow-up. The former and latter patients had increased and decreased CBF, respectively, in the affected cerebral hemisphere at the end of the 5-year follow-up compared with preoperative brain perfusion., (© 2021 The Author(s). Published by S. Karger AG, Basel.)

Background: In nonsurgical adult moyamoya disease (MMD) patients with ischemic onset and stable hemodynamics, the cerebral hemodynamic and cognitive course remains unclear., Objective: To clarify, through a prospective cohort study, 2-yr clinical, cerebral hemodynamic, and cognitive outcomes of adult patients receiving medication alone for symptomatically ischemic MMD without cerebral misery perfusion., Methods: Seventy patients without cerebral misery perfusion on the first 15O gas positron emission tomography (PET) were clinically followed up for 2 yr. The second PET was performed at the end of the 2-yr follow-up. Neuropsychological tests were also performed at the study entry and the end of the 2-yr follow-up., Results: During the 2-yr follow-up period, 2 patients (3%) developed further cerebral ischemic symptoms and showed new cerebral misery perfusion on PET performed at recurrence. None of the 68 patients without further ischemic symptoms showed cerebral misery perfusion on second PET. All 66 patients who underwent the first and second neuropsychological tests displayed unchanged interval cognition at the 2-yr follow-up., Conclusion: Among adult patients receiving medication alone for symptomatically ischemic MMD without cerebral misery perfusion, the incidence of recurrent ischemic events was 3% per 2 yr. In patients without recurrent ischemic events, cerebral hemodynamics and cognitive function had not deteriorated by 2 yr after the last event., (Copyright © 2018 by the Congress of Neurological Surgeons.)

Objective: Adult patients with ischemic moyamoya disease (MMD) who receive treatment with antiplatelet drugs reportedly show improvements in neuropsychological test scores after around 2 years. The purpose of the present study subanalyzing the same patient cohort used in a previous study was to determine which antiplatelet drug, clopidogrel or cilostazol, results in better improvement of cognitive function among non-surgical adult patients showing ischemic MMD without severe hemodynamic compromise., Methods: Sixty-six patients without cerebral misery perfusion on 15 O gas positron emission tomography were treated with pharmacotherapy alone. Patients ≥50 years old and <50 years old initially received clopidogrel and cilostazol, respectively. Any patient suffering side effects of the antiplatelet drug switched to the other antiplatelet drug. Neuropsychological tests were performed at study entry and at the end of the 2-year follow-up, and differences in each neuropsychological test score between the two time points (second test score - first test score) were calculated and defined as Δ scores., Results: Among the five neuropsychological tests, Δ scores for two tests were significantly greater in patients treated with cilostazol (n = 36) than in those treated with clopidogrel (n = 30), and Δ scores of the remaining three tests did not differ between patient groups. Based on Δ scores, 15 patients (23%) were defined as showing interval cognitive improvement. On multivariate analysis, cilostazol administration (95% confidence interval, 1.19-193.98; P = 0.0361) represented an independent predictor of interval cognitive improvement., Conclusions: Cilostazol may improve cognition better than clopidogrel in non-surgical adult patients with ischemic MMD.

Objective: Some adult patients with moyamoya disease (MMD) undergoing revascularization surgery show an improvement or decline in cognition postoperatively. Revascularization surgery for ischemic MMD augments cerebral blood flow (CBF) and improves cerebral oxygen metabolism. However, cerebral hyperperfusion, which is a short-term, major increase in ipsilateral CBF that is much greater than the metabolic needs of the brain, sometimes occurs as a complication. Cerebral hyperperfusion produces widespread, minimal injury to the ipsilateral white matter and cortical regions. The aim of the present prospective study was to determine how changes in CBF due to arterial bypass surgery affect cognitive function in adult patients with symptomatic ischemic MMD and misery perfusion., Methods: Thirty-two patients with cerebral misery perfusion, as determined on the basis of 15O gas positron emission tomography, underwent single superficial temporal artery-middle cerebral artery (M4 in the precentral region) anastomosis. Brain perfusion single-photon emission computed tomography (SPECT) studies were performed preoperatively, on the 1st postoperative day, and 2 months after surgery. Neuropsychological tests were also performed preoperatively and 2 months after surgery., Results: Postoperative neuropsychological assessments demonstrated cognitive improvement in 10 cases (31%), no change in 8 cases (25%), and decline in 14 cases (44%). Based on brain perfusion SPECT and symptoms, 10 patients were considered to have cerebral hyperperfusion syndrome, and all of these patients exhibited a postoperative decline in cognition. Relative precentral CBF on the 1st postoperative day was significantly greater in patients with postoperative cognitive decline (167.3% ± 15.3%) than in those with improved (105.3% ± 18.2%; p < 0.0001) or unchanged (131.4% ± 32.1%; p = 0.0029) cognition. The difference between relative precentral CBF 2 months after surgery and that before surgery was significantly greater in patients with postoperative cognitive improvement (17.2% ± 3.8%) than in those with no postoperative change (10.1% ± 2.4%; p = 0.0003) or with postoperative decline (11.5% ± 3.2%; p = 0.0009) in cognition., Conclusions: Cerebral hyperperfusion in the acute stage after arterial bypass surgery impairs cognitive function. An increase in CBF in the chronic stage without acute-stage cerebral hyperperfusion improves cognitive function in adult patients with symptomatic ischemic MMD and misery perfusion.

Cerebral hyperperfusion syndrome following arterial bypass surgery is known as a surgical complication of moyamoya disease (MMD). How cerebral hyperperfusion affects neural function and causes neurological deficits remains unknown. We report here a case with cerebral hyperperfusion syndrome after arterial bypass surgery for ischemic MMD. Chronological changes of brain perfusion and central benzodiazepine receptor biding potential were observed using single-photon emission computed tomography. A 20-year-old woman with ischemic MMD underwent arterial bypass surgery. Six days later, cerebral hyperperfusion syndrome developed. During this syndrome, contralateral-to-ipsilateral cerebellar asymmetry of blood flow and a decrease in central benzodiazepine receptor binding potential in the area with hyperperfusion were observed. Four months later, these two findings resolved and a neurological examination revealed no abnormal signs. Cerebral hyperperfusion after arterial bypass surgery for ischemic MMD may lead to transient, reversible reduction of cerebral metabolism and downregulation of cortical neurotransmitter receptor function, resulting in transient neurological deficits.

Background and Purpose: Adult patients with symptomatically ischemic moyamoya disease (MMD) initially undergo medical treatment alone including antiplatelet drugs when symptomatic cerebral hemispheres do not exhibit hemodynamic compromise. The purpose of the present study subanalyzing the same patient cohort used in a previous study was to determine which antiplatelet drug, clopidogrel or cilostazol, provides better improvement of cerebral perfusion in such patients., Methods: All patients without cerebral misery perfusion on 15 O gas positron emission tomography (PET) did not undergo revascularization surgery and were treated with medication alone, including antiplatelet therapy. Patients ≥50years and <50years initially received clopidogrel and cilostazol, respectively. When a patient suffered side effects of an antiplatelet drug, they were switched to the other antiplatelet drug. Cerebral blood flow (CBF) in the symptomatic hemisphere was measured at inclusion and at 2years after inclusion using 15 O gas PET., Results: Of 68 patients, 31 and 38 were treated with clopidogrel and cilostazol, respectively, for 2years after inclusion. For patients treated with clopidogrel, CBF did not differ between first and second PET. For patients treated with cilostazol, CBF was significantly greater in the second PET than in the first PET. On multivariate analysis, cilostazol administration was an independent predictor of CBF improvement in the symptomatic hemisphere (95% confidence interval, 1.34-139.20; P =.0271)., Conclusions: Cilostazol improves cerebral perfusion better than clopidogrel in adult patients with symptomatically ischemic MMD not accompanied by misery perfusion., (Copyright © 2018. Published by Elsevier Inc.)

The present study examined whether preoperatively reduced cerebrovascular contractile reactivity to hypocapnia by hyperventilation is associated with development of cerebral hyperperfusion syndrome after arterial bypass surgery for adult patients with cerebral misery perfusion due to ischemic moyamoya disease. Among 65 adult patients with ischemic moyamoya disease, 19 had misery perfusion in the precentral region on preoperative 15 O positron emission tomography and underwent arterial bypass surgery for that region. Brain technetium-99 m-labeled ethyl cysteinate dimer single-photon emission computed tomography (SPECT) was preoperatively performed with and without hyperventilation challenge and relative cerebrovascular contractile reactivity to hypocapnia (RCVCR hypocap ) (%/mmHg) was calculated in the precentral region. Development of cerebral hyperperfusion syndrome was determined using perioperative changes of symptoms and brain N-isopropyl-p-[ 123 I]-iodoamphetamine SPECT performed after surgery. RCVCR hypocap was significantly lower in the 6 patients with cerebral hyperperfusion syndrome (-2.85 ± 1.10%/mmHg) than in the 13 patients without cerebral hyperperfusion syndrome (0.18 ± 1.97%/mmHg; p = 0.0050). Multivariate analysis demonstrated low RCVCR hypocap as an independent predictor of cerebral hyperperfusion syndrome (95% confidence interval, 0.04-0.96; p = 0.0433). Preoperatively reduced cerebrovascular contractile reactivity to hypocapnia by hyperventilation is associated with development of cerebral hyperperfusion syndrome after arterial bypass surgery for adult patients with cerebral misery perfusion due to ischemic moyamoya disease.

Here we describe the case of a patient with moyamoya disease who developed cerebral infarction followed shortly by subarachnoid hemorrhage and intracerebral hemorrhage. A 50-year-old woman presenting with sudden and transient weakness of the left lower limb was transferred to a local hospital. Magnetic resonance imaging(MRI)revealed cerebral infarction in the corpus callosum and in the bilateral frontal lobes induced by moyamoya disease. Pyramidal tract was not affected. The patient was treated with an anti-platelet agent(ozagrel sodium 160mg/day)and did not undergo intentional antihypertensive therapy. The patient's averaged blood pressure was 150/90mmHg. Six days after symptom onset, the patient's orientation deteriorated. Head computed tomography scans revealed a subarachnoid hemorrhage in the right sylvian fissure and in the bilateral frontal lobes and an intracerebral hemorrhage adjacent to the left corona radiata. In addition, MRI revealed a significant expansion of the cerebral infarction in the bilateral frontal lobes. Angiography with arterial catheterization confirmed that the patient's symptoms met diagnostic criteria of stage III moyamoya disease. Positron emission tomography revealed hemodynamic compromise in the left cerebral hemisphere. The patient underwent a superficial temporal artery-middle cerebral artery anastomosis with encephalo-duro-myo-synangiosis. The patient's postoperative course was uneventful. Postoperative T2 * -weighted MRI revealed multiple microbleeds in both cerebral hemispheres.

Objective and Importance: The prognosis of cerebral hyperperfusion syndrome after vascular reconstructive surgery, including extracranial-intracranial arterial bypass, is not poor unless intracerebral hemorrhage develops secondary to hyperperfusion., Clinical Presentation: A 48-year-old man with symptomatic moyamoya disease with misery perfusion in the right cerebral hemisphere underwent double right superficial temporal artery-to-middle cerebral artery bypasses. The postoperative course was uneventful until the patient developed headache and agitated delirium on the 4th postoperative day., Intervention: Perfusion computed tomographic imaging demonstrated hyperperfusion in the right temporal lobe. The symptoms resolved by institution of intensive blood pressure control. Positron emission tomography performed 2 months after surgery demonstrated a postoperative reduction of the cerebral metabolic rate of oxygen in the right temporal lobe, where brain atrophy was observed on magnetic resonance images 3 months postoperatively. Neuropsychological testing performed 3 months postoperatively showed worsening digit span, which adversely affected the patient's quality of life., Conclusion: The current case suggests that cerebral hyperperfusion after vascular reconstructive surgery can cause irreversible neural damage, which results in cognitive impairment.

This study aimed to determine the influence of atherosclerotic risk factors on initial and further cerebrovascular events in adult patients with moyamoya disease (MMD) by combined analysis of two prospective cohorts in which patients received pharmacotherapy alone and were prospectively followed-up for 5 years. In 71 patients, smoking status, home blood pressure, hemoglobin (Hb)A1c and low-density lipoprotein cholesterol (LDL-chol) were checked at inclusion and at further cerebrovascular event or at the end of 5-year follow-up. When a patient had daily smoking, increased HbA1c, increased LDL-chol, increased systolic blood pressure, or increased diastolic blood pressure, the patient was categorized as showing atherosclerotic burden. Angiographic disease progression was determined using changes on magnetic resonance angiography. Eleven patients showed angiographic disease progression and seven of these 11 patients experienced further cerebrovascular events during the follow-up period. The remaining 60 patients did not exhibit either condition. At inclusion, the incidence of atherosclerotic burden was significantly greater in patients without angiographic disease progression (80%) than in those with such progression (45%; p = 0.0249). For patients without angiographic disease progression, values or incidence of almost all variables showed significant interval decreases at the end of 5-year follow-up (p < 0.05). Adult patients with ischemic MMD who do not exhibit angiographic disease progression appear more strongly affected by atherosclerotic burden at the initial onset of cerebrovascular events than those exhibiting angiographic disease progression. A reduction in atherosclerotic burden by medical treatments for the former patients prevents further cerebrovascular events. [ABSTRACT FROM AUTHOR]

Objective: The present prospective study aimed to determine whether the addition of 123I-iomazenil (IMZ) single-photon emission computed tomography (SPECT) to brain perfusion SPECT could improve the detection accuracy of misery perfusion on positron emission tomography (PET) in adult patients with ischemic moyamoya disease (MMD). Methods: Oxygen extraction fraction (OEF) and brain perfusion were assessed using 15O gas PET and N-isopropyl-p-[123I]-iodoamphetamine (IMP) SPECT, respectively, in 137 patients. IMZ SPECT was also performed. Regions of interest (ROIs) were automatically placed in the five middle cerebral artery (MCA) territories ipsilateral to the symptomatic cerebral hemisphere and in the contralateral posterior cerebral artery territory using a three-dimensional stereotaxic ROI template. The radioactive count of the MCA ROI to the contralateral posterior cerebral artery ROI was calculated on IMP SPECT (relative SPECT-IMP uptake) and IMZ SPECT (relative SPECT-IMZ uptake). The relative SPECT-IMZ uptake to the relative SPECT-IMP uptake was also calculated (relative SPECT-IMZ/IMP uptake). Of the five MCA ROIs in the symptomatic cerebral hemisphere in each patient, the ROI with the highest PET-OEF value (one ROI per patient) was selected for analysis. Results: Significant correlations were observed between the PET-OEF and relative SPECT-IMP uptake (correlation coefficient, – 0.683) and relative SPECT-IMZ/IMP uptake (correlation coefficient, 0.875). The area under the receiver operating characteristic curve for detecting misery perfusion (PET-OEF > 51.3%) was significantly greater for the relative SPECT-IMZ/IMP uptake than for the relative SPECT-IMP uptake (difference between areas, 0.080; p = 0.0004). The sensitivity, specificity, and positive- and negative-predictive values for the relative SPECT-IMZ/IMP uptake for detecting misery perfusion were 100%, 92%, 81%, and 100%, respectively. The specificity and positive-predictive value were significantly greater for the relative SPECT-IMZ/IMP uptake than for the relative SPECT-IMP uptake. Conclusions: The addition of IMZ SPECT to brain perfusion SPECT improves the detection accuracy of misery perfusion on PET in adult patients with ischemic MMD. [ABSTRACT FROM AUTHOR]

Aim: Indirect revascularization surgery for moyamoya disease (MMD) sometimes results in swelling of the soft tissues (including the temporalis muscle) compressing the cerebral hemispheres postoperatively. The present supplementary analysis of two prospective cohorts aimed to elucidate the effects of Japanese traditional medicines, keishibukuryogan and jidabokuippo, on postoperative soft tissue swelling in adult patients undergoing revascularization surgery for ischemic MMD. Methods: Thirty‐two patients treated using direct revascularization surgery alone without Japanese traditional medicine (the first original study) and another 22 patients treated using indirect revascularization surgery alone with perioperative administration of keishibukuryogan and jidabokuippo (the second original study) underwent computed tomography of the head before and 3 days after surgery. The postoperative swelling rate of soft tissues in the head ipsilateral to the surgery was calculated. Results: The postoperative swelling rate of the soft tissues was significantly lower in patients participating in the second original study (88.8 ± 31.6%) than in those participating in the first original study (127.1 ± 31.1%) (p = 0.0002). When postoperative swelling rates of soft tissues in all 54 patients were arranged in descending order and divided into tertiles (186.7–132.0%, 130.0–92.4%, 92.3–44.3%), logistic regression analysis revealed that participation in the second original study was significantly associated with the bottom tertile of postoperative swelling rate of soft tissues (95% confidence interval, 4.90–100.12; p = 0.0098). Conclusion: Combined administration of Japanese traditional medicines, keishibukuryogan and jidabokuippo, can reduce postoperative soft tissue swelling in adult patients undergoing revascularization surgery for ischemic MMD. [ABSTRACT FROM AUTHOR]