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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]

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. 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. 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. 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. [ABSTRACT FROM AUTHOR]

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 15O 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. [ABSTRACT FROM AUTHOR]

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 15O 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 15O 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. [ABSTRACT FROM AUTHOR]

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 15O 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 15O 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. [ABSTRACT FROM AUTHOR]

Reports a case of a patient with 3&beta-hydroxy-&Delta&sup5-C[sub 27]-steroid dehydrogenase/isomerase deficiency. Effect of enzyme deficiency; Characterization of patients with enzyme deficiency; Types of successful treatment that have been previously reported.

Brain 123I-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 123I-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. Twenty-two patients who underwent indirect revascularization surgery alone also underwent brain SPECT scanning at 180 minutes after 123I-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. 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. 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. [ABSTRACT FROM AUTHOR]