Tc99m-ECD brain perfusion SPECT Images in transaxial, sagittal and coronal views showing extensive perfusion defects in the left frontal and parietal lobes

Tc99m-ECD brain perfusion SPECT Images in transaxial, sagittal and coronal views showing extensive perfusion defects in the left frontal and parietal lobes

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Moyamoya disease is a rare, progressive cerebrovascular disorder caused by intracranial stenosis of the circle of Willis, resulting in successive ischemic events. Computed tomography (CT) and magnetic resonance imaging (MRI) play a major role in diagnosis. The aim of the study was to describe the spectrum of findings on brain SPECT in patients with...

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... Despite the diagnostic significance of hemodynamic parameters derived from BOLD and ASL imaging in pediatric MMV, reports on the effects of anesthesia on these parameters during ACZ administration are limited. Some focus has been placed on the effect of different anesthetic agents on CBF (e.g., using propofol versus sevoflurane anesthesia [24]), but ACZ is most often used according to the same protocol as in awake patients [25][26][27]. This is performed under the assumption that mechanisms of CVR are unaffected by the anesthetic agent. ...
... The CVR in the anesthetized patients was lower compared to healthy, awake volunteers, but cannot be directly compared, since both the disease and anesthesia can affect CVR (only the supratentorial regions were compared). Other studies included patients scanned under anesthesia, but did not provide quantifiable measures to compare the CVR of the awake and anesthesia patients [27,37]. Furthermore, there are papers describing CVR in pediatric MMV patients using sedatives instead of anesthesia [26], or the use of anesthesia is not mentioned in the paper. ...
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Citation: Deckers, P.T.; Siero, J.C.W.; Mensink, M.O.; Kronenburg, A.; Braun, K.P.J.; van der Zwan, A.; Bhogal, A.A. Anesthesia Depresses Cerebrovascular Reactivity to Acetazolamide in Pediatric Moyamoya Vasculopathy. J. Clin. Med. 2023, 12, 4393. https://doi. Abstract: Measurements of cerebrovascular reactivity (CVR) are essential for treatment decisions in moyamoya vasculopathy (MMV). Since MMV patients are often young or cognitively impaired, anesthesia is commonly used to limit motion artifacts. Our aim was to investigate the effect of anesthesia on the CVR in pediatric MMV. We compared the CVR with multidelay-ASL and BOLD MRI, using acetazolamide as a vascular stimulus, in all awake and anesthesia pediatric MMV scans at our institution. Since a heterogeneity in disease and treatment influences the CVR, we focused on the (unaffected) cerebellum. Ten awake and nine anesthetized patients were included. The post-acetazolamide CBF and ASL-CVR were significantly lower in anesthesia patients (47.1 ± 15.4 vs. 61.4 ± 12.1, p = 0.04; 12.3 ± 8.4 vs. 23.7 ± 12.2 mL/100 g/min, p = 0.03, respectively). The final BOLD-CVR increase (0.39 ± 0.58 vs. 3.6 ± 1.2% BOLD-change (mean/SD), p < 0.0001), maximum slope of increase (0.0050 ± 0.0040%/s vs. 0.017 ± 0.0059%, p < 0.0001), and time to maximum BOLD-increase (~463 ± 136 and~697 ± 144 s, p = 0.0028) were all significantly lower in the anesthesia group. We conclude that the response to acetazolamide is distinctively different between awake and anesthetized MMV patients, and we hypothesize that these findings can also apply to other diseases and methods of measuring CVR under anesthesia. Considering that treatment decisions heavily depend on CVR status, caution is warranted when assessing CVR under anesthesia.
... [7] Brain perfusion single-photon emission computed tomography (SPECT) is also used to demonstrate preoperative impaired perfusion or postoperative effects of revascularization surgery. [8][9][10][11] MMD is associated with cortical microvascularisation and basal collaterals to compensate for arterial stenosis, which is a specific finding. [12] These compensatory changes in arterial blood flow are expected to cause a corresponding change in the venous structure. ...
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This study analyzed the clinical significance and characteristics of asymmetric venous blood flow in patients with Moyamoya disease (MMD) using minimum intensity projection (minIP) susceptibility-weighted imaging. The minIP views of 30 patients diagnosed with MMD were retrospectively analyzed using clinical features, brain magnetic resonance angiography, electroencephalography, and brain single-photon emission computed tomography (SPECT). Simultaneously, differences between patients with acute cerebral infarction and non-MMD causes were analyzed. Twelve (40.0%) of the 30 patients had asymmetrical venous flow, which is usually seen in patients with acute cerebral infarction (P = .146). They also had significantly higher Suzuki stages than symmetric patients (P = .014), with five (41.7%) and three (25.0%) of them in stages 4 and 5, respectively. When the Suzuki stages of both hemispheres were different, more veins were found in the stenotic hemisphere (88.9%). Brain SPECT showed more severe hypoperfusion on the side with prominent vascularity in the minIP view (100.0%). Additionally, asymmetric blood flow was observed in 66.7% of the patients with cerebral infarction caused by MMD, whereas only 11.1% of the children with cerebral infarction caused by non-MMD had asymmetry (P = .005). Patients with MMD showed asymmetric hypointensity of the cortical veins with a minIP appearance. The venous structure showed greater signal loss on SWI and was more prominent in the hemisphere where stenosis was advanced or infarction occurred in other examinations. Cerebral infarction in patients with MMD tended to occur with asymmetrically prominent venous patterns with damaged areas in minIP images, which had distinct characteristics from those of patients without MMD.
... This case was done using oral ACZ following reports of oral ACZ administration at doses of 1.2 -2 gm [8,9]. ...
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Background: Assessment of cerebrovascular reserve (CVR) by acetazolamide (ACZ) stress brain perfusion SPECT aids to choose appropriately between cerebral revascularization surgery and medical management. Aim of this report was to describe deterministic
... DSA is considered as the procedure of choice for confirming the diagnosis of MMD,[8] and conventional angiography is considered as the official criteria for diagnosis of MMD by the Japanese Ministry of Health and Welfare.[11] Brain perfusion scintigraphy is an important adjunct in evaluation of patients with MMD.[12] Kashyap et al.,[12] in their study on 17 patients of MMD found that Tc99m-ethylcysteinate dimer (ECD) brain single-photon emission computed tomography (SPECT) showed perfusion defects that were more extensive compared to those detected on MR. Initially it was believed that MMD was restricted to Japan, but later cases were reported from all over the world especially from China and Korea.[5] ...
... DSA is considered as the procedure of choice for confirming the diagnosis of MMD,[8] and conventional angiography is considered as the official criteria for diagnosis of MMD by the Japanese Ministry of Health and Welfare.[11] Brain perfusion scintigraphy is an important adjunct in evaluation of patients with MMD.[12] Kashyap et al.,[12] in their study on 17 patients of MMD found that Tc99m-ethylcysteinate dimer (ECD) brain single-photon emission computed tomography (SPECT) showed perfusion defects that were more extensive compared to those detected on MR. Initially it was believed that MMD was restricted to Japan, but later cases were reported from all over the world especially from China and Korea.[5] ...
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Aim: Moyamoya disease (MMD) is a slowly progressive bilateral stenocclusive process of the distal internal carotid and proximal portions of the anterior and middle cerebral arteries and the formation of an abnormal vascular network at the base of the brain. The purpose of this retrospective study was to identify clinical features, salient features, radiological features and yield of diagnostic cerebral angiography in MMD. Materials and Methods: We analyzed the records of 26 patients with MMD evaluated and treated at our institute from August 2010 until March 2013. Diagnosis of MMD was made on the basis of features of angiographic findings. Cerebral angiography showed typically fine network of vessels at the base of the brain with puff of smoke appearance suggestive of MMD. CT angiography (CTA) was done in 25 (96.15%) patients where as Digital substraction angiography (DSA) was done in 18 (69.23%) patients. Results: Out of the 26 patients 13 were in the pediatric age group and 13 were adults. At presentation 14 patients had infarcts and 10 patients had hemorrhages. Among the hemorrhagic group 20% had isolated intracerebral hemorrhage (ICH), 50% patients had ICH with intraventricular extension (IVE) and 30% patients had primary intraventricular hemorrhage (PIVH). 50 % of the patients had involvement of the posterior circulation. Conclusion: Posterior circulation involvement is frequent in MMD. Though parenchymal bleed with/without intraventricular extension is the usual presentation of hemorrhagic MMD, isolated intraventricular hemorrhage could also be the mode of presentation.
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Genetic stroke syndromes represent a small portion of stroke patients usually affecting younger patients. Recognition of systemic manifestations of genetic disorders with stroke as a phenotype, and low clinical suspicion especially in young patients with stroke, would provide important clues in establishing an accurate diagnosis. Along with clinical presentation, neuroimaging characteristics suggestive of genetic stroke syndromes provide a useful tool in establishing the diagnosis. Here we discuss neuroimaging findings of these genetic disorders.