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A 72-year-old man presented with chorea while undergoing treatment for recurrence of nodal peripheral T-cell lymphoma with T follicular helper (TFH) phenotype. An examination by brain N-isopropyl-p-iodoamphetamine (I-123-IMP)-single photon emission computed tomography (SPECT) revealed no abnormalities other than a decreased cerebral blood flow (CBF) in the left striatum. After four courses of salvage chemotherapy, his clinical symptoms and asymmetric cerebral perfusion improved, suggesting that the decreased CBF had caused chorea. The significance of brain SPECT has not been fully clarified in patients with chorea-associated malignant lymphoma, warranting further investigations. Brain SPECT is an alternative approach to identify abnormalities in such patients.

Deep convolutional generative adversarial networks (GAN) allow for creating images from existing databases. We applied a modified light-weight GAN (FastGAN) algorithm to cerebral blood flow SPECTs and aimed to evaluate whether this technology can generate created images close to real patients. Investigating three anatomical levels (cerebellum, CER; basal ganglia, BG; cortex, COR), 551 normal (248 CER, 174 BG, 129 COR) and 387 pathological brain SPECTs using N-isopropyl p-I-123-iodoamphetamine (123I-IMP) were included. For the latter scans, cerebral ischemic disease comprised 291 uni- (66 CER, 116 BG, 109 COR) and 96 bilateral defect patterns (44 BG, 52 COR). Our model was trained using a three-compartment anatomical input (dataset ‘A’; including CER, BG, and COR), while for dataset ‘B’, only one anatomical region (COR) was included. Quantitative analyses provided mean counts (MC) and left/right (LR) hemisphere ratios, which were then compared to quantification from real images. For MC, ‘B’ was significantly different for normal and bilateral defect patterns (P P = 0.77). Comparable results were recorded for LR, as normal and ischemia scans were significantly different relative to images acquired from real patients (P ≤ 0.01, respectively). Images provided by ‘A’, however, revealed comparable quantitative results when compared to real images, including normal (P = 0.8) and pathological scans (unilateral, P = 0.99; bilateral, P = 0.68) for MC. For LR, only uni- (P = 0.03), but not normal or bilateral defect scans (P ≥ 0.08) reached significance relative to images of real patients. With a minimum of only three anatomical compartments serving as stimuli, created cerebral SPECTs are indistinguishable to images from real patients. The applied FastGAN algorithm may allow to provide sufficient scan numbers in various clinical scenarios, e.g., for “data-hungry” deep learning technologies or in the context of orphan diseases.

ObjectiveTo clinically diagnose MM2-cortical (MM2C) and MM2-thalamic (MM2T)-type sporadic Creutzfeldt-Jakob disease (sCJD) at early stage with high sensitivity and specificity.MethodsWe reviewed the results of Creutzfeldt-Jakob disease Surveillance Study in Japan between April 1999 and September 2019, which included 254 patients with pathologically confirmed prion diseases, including 9 with MM2C-type sCJD (MM2C-sCJD) and 10 with MM2T-type sCJD (MM2T-sCJD), and 607 with non-prion diseases.ResultsAccording to the conventional criteria of sCJD, 4 of 9 patients with MM2C- and 7 of 10 patients with MM2T-sCJD could not be diagnosed with probable sCJD until their death. Compared with other types of sCJD, patients with MM2C-sCJD showed slower progression of the disease and cortical distribution of hyperintensity lesions on diffusion-weighted images of brain MRI. Patients with MM2T-sCJD also showed relatively slow progression and negative results for most of currently established investigations for diagnosis of sCJD. To clinically diagnose MM2C-sCJD, we propose the new criteria; diagnostic sensitivity and specificity to distinguish ‘probable’ MM2C-sCJD from other subtypes of sCJD, genetic or acquired prion diseases and non-prion disease controls were 77.8% and 98.5%, respectively. As for MM2T-sCJD, clinical and laboratory features are not characterised enough to develop its diagnostic criteria.ConclusionsMM2C-sCJD can be diagnosed at earlier stage using the new criteria with high sensitivity and specificity, although it is still difficult to diagnose MM2T-sCJD clinically.

Objective The γ-Ray Evaluation with iodoamphetamine for Cerebral Blood Flow Assessment (REICA) is a new method for quantifying cerebral blood flow (CBF) using single-photon emission computed tomography (SPECT) and [123I]N-isopropyl-p-iodoamphetamine (123I-IMP). The present study aimed to validate the REICA method using data including acetazolamide challenge test. Methods The REICA and Graph-Plot (GP) methods were used to calculate mean CBF (mCBF) for 92 acquisitions (rest: 57, stress: 35) and cerebrovascular reactivity (CVR) in 33 patients. To obtain stress data, 15 mg/kg of acetazolamide was injected intravenously 10 min before the administration of 123I-IMP, and blood samples were collected under the same conditions as rest data. The reference standard was the Autoradiograph (ARG) method using arterial blood sampling, and the accuracy of the REICA method was analyzed by comparing it with each method. Results For mCBF, the correlation coefficients (r) were 0.792 for the REICA method and 0.636 for the GP method. For CVR, r values were 0.660 for the REICA method and 0.578 for the GP method. In both acquisitions, the REICA method had a stronger correlation with the ARG method than the GP method. For mCBF, there was a significant difference in the correlation coefficient between the two correlation coefficients (p Conclusions The REICA method was more accurate than the GP method in quantifying CBF and closer to the ARG method. The REICA method, which is a noninvasive method of cerebral blood flow quantification using 123I-IMP, has great medical usefulness.

Using N-isopropyl-p-[123I]-iodoamphetamine(123I-IMP) and single-photon emission computed tomography (SPECT), the relationship between cerebrovascular reserve and the 123I-IMP redistribution phenomenon was investigated. The 50 patients who matched the inclusion criteria were divided into control and ischemia groups, and the redistribution phenomenon was examined on resting images. The delayed images showed higher 123I-IMP accumulation in lesions in the middle cerebral artery(MCA) area and anterior cerebral artery(ACA) area, these watershed areas in the ischemia group than in the control group, confirming that the redistribution phenomenon exists with statistical significance (Wilcoxon test; control group vs ischemic group in the ACA area[P = .002], ACA-MCA watershed area(P = .014), MCA area(P = .025), and MCA-posterior cerebral artery(PCA) watershed area(P = .002). The patients were then divided into 4 types according to the Kuroda grading system, and the difference in the redistribution phenomenon was investigated between type III and the other 3 types. Compared with type I and type II, type III had a significantly lower rate of decrease in the radioisotope (RI) count, verifying the redistribution phenomenon (Student t test: type I vs type III in the ACA area(P = .008), ACA-MCA watershed area(P = .009), MCA area(P

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

We describe the case of a 61-year-old woman diagnosed with Borreliosis at the age of 57. Subsequently, the patient developed depression, anxiety, and behavioral disturbances. A lumbar puncture excluded the condition of Neuroborreliosis. The diagnostic workup included: an MRI scan, a 18F-FDG PET, a 123I-ioflupane-SPECT, an amyloid-β PET, a specific genetic analysis, and a neuropsychological evaluation. Based on our investigation, the patient was diagnosed with probable behavioral-frontotemporal dementia (bvFTD), whereas in the previous years, the patient had been considered firstly as a case of Post-Treatment-Lyme Disease and, secondly, a psychiatric patient. We believe that, in the present case, such initial symptoms of Borrelia infection may have superimposed on those of bvFTD rather than playing as a contributory cause.

OBJECTIVE Transient neurological symptoms are frequently observed during the early postoperative period after direct bypass surgery for moyamoya disease. Abnormal signal changes in the cerebral cortex can be seen in postoperative MR images. The purpose of this study was to reveal the radiological features of the “cortical hyperintensity belt (CHB) sign” in postoperative FLAIR images and to verify its relationship to transient neurological events (TNEs) and regional cerebral blood flow (rCBF). METHODS A total of 141 hemispheres in 107 consecutive patients with moyamoya disease who had undergone direct bypass surgery were analyzed. In all cases, FLAIR images were obtained during postoperative days (PODs) 1–3 and during the chronic period (3.2 ± 1.13 months after surgery). The CHB sign was defined as an intraparenchymal high-intensity signal within the cortex of the surgically treated hemisphere with no infarction or hemorrhage present. The territory of the middle cerebral artery was divided into anterior and posterior parts, with the extent of the CHB sign in each part scored as 0 for none; 1 for presence in less than half of the part; and 2 for presence in more than half of the part. The sum of these scores provided the CHB score (0–4). TNEs were defined as reversible neurological deficits detected both objectively and subjectively. The rCBF was measured with SPECT using N-isopropyl-p-[123I]iodoamphetamine before surgery and during PODs 1–3. The rCBF increase ratio was calculated by comparing the pre- and postoperative count activity. RESULTS Cortical hyperintensity belt signs were detected in 112 cases (79.4%) and all disappeared during the chronic period. Although all bypass grafts were anastomosed to the anterior part of the middle cerebral artery territory, CHB signs were much more pronounced in the posterior part (p < 0.0001). TNEs were observed in 86 cases (61.0%). Patients with TNEs showed significantly higher CHB scores than those without (2.31 ± 0.13 vs 1.24 ± 0.16, p < 0.0001). The CHB score, on the other hand, showed no relationship with the rCBF increase ratio (p = 0.775). In addition, the rCBF increase ratio did not differ between those patients with TNEs and those without (1.15 ± 0.033 vs 1.16 ± 0.037, p = 0.978). CONCLUSIONS The findings strongly suggest that the presence of the CHB sign during PODs 1–3 can be a predictor of TNEs after bypass surgery for moyamoya disease. On the other hand, presence of this sign appears to have no direct relationship with the postoperative local hyperperfusion phenomenon. Vasogenic edema can be hypothesized as the pathophysiology of the CHB sign, because the sign was transient and never accompanied by infarction in the present series.

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.

We aimed to investigate whether the frontal lobe bottom and cerebellum tuber vermis (FLB-CTV) line on brain perfusion scintigraphy, using iodine-123 isopropyl iodoamphetamine single photon emission computed tomography (I-123 IMP SPECT) images, is useful to determine an axial angle parallel to the anterior commissure-posterior commissure (AC-PC) line. We measured the angular differences between the AC-PC line and the FLB-CTV line on midsagittal brain magnetic resonance imaging (MRI) scans of 100 patients. We also evaluated the angular differences of the FLB-CTV line between the IMP SPECT images and the computed tomography for attenuation correction (CTAC) images in the same 100 patients, using a reference line on the CTAC images. Furthermore, the inter-reader reproducibility of the FLB-CTV line measurements on IMP SPECT images of 50 patients between two readers was evaluated using the intra-class correlation coefficient (ICC) and 95% confidence interval (CI). The mean and standard deviation of the angular differences between the AC-PC and FLB-CTV lines on midsagittal brain MRI scans were - 1.24° and 1.14°, respectively. The mean and the standard deviation of the angular differences of the FLB-CTV line in the IMP SPECT and CTAC images were 0.87° and 0.48°, respectively. The ICC of the FLB-CTV line measurements on IMP SPECT images was 0.99 (95% CI 0.98-0.99). We demonstrated that the FLB-CTV line was almost parallel to the AC-PC line and could be reconstructed using IMP SPECT images. The FLB-CTV line can be used as additional evidence to set the axial angle parallel to the AC-PC line.

Patients with Alzheimer's disease (AD) experience a wide array of cognitive deficits, which typically include the impairment of explicit memory. In previous studies, the authors reported that a flavonoid, quercetin, reduces the expression of ATF4 and delays memory deterioration in an early-stage AD mouse model. In the present study, the effects of long-term quercetin intake on memory recall were assessed using contextual fear conditioning in aged wild-type mice. In addition, the present study examined whether memory recall was affected by the intake of quercetin-rich onion (a new cultivar of hybrid onion 'Quergold') powder in early-stage AD patients. In-vivo analysis indicated that memory recall was enhanced in aged mice fed a quercetin-containing diet. Memory recall in early-stage AD patients, determined using the Revised Hasegawa Dementia Scale, was significantly improved by the intake of quercetin-rich onion (Quergold) powder for 4 weeks compared with the intake of control onion ('Mashiro' white onion) powder. These results indicate that quercetin might influence memory recall.

Both (18)F-fluorodeoxyglucose (FDG) positron emission tomography (PET) and (123)I-iodoamphetamine (IMP) single-photon emission computed tomography (SPECT) have been used for the differential diagnosis of Alzheimer's disease (AD) and dementia with Lewy bodies (DLB). Less information is available, however, regarding the differential diagnosis of mild cognitive impairment (MCI) due to AD and MCI due to DLB. We examined nine AD patients (AD group), nine DLB patients (DLB group), eight MCI due to AD patients (MCI-AD group), and nine MCI due to DLB patients (MCI-DLB group) with FDG PET and IMP SPECT using a well-characterized normal database and a stereotactic extraction estimation method. In the AD and DLB groups, receiver operating characteristic (ROC) analysis in the occipital regions showed significant accuracy of both FDG PET and IMP SPECT for the differential diagnosis. In the MCI-AD and MCI-DLB groups, ROC analysis showed significant accuracy of only FDG PET for the differential diagnosis. Both FDG PET and IMP SPECT would be useful for the differential diagnosis between AD and DLB. For the differential diagnosis of MCI-AD versus MCI-DLB, FDG PET would be more useful than IMP SPECT.

In clinical practice, measurement of the rCBF has mainly been conducted by I-123-N-isopropyl-p-iodoamphetamine (123I-IMP) SPECT using the microsphere (MS) method, with continuous arterial blood sampling. While several non-invasive 123I-IMP quantification methods have been developed, their accuracy has been shown to be lower than that of the MS method. Therefore, a non-invasive quantification method for use in routine clinical practice is being sought. The purpose of this study was to develop a simple non-invasive 123I-IMP quantification method (SIMS method) with a simple input function-determining protocol based on the MS method. The input function for the SIMS method was determined using the administered dose and the integrated lung washout ratio obtained by analyzing the count–time activity curve of the pulmonary artery and lung on dynamic chest images. The mean CBF (mCBF) and input function measured in 80 patients by the SIMS method was compared with those determined using the MS method. A good correlation was observed between the counts measured by continuous arterial blood sampling in the MS method and the estimated counts by image analysis in the new method (r = 0.94, p

In pediatric cases requiring quantification of cerebral blood flow (CBF) using123I-N-isopropyl-p-iodoamphetamine (123I-IMP) single-photon emission computed tomography (SPECT), arterial blood sampling is sometimes impossible due to issues such as movement, crying, or body motion. If arterial blood sampling fails, quantitative diagnostic assessment becomes impossible despite radiation exposure. We devised a new easy non-invasive microsphere (e-NIMS) method using whole-body scan data. This method can be used in conjunction with autoradiography (ARG) and can provide supportive data for invasive CBF quantification. In this study, we examined the usefulness of e-NIMS for pediatric cerebral perfusion semi-quantitative SPECT and compared it with the invasive ARG. The e-NIMS estimates cardiac output (CO) using whole-body acquisition data after123I-IMP injection and the body surface area from calculation formula. A whole-body scan was performed 5 minutes after the123I-IMP injection and CO was estimated by region of interest (ROI) counts measured for the whole body, lungs, and brain using the whole-body anterior image. The mean CBF (mCBF) was compared with that acquired via ARG in 115 pediatric patients with suspected cerebrovascular disorders (age 0–15 years). Although the mCBF estimated by the e-NIMS indicated a slight deviation in the extremely low- or high-mCBF cases when compared with the values acquired using the invasive ARG, there was a good correlation between the two methods (r = 0.799;p< 0.001). There were no significant differences in the mCBF values based on physical features, such as patients’ height, weight, and age. Our findings suggest that123I-IMP brain perfusion SPECT with e-NIMS is the simplest semi-quantitative method that can provide supportive data for invasive CBF quantification. This method may be useful, especially in pediatric brain perfusion SPECT, when blood sampling or identifying pulmonary arteries for CO estimation using the graph plot method is difficult.

Cerebral vascular reserve (CVR) is an important indicator for the management of and therapy for cerebral arterial occlusive disease (CAOD). Vasodilatory function is measured using the standard IMP-ARG method. The IMP autoradiography (IMP-ARG) method employed here uses a standardized input function, which was derived from 12 patients between 31 and 71 years of age. Because the population of elderly patients continues to increase in Japan, additional therapies are required to assess CVR in elderly patients with chronic cardiopulmonary disease or a history of smoking, in particular. Despite its popularity, alternatives to the IMP-ARG method are necessary. Here, we proposed the microsphere (MS) method without an input function. Using this method and the IMP-ARG method, we measured the CVRs of 18 CAOD patients. The CVRs derived with these two methods were significantly and linearly correlated (r = 0.89, p

Anterior choroidal artery (AchA) infarction remains a challenging diagnosis although it was first described almost 100 years prior. N-isopropyl-p-[

Enlarged perivascular spaces (EPVS) are often observed in small vessel disease on T2-weighted images. However, their role in ischemic conditions caused by cerebral large vessel disease remains unclear. We evaluated EPVS in patients with hemodynamic compromise associated with atherosclerotic large vessel disease and aimed to identify the pathophysiology of EPVS.We examined 28 adults with atherosclerotic large vessel disease. EPVS numbers in the basal ganglia and the centrum semiovale were assessed. For each affected hemisphere, the total numbers of EPVS were compared with those on the unaffected side. EPVS in the impaired hemodynamics group were compared with those in the unimpaired hemodynamics group. Moreover, EPVS were compared in the presence/absence of large stroke.The number of EPVS was significantly increased on the affected side in the centrum semiovale (p = 0.023), particularly in the impaired hemodynamics group (p = 0.006). Moreover, in the small stroke subgroup of the impaired hemodynamics group, the number of EPVS was significantly increased on the affected side (p = 0.002), although this number was insignificant in the large ischemic stroke subgroup.The number of EPVS was increased in patients with atherosclerotic large vessel disease with hemodynamic compromise and decreased in the presence of a large stroke. EPVS might act as fluid absorbers in a hemodynamically compromised state until the occurrence of an ischemic stroke.

With the advancement of reperfusion therapy in stroke patients, assessment of perfusion status after therapy is gaining importance. Hyperperfusion tends to be underestimated by the conventional early imaging ofSixty-seven patients who underwentThe frequency of postischemic hyperperfusion was significantly higher using both the super-early and early images (28/67 patients) than using the early images alone (17/67 patients, p 0.001). In 56 patients in whom judgement regarding the presence or absence of postischemic hyperperfusion was unchanged, the confidence level was increased in 8 patients using both image sets. The addition of the super-early SPECT images was judged to be useful and marginally useful in 14 and 15 patients, respectively. The contrast ratio was significantly higher on the super-early images (1.48 ± 0.25) than on the early images (1.26 ± 0.18, p 0.001).The addition of super-early imaging to the conventional early imaging aids assessment of postischemic hyperperfusion by

Introduction Glucocerebrosidase gene (GBA) variants are associated with Parkinson's disease (PD) and dementia with Lewy bodies (DLB). The molecular mechanisms underlying these diseases with GBA variants, however, are not well understood. In order to determine the effect of a deletion mutation in GBA, we performed a neuroimaging, genetic, and enzymatic study in a Japanese family with a gross deletion of exons 3 to 11 in GBA. Methods We performed [123I] FP-CIT SPECT and [123I] N-isopropyl-p-iodoamphetamine SPECT (IMP-SPECT), and determined GBA expression and glucocerebrosidase (GCase) activity in leukocytes in two GBA-associated PD patients and nine unaffected individuals (including four mutation carriers) in a Japanese family with a heterozygous gross deletion mutation in the GBA gene. Results The two PD patients and two of the four clinically unaffected carriers showed decreased [123I] FP-CIT uptake. IMP-SPECT showed a pattern like that in DLB in one patient. When we compared PD patients with GBA mutations with clinically unaffected carriers, there was a poor correlation between the development of PD and the expression level of GBA or GCase activity. Conclusion We confirmed the gross deletion mutation in the GBA gene, which appeared to be associated with the PD or reduced [123I] FP-CIT in this family. However, since we cannot conclude whether a reduction of GCase activity is directly correlated with the pathogenesis of PD or not, longitudinal follow-up of this family is needed.

Background Cerebral hyperperfusion (CHP) syndrome is a potential complication of superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis for moyamoya disease (MMD), but its biphasic and delayed development is extremely rare. Case report A 47-year-old woman with autosomal dominant kidney disease (ADPKD) presented with transient ischemic attacks due to MMD, and underwent left STA-MCA anastomosis. N-isopropyl-p-[123I] iodoamphetamine single-photon emission computed tomography (123IMP-SPECT) 1 day after surgery revealed asymptomatic CHP at the site of anastomosis. Strict blood pressure control and minocycline hydrochloride relieved CHP at postoperative day 7. However, 2 days later, the patient complained of sensory aphasia, and 123IMP-SPECT demonstrated significant focal CHP at the site of anastomosis accompanying high-intensity signal on magnetic resonance (MR) imaging of fluid attenuated inversion recovery (FLAIR) in her left temporal lobe near the site of anastomosis. We continued strict blood pressure control and additionally administered free radical scavenger (Edaravone) and antiepileptic agents, which gradually improved sensory aphasia. MR imaging and 123IMP-SPECT also confirmed the amelioration of the FLAIR-high lesion and focal CHP in her left temporal lobe. Two months later, the patient underwent right STA-MCA anastomosis without complications. Conclusions Although the underlying mechanism is unknown, biphasic development of focal CHP after revascularization surgery in an MMD patient with ADPKD is unique. Due to the potential vulnerability of the systemic vessels in ADPKD, it is conceivable that intrinsic vascular wall fragility in MMD could be enhanced by ADPKD and have partly led to this rare complication.

Background: Falls and fractures due to impaired balance in patients with Alzheimer’s disease (AD) have an adverse effect on the clinical course of the disease. Objective: To evaluate balance impairment in AD from the viewpoint of vestibular functional impairment. Methods: The subjects were 12 patients with AD, 12 dementia-free elderly adults, and 12 younger adults. Vestibular function was assessed using a stepping test, caloric nystagmus, and a visual suppression (VS) test. Results: The stepping test was abnormal in 9 of the 12 patients in the AD group. An abnormal stepping test was not associated with self-reported dizziness or tendency to fall. Significant VS abnormalities were present in the AD group. The suppression rate of VS was lower in AD patients with either a tendency to fall or constructional apraxia than in AD patients without either. The velocity of the rapid phase of caloric nystagmus before the VS test was similar in the AD group and the elderly control group. Significant abnormalities of both caloric nystagmus and VS were not present in either the elderly or the younger control groups. Conclusion: AD could involve impairments in the vestibular control of balance. The VS test is useful for assessing the tendency to fall in AD. Impairment of VS in AD might arise from cerebral vestibular cortex impairment rather than comorbid peripheral vestibular disorders.

To quantitatively evaluate regional cerebral blood flow (rCBF) and regional developmental changes during childhood usingWe retrospectively analyzed quantitative values of rCBF in 75 children (29 girls) aged between 16 days and 178 months (median: 12 months), whose brain images, including magnetic resonance imaging and SPECT data, were normal under visual inspection at Saitama Children's Medical Center between 2005 and 2015. The subjects had normal psychomotor development, no focal neurological abnormalities, and neither respiratory nor cardiac disease at the time of examination. Regions of interest were placed automatically using a three-dimensional stereotactic template.rCBF was lowest in neonates, who had greater rCBF in the lenticular nucleus, thalamus, and cerebellum than the cerebral cortices. rCBF increased rapidly during the first year of life, reaching approximately twice the adult levels at 8 years, and then fell to approximately adult levels in the late teenage years. Cerebral cortex rCBF sequentially increased in the posterior, central, parietal, temporal, and callosomarginal regions during infancy and childhood.rCBF changed dramatically throughout childhood and ranged from lower than adult values to approximately two times higher than adult values. It had different trajectories in each region during brain development. Understanding this dynamic developmental change is necessary for SPECT image evaluation in children.

Objective The multicenter prospective cohort study (Japan Cooperative SPECT Study on Assessment of Mild Impairment of Cognitive Function: J-COSMIC) aimed to examine the value of 123I-N-isopropyl-4-iodoamphetamine cerebral blood flow (IMP-CBF) SPECT in regards to early diagnosis of Alzheimer’s disease (AD) in patients with mild cognitive impairment (MCI). Methods Three hundred and nineteen patients with amnestic MCI at 41 participating institutions each underwent clinical and neuropsychological examinations and 123I-IMP-CBF SPECT at baseline. Subjects were followed up periodically for 3 years, and progression to dementia was evaluated. SPECT images were classified as AD/DLB (dementia with Lewy bodies) pattern and non-AD/DLB pattern by central image interpretation and automated region of interest (ROI) analysis, respectively. Logistic regression analyses were used to assess whether baseline 123I-IMP-CBF SPECT was predictive of longitudinal clinical outcome. Results Ninety-nine of 216 amnestic MCI patients (excluding 3 cases with epilepsy (n = 2) or hydrocephalus (n = 1) and 100 cases with incomplete follow-up) converted to AD within the observation period. Central image interpretation and automated ROI analysis predicted conversion to AD with 56 and 58 % overall diagnostic accuracy (sensitivity, 76 and 81 %; specificity, 39 and 37 %), respectively. Multivariate logistic regression analysis identified SPECT as a predictor, which distinguished AD converters from non-converters. The odds ratio for a positive SPECT to predict conversion to AD with automated ROI analysis was 2.5 and combining SPECT data with gender and mini-mental state examination (MMSE) further improved classification (joint odds ratio 20.08). Conclusions 123I-IMP-CBF SPECT with both automated ROI analysis and central image interpretation was sensitive but relatively nonspecific for prediction of clinical outcome during the 3-year follow-up in individual amnestic MCI patients. A combination of statistically significant predictors, both SPECT with automated ROI analysis and neuropsychological evaluation, may increase predictive utility.

Background: Prader–Willi syndrome (PWS) is a genetically determined neurodevelopmental disorder and is generally regarded as a genetic model of obesity. Individuals with PWS exhibit behavioral symptoms including temper tantrums, rigid thinking, and compulsive behavior. The most striking feature of PWS is abnormal eating behavior, including hyperphagia, intense preoccupation with food, and incessant food seeking. To explore brain regions associated with the behavioral symptoms of PWS, we investigated differences in resting-state regional cerebral blood flow (rCBF) between individuals with PWS and healthy controls. Correlation analyses were also performed to examine the relationship between rCBF and altered eating behavior in PWS individuals. Methods: Twelve adults with PWS and 13 age- and gender-matched controls underwent resting-state single photon emission computerized tomography (SPECT) with N-isopropyl-p-[ 123 I] iodoamphetamine (IMP). The rCBF data were analyzed on a voxel-by-voxel basis using SPM5 software. Results: The results demonstrated that compared with controls, individuals with PWS had significantly lower rCBF in the right thalamus, left insular cortex, bilateral lingual gyrus, and bilateral cerebellum. They had significantly higher rCBF in the right inferior frontal gyrus, left middle/inferior frontal gyrus (anterior and posterior clusters), and bilateral angular gyrus. Additionally, rCBF in the left insula, which was significantly lower in PWS individuals, was negatively correlated with the eating behavior severity score. Conclusions: These results suggest that specific brain regions, particularly the left insula, may be partly responsible for the behavioral symptoms in PWS.

Hemiparkinsonism secondary to a vascular mesencephalic lesion is infrequent; these patients offer an exceptional opportunity to study neuropsychological alterations attributable to unilateral dopaminergic denervation, shedding light on the pathophysiology of cognitive disorders in early-stage idiopathic Parkinson's disease (PD). From the investigation of our case, we conclude that destruction of the right nigrostriatal pathway is accompanied by deficits in executive functioning and verbal/visual memory similar to those observed in many patients with early-stage idiopathic PD. The more complex neuropsychological dysfunction developed by other PD patients must therefore be related to the additional involvement of other brain structures. (JINS, 2013, 19, 1–6)

We report a 74-year-old female patient with Parkinson disease (PD). Around 2010, she developed depression and bradykinesia and was diagnosed as PD. In July 2014, she came to our hospital, of which she lived in the neighborhood. In the last part of December 2014, she felt uneasy about her fecal smell and saw a psychiatrist in the first part of January 2015. Quetiapine (25 mg/day) was added. In the last part of January, she complained of fecal smell everywhere and could not take a meal. No-one else could detect the smell. A diagnosis of olfactory hallucination was made. The next day after increasing to 75mg/day, however, she was admitted to our hospital because of refusing to take medicine. After introducing donepezil, olfactory hallucination subsided and her appetite was improved. Brain MRI showed atrophy of the bilateral temporal lobes and N-isopropyl-p-(iodine-123)-iodoamphetamine single photon emission computed tomography ((123)I-IMP-SPECT) revealed hypoperfusion in the bilateral mesial temporal lobes. We suppose that cholinergic denervation in the mesial temporal lobes is an important determinant of her olfactory hallucination.

Since the clinical symptoms of different types of dementia frequently overlap, especially in the earlier stages at onset, it is difficult to distinguish dementia with Lewy bodies (DLB) from other neurodegenerative dementias based on their clinical manifestations alone. Nuclear medicine imaging has been reported as a high-value index for the objective evaluation and diagnosis of DLB. The aim of this study was to evaluate whether nuclear medicine imaging findings may yield core features to be added to the diagnosis of DLB.We enrolled 332 patients with suspected DLB. All were evaluated by bothUnivariate and multivariate analyses of fluctuating cognition, visual hallucinations, Parkinsonism and early H/M ratio in patients with probable and without DLB revealed significant differences. Parameters based onThe early H/M ratio obtained by

SPECT images of the brain can be obtained using either 123I labelled amines or 99mTc-hexamethylpropyleneamineoxime (HM-PAO). Both materials produce images which are blood flow dominated and so appear similar in normal subjects, although the respective mechanisms of uptake are not yet finally established. It seems likely, however, that the different mechanisms of uptake are responsible for recent reports of some differences seen in images obtained with the two types of agent in patients with cerebral pathology, mainly cerebrovascular disease. In this study, 12 demented patients, 6 with dementia of the Alzheimer type (DAT) and 6 with multi infarct dementia (MID), were imaged with 123I-isopropylamphetamine (IMP) and 99mTc-HM-PAO and the images compared. Significantly more lesions were seen with IMP than HM-PAO (P less than 0.02); out of a possible 120 sites, 41 lesions were seen with IMP compared to 28 with HM-PAO, 23 being seen with both agents. However, it is concluded that either agent can be used for the differential diagnosis of dementia, a task for which the new cerebral blood flow agents seem well suited.

The acetazolamide challenge test in conjunction withTwenty consecutive patients with unilateral internal carotid artery or middle cerebral artery steno-occlusive disease were enrolled.The asymmetry ratio of MTT as measured by CT perfusion showed a strong correlation with the CVR to acetazolamide as measured byMTT as measured by CT perfusion in combination with CBF as measured by resting state

Objective We examined regional benzodiazepine receptors (rBZR) using single photon emission CT (SPECT) in patients with Alzheimer disease (AD), vascular dementia (VaD), and mixed AD/VaD dementia (MD) and compared the changes in the availability of rBZR with those of regional cerebral blood flow (rCBF). Methods A total of 7 patients with AD, 6 with MD, and 9 with VaD underwent SPECT studies with N-isopropyl-p-[ 123 I] iodoamphetamine and 123 I-iomazenil to measure rCBF and rBZR. The ratios of rCBF and rBZR uptake in brain subregions to the average global activity were compared among these diseases. In addition, we acquired z-score maps using 3-dimensional stereotactic surface projections of SPECT data. Results Compared with AD, VaD and MD showed rCBF and rBZR reduction predominantly in the frontal lobe, but rBZR images revealed more extensive and severe defects than rCBF images. In contrast, AD showed rCBF and rBZR reduction predominantly in the parietotemporal lobe compared with VaD and MD, but rCBF images revealed more extensive defects than rBZR images. Conclusion rCBF imaging can detect parietotemporal abnormalities in AD, while rBZR imaging may enable the demonstration of underlying pathophysiological differences in the frontal lobe between VaD, MD and AD, reflecting neuronal integrity in the cerebral cortex.

In this paper, data available on nuclear medicine imaging using commercially available radiopharmaceuticals for the differentiation, staging, and prediction or assessment of the response to treatment in tuberculosis (TB) are reviewed. Limited available studies suggest that single photon emission computed tomography (SPECT) using either 201Tl, 99mTc-sestamibi, or 99mTc-tetrofosmin is accurate (≥85%) and has a high negative predictive value (≥90%) for the differentiation of TB from carcinoma in patients presenting with a solitary pulmonary nodule (SPN). The criteria for detection of TB on 201Tl SPECT are nondepiction of the suspicious lesion in the delayed image or a negative retention index [washout on the delayed images (3–4 h postinjection) vs. the early image (5–15 min postinjection)] and a comparable-to-background uptake on 99mTc-sestamibi or 99mTc-tetrofosmin SPECT. Another SPECT tracer of potential interest for the differentiation of TB from malignant SPN that warrants further exploration, is N-isopropyl-p-[123I]iodoamphetamine (123I-IMP). In contrast, 18F-fluorodeoxyglucose (18F-FDG) PET is unable to differentiate malignancy from TB and thus cannot be used as a tool to reduce futile biopsy/thoracotomy in these patients. A limited number of studies have reported on the potential of nuclear medicine imaging in assessment of the extent of disease in patients with extrapulmonary TB using 67Ga-citrate SPECT and 18F-FDG PET, respectively. 67Ga-citrate SPECT was shown to be as sensitive as bone scintigraphy for the detection of bone infection and was found to be complementary to computed tomography (CT) imaging. 18F-FDG PET was found to be significantly more efficient when compared with CT, respectively, in over half of patients for the identification of sites of lymph node involvement that were missed by CT and often the only sites of extrapulmonary TB identified. Unfortunately, 18F-FDG PET findings did not lead to alterations in treatment planning in any of the patients under study. Additional studies confirming these findings are urgently required. Similar to the setting of SPN, 18F-FDG PET cannot differentiate malignant lymph node involvement from lymph node involvement by TB. These results and the recent findings of Demura and colleagues using 18F-FDG PET further suggest that nuclear medicine imaging techniques could be used for the evaluation of therapeutic response. Prospective studies, focusing on specific subgroups of patients in whom such an imaging approach might be clinically relevant, for example in multidrug-resistant TB patients, are warranted. In acquired immunodeficiency syndrome patients, 67Ga scintigraphy proved to be a reliable and sensitive method for the primary detection and follow-up of opportunistic pneumonias, including TB. Combining 201Tl scintigraphy with 67Ga scintigraphy was shown to increase the specificity for both pulmonary and extrapulmonary TB, which is a 67Ga(+) and 201Tl(-) mismatch pattern in acquired immunodeficiency syndrome patients that is specific for mycobacterial infections. Finally, the results obtained using both SPECT and PET indicate that nuclear medicine could be an important noninvasive method for the determination of disease activity, detection of extrapulmonary TB, and determination of response to therapy.

PURPOSE The present study aims to quantitatively investigate a normal database (NDB) created under the same acquisition and reconstruction conditions for three gamma camera systems (four types of collimator systems) with use of three-dimensional stereotactic surface projections (3D-SSP). We rebuilt a NDB with use of the N-isopropyl-p-(123)I-iodoamphetamine ((123)I-IMP) SPECT data derived from 30 healthy individuals at 20 institutions nationwide. We standardized the acquisition and reconstruction conditions, evaluated Z scores using patient data (PD) and examined each compensation effect. RESULTS Z scores determined using the advanced NDB were the same value. Artifacts were often generated in Z score maps derived from the conventional NDB (CONDB). The Z score of the own site NDB (OWNDB) was 70% of that calculated based on the CONDB. The combinatorial difference in compensation (scatter and attenuation) resulted in many artifacts being generated in Z score map images. DISCUSSIONS More artifacts were generated in Z score map images using the novel NDB compared with the CONDB. The novel NDB was comparable to the performance of OWNB. The accuracy of brain function image analysis can be improved the reconstruction conditions and correcting for scatter and attenuation on both the novel NDB and PD.

Alexia with agraphia results from lesions of the left angular gyrus or the left posteroinferior temporal lobe. In Japanese or Korean persons, lesions of the latter cause alexia with agraphia for ideograms. We describe a case of alexia with agraphia for kanji (Japanese ideograms) caused by temporal lobe epilepsy. After generalized convulsions, a 32-year-old man noticed that he had difficulty in reading and writing kanji, although he could read and write kana (Japanese syllabograms). His EEG showed frequent sharp waves on the left occipitotemporal region. MRI of the brain revealed a hyperintense lesion on the left hippocampus. Single-photon-emission computed tomography revealed marked hyperperfusion at the left hippocampus and the left posteroinferior temporal cortex. Antiepileptic drugs improved his alexia with agraphia for kanji. This is the first report describing alexia with agraphia for kanji caused by temporal lobe epilepsy.

We describe a case of left temporal lobe epilepsy without hippocampal atrophy. A 31-year-old woman presented with typical symptoms of complex partial seizures. Magnetic resonance imaging demonstrated slightly obscure internal structures in the left hippocampus. Scalp electroencephalography revealed interictal epileptiform discharges in the left temporal lobe. A Wada test with propofol determined the language-dominant hemisphere to be the left. Intraoperative electrocorticography revealed active epileptic discharges in the hippocampus and the anterior temporal basal area. The hippocampal epileptic area was treated with multiple transection, which led to the complete cessation of epileptic discharges. After surgery, the Rey Auditory Verbal Learning Test score decreased from 12 to 9. However, it returned to the preoperative level 6 months after surgery. We describe this case as a typical example demonstrating the efficacy of hippocampal transection for seizure control and the preservation of verbal memory.

Oxygen extraction fraction (OEF) is a key predictor of stroke recurrence in patients with symptomatic major cerebral arterial occlusive disease. The purpose of the present study was to compare central benzodiazepine receptor binding potential (BRBP) and cerebral blood flow (CBF) images on SPECT with OEF images on PET in patients with chronic unilateral middle cerebral artery (MCA) or internal carotid artery (ICA) occlusive disease.OEF, CBF, and BRBP were assessed using (15)O PET and N-isopropyl-p-(123)I-iodoamphetamine and (123)I-iomazenil SPECT, respectively, in 20 healthy subjects and in 34 patients with unilateral MCA or ICA occlusive disease. All images were transformed into the standard brain size and shape by linear and nonlinear transformation using statistical parametric mapping for anatomic standardization. A region of interest (ROI) was automatically placed according to the arterial supply using a 3-dimensional stereotactic ROI template, and the ratio of the value in the affected side to that in the contralateral side was calculated in each image.Among patients with occlusive disease, a significant positive correlation was observed between PET OEF and SPECT BRBP/CBF ratios in 3 cerebral cortical regions (r = 0.851, P0.0001, for anterior cerebral artery [ACA] ROI; r = 0.807, P0.0001, for MCA ROI; and r = 0.774, P0.0001, for posterior cerebral artery [PCA] ROI), but there were no correlations between these 2 parameters in the basal ganglia or the cerebellum. When an abnormally elevated PET OEF ratio was defined as a value greater than the mean + 2 SDs obtained in healthy subjects, sensitivity and specificity were, respectively, 100% and 96% for the ACA ROI, 100% and 89% for the MCA ROI, and 100% and 93% for the PCA ROI for the SPECT BRBP/CBF ratio for detecting an abnormally elevated PET OEF ratio.BRBP/CBF images on SPECT correlate with OEF images on PET in a specific clinical setting-that is, in the cerebral cortex of patients with chronic unilateral MCA or ICA occlusive disease.

To semiquantify the uptake of N-isopropyl-p-[(123)I]-iodoamphetamine (I-123 IMP) in patients with uveal malignant melanoma reproducibly and objectively.Fifty-two patients were examined. Twenty-nine patients had malignant melanoma (group A), three were clinically diagnosed with metastatic choroidal tumor, and 20 patients were given either histological or clinical diagnoses of either benign pigmented lesion or tumor (group B). Early and delayed I-123 IMP images were obtained and standardized by Neurostat software. Using fusion software, we applied a three-dimensional region of interest (3D-ROI) template to the standardized I-123 IMP, and calculated the retention index and tumor-to-nontumor (T/N) ratio of the delayed phase using the maximum count for each ROI.Sensitivity at the retention index cutoff of 30 was 82.4%, specificity was 85.2%, and accuracy was 83.6%. Sensitivity at the T/N ratio of the delayed phase cutoff of 1.3 was 91.2%, specificity was 77.8%, and accuracy was 85.2%. The positive predictive value of the T/N ratio was better than that of the retention index. The negative predictive value of the retention index was better than that of the T/N ratio.This new semiquantitative estimation method is reproducible and objective, especially when the examinations are performed repeatedly for evaluation of both therapy and follow-up.

In patients with idiopathic normal pressure hydrocephalus (iNPH), ventriculomegaly and narrowed subarachnoid spaces at the high convexity appear in magnetic resonance (MR) images before the occurrence of objective symptoms. In addition, quantitative regional cerebral blood flow (rCBF) has been reported to be reduced in iNPH patients with objective symptoms. To determine whether reduced rCBF is responsible for the appearance of symptoms, we compared rCBF in patients with suspected iNPH with no objective triad symptoms (NOS), iNPH patients with apparent objective triad symptoms (AOS) and normal control subjects (NC). Regional CBF was quantified in 35 Regions-of-interest (ROIs) by 123I-IMP single photon emission computed tomography (SPECT) using the autoradiography (ARG) method. Multiple comparisons showed that, in all brain regions examined except for in the frontal white matter, rCBF in the NOS group was significantly lower than that in the NC group, but in all brain regions, not significantly different from that of the AOS group. These results suggest that factors other than rCBF in the resting state are responsible for the occurrence of objective symptoms of iNPH.

An 15O-labeled water (H215O) steady-state method for quantitative measurement of cerebral blood flow ( CBF), which is less stressful to small animals with a few point blood sampling, was developed. After a simulation using a dose meter to achieve stable H215O radioactivity in the blood with a multiprogramming syringe pump programmed for slowly increasing injection volume, 10 rats were studied with the injection method. Arterial blood was sampled every minute during 6-minute positron emission tomography (PET) scans. After the PET scan, N-isopropyl- p-[125I]-iodoamphetamine (125I-IMP) was injected into the same rat to measure CBF using the autoradiography method based on a microsphere model. Regions of interest were placed on the whole brain in H215O-PET and 125I-IMP-autoradiography images, and CBF values calculated from both methods were compared. Radioactivity in the dose meter achieved equilibrium ~1 minute after starting the H215O injection. In rat studies, radioactivity in the blood and brain rapidly achieved equilibrium at 2 minutes after administration. The correlation of CBF values of H215O PET (49.2 ± 5.4 mL per 100 g per minute) and those of 125I-IMP autoradiography (49.1 ± 5.2 mL per 100 g per minute) was excellent ( y = 1.01 x −0.37, r2 = 0.97). The H215O steady-state method with a continuously increasing injection is useful for CBF measurement in small animal studies, especially when multiple scans are required in the same animal.

To evaluate the effect of donepezil hydrochloride, an agent for the treatment of Alzheimer's disease (AD), on the cerebral and optic nerve head (ONH) blood flow, visual field defect in normal-tension glaucoma (NTG) patients with decreased cerebral blood flow (CBF) that demonstrates an AD-like perfusion pattern.The subjects were 5 NTG patients who exhibited AD-like decreased CBF upon (123)I-iodoamphetamine single photon emission computed tomography ((123)I-IMP SPECT). Donepezil hydrochloride (5 mg/day) was prescribed for each patient during a period of 12 months. Intraocular pressure (IOP), mean deviation (MD) of the Humphrey visual field, ONH blood flow determined by a laser speckle flowgraphy, and regional CBF (rCBF) determined by (123)I-IMP SPECT were measured before and every 6 months during the treatment.MD, ONH blood flow, and rCBF were improved significantly after 6 months of the treatment, although IOP did not change significantly. No deterioration of NTG morbidity was found in any of the measured parameters after 12 months of the treatment.Oral administration of donepezil hydrochloride in NTG patients might prevent deterioration of visual field defect, ONH blood flow, and rCBF in the temporal, parietal, and posterior lobes. This pilot study suggested the possibility that donepezil hydrochloride might ameliorate glaucomatous optic neuropathy in NTG.