向井 宏樹

We report the case of a woman in her 40s who presented with sensory disturbances in all 4 limbs and left facial palsy. MRI revealed asymmetric enlargement of the dorsal root ganglia, which was enhanced by gadolinium-a chest CT scan identified enlarged supraclavicular, mediastinal, and hilar lymph nodes. A biopsy of a hilar lymph node showed noncaseating epithelioid granulomas, confirming a sarcoidosis diagnosis. Prednisolone treatment led to symptomatic improvements. In sarcoidosis of the peripheral nervous system, there might be observable enlargement of the dorsal root ganglion alongside enhanced gadolinium contrast. Obtaining a biopsy from the dorsal root ganglion poses challenges, and radiologists should be mindful of this specific imaging characteristic.

The most common genetic Creutzfeldt-Jakob disease (gCJD) in Japan is caused by a point mutation in which isoleucine replaces valine at codon 180 of the prion protein (PrP) gene (V180I gCJD). Evidence suggests that cerebral cortex swelling, which appears as abnormal hyperintensities on diffusion-weighted imaging (DWI), is a characteristic magnetic resonance imaging (MRI) finding of V180I gCJD. However, no study has directly compared the MRI findings between V180I gCJD and sporadic CJD (sCJD). The current study, therefore, aims to clarify the imaging features of V180I gCJD, which would lead to prompt genetic counselling and analysis of the PrP gene, particularly focusing on cerebral cortex swelling. We included 35 patients with sCJD (n = 23) or V180I gCJD (n = 12). Cerebral cortex swelling on T2-weighted imaging (T2WI) or fluid-attenuated inversion recovery (FLAIR) wherein abnormal cortical hyperintensities were observed on DWI, and the distribution of grey matter hyperintensities on DWI were visually evaluated. V180I gCJD patients had significantly more cerebral cortex swelling (100% vs. 13.0%, p < 0.001), an overall correct classification of 91.4%, and parahippocampal gyrus hyperintensities on DWI (100% vs. 39.1%, q = 0.019) than sCJD patients. Cerebral cortical hyperintensities on DWI with swelling on T2WI or FLAIR are characteristic imaging findings of V180I gCJD and are useful for differentiating it from sCJD.

BACKGROUND: Cerebral blood flow (CBF) and dopamine transporter (DAT) images are clinically used for the differential diagnosis of parkinsonian disorders. OBJECTIVES: This study aimed to examine the correlation of CBF with striatal DAT in patients with Parkinson's disease (PD) and atypical parkinsonian syndromes (APS) and evaluate the diagnostic power of DAT-correlated CBF in PD through machine learning with each imaging modality alone or in combination. METHODS: Fifty-eight patients with PD and 71 with APS (24 with multiple system atrophy, 21 with progressive supranuclear palsy, and 26 with corticobasal syndrome) underwent 123 I-IMP and 123 I-FP-CIT single-photon emission computed tomography. Multiple regression analyses for CBF and striatal DAT binding were conducted on each group. PD probability was predicted by machine learning and receiver operating characteristic curves. RESULTS: The PD group showed more affected striatal DAT binding positively correlated with the ipsilateral prefrontal perfusion and negatively with the bilateral cerebellar perfusion. In corticobasal syndrome, striatal DAT binding positively correlated with the ipsilateral prefrontal perfusion and negatively with the contralateral precentral perfusion. In Richardson's syndrome, striatal DAT binding positively correlated with perfusion in the ipsilateral precentral cortex and basal ganglia. Machine learning showed that the combination of CBF and DAT was better for delineating PD from APS (area under the curve [AUC] = 0.87) than either CBF (0.67) or DAT (0.50) alone. CONCLUSIONS: In PD and four-repeat tauopathy, prefrontal perfusion was related to ipsilateral nigrostriatal dopaminergic function. This dual-tracer frontostriatal relationship may be effectively used as a diagnostic tool for delineating PD from APS. © 2022 International Parkinson and Movement Disorder Society.

Magnetic resonance vessel wall imaging is desirable for evaluating Kawasaki disease (KD)-associated coronary arterial lesions. To evaluate the reproducibility of three-dimensional turbo spin-echo (3D-TSE) and two-dimensional dual inversion-recovery turbo spin-echo (2D-DIR-TSE) for coronary vessel wall imaging in KD. Ten patients were prospectively enrolled. Coronary vessel wall imaging with axial-slice orientation 3D-TSE and 2D-DIR-TSE were acquired for cross-sectional images in aneurysmal and normal regions. Lumen area (LA), wall area (WA), and normalized wall index (NWI) of cross-sectional images were measured in both regions. Reproducibility between 3D-TSE and 2D-DIR-TSE was evaluated via intraclass correlation coefficients (ICCs) and Bland-Altman plots. 48 points (aneurysmal, 27; normal, 21) were evaluated. There were high ICCs between 3D-TSE and 2D-DIR-TSE in LA (0.95) and WA (0.95). In aneurysmal regions, 95% limits of agreement were LA, WA, and NWI of - 29.9 to 30.4 mm2, - 18.8 to 15.0 mm2, and - 0.22 to 0.20, respectively. In normal regions, the 95% limits of agreement were LA, WA, and NWI of - 4.44 to 4.38 mm2, - 3.51 to 4.30 mm2, and - 0.14 to 0.16, respectively. No fixed and proportional biases between 3D-TSE and 2D-DIR-TSE images in aneurysmal and normal regions were noted. 3D-TSE was reproducible with conventional 2D-DIR-TSE for coronary vessel wall assessment on KD.

Since leptomeningeal carcinomatosis is rarely observed before diagnosis of the primary cancer, its detection is often delayed. We report the case of a 60-year-old woman who presented with lung adenocarcinoma with leptomeningeal carcinomatosis. Magnetic resonance imaging showed the characteristic abnormal hyperintensity along the ventral surface of the brain stem on fluid-attenuated inversion recovery and diffusion weighted imaging. It had no contrast uptake. Based on these findings, we were able to make an early diagnosis of leptomeningeal carcinomatosis of lung adenocarcinoma. This condition was resolved after treatment with a tyrosine kinase inhibitor.

INTRODUCTION: This study assessed the morphological changes and diffusion tensor imaging (DTI)-derived parameters of the brachial plexus using magnetic resonance neurography (MRN) in patients with anti-myelin-associated glycoprotein (anti-MAG) neuropathy. METHODS: Eight patients with anti-MAG neuropathy underwent MRN of the brachial plexus with 3-dimensional (3D) short tau inversion recovery (STIR) and DTI sequences. Two neuroradiologists and a neurologist qualitatively assessed nerve hypertrophy on 3D STIR MRN. The cross-sectional area (CSA) of the nerve roots was measured. Quantitative analyses of fractional anisotropy (FA) and axial, radial, and mean diffusivity (AD, RD, and MD) were obtained after postprocessing on DTI and manual segmentation. RESULTS: There was nerve hypertrophy in 37.5% of the patients with anti-MAG neuropathy. All patients with anti-MAG neuropathy with nerve hypertrophy were refractory to rituximab therapy. The CSA of the nerve roots was inversely correlated with FA and positively correlated with MD and RD. FA decreased in the nerve roots and inversely correlated with disease duration. CONCLUSIONS: Nerve hypertrophy appears in the proximal portion of peripheral nerves, such as the brachial plexus, in patients with anti-MAG neuropathy. Altered diffusion in the nerve roots might be associated with the loss of myelin integrity due to the demyelination process in anti-MAG neuropathy.

Frontotemporal brain sagging syndrome (FBSS) is a progressive disorder characterized by symptoms similar to the behavioral variant of frontotemporal dementia (FTD), with a sagging appearance of the brain on imaging similar to that observed in spontaneous intracranial hypotension (SIH). The onset of behavioral and cognitive symptoms of FBSS is insidious and progressive, similar to those of FTD. Here, we report a case involving a 53-year-old man with progressive hypersomnolence, apathy, forgetfulness, and personality changes but without headache or auditory symptoms. The combination of frontotemporal dysfunction, hypersomnolence, and the appearance of a sagging brain on magnetic resonance imaging suggested a diagnosis of FBSS. Although a definite site of cerebrospinal fluid leakage could not be identified in our case, clinical symptoms and imaging findings were improved after an epidural blood patch. Considering FBSS as a differential diagnosis of FTD is important even in the absence of typical SIH symptoms, such as headache or auditory symptoms.

Taq1A polymorphism is a DRD2 gene variant located in an exon of the ANKK1 gene and has an important role in the brain's dopaminergic functions. Some studies have indicated that A1 carriers have an increased risk of developing Parkinson's disease (PD) and show poorer clinical performance than A2 homo carriers. Previous studies have suggested that A1 carriers had fewer dopamine D2 receptors in the caudate and increased cortical activity as a compensatory mechanism. However, there is little information about morphological changes associated with this polymorphism in patients with PD. The study's aim was to investigate the relationship between brain volume and Taq1A polymorphism in PD using voxel-based morphometry (VBM). Based on Taq1A polymorphism, 103 patients with PD were divided into two groups: A1 carriers (A1/A1 and A1/A2) and A2 homo carriers (A2/A2). The volume of the left prefrontal cortex (PFC) was significantly decreased in A2 homo carriers compared to A1 carriers. This finding supports the association between Taq1A polymorphism and brain volume in PD and may explain the compensation of cortical function in A1 carriers with PD.

BACKGROUND: The standardized T1-weighted/T2-weighted (sT1w/T2w) ratio for the middle cerebellar peduncle (MCP) has been reported to be sensitive for detecting degenerative changes in the cerebellar subtype of multiple system atrophy (MSA-C), even in the early stages. We aimed to investigate the diagnostic value of the MCP sT1w/T2w ratio for differentiating between MSA-C and spinocerebellar ataxia (SCA). METHODS: We included 32 MSA-C, 8 SCA type 3 (SCA3), 16 SCA type 6 (SCA6) patients, and 17 controls, and the MCP sT1w/T2w ratio was analyzed using a region-of-interest approach. The diagnostic performance of the MCP sT1w/T2w ratio in discriminating among MSA-C, SCA3, and SCA6 was assessed and compared with diagnosis based on visual interpretation of MCP hyperintensities and the "hot cross bun" (HCB) sign. RESULTS: MCP sT1w/T2w ratio values were markedly lower in patients with MSA-C than in those with SCA3, those with SCA6, and controls (p < 0.001). The MCP sT1w/T2w ratio showed high diagnostic accuracy for distinguishing MSA-C from SCA3 (area under curve = 0.934), SCA6 (area under curve = 0.965), and controls (area under curve = 0.980). The diagnostic accuracy of the MCP sT1w/T2w ratio for differentiating MSA-C from SCA3 or SCA6 (90.0% for MSA-C vs. SCA3, and 91.7% for MSA-C vs. SCA6) was comparable to or superior than that of visual interpretation of MCP hyperintensities (80.0-87.5% in MSA-C vs. SCA3 and 87.6-97.9% in MSA-C vs. SCA6) or the HCB sign (72.5-80.0% in MSA-C vs. SCA3 and 77.1-93.8% in MSA-C vs. SCA6). CONCLUSIONS: The MCP sT1w/T2w ratio might be a sensitive imaging-based marker for detecting MSA-C-related changes and differentiating MSA-C from SCA3 or SCA6.

OBJECTIVE: The impact of clinical information on radiological diagnoses and subsequent clinical management has not been sufficiently investigated. This study aimed to compare diagnostic performance between radiological reports made with and without clinical information and to evaluate differences in the clinical management decisions based on each of these reports. METHODS: We retrospectively reviewed 410 patients who presented with acute abdominal pain and underwent unenhanced (n = 248) or enhanced CT (n = 162). Clinical information including age, sex, current and past history, physical findings, and laboratory tests were collected. Six radiologists independently interpreted CTs that were randomly assigned with or without clinical information, made radiological diagnoses, and scored the diagnostic confidence level. Four general and emergency physicians simulated clinical management (i.e., followed up in the outpatient clinic, hospitalized for conservative therapy, or referred to other departments for invasive therapy) based on reports made with or without the clinical information. Reference standards for the radiological diagnoses and clinical management were defined by an independent expert panel. RESULTS: The radiological diagnoses made with clinical information were more accurate than those made without clinical information (93.7% vs. 87.8%, p = 0.008). Median interpretation time for radiological reporting with clinical information was significantly shorter than that without clinical information (median 122.0 vs. 139.0 s, p < 0.001). Clinical simulation better matched the reference standard for clinical management when radiological diagnoses were made with reference to clinical information (97.3% vs. 87.8%, p < 0.001). CONCLUSION: Access to adequate clinical information enables accurate radiological diagnoses and appropriate subsequent clinical management of patients with acute abdominal pain. KEY POINTS: • Radiological interpretation improved diagnostic accuracy and confidence level when clinical information was provided. • Providing clinical information did not extend the interpretation time required by radiologists. • Radiological interpretation with clinical information led to correct clinical management by physicians.

BACKGROUND: Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disease. Pathologically, it is characterized by eosinophilic hyaline intranuclear inclusions in the cells of the visceral organs as well as central, peripheral, and autonomic nervous system cells. Recently, a GGC repeat expansion in the NOTCH2NLC gene has been identified as the etiopathological agent of NIID. Interestingly, this GGC repeat expansion was also reported in some patients with a clinical diagnosis of amyotrophic lateral sclerosis (ALS). However, there are no autopsy-confirmed cases of concurrent NIID and ALS. CASE PRESENTATION: A 60-year-old Taiwanese woman reported a four-month history of progressive weakness beginning in the right foot that spread to all four extremities. She was diagnosed with ALS because she met the revised El Escorial diagnostic criteria for definite ALS with upper and lower motor neuron involvement in the cervical, thoracic, and lumbosacral regions. She died of respiratory failure at 22 months from ALS onset, at the age of 62 years. Brain magnetic resonance imaging (MRI) revealed lesions in the medial part of the cerebellar hemisphere, right beside the vermis (paravermal lesions). The subclinical neuropathy, indicated by a nerve conduction study (NCS), prompted a potential diagnosis of NIID. Antemortem skin biopsy and autopsy confirmed the coexistence of pathology consistent with both ALS and NIID. We observed neither eccentric distribution of p62-positive intranuclear inclusions in the areas with abundant large motor neurons nor cytopathological coexistence of ALS and NIID pathology in motor neurons. This finding suggested that ALS and NIID developed independently in this patient. CONCLUSIONS: We describe a case of concurrent NIID and ALS discovered during an autopsy. Abnormal brain MRI findings, including paravermal lesions, could indicate the coexistence of NIID even in patients with ALS showing characteristic clinical phenotypes.

BACKGROUND: In double-bundle anterior cruciate ligament reconstruction (ACLR), fixed-loop and adjustable-loop cortical suspensory devices are commonly used to fix the soft graft on the femoral side. However, few studies have compared in vivo elongation of the two devices. The purpose of this study was to determine whether EndoButton CL (EB) and TightRope RT (TR), the suspensory fixation devices used in ACLR, maintained their length in vivo from the time of surgery through the postoperative period in a randomized controlled trial. METHODS: This study prospectively incorporated 30 patients undergoing initial ACLR at a single center. Participants were divided into two groups using a stratified randomization method with age and sex as assignment adjustment factors. EB or TR was used for fixation of the soft graft on the femoral side. The primary endpoint was to compare the elongation distance of the suspensory device. MRIs were taken within seven days after ACLR and 3,6,12 months postoperatively and measured by a radiologist in a blinded fashion. Secondary endpoints included the side-to-side difference in anterior translation, one leg hop test (HOP index), Lachman test, lateral pivot shift test, and Lysholm score one year postoperatively. RESULTS: Twenty-eight patients (EB, n = 13; TR, n = 15) were followed for one year. There was no significant difference between EB and TR groups in elongation from the immediate postoperative period to 3, 6, 12 months after surgery. However, the non-inferiority of TR to EB (non-inferiority margin: 1.5 mm) was not proved by the difference in measured elongation between the two groups (TR - EB, lower 95% CI. AM: 1.80 mm; PL: 1.86 mm) at 6 months. There was no significant difference in anterior translation, HOP index, Lachman test, lateral pivot shift test, or Lysholm score. CONCLUSION: EB and TR had similar graft retaining ability in vivo for 12 months, but the non-inferiority of TR against EB was not verified statistically.

BACKGROUND: This study aimed to investigate the frequency and risk factors for cerebral artery stenosis and occlusion in patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome. METHODS: We reviewed results of magnetic resonance angiography (MRA) or computed tomography angiography (CTA) in 61 patients with POEMS syndrome seen between 2010 and 2017. Stenosis or occlusion was assessed in the initial MRA/CTA. Multivariate analysis was used to identify risk factors for artery stenosis/occlusion. In an autopsy case, pathologic examination was conducted of the occluded middle cerebral arteries. RESULTS: Stenosis (> 50 %) or occlusion of the major cerebral arteries was found in 29 (47.5 %) patients on the initial MRA/CTA. The internal carotid artery was involved most frequently (32.8 %), followed by the anterior (21.3 %) and middle (16.4 %) cerebral arteries. The basilar (1.3 %) and vertebral (3.6 %) arteries were rarely affected. Cerebral infarction developed in eight (13.1 %) patients. The serum vascular endothelial growth factor (VEGF) level was an independent predictor for stenosis/occlusion (odds ratio, 1.228; 95 % confidence interval, 1.042-1.447; P = 0.014). An autopsy study showed occluded middle cerebral arteries by fibrous and myxomatous thickening of intima with splitting of the internal elastic lamina. Follow-up MRA in 23 patients showed improved, worsened, and unchanged stenosis in 20.7 %, 8.7 %, and 69.6 %, respectively. CONCLUSIONS: Cerebral large-vessel stenosis or occlusion is frequently seen in approximately half of patients with POEMS syndrome. Vasculopathy was related to serum VEGF levels and thereby disease activity. Assessment of cerebral vessels is recommended in these patients to improve management.

OBJECTIVE: We aimed to investigate the use of a myelin-sensitive MRI contrast, the standardized T1-weighted/T2-weighted (sT1w/T2w) ratio, for detecting early changes in the middle cerebellar peduncle (MCP) in cerebellar subtype multiple system atrophy (MSA-C) patients. METHODS: We included 28 MSA-C patients, including a subset of 17 MSA-C patients within 2 years of disease onset (early MSA-C), and 28 matched healthy controls. T1w and T2w scans were acquired using a 3-T MR system. The sT1w/T2w ratio in MCP was analyzed using SPM12 by utilizing a region-of-interest approach in normalized space. The diagnostic performance of the MCP sT1w/T2w ratio in discriminating MSA-C and the subgroup of early MSA-C from the matched controls was assessed. Correlation analyses were performed to evaluate the relationship between the MCP sT1w/T2w ratio and other clinical parameters including the International Cooperative Ataxia Scale (ICARS) score for quantifying cerebellar ataxia. RESULTS: Compared to controls, the sT1w/T2w ratio in the MCP was markedly lower in all (p < 0.001) MSA-C patients and 17 early (p < 0.001) MSA-C patients. The MCP sT1w/T2w ratio had high sensitivity (96%) and specificity (100%) to distinguish MSA-C from controls (area under the curve = 0.99), even for the early MSA-C group (area under the curve = 0.99; sensitivity = 94%, specificity = 100%). The MCP sT1w/T2w ratio correlated with the ICARS score in early MSA-C. CONCLUSIONS: The sT1w/T2w ratio can detect MSA-C-related changes in the MCP, even in the early stages of the disorder, and could be a sensitive biomarker for MSA-C. KEY POINTS: • The sT1w/T2w ratio can detect MSA-C-related changes in the middle cerebellar peduncle, even in the early stages of the disorder. • The middle cerebellar peduncle sT1w/T2w ratio correlated with a cerebellar ataxia score in early MSA-C patients.

A man and a woman were found dead in the same car with a burned coal briquette. The cause of death of the woman was assigned to acute carbon monoxide (CO) poisoning without difficulty based on typical findings associated with this condition, including elevation of carboxyhaemoglobin (COHb). However, the man had an unremarkable elevation of COHb and a higher rectal temperature compared to that of the woman. Postmortem computed tomography (PMCT) revealed ambiguous low-density areas in the bilateral globi pallidi. Further analysis by postmortem magnetic resonance (PMMR) imaging showed these lesions more clearly; the lesions appeared as marked high signal intensity areas on both the T2-weighted images and the fluid-attenuated inversion recovery sequences. A subsequent autopsy revealed signs of pneumonia, dehydration, starvation, and hypothermia, suggesting that the man died from prolonged CO poisoning. Both globi pallidi contained grossly ambiguous lesions, and a detailed neuropathologic investigation revealed these lesions to be coagulative necrotic areas; this finding was compatible with a diagnosis of prolonged CO poisoning. This case report shows that postmortem imaging, especially PMMR, is useful for detecting necrotic lesions associated with prolonged CO poisoning. This report further exemplifies the utility of PMMR for detecting brain lesions, which may be difficult to detect by macroscopic analysis.

Current deep learning-based cerebral aneurysm detection demonstrates high sensitivity, but produces numerous false-positives (FPs), which hampers clinical application of automated detection systems for time-of-flight magnetic resonance angiography. To reduce FPs while maintaining high sensitivity, we developed a multidimensional convolutional neural network (MD-CNN) designed to unite planar and stereoscopic information about aneurysms. This retrospective study enrolled time-of-flight magnetic resonance angiography images of cerebral aneurysms from three institutions from June 2006 to April 2019. In the internal test, 80% of the entire data set was used for model training and 20% for the test, while for the external tests, data from different pairs of the three institutions were used for training and the remaining one for testing. Images containing aneurysms > 15 mm and images without aneurysms were excluded. Three deep learning models [planar information-only (2D-CNN), stereoscopic information-only (3D-CNN), and multidimensional information (MD-CNN)] were trained to classify whether the voxels contained aneurysms, and they were evaluated on each test. The performance of each model was assessed using free-response operating characteristic curves. In total, 732 aneurysms (5.9 ± 2.5 mm) of 559 cases (327, 120, and 112 from institutes A, B, and C; 469 and 263 for 1.5T and 3.0T MRI) were included in this study. In the internal test, the highest sensitivities were 80.4, 87.4, and 82.5%, and the FPs were 6.1, 7.1, and 5.0 FPs/case at a fixed sensitivity of 80% for the 2D-CNN, 3D-CNN, and MD-CNN, respectively. In the external test, the highest sensitivities were 82.1, 86.5, and 89.1%, and 5.9, 7.4, and 4.2 FPs/cases for them, respectively. MD-CNN was a new approach to maintain sensitivity and reduce the FPs simultaneously.

BACKGROUND: Individuals with early-onset Alzheimer disease (EOAD) differ from those with late-onset Alzheimer disease (LOAD) not only in genetics and age at onset but also in their clinical symptoms. OBJECTIVE: To differentiate the neuropathological and neurocognitive features of EOAD and LOAD by comparing the pattern of regional gray matter volume (GMV) reduction and its symptomatic correlates. METHOD: Three-dimensional T1-weighted MRIs and Mini-Mental State Examination (MMSE) scores were obtained from 12 individuals with EOAD, 65 with LOAD, and 49 healthy controls (HC). Regional GMV reduction between the three groups was assessed using voxel-based morphometry. Multiple regression analyses were conducted with MMSE total score as an independent variable. RESULTS: Compared to the HC, both AD groups showed a significant GMV reduction in the bilateral hippocampus and the left temporoparietal junction; in addition, the LOAD group showed one in the bilateral anterior temporal lobes. Multiple regression analyses revealed a positive correlation between MMSE total score and GMV in the left anterior temporal lobe in both AD groups; that is, lower scores were associated with reduced GMV. Interestingly, a positive correlation in hippocampal GMV was revealed only in the LOAD group. CONCLUSION: MMSE total score is associated with the anterior temporal lobe volume in individuals with AD. Hippocampal volume and its relationship with MMSE total score are associated with LOAD pathophysiology but not EOAD pathophysiology. The hippocampal volume reduction and low MMSE scores are hallmarks of LOAD but are less specific to EOAD, which may cause a delay in diagnosis.

BACKGROUND: The "hot cross bun" (HCB) sign, a cruciform hyperintensity in the pons on magnetic resonance imaging (MRI), has gradually been identified as a typical finding in multiple system atrophy, cerebellar-type (MSA-C). Few reports have evaluated the sensitivity of an HCB, including a cruciform hyperintensity and vertical line in the pons, which precedes a cruciform hyperintensity, in the early stages of MSA-C. Moreover, the difference in frequency and timing of appearance of an HCB between MSA-C and spinocerebellar ataxia type 3 (SCA3) has not been fully investigated. METHODS: This study investigated the time at which an HCB and orthostatic hypotension (OH) appeared in 41 patients with MSA-C, based on brain MRI and head-up tilt test. The MRI findings were compared with those of 26 patients with SCA3. The pontine signal findings on T2-weighted MRI were graded as 0 (no change), 1 (a vertical T2 high-intensity line), or 2 (a cruciform T2 high-intensity line), with grades 1 or 2 considered as an HCB. OH 30/15 was defined as a decrease in systolic blood pressure of > 30 mmHg or diastolic blood pressure of > 15 mmHg. RESULTS: Among the 24 patients with MSA-C within 2 years from the onset of motor symptoms, an HCB was detected in 91.7%, whereas OH 30/15 was present in 60.0%. Among the 36 patients with MSA-C within 3 years from the onset of motor symptoms, a grade 2 HCB was detected in 66.7% of those with MSA-C but in none of those with SCA-3. CONCLUSIONS: HCB is a highly sensitive finding for MSA-C, even in the early stages of the disease. A grade 2 HCB in the early stage is an extremely specific finding for differentiating MSA-C from SCA-3.

Only a few reports have described radiological findings of parasite depicted by postmortem computed tomography (PMCT). We herein present a case in which the PMCT of a 29-year-old man revealed Taenia saginata, a species of tapeworms, inside the intestine. The image of the parasite appeared as a low-density linear or cystic pattern of gas bubbles located within the lumen of the small bowel. We named this finding as 'PseudoPneumatosis intestinalis' sign, since which mimicked the findings of Pneumatosis intestinalis in a clinical setting. In this report, we present the image and discuss the importance of the findings of parasites on PMCT performed before autopsy.

BACKGROUND: Neuropsychological tests, structural neuroimaging, and functional neuroimaging are employed as diagnostic and monitoring biomarkers of patients with Alzheimer's disease (AD)Objective:We aimed to elucidate the similarities and differences in neuropsychological tests and neuroimaging with the use of the Mini-Mental State Examination (MMSE), Alzheimer's Disease Assessment Scale cognitive subscale (ADAS-cog), structural magnetic resonance image (MRI), and perfusion single photon emission computed tomography (SPECT), and parametric image analyses to understand its role in AD. METHODS: Clinically-diagnosed AD patients (n = 155) were scanned with three-dimensional T1-weighted MRI and N-isopropyl-p-[123I] iodoamphetamine SPECT. Statistical parametric mapping 12 was used for preprocessing images, statistical analyses, and voxel-based morphometry for gray matter volume analyses. Group comparison (AD versus healthy controls), multiple regression analyses with MMSE, ADAS-cog total score, and ADAS-cog subscores as variables, were performed. RESULTS: The AD group showed bilateral hippocampal volume reduction and hypoperfusion in the bilateral temporo-parietal lobe and posterior midline structures. Worse MMSE and ADAS-cog total score were associated with bilateral temporo-parietal volume loss and hypoperfusion. MMSE, but not ADAS-cog, was associated with the posterior midline structures. The ADAS-cog subscores were associated with the temporal volume, while perfusion analyses were linked to the left temporo-parietal region with the language function and right analogous region with the constructional praxis subscore. CONCLUSION: MMSE and ADAS-cog are associated with temporo-parietal regions, both in volume and perfusion. The MMSE score is associated with posterior midline structures and linked to an abnormal diagnostic AD pattern. Perfusion image analyses better represents the cognitive function in AD patients.

PURPOSE: To evaluate the feasibility of coronary vessel wall visualization using three-dimensional turbo spin-echo black blood imaging (3D-TSE) in children with Kawasaki disease. MATERIALS AND METHODS: Nine patients (6 girls and 3 boys; mean age ± standard deviation, 5.6 ± 3.3 years; range, 1.4-10.3 years) were included. Coronary magnetic resonance angiography (MRA) with an axial slice orientation and 3D-TSE with axial and sagittal slice orientations (3D-TSE-axi and 3D-TSE-sag) were acquired for the whole heart. Coronary vessel walls were evaluated separately in aneurysm and normal-proximal regions. The internal diameter and wall thickness of the reformatted cross-sectional images were measured in both the regions. Reproducibility between MRA and 3D-TSE was evaluated via interclass correlation coefficients (ICCs) and Bland-Altman plots. RESULTS: In total, 164 points (aneurysmal regions, 73; normal-proximal regions, 64; normal-distal regions, 27) were evaluated. The ICC for 3D-TSE-axi was higher than that for 3D-TSE-sag (aneurysmal regions, ICC = 0.88 and 0.81; normal-proximal regions, ICC = 0.90 and 0.32, respectively). Bland-Altman plots of the internal diameter via MRA and 3D-TSE-axi showed a wide 95% limit of agreement (-0.13 to 2.89 mm) and significant fixed and proportional biases (P < 0.001 and P = 0.002) in the aneurysmal regions. However, the 95% limit of agreement was narrow (-0.14 to 0.57 mm) in the normal-proximal regions. If 1 mm was set as the cut-off for a thickened wall, wall thickness via 3D-TSE-axi was found to be abnormal across many points (84.0% of aneurysmal regions; 18.4% of normal-proximal regions). CONCLUSIONS: 3D-TSE imaging of the normal-proximal regions of the coronary vessel in individuals with Kawasaki disease was found to be feasible. However, in aneurysmal regions, larger aneurysmal diameters led to an increased bias between MRA and 3D-TSE.

In forensic investigations, it is important to detect traumatic axonal injuries (TAIs) to reveal head trauma that might otherwise remain occult. These lesions are subtle and frequently ambiguous on macroscopic evaluations. We present a case of TAI revealed by pre-autopsy postmortem magnetic resonance imaging (PMMR). A man in his sixties was rendered unconscious in a motor vehicle accident. CT scans revealed traumatic mild subarachnoid hemorrhage. Two weeks after the accident he regained consciousness, but displayed an altered mental state. Seven weeks after the accident, he suddenly died in hospital. Postmortem computed tomography (PMCT) and PMMR were followed by a forensic autopsy. PMMR showed low-intensity lesions in parasagittal white matter, deep white matter, and corpus callosum on three-dimensional gradient-echo T1-weighted imaging (3D-GRE T1WI). In some of these lesions, T2∗-weighted imaging also showed low-intensity foci suggesting hemorrhagic axonal injury. The lesions were difficult to find on PMCT and macroscopic evaluation, but were visible on antemortem MRI and confirmed as TAIs on histopathology. From this case, it can be said that PMMR can detect subtle TAIs missed by PMCT and macroscopic evaluation. Hence, pre-autopsy PMMR scanning could be useful for identifying TAIs during forensic investigations.

PURPOSE: Preoperative identification of Adamkiewicz artery (AKA) for preventing postoperative spinal cord ischemia is still challenging because of its small diameter. Low-tube-voltage technique might improve the delineation of AKA due to its higher contrast enhancement and contrast-to-noise ratio (CNR). Our purpose was to evaluate the usefulness of low-tube-voltage CTA in visualization of AKA compared with the conventional voltage protocol on the condition with the same imaging parameters aside from tube voltage. METHODS: Eighty-three patients undergoing CTA for the evaluation of aorta were retrospectively included. All CTA was performed with 320-detector-row CT with the tube voltage of either 100-kVp (41 patients) or 120-kVp (42 patients). The CNR, CT value of aorta and objective image noise were assessed. Visualization of AKA was evaluated based on the continuity from aorta using the four-grade score by two independent reviewers. The estimated radiation dose (volumetric CT dose index) was also compared. RESULTS: The 100-kVp group showed significantly higher CNR and CT value than 120-kVp protocol (P =  0.010 and < 0.001, respectively). The visual score was also significantly higher in 100-kVp group than in 120-kVp group (2.73 ± 0.98 and 2.02 ± 1.00, respectively; P =  0.002). There was no significant difference on objective image noise and radiation dose between the groups (P =  0.24 and 0.72, respectively). CONCLUSION: CTA with low-tube-voltage was significantly more sensitive for AKA visualization than conventional voltage protocol.

<p>脳動脈瘤はくも膜下出血と呼ばれる重大疾患の主要因であり，破裂すると多くの場合死に至るため，医師の診断による早期発見・治療が求められる． 医師の診断を支援する目的として，これまでに，磁気共鳴血管画像（Magnetic Resonance Angiography; MRA）を用いて脳動脈瘤を自動検出する機械学習手法がいくつか提案されている． 近年では，画像認識タスクで広く利用されてる畳み込みニューラルネットワーク（Convolutional Neural Networks; CNN）を用いた脳動脈瘤検出手法が提案されており，高い精度で瘤を検出できることで知られている． これまでに提案されているCNNを用いた脳動脈瘤検出手法は，いずれも2次元画像を入力としたネットワーク構成となっており，動脈の屈折部に隠れた瘤が正確に検出することが難しいという問題があった． そこで本研究では，入力として2次元画像を用いたネットワークと3次元ボクセルを用いたネットワークで検出感度を比較し，その精度を評価する． 実験の結果，2Dネットワークと比較し3Dネットワークでは，高い検出感度で少ない誤検出率を達成した．</p>

PURPOSE: The aim of this study was to evaluate the utility of merged balanced steady-state free precession (bSSFP) magnetic resonance cisternography images. MATERIALS AND METHODS: Twenty ears of 10 healthy volunteers (six men, four women; mean age ± standard deviation, 26.7 ± 1.6 yr) and 10 patients (two men, eight women; mean age, 46.3 ± 10.9 yr) with neoplasm around the sella turcica were included. Two different devices (A and B) were used to confirm the versatility of our method for MR devices with different local magnetic field homogeneity. Images with different central frequencies (±10, ±20, ±30, ±40, and ±50 Hz) were merged with the maximum magnitude of corresponding pixels from the images acquired using both devices. Two neuroradiologists visually graded the image quality of 11 sites in the inner ear and three sites around the sella turcica (scale: 0-2) and compared the quality with that of the corresponding basic image (0 Hz). RESULTS: The image quality was better in merged images of the vestibule, superior semicircular canal (SCC), posterior SCC, and horizontal SCC (P = 0.005 to 0.020 mainly at ±40 and ±50 Hz on devices A and B), as well as in merged images of the sella turcica and right cavernous sinus (±50 Hz, P = 0.003 and 0.020 on device B, respectively), than it was in the corresponding basic images. CONCLUSIONS: The maximum magnitude merging of images with different central frequencies makes it possible to reduce banding artifacts on bSSFP images without the need for special pulse sequences and image processing programs.

OBJECTIVE: To assess the cerebral blood flow (CBF) in patients with diabetic neuropathic pain, and its changes after duloxetine therapy. METHODS: Using iodine-123-N-isopropyl-p-iodoamphetamine single-photon emission computed tomography (IMP-SPECT), we performed a cross-sectional study of 44 patients with diabetes, and compared CBF in those with (n = 24) and without neuropathic pain (n = 20). In patients with neuropathic pain, we also longitudinally assessed changes in CBF 3 months after treatment with duloxetine. RESULTS: IMP-SPECT with voxel-based analyses showed a significant increase in cerebral blood flow in the right anterior cingulate cortex and a decrease in the left ventral striatum in patients with neuropathic pain, compared with those without pain. After duloxetine treatment, volume of interest analyses revealed a decrease in cerebral blood flow in the anterior cingulate cortex in patients with significant pain relief but not in non-responders. Furthermore, voxel-based whole brain correlation analyses demonstrated that greater baseline CBF in the anterior cingulate cortex was associated with better pain relief on the numerical rating scale. CONCLUSIONS: Our results suggest that the development of neuropathic pain is associated with increased activity in the anterior cingulate cortex, and greater baseline activation of this region may predict treatment responsiveness to pharmacological intervention. TRIAL REGISTRATION NUMBER: UMIN000017130;Results.

Background and Purpose: The superior cervical ganglion and inferior ganglion of the vagus nerve can mimic pathologic retropharyngeal lymph nodes. We studied the cross-sectional anatomy of the superior cervical ganglion and inferior ganglion of the vagus nerve to evaluate how they can be differentiated from the retropharyngeal lymph nodes. MATERIALS AND METHODS: This retrospective study consists of 2 parts. Cohort 1 concerned the signal intensity of routine neck MR imaging with 2D sequences, apparent diffusion coefficient, and contrast enhancement of the superior cervical ganglion compared with lymph nodes with or without metastasis in 30 patients. Cohort 2 used 3D neurography to assess the morphology and spatial relationships of the superior cervical ganglion, inferior ganglion of the vagus nerve, and the retropharyngeal lymph nodes in 50 other patients. RESULTS: All superior cervical ganglions had homogeneously greater enhancement and lower signal on diffusion-weighted imaging than lymph nodes. Apparent diffusion coefficient values of the superior cervical ganglion (1.80 ± 0.28× 10-3mm2/s) were significantly higher than normal and metastatic lymph nodes (0.86 ± 0.10-3 mm2/s, P &lt .001, and 0.73 ± 0.10 × 10-3mm2/s, P &lt .001). Ten and 13 of 60 superior cervical ganglions were hypointense on T2-weighted images and had hyperintense spots on both T1- A nd T2-weighted images, respectively. The latter was considered fat tissue. The largest was the superior cervical ganglion, followed in order by the retropharyngeal lymph node and the inferior ganglion of the vagus nerve (P&lt .001 to P&lt .004). The highest at vertebral level was the retropharyngeal lymph nodes, followed, in order, by the inferior ganglion of the vagus nerve and the superior cervical ganglion (P &lt .001 to = .001). The retropharyngeal lymph node, superior cervical ganglion, and inferior ganglion of the vagus nerve formed a line from anteromedial to posterolateral. CONCLUSIONS: The superior cervical ganglion and the inferior ganglion of the vagus nerve can be almost always differentiated from retropharyngeal lymph nodes on MR imaging by evaluating the signal, size, and position.

Dandy-Walker malformation (DWM) is a posterior fossa anomaly characterized by hypoplasia and upward rotation of the cerebellar vermis and cystic dilation of the fourth ventricle. The cyst of DWM rarely extends posteriorly to almost completely fill the entire posterior fossa, which mimics primary cerebellar agenesis, a cerebellar porencephalic cyst, and an arachnoid cyst due to the lack of clarity of the thin cystic wall. A 10-month-old female born at 23 weeks' gestation with cerebellar hemorrhage in the neonatal period was admitted to our hospital with dysphagia and side-to-side head bobbing. The detection of hemosiderin deposits enveloping the cyst wall by T2 star-weighted angiography (SWAN) was useful for the differential diagnosis of an acquired form of DWM from primary cerebellar agenesis. Cyst fenestration successfully improved dysphagia and head bobbing. A pathological specimen of the perforated cyst consisted of collagen fibers with hemosiderin deposits but lacked congenital cyst components. In infants with posterior fossa cysts, SWAN will be useful for a differential diagnosis between DWM and primary cerebellar agenesis.

OBJECTIVES: Among the benign tumours of the parotid gland, basal cell adenoma (BCA) is far less common than pleomorphic adenoma (PA). MR features of BCA, including diffusion-weighted imaging and dynamic contrast-enhanced study, have not been previously described. Assessment of the crucial MR features of BCA appears to offer beneficial clues for distinguishing BCA from PA. METHODS: We retrospectively reviewed 14 BCAs and 179 PAs in the parotid gland, collected between March 2000 and May 2012, from the MRI database. RESULTS: Nearly half of the BCAs had cystic components. The average ratio of the maximum diameter of the cysts to the BCAs (cystic ratio) was 0.80 ± 0.11 [standard deviation (SD)]. The BCA cystic ratio was significantly higher (p = 0.00232) than that of PAs. The cystic ratio threshold was 0.65 between cystic BCA and cystic PA. Sensitivity and specificity were 76.5% and 100%, respectively. The average of the apparent diffusion coefficient (ADC) values of the 12 BCAs [1.24 ± 0.18 (SD) ×10(-3) mm(2) s(-1)] was significantly lower than that of the 151 PAs [1.86 ± 0.40 (SD) ×10(-3) mm(2) s(-1)] (p < 0.001) and also lower than that of the cystic PAs [1.83 ± 0.57 (SD) ×10(-3) mm(2) s(-1)] (p < 0.00495). The ADC threshold was 1.31 × 10(-3) mm(2) s(-1) between BCA and cystic PA, with sensitivity and specificity of 81.2% and 91.7%, respectively, and also the same threshold 1.31 × 10(-3) mm(2) s(-1) between BCA and PA, with sensitivity and specificity of 92.7% and 91.7%, respectively. Time-signal intensity curves (TICs) showed various patterns. CONCLUSIONS: A small cystic mass with well-circumscribed borders and slightly lower ADC value may suggest BCA, although TICs showed various patterns.

OBJECTIVE: To identify useful MRI abnormalities in the putamen for diagnosing multiple system atrophy. METHODS: Patients with multiple system atrophy (n=15), Parkinson's disease (n=16), or progressive supranuclear palsy (n=9) and healthy controls (n=10) were enrolled. Using a visual analog scale, 4 examiners independently rated high-intensity signals along the lateral putamen on T2-weighted and T2*-weighted images, low-intensity signals within the putamen on T2-weighted and T2*-weighted images, and putaminal atrophy. Receiver operating characteristic analyses were performed, and the area under the receiver operating characteristic curve was calculated. RESULTS: For differentiating multiple system atrophy from progressive supranuclear palsy, Parkinson's disease, and healthy controls, the mean area under the curve values was the highest for low-intensity signals within the putamen on T2*-weighted images (0.797, 0.867, 0.896, respectively). Variations in the area under the curve values among the 4 examiners were the smallest in low-intensity signals within the putamen on T2*-weighted images. Good inter-rater reliability was achieved for low-intensity signals within the putamen on T2*-weighted images and high-intensity signals along the lateral putamen on T2*-weighted images. CONCLUSION: Low-intensity signals within the putamen on T2*-weighted images is the most useful MRI abnormality for diagnosing multiple system atrophy.